Resident Survival Guide 2012-2013, 1st edition This book is intended to be a reference guide for common pediatric diseases.

We have borrowed heavily from our favorite sources and teachers and have done our best to cite our references. Please use this book as a starting point in caring for your patients. It should not, however, replace clinical judgment, especially for complex decision-making. Please do not hesitate with any suggestions or criticisms on how to continue to improve this book over the years. Enjoy!

Editor: Julia Wirjo, MD Questions, comments, suggestions? Email julia.wirjo@unmc.edu

Contributors: Heidi Barker, DO David Bearl, MD Phil Boucher, MD Ojasvini Choudhry, MD Kathleen Davis, MD Sabrina Malone-Jenkins, MD Nicholas Torbert, DO Jennifer Pham, MD Brandi Reeve-Iverson, MD Gwenn Skar, MD Cody Wilson, DO Julia Wirjo, DO

Useful Numbers............................................................................................................................... 4 Allergy/Immunology........................................................................................................................ 9 DDx of Wheezing......................................................................................................................... 9 Asthma-related History Questions............................................................................................... 9 Spirometry................................................................................................................................... 9 Level of Asthma Control............................................................................................................. 11 Stepwise Approach for Managing Asthma.................................................................................. 12 When to refer............................................................................................................................. 13 Allergy Medications.................................................................................................................... 13 D Dx of Hives............................................................................................................................. 13 Food Allergies in Children.......................................................................................................... 14 Anaphylaxis................................................................................................................................ 14 Eczema...................................................................................................................................... 14 Immunodeficiency...................................................................................................................... 15 Primary Immunodeficiency Syndromes................................................................................... 17 Cardiology..................................................................................................................................... 18 Physical Exam............................................................................................................................ 18 Murmurs..................................................................................................................................... 18 EKGs........................................................................................................................................... 19 Arrhythmias............................................................................................................................... 20 Kawasaki Disease....................................................................................................................... 21 Congenital Heart Disease........................................................................................................... 22 Carditis....................................................................................................................................... 23 Endocrinology............................................................................................................................... 28 Diabetes..................................................................................................................................... 28 DI............................................................................................................................................... 28 SIADH......................................................................................................................................... 29 Short Stature.............................................................................................................................. 29 Precocious Puberty..................................................................................................................... 29 Adrenal Insufficiency.................................................................................................................. 29 FEN................................................................................................................................................ 31 Dehydration............................................................................................................................... 31 Fluids......................................................................................................................................... 31 Oral Rehydration........................................................................................................................ 31 Neonatal Fluids.......................................................................................................................... 32 Formula...................................................................................................................................... 32 Growth....................................................................................................................................... 32 2

Hypoglycemia............................................................................................................................ 32 Hypo/hypernatremia.................................................................................................................. 34 Hyperkalemia............................................................................................................................. 35 Gastroenterology.......................................................................................................................... 37 GERD.......................................................................................................................................... 37 FTT............................................................................................................................................. 37 IBD............................................................................................................................................. 39 Pancreatitis................................................................................................................................ 40 Hematology/Oncology................................................................................................................... 41 Transfusion reactions................................................................................................................. 41 Thrombocytopenia..................................................................................................................... 45 Coagulation................................................................................................................................ 46 Chemotherapeutic agents.......................................................................................................... 49 Infectious Diseases....................................................................................................................... 51 Bacterial Classification............................................................................................................... 51 Fever without a Localizing Source.............................................................................................. 53 Common Pediatric Infections..................................................................................................... 54 Children's Antibiogram............................................................................................................... 64 Metabolism.................................................................................................................................... 69

USEFUL NUMBERS

USEFUL NUMBERS Hospitalists (Childrens) Pager On call pager Dolter, Stephen Killefer, Heidi Lerner, Gary Sieczkowski, Lisa 888-8616 888-8404 888-8718 888-8600 888-8430 Dictatio n 5812 5851 0240 5895 Snow, Jay Snyder, Sheila Stoolman, Sharon Susman, Cassandra Vernon, Yohanna Pager 888-8662 888-1127 888-8705 888-8724 888-8143 Dictation 1305 5642 5589 5856 5971

General Peds (UNMC) Finken, David 888-2809 112 4 Hollins, Susan 888-3403 Lacroix, Amy 888-2313 027 2 Parker, Jennifer 888-1157 560 4 Pitner, Sheryl 888-2298 089 9 Private Attendings Abraham, Nadia Amstutz. Kenton Anderson, Karoline Andresen, John Anglim, Katherine Arispe, Emilio Asher, Nathan Belin, Marie Bruce, Jason Brunner-Buck, Lori Byrne, Thomas Calderon, Cicero Carnazzo, Jane Cohen, Michael Davey, Kurt Dawson, Michael Dek, Mary Desai, Alka Dethlefs, Henry Drake, Jamie Dulac, Michael Dworak, Alex Ellison, Joseph Fellman, Susan Fernandez, Cristina Feuerstein, Frederick Gary, Dawn Grahn, Kirstin Grush, Michael Hardisty, Lisa Harrison, Francis Hart, Roseanne Hennessy, Therese Herman, Andrea Kaufman, David Kaushik, Ruchi Kobayashi, Ai Lan Kratochvil, Jill Krenzer, Kari Loucks, Erin Pager 888-8443 579-4777 579-1001 977-0338 221-1179 221-6410 579-6235 579-3097 579-1002 977-2001 955-7474 579-1003 579-1004 579-9632 579-1005 299-1960 546-2661 221-5124

Scott-Mordhorst, Tina 888-5215 1177 Sievert, Patricia Shearer, Bonnie Walburn, John Wilwerding, Laura 888-5216 888-2157 888-1365 0676 888-5217 1222

Phone 955-5400 827-0636 573-7337 955-8400 955-3000 815-1325 391-7684 573-7337 498-6363 354-7842 573-7337 955-7575 955-7474 573-7337 573-7337 343-8585 573-7337 955-3000 734-4110 955-7676

Dictation 5820 1062 5624 5583 5631 0940 5861 5950 5753 5775 0730 0914 0860 5517 0993 1297 0944 0806 5695 5952 0158 5688 5660 0892 0660 0447 5984 0417 5760 5663 5879 1161 5762 0461 1039 1210 5678

734-4110 330-5690 955-8300 978-0542 280-4580 354-7500 221-6316 955-7500 955-7474 955-7474 579-7166 498-6363 778-6900 977-0021 955-8400 577-2284 330-5690 271-1892 734-4110 733-4433 221-1975 955-3000 343-8585 592-1700 978-0104 955-7676 636-4894 955-5437 271-8177 955-7676

USEFUL NUMBERS

Macklem, Monique Maxwell, Andrew May, Erin McCann, Rachel McNally, Clancy McVea, Kristine Moffatt, Kody Mogenson, Michelle Moore, John Moore, Michael Nielsen, Laura Paradis, Mara Penny, Gregory Penny, Katherine Reel, Jill Reiser, Jennifer Rizal, Chandrika Roy, Chitrita Runge, Rebecca Rupp, Ellen Rush, Charles Russell, Ann Sawyer, Alexis Saxena, Kristin Severson, Gregory Shidler, Kelli Sindelar, Steven Smith, Jennifer Specht, Patrick St. Germain, Melissa Steinauer, Patrick Stephenson, Betsy Straley, Joseph Uzendoski, Donald Vanderbur, Lars Vann, John Vargas, Monica Walenz, Elizabeth Whaley, Debra Whitcomb, Lisa Wilczewski, Michael Woodford, Robert Yaghmour, Anthony Zimmerman, Heather

978-0045 955-7600 977-6614 (712) 396-4321 955-6877 221-1111 955-8400 978-0152 955-7600 734.4110 978-0478 955-7600 955-7474 955-7474 221-5082 955-5437 978-0097 955-7676 955-5437 579-3077 758-5125 579-7149 391-7684 578-5573 391-7684 579-7160 778-6900 815-1325 977-3107 955-7575 991-5437 595-3939 579-7162 498-6363 579-7151 398-6600 579-7148 391-7684 498-6363 827-0636 354-0620 597-8918 758-5125 978-0020 955-7600 977-0087 955-7500 978-0103 955-7676 221-6225 955-5437 579-1006 573-7337 221-1610 955-7575 221-8770 955-3000 597-7147 391-7684 221-9942 955-7500 330-5690 271-8467 734-4110 221-8127 354-1325 579-6957 758-5125 354-7630 977-0529 955-7575 221-5349 354-1325 978-0070 280-4580 579-5325 778-6900

0915 1099 5728 5860 5532 5601 5929 0399 0916 0808 5591 1042 5605 0498 5983 1030 0983 0228 0321 0544 5990 5991 0327 5747 0917 0737 1288 5928 1108 0406 0654 0363 0436 5800 5745 5867 2001 0926 0556 1227 5823

Cardiology: Cardiology clinic 402-955-4350, Cardiothoracic surgery clinic 955-4360 Danford, David 977-8850 015 Hsu, Hao 977-8803 6 Delaney, Jeffrey 977-8849 585 Jenkins, Lindsay 955-8062 (phone) 2 Erickson, Christopher 977-8851 131 Kenkel, Amy 966-8062 (phone) 7 Fletcher, Scott 977-8852 050 Kugler, John 977-8854 0463 2 Goesch, Tara 955-4850 (phone) Kutty, Shelby 977-8855 5850 Gumbiner, Carl 977-8853 0425 Critical Care Chaplin, Robert DeMare, Jeffrey Kadlec, Kelly

888-8491 586 8 888-8520 011 2 888-8704 581 8

Noronha, Luke Norton, Bridget Thakker, Jayesh

888-8528 1322 888-8403 5752 888-8154 1048

USEFUL NUMBERS

McFadyen, Andrew 888-8541 Truemper, Edward 888-8152 5602 Mysore, Mohan 888-8151 1001 Endocrinology, clinic 955-3871 Cabrera, 888-8190 128 Desmangles, Jean-Claude 888-8205 1309 Monina 5 Corley, Kevin 888-0344 0579 Gastroenterology: Childrens clinic 955-5700, Boystown clinic 778-6820 Antonson, Dean 888-1837 040 Prestridge, Laurel 579-8051 1205 4 Fischer, Ryan 888-1536 594 Quiros, Ruben 888-0191 5956 7 Infantino, Benjamin 888-0562 Shelby, Shaija 888-6169 Kunnath, Sharad 579-8387 580 Trauernicht, 9 Anna Lirio, Richard 888-1741 Vanderhoof, Jon 0523 Palomo, Pablo 888-2609 Zapata, 888-8566 5651 Fernando Genetics Buehler, Bruce 231-9664 058 Rush, 888-5766 5 Eric Lutz, Richard 888-0404 123 Starr, Lois 888-3038 2 Olney, Ann 888-0389 0735 Hematology/Oncology: Childrens clinic 955-5700, UNMC clinic 559-8022 Abromowitch, Minnie 888-8530 013 Gnarra, David 888-8531 5 Arens, Mandy 888-0224 Gordon, Bruce 888-1125 Beck, Jill 888-8161 597 Harper, James 888-2245 2 Belsky-Lohr, Linda 955-8486 Lowas, Stefanie 888-8490 Coccia, Peter 888-1226 106 Thompson, Elizabeth 888-8215 7 Coulter, Don 888-8593 584 Warkentin, Phyllis 559-7257 5 Infectious Disease: clinic 955-4005 Chatterjee, Archana 978-0548 120 7 Delair, Shirley 888-2256 598 2 Green, Andrea 888-2772 Hoffman, Michelle 888-2176 Metabolism: clinic 955-4199 Lutz, 888-0404 123 Richard 2 Neonatology Berry, Ann Birge, Nicole Bolam, David 888-5221 564 5 888-8719 Simonsen, Kari 221-1386

0186 0589 0123 6010 1241 1091

Snowden, Jessica 888-1127 5945 Varman, Meera 978-0504 5582

Rizzo, William 888-0742 0117

Lees, Bonnie Needelman, Howard Olney, Richard Schmidt, John Willett, Lynne

888-8144 5980 888-8547 0600 888-5222 888-5225 5934 888-8298 5942

888-5218 041 4 Fletcher, Garth 888-5220 Grebe, John 888-8504 596 9

USEFUL NUMBERS

Kaftan, Harold

888-5223 010 5

Zach, Terence

888-5224 0537

Nephrology: clinic 559-6780 Lovell, Helen 888-3102 Neurology: clinic 955-5372 Kader, Fred 884-1590 (cell) Larsen, Paul 888-2381 McAllister, Janice 888-2721 024 2 046 4 056 8

0257 Nelson, James Oliver, Young 888-8418 5963 888-8452 1024

Wright, Rhonda 888-8189 0908

Pulmonology: Childrens clinic 955-5570, UNMC clinic 559-6275 Barker, Carrie 888-8514 Sammut, Paul 888-1165 0790 Colombo, John 888-1025 057 Thomas, Heather 888-2408 5692 8 Noronha, Luke 888-8528 132 Wilson, Mark 888-8550 0168 2 Rheumatology: clinic 955-4070 Reinhardt, Adam 888-8737 Surgery: clinic 955-7400 Surgery Chief 888-8331 Surgery Junior 888-8330 Abdessalam, Shahab 224-8871 574 1 Azarow, Kenneth 206-1235 571 1 Childrens Hospital Behav Health 955-3900 Blatchford, Allison 955-6061, 8888683 Charge nurse 955-790_ (floor #) Dictation 955-4030 Emergency Dept 955-5150 Long distance code 22850 Main fax: 4th floor 955-3670 th 5 floor 955-3651 6th floor 955-3660 Main number 955-5700 Pathology 955-5518, 9555500 UNMC Clinic: Appointments Conf room Backline Chief resident Colpitts, Cindy Doherty, Patrick Emergency department Inpatient units: 6 N/S BMT PICU NICU NICU Call Room Nursery 559-4208 559-6711 559-6898 888-1616 888-5730, 5380 888-0977, 5380 559-4020 559-6243 559-7402 559-5257 559-4442 559-5806 559-4309 5858 Brester, Michelle 955-8687 (phone) Cusick, Robert 221-6126 1266 Lao, Oliver 444-8551 Raynor, Stephen 444-0742 0125

Pharmacist PICC Radiology Respiratory Therapy Scheduling Security Social work Spanish interpreter

955-8035, 955-5470 888-8482 955-5637 955-8615 955-5800 955-5300 888-8420 888-8416

Lab Inpatient Lab Outpatient Main Medical Records 559- Micro 559- Pharmacy Inpatient

559-4255 559-8780 559-4000 559-4024 559-7667 559-7235

Pharmacy Outpatient 559-5215 Radiology Resident library Resident Supervisor Security Virology PCR 559-1000 559-6773, 559-8918 888-1859 559-5111 559-7744

Creighton Clinic: Appointments Conf room (main) Teaching conf room Continuity clinic room Nurse backline Nurse backline #2 Fax Emergency Dept

280-4580 280-4690 280-5951 280-4162 280-4454 280-4452 2804159/4304 449-4590

Inpatient Units L&D Main NICU Nursery Pharmacy Radiology Zach, Terry

449-5900 449-4190 449-4000 449-4195 449-4417 280-4567 449-4540 280-1231, 280-4132

ALLERGY/IMMUNOLOGY D Dx of Wheezing (* indicates more likely during the first year of life) Acute Persistent/Recurrent: Recurrent/Chronic: Functional Structural Asthma Tracheo-bronchomalacia* Asthma Bronchiolitis Vascular compression/rings* Post-RSV Wheeze Bronchitis Tracheal stenosis/webs* GERD Laryngotracheobronchitis Cystic lesions/masses* Recurrent Aspiration Bacterial Tracheitis Tumors/lymphadenopathy Cystic Fibrosis Foreign Body Aspiration Cardiomegaly Immunodeficiency Esophageal Foreign Primary ciliary dyskinesia Body Bronchopulmonary dysplasia* Retained foreign body (trachea/esophagus) Pulmonary edema Vocal cord dysfunction ***all of the above aggravated by environmental tobacco smoke (ETS) Asthma-related History Questions HPI: Symptoms (cough, wheezing, dyspnea, sputum production), duration, timing, nighttime wakening, impact on normal activity, triggers, recent illness. PMH: Resp meds, time/means of diagnosis, # courses oral steroids in past year, hospitalizations, PICU, intubation, prematurity, lung disease at birth Hx allergic rhinitis, eczema, food allergies, or eosinophilic esophagitis Meds: Current and past meds, frequency, correct use, last time refilled? F Hx: Asthma, allergies, inhaler use S Hx: Smoke (onset, amount), smoke exposure (including intrauterine), pets, mold, activity level. Spirometry Forced Vital Capacity (FVC). Maximum volume of air that can be forcibly expired after full inspiration Forced Expired Volume in 1 Second (FEV1). Measured after maximum inspiration, the volume of air that can be expelled in 1 s Forced Expiratory Flow at 25-75% FVC (FEF2575). Mean flow that is measured when between 25 and 75% of the FVC has been expired. Obstructive Lung Disease FVC FEV1
Asthma Flow-Volume Curve

ALLERGY / IMMUN

Restrictive Lung Disease Decreased Decreased Normal or increased Normal, increased, or decreased

Normal or decreased Decreased

FEV1:FVC Decreased ratio FEF2575 Decreased

Classifying Asthma Severity (University of Michigan Health System Clinical Care Guidelines )

ALLERGY / IMMUN

Level of Asthma Control ( University of Michigan Health System Clinical Care Guidelines )

ALLERGY / IMMUN

Stepwise Approach for Managing Asthma (University of Michigan Health System Clinical Care Guidelines)

ALLERGY / IMMUN

When to refer an asthmatic to a pulmonologist or an allergist A life-threatening exacerbation has occurred The patient has been hospitalized The patient has required more than two steroid bursts in a year The patient >5 years old requires Step 4 care or higher (severe persistent) The patient <5 years old requires Step 3 care or higher (moderate persistent) The patient appears unresponsive to therapy The diagnosis of asthma is uncertain Additional testing is needed (allergen skin testing, bronchoscopy, etc.) The patient is a candidate for allergen immunotherapy Asthma Medications - Brand Names (Generics) Type Nebulized Bronchodilators (SABA Short-acting Beta-2-Agonists) Metered Dose Inhaler (MDI) SABA Examples Ventolin, Proventil, Accuneb (Albuterol) Xopenex (Levalbuterol) Ventolin HFA, Proventil HFA, ProAir HFA (Albuterol) Xopenex HFA (Levalbuterol) Maxair (Pirbutrol) Atrovent (Ipratropium bromide) Atrovent HFA (Ipratroprium bromide) Combivent (Ipratroprium bromide and Albuterol) QVAR (Beclomethasone) Pulmicort (Budesonide) Flovent HFA (Fluticasone) Alvesco (Ciclesonide) Flovent Diskus (Fluticasone) Asmanex Twisthaler (Mometasone) Pulmicort Respules (Budesonide) Advair HFA (Fluticasone and Salmeterol) Symbicort HFA (Budesonide and Formoterol) Advair Diskus (Fluticasone and Salmeterol) Intranasal steroids Flonase (Fluticasone) Nasonex (Mometasone) Rhinocort (Budesonide) Nasacort (Triamcinolone) Omnaris (Ciclesonide)

ALLERGY / IMMUN

Nebulized Anticholinergics MDI Anticholinergics Combination Anticholinergics with SABA MDI Inhaled Corticosteroids (ICS)

Dry Powder Inhaler (DPI) ICS Nebulized ICS MDI Combination ICS with LABA (Long-acting Beta-2-Agonists) DPI Combination ICS with LABA

Allergy Medications - Brand Names (Generics) Antihistamines 1st-gen (more sedating): Benadryl (Diphenhydramine), Atarax (Hydroxyzine) 2nd-gen: Allegra (fexofenadine), Zyrtec (cetirizine), Claritin (loratidine)

D Dx of Hives Infections IgE-mediated Allergic Reactions

Direct Mast Cell Activation Physical Stimuli

Medication-related Systemic Disorders

Viral, Bacterial, or Parasitic Medications Insect Bites/Stings Latex Aeroallergens (Pollens, etc.) Foods/Food Additives Medications (Narcotics, Muscle Relaxants, Vancomycin) Radiocontrast Medium Temperature Change Pressure Vibration Sunlight Exercise NSAIDs Hormone-related (OCPs, HRT, etc.) Vasculitis Cryoglobulinemia

Mastocytosis Most Common Food Allergies in Children 1. Milk (2.5%) 2. Egg (1.3%) 3. Peanut (0.8%) 4. Soy (0.4%) 5. Wheat (0.4%) 6. Tree nuts almonds, walnuts, cashews, pecans, pine nuts, hazelnuts, chestnuts, macadamia nuts, coconut, and pistachios (0.2%) *Most children outgrow their allergy to milk, egg, wheat, and soy but not to peanut or tree nut. Anaphylaxis Management ( UpToDate Anaphylaxis: Rapid Recognition and Treatment) The first and most important therapy in anaphylaxis is epinephrine. There are NO absolute contraindications to epinephrine in the setting of anaphylaxis. Airway: Immediate intubation if evidence of impending airway obstruction from angioedema; delay may lead to complete obstruction; intubation can be difficult and should be performed by the most experienced clinician available; cricothyrotomy may be necessary IM Epinephrine (1 mg/mL preparation): Give epinephrine 0.01 mg/kg IM (maximum dose: 0.5 mg), preferably in the mid-anterolateral thigh, can repeat every 5 to 15 minutes as needed. If signs of poor perfusion are present or symptoms are not responding to epinephrine injections, prepare IV epinephrine for infusion. Place patient in recumbent position , if tolerated, and elevate lower extremities Oxygen: Give 6 to 8 liters per minute via face mask, or up to 100 percent oxygen as needed Normal saline rapid bolus: Treat poor perfusion with rapid infusion of 20 mL/kg; reevaluate and repeat fluid boluses (20 mL per kilogram) as needed; massive fluid shifts with severe loss of intravascular volume can occur; monitor urine output Albuterol: For bronchospasm resistant to IM epinephrine, give albuterol 0.15 mg/kg (min dose: 2.5 mg) in 3 mL saline inhaled via nebulizer; repeat as needed H1 antihistamine: Consider diphenhydramine 1 mg/kg (max 40 mg) IV H2 antihistamine: Consider ranitidine 1 mg/kg (max 50 mg) IV Glucocorticoid: Consider methylprednisolone 1 mg/kg (max 125 mg) IV Monitoring: Continuous non-invasive hemodynamic monitoring and pulse oximetry; urine output in patients receiving IV fluid resuscitation for severe hypotension or shock Eczema Management (UpToDate Treatment of Atopic Dermatitis (Eczema)) 1. Eliminate Exacerbating Factors - Avoid triggers such as heat, perspiration, and low humidity - Treat skin infections (Antibiotics, bleach baths, etc.) 2. Maintain Skin Hydration - Hydrating bath followed by immediate application of cream, ointment, or emollient - Creams: Eucerin, Cetaphil, Nutraderm - Ointments: Vaseline, Aquaphor - Emollients: Mimyx, Biafine, Hylatopic, Tropazone 3. Control Pruritis - Antihistamines: Diphenyhydramine, Hydroxyzine, Fexofenadine, Loratidine, Doxepin - Wet dressings soothe the skin, reduce pruritus, reduce redness, and limit access to the skin. Apply a cream, ointment or emollient to the affected area, cover with damp cloth(s) and cover with a dry cloth. 4. Treat Inflammation - Low Potency Topical Corticosteroids *best option for eczema on the face o Hydrocortisone 1 or 2.5% Cream (available over the counter) - Medium Potency Topical Corticosteroids *best option for eczema on the body o Triamcinolone 0.1% Cream (Kenalog) o Fluocinolone 0.025% Ointment (Synalar) o Mometasone 0.1% Cream (Elocon) - High Potency Topical Corticosteroids *best used for acute flares for up to 10 days (in general, ointments are higher potency than creams)

ALLERGY / IMMUN

o Triamcinolone 0.5% Cream (Aristocort) o Triamcinolone 0.1% Ointment (Aristocort) o Fluticasone 0.005% Ointment (Cutivate) o Mometasone 0.1% Ointment (Elocon) o Betamethasone 0.05% Cream (Diprosone) 5. Other Therapies - UV Light Therapy - Calcineurin Inhibitors - Immunosuppressants (Methotrexate, Azathioprine, etc.) Immunodeficiency (The Jeffrey Modell Foundation)

ALLERGY / IMMUN

ALLERGY / IMMUN

Clinical Features of Primary Immunodeficiency Syndromes ( UpToDate Approach to the Child with Recurrent Infections) Adaptive Immunity B-cell defects (IgA Deficiency, Common Variable Immunodeficiency, IgG Subclass Deficiencies, X-Linked Agammaglobulinemia) - Recurrent bacterial sinopulmonary infections or sepsis, particularly with polysaccharide encapsulated organisms (S. pneumoniae, H. influenzae type b) - Unexplained bronchiectasis - Chronic or recurrent gastroenteritis (often with Giardia or enterovirus) - Failure to thrive - Chronic enteroviral meningoencephalitis - Arthritis T-cell defects (Wiskott-Aldrich Syndrome, DiGeorge Syndrome) - Recurrent, severe, or unusual viral infections (varicella-zoster, CMV, HSV) - Failure to thrive - Chronic candidiasis - Chronic diarrhea - Lymphopenia during the neonatal period or in infancy - Pneumocystis pneumonia - Graft-versus-host disease (maculopapular and/or desquamating skin, abnormal liver function tests and/or chronic diarrhea) - Severe/neonatal eczematoid or seborrheic rashes Combined B-cell and T-cell defects (SCID, Hyper-IgM Syndrome, Adenosine Deaminase Deficiency, X-Linked Lymphoproliferative Disease) Innate Immunity Phagocytic defects (Cyclic Neutropenia, Chronic Granulomatous Disease, Leukocyte Adhesion Deficiency, Chediak-Higashi Syndrome) - Poor wound healing - Delayed separation of the umbilical cord - Lymphadenitis or soft tissue abscesses - Hepatosplenomegaly - Chronic gingivitis and periodontal disease; oral mucosal ulcerations - Infection with catalase positive bacteria and fungi - Recurrent gastrointestinal or genitourinary tract obstruction Complement defects (Hereditary Angioedema, Various Complement component disorders) - Angioedema of face, hands, feet, GI tract - Autoimmune disease; lupus-like symptoms - Pyogenic bacterial infections (eg, N. meningitidis) - History suggestive of autosomal dominant inheritance

ALLERGY / IMMUN

CARDIOLOGY Have your PALS card handy!

Physical Exam 1. Vitals HR, BP, O2 sats 2. I/Os: a. Ins both in ml/kg/hr (goal between 120 and 150) and cal/kg/day (goal for post-op hearts is often >120) b. Outs ml/kg/hr (goal is usually >1) 3. Rate can be normal, tachy or brady (NOT regular) 4. Rhythm can be regular, regularly irregular or irregularly irregular
5. Murmurs 3 key pieces: grade (I-VI), systolic vs. diastolic, location heard on the chest

a. Simple grading a. Grade I = murmur softer than S1, S2 b. Grade II = murmur same volume as S1, S2 c. Grade III = murmur louder than S1, S2 d. Grade IV = palpable thrill e. Grade V = thrill + can hear with stethoscope on its side f. Grade VI = thrill + can hear with stethoscope off the chest b. Systolic from S1 to S2 c. Diastolic from S2 to S1 d. Locations a. Right upper sternal border (RUSB) b. Left upper sternal border (LUSB) c. Left mid sternal border (LMSB) d. Left lower sternal border (LLSB) e. Left mid-clavicular 5th intercostal space (Apex) f. Infraclaviular right and left g. Posterior chest/back 6. Specific common murmurs a. Innocent a. Stills (vibratory) systolic LLSB b. Pulmonary ejection murmur early systolic LUSB c. Pulmonary flow murmur of infancy systolic LUSB d. Venous hum supraclavicular/infraclavicular areas b. ASD fixed split S2, systolic ejection murmur LUSB and diastolic rumble LLSB c. VSD holosystolic murmur LLSB (large VSDs can have diastolic rumble as well) d. AS systolic ejection murmur RUSB

CARDIOLOGY

e. AR early diastolic murmur RUSB, LMSB or LLSB f. MR holosystolic murmur Apex g. MS mid-late diastolic rumble Apex h. MVP mid-systolic click w/ or w/o late systolic murmur or diastolic rumble Apex i. PR diastolic LUSB, LMSB j. PS systolic LUSB, RUSB and back k. PDA continuous murmur LUSB l. BT shunt continuous murmur RUSB or LUSB (depends on the site of the shunt)

EKGs 1. Rate 300/number of big squares between consecutive QRS complexes 2. Rhythm regular vs. irregular, does a P-wave precede each QRS? 3. Axis (normals vary by age) a. if P and QRS are up in I and aVF = nl b. if QRS is down in I = R axis deviation (nl in newborns) c. if QRS is up in I and down in aVF = L axis deviation d. if QRS is down in I and AVF = northwest axis 4. Intervals vary with age, with intervals usually increasing as children get older a. PR 0.08 to 0.2 sec (5 small boxes/1 large box) i. First-degree heart block fixed prolonged PR interval ii. Second-degree heart block Mobitz Type I (Wenckebach) progressive lengthening of the PR interval until a QRS is not conducted iii. Second-degree heart block Mobitz Type II loss of QRS conduction without lengthening of PR interval iv. Third-degree (complete) heart block complete dissociation of P-wave and QRS b. QRS 0.05 to 0.08 sec (2 small boxes for <15yo), to 0.12 sec (3 boxes for older teens and adults) i. Tachycardia can be defined by narrow vs wide QRS complex, long or short RP, regular vs irregular, stable vs unstable c. QTc = QT (sec) measured / (mean R-R interval) nl is <0.46 in M and <0.47 in F i. QTc is calculated because QT will vary with heart rate ii. QTc calculated by ECG is often wrong requiring manual measurement and calculation iii. A prolonged QT can be associated with ventricular arrhythmias (torsades de pointes), syncope and sudden death 5. Waves a. P-wave

CARDIOLOGY

6. Arrhythmias

a. Sinus tachycardia (HR > 95th percentile for age) or bradycardia (HR<95th percentile for age) b. Supraventricular (can be narrow or wide complex QRS) i. PACs ii. SVT (9 types) a. Intra-atrial Reentrant Tachycardia (IART) including atrial flutter b. Sinoatrial nodal reentrant tachycardia (SNRT) - rare c. Orthodromic AV reentrant tachycardia (ORT) (Wolff-ParkinsonWhite syndrome) most common in infants and young children d. Antidromic AV reentrant tachycardia (ART) wide QRS e. Junctional ectopic tachycardia (JET) can be in post-op hearts f. Atrial ectopic tachycardia g. AV node reentrant tachycardia (AVNRT) more common in older teens h. Persistent junctional reciprocating tachycardia (PJRT) i. Mahaim tachycardia wide QRS c. Ventricular (wide-QRS) i. PVCs a. Bigeminy alternating normal and PVC b. Trigeminy two normal QRS followed by PVC

c. Couplet two consecutive PVCs ii. Ventricular tachycardia 3 or more PVCs (120-250 beats/min), wide QRS and dissociated, retrograde or no P wave iii. Ventricular fibrillation disorganized, irregular QRS with rapid rate 7. Electrolyte abnormalities a. Hyperkalemia - >6 = tall T-waves, >7 = wide P-wave, >8 = absent P-wave, >9 = AV block, V-tach, V-fib b. Hypokalemia flat T-waves, ST depression, prominent U-wave c. Hypercalcemia short ST segment, sinus bradycardia d. Hypocalcemia long ST segment -> long QTc, Nl QRS CARDIOLOGY e. Hypermagnesemia Long PR interval, short ST segment f. Hypomagnesemia Long ST segment, prominent U-wave

Kawasaki Disease actually an inflammatory immune response, but the most dangerous sequelae involves the heart. 1. Diagnostic criteria a. Fever 5 or more days b. 4 of the following criteria (although can be incomplete) i. ii. iii. iv. v. Non-exudative conjunctivitis Oral mucous membrane changes injected or fissured lips, injected pharynx or buccal mucosa, strawberry tongue Rash can be many variations Extremity changes erythema of palms or soles, edema of the hands or feet, desquamation (late finding) Cervical lymphadenopathy one node >1.5cm, often unilateral

c. Can also present with aseptic meningitis, arthritis, sterile pyuria, abdominal pain, Hydrops of gallbladder 2. Risk factors: < 1 y/o, > 9 y/o, prolonged fever, low platelets, hyponatremia. 3. Labs: a. CBC: Leukocytosis with left shift, anemia, thrombocytosis (2 nd week) b. ESR/CRP: Elevated c. CMP: Hypoalbuminemia, hyponatremia, elevated transaminases 4. Cardiac sequelae coronary artery aneurysms or dilation develop in 15-25% of untreated patients, which can lead to myocardial infarction or sudden death 5. Management a. IVIG 2 gm/kg x1 dose over 12 hours, should be given within 10 days of onset of symptoms. May repeat if fever persists for > 36 hours after completion b. Aspirin Start high dose ASA (80-100mg/kg/day divided QID) until afebrile for 48hrs, then continue low dose ASA (3-5mg/kg/day) and continue for at least 6 weeks, until ESR/CRP resolve. Will continue indefinitely if evidence of aneurysm. c. Ranitidine while on high dose ASA for GI prophylaxis

Congenital Heart Disease NOTE: Remember that blood flow will take the path of least resistance, always. If resistance is high, then flow is low. If resistance is low, then flow is high. See PedHeart Companion on Childrens Homepage -> Virtual Library 1. Acyanotic heart lesions a. ASD primum, secundum, unroofed coronary sinus, or sinus venosus often asymptomatic, can present with wide, fixed-splitting of S2 and a systolic ejection murmur due to the increased blood flow across the pulmonary valve. There can also be a diastolic regurgitation component. VSD membranous (80%) vs. muscular most common congenital heart lesion classical holosystolic murmur but not always, large VSDs lead to FTT, diaphoresis and tachypnea especially with feeding most do not require surgery. PDA persistent PDA may cause CHF symptoms, closure can be done with indomethacin (prostaglandin inhibitor), surgical ligation, or catheter coiling

b.

CARDIOLOGY

c.

2. Cyanotic heart lesions a. b. NOTE: If concern for ductal-dependent lesion, give Alprostadil (Prostaglandin E1 PGE1) 0.01-0.1 mcg/kg/min IV. Truncus arteriosus a single vessel (aorta + pulmonary artery) arises from the left and right ventricles. Several types depending upon how the pulmonary artery separates from the trunk. Always accompanied by a VSD, given that both ventricles share the same outflow tract. Definitive repair is done as an infant. Transposition of the great arteries aorta arises from RV and pulmonary artery arises from LV, mixing occurs via PDA, ASD and/or VSD. Most commonly repaired via arterial switch. Older approach was the atrial switch (Mustard/Senning procedure). Triscuspid atresia/severe stenosis similar 3 stage repair to the HLHS Tetralogy of Fallot Pulmonary valve stenosis/RV outflow obstruction Right ventricular hypertrophy Overriding aorta Ventricular septal defect (VSD) Total anomalous pulmonary venous return (TAPVR) pulmonary venous return to either vena cava or right atrium, not the left atrium. Repair involves reinserting the veins into the left atrium.

c.

d. e. i. ii. iii. iv. f.

3. Left ventricular outflow obstruction a. Hypoplastic left heart syndrome (HLHS) hypoplastic left ventricle often with hypoplastic ascending aorta, aortic and mitral atresia/stenosis. Requires 3 procedures for repair: i. Stage 1) Norwood w/ Sano (within the first few days of life) reconstruction of the aorta then connected to the right ventricle, with a Sano conduit that runs directly from the right ventricle to the pulmonary arteries as the only pulmonary flow. ii. Stage 2) Bi-directional Glenn (between 4-6 months usually) take down of the Sano conduit, followed by re-routing the SVC to the pulmonary artery. iii. Stage 3) Fontan (between 18 36 months usually) further routing of IVC to the pulmonary arteries usually via extracardiac conduit, thereby all systemic venous return is routed to the pulmonary arteries.

iv. Done in a step-wise process to keep the pulmonary resistance down. b. Coarctation of the aorta increased HTN in the upper extremities compared to lower extremities, may also include murmur. c. Interrupted aortic arch presentation is often similar to coarctation.

Carditis 1. Endocarditis a. b. c. Presentation can be acute (hemodynamic instability and heart failure), or subacute (prolonged low-grade fevers and non-specific complaints) Modified Duke Criteria (simplified here) 2 major or 1 major and 3 minor Major criteria i. Positive blood cultures typical organisms x2 Strep viridians, Staph. aureus, Strep bovis, HACEK group (Haemophilus, Actinobacillus, Cardiobacterium, Eikenella, Kingella); OR Evidence of endocardial involvement on echo vegetation, new valvular regurgitation, abscess or dehiscence of prosthetic valve

ii. d.

Minor criteria i. ii. iii. Predisposing heart condition or intravenous drug use Fever - 38.0C (100.4F) Vascular phenomena - major arterial emboli, septic pulmonary infarcts, mycotic aneurysm, intracranial hemorrhage, conjunctival hemorrhages, Janeway lesions Immunologic phenomena - glomerulonephritis, Osler's nodes, Roth spots,rheumatoid factor Microbiologic evidence - positive blood culture but not meeting major criterion as noted previously (excluding single positive cultures for coagulase-negative straphylococci and organisms that do not cause endocarditis) Serologic evidence of active infection with organism consistent with IE

iv. v.

vi. 2. Myocarditis a. b. c.

Suspect in a child with acute heart failure without structural heart disease. Get CXR, EKG, cardiac enzymes and ECHO to rule out structural heart disease. There is a high risk of arrhythmias and hemodynamic instability, send to PICU.

3. Pericarditis a. b. c. Presentation pleuritic chest pain and shortness of breath, refuses to lay down Causes mostly idiopathic or viral, dont forget rare etiologies - cancer, TB, uremia, SLE, meds Diagnosis must have at least 2 i. Typical chest pain pleuritic, may be better sitting and leaning forward ii. Pericardial friction rub often heard best of LSB iii. Suggestive changes on EKG widespread ST elevation iv. New or worsening pericardial effusion as seen on ECHO

d.

Treatment treat underlying etiology, if none, colchicines + NSAIDS

CARDIOLOGY

DERMATOLOGY Terms to use: Abscess: a collection of pus or suppurative exudate found in the body often times associated with erythema (redness) and induration (hardening or swelling) Annular: lesion(s) grouped in a circle (typically think fungal infection) Aplasia: skin tissue that has failed to grow (aplasia cutis in infants where the skin and hair have not met and create a bald spot). Bullae: typically clear fluid filled blister larger than 1cm wide Cyst: membranous sack containing fluid, gas or semi-fluid Dermatome: finding is typically a unilateral distribution in a very limited area that is distributed by a branch of nerves Dermatosis: fancy name for skin disease Desquamation: skin falling or peeling off Ecchymosis: bruise please note that you can NOT characterize the age of a bruise Erosion: an ulceration of the skin, occasionally covered by a scab Errythema: redness Excoriation: scratch mark Extensor surface: the front part of the leg or outside part of the arm (think eczema) Fissure: a crack in the skin (think oral fissure in chapped lips, or anal fissures in constipation) Flexural Distribution: a dermatosis found where the skin folds around itself (elbow pit) Follicular: arising from hair follicles Granulation tissue: new tissue composed of neovasculature, and fibrous tissue (looks gross) Granuloma: skin finding associated with chronic inflammation, will often times be raised, and pigmented differently Herpetiform: dermatosis secondary to a herpes infection composed of clusters of vesicles Hyperkeratosis: scaling on the epidermis Hyperpigmentation: darkening of the skin secondary to hypermelanosis or haemosiderin Hypertrophy: skin findings growing often times excessively Induration: feeling hard or swollen Lichinification: thickened or hypertrophic skin secondary to rubbing from things like chronic eczema Linear lesion: skin finding found making a line Macule: smooth skin finding often with color change <1.5cm diameter Nikolskys sign: you can shear off the skin by rubbing the lesion lightly Nodule: a larger papule in height, width and length Nummular lesion: coin shaped lesion, also called discoid Papule: <1.5 cm lesions that are raised, palpable, and often have color change Patch: large area of color change Petechiae: small pinpoint red or brown lesions, a form of purpura Plaque: palpable finding >1.5 cm diameter that is elevated or thickened Polymorphic: lesion with varied shapes Purpura: bleeding into the skin, NON-BLANCHING Pustule: vesicle filled with purulent material Target lesion: lesion with concentric circles Ulcer: full thickness loss of epithelium or epidermis Umbilicated: central divot kind of like an inny umbilicus think molluscum Verrucous: wartlike, thick and scaly

DERMATOLOGY

Vesicles: <0.5 cm raised lesions filled with clear fluid Wheal: edematous papule or plaque caused by swelling, think urticaria

Common Dermatoses: 1. Neonatal Herpes: looks like erythematous base with vescicles on top, diagnosis by Wright stain (multinucleated giant cells), make sure that its not fetal monitoring probes, and treatment is acyclovir 2. Neonatal pustualr melanosis (PM) vs staph infection: both are pustules and hyperpigmented macules. Tzanck smear in PM shows PMNs without organisms, a staph infection with have positive gram stain. Dont tx PM, tx staph infection with abx 3. Erythema Toxicum: yellow pustules on erythematous base, with an occasional vesicle. Wright stain reveals eosinophils (both start with E). no tx 4. Atopic Dermatitis: lichenification with scratching, flexor surfaces, atopic picture, prone to super-infection with staph aureus and treat that 5. Eczema: plaques on extensor surfaces of extremities, treat with topical steroid. WiskottAldrich Syndrome and Hyper IgE can present with eczema 6. Seborrheic Dermatitis: greasy yellow patches on scalp, face skin folds. Tx with mild topical steroids or topical antifungal agents. Consider histiocytosis X if there is ear discharge or profuse UOP 7. Poison Ivy: linear vesicles and papules, Type IV reaction (get it IV=IVY), wash, and use steroids 8. Psoriasis silvery lesions on extensor surfaces, when picked have pinpoint hemorrhage (Auspitz sign), get them to a dermatologist 9. Pityriasis Rosea: Christmas tree pattern on the pack that arrive in winter and early spring. Tx is sunlight 10. Tinea Corporis: ringworm looks like granuloma annulare but is scaly! 11. Impetigo: honey crusting usually caused by strep or staph, but usually staph aureus. Tx with abx. 12. Staphylococcal Scalded Skin Syndrome: sheet-like loss of skin caused by an exotoxin. Treat with abx some consider clindamycin, and careful monitoring for shock 13. Erythema multiforme hypersensitivity rxn, Major (SJS or TEN) Minor. Typically older children. Usually triggered by virus or med (sulfa drugs, anticonvulsants, NSAIDs). Tx stop offending agent, rehydrate, steroid only in debilitating disease. Different than SSSS as it will appear erythematous NOT white 14. Cellulitis: tender well-demarcated area of erythema, usually 2/2 to strep pyo, or MRSA. Look at the hospitals biogram and consider clinda, bactrim, augmentin, cephalexin 15. Scabies: linear lesions that is papular or pustular in the wrists, axilla, groin. Tx with permethrin, 16. Lice: nits on the hair shaft. Tx with permethrin immediately and week after, with thorough cleaning of the home. 17. Molluscum: pearly papules with central dimpling, no therapy needed 18. Acne: inflammation caused often times by bacteria (propionibacterium acnes), closed comedones (white heads) and open comedones (black heads), always consider early puberty in acne <8 yo. Tx dont scrub or pick this will increase oil production, use mild soap, start with topical benzoyl peroxide/clinda/retin-A. Low dose Abx (tetracycline, doxycycline, minocycline) will help decrease inflammation, but dont use for ~4mos. If acne persists or progresses refer to dermatology for accutane therapy. 19. Hemangioma consider PHACE syndrome or vascular malformation of the brain. May treat conservatively if they are not increasing in size, and they should disappear at a minimum of 10% per year. Take them out if they will cause disfigurement, oral airway compromise. Steroids and propranolol are first line agents. 20. Port-Wine stain over the face, consider Sturge-Weber syndrome Dont forget to: Ask progression of the rash is it clearing? Is it migrating? Is it changing? Ask when it started, and what makes it better and worse

DERMATOLOGY

Ask if there are any associated symptoms (itching, burning, fever) Ask if it ever drains or weeps and what does that look like? Ask if anyone else has the rash, or if the patient has had the rash previously Measure the lesion Remark on bodily location (intertrigenous, dermatomal) Remark on inflammation Remark on color Remark on inflammation Remark on elevation Remark on mucous membrane involvement Sources: Laughing Your Way to Passing the Pediatric Boards 5th Edition - Stu Silverstein MD, FAAP MedStudy Pediatrics Board Review - Dr. Thomas Cross, Jr, MD and Robert Hannaman, MD Uptodate Dermnet NZ

DERMATOLOGY

ENDOCRINOLOGY (Harriet Lane, Pocket Pediatrics) Diabetes and DKA DKA: Mild: pH <7.30, bicarb <15, Moderate: pH<7.2, bicarb <10, Severe: <pH 7.1, bicarb <5 Pseudohyponatremia: Corrected Na= measured Na + 1.6x [(glucose-100)/100] Remember total potassium can be low even if measured K is normal. Will shift intracellular with treament, add K+ if serum <5 and once voided Fluids: assume 5-7% dehydration in mild/mod DKA, 10% if severe. Will need 10 ml/kg for each % of dehydration. Want to give 10-20cc/kg bolus over 1-2 hours (max 2 boluses) and then correct fluid deficit over 48 hours. After bolus, use S and D bag system. o S bag: NS + 20 KCl, 20 KAcetate, D bag: 10% Dextrose. o Create sliding scale: o when sugars >300, 100% fluids are S bag, 251-300: 75% S, 25% D 201-250: 50% S, 50% D 150-200: 25% S, 75% D <150: 100% D Once eating, can continue fluids to finish correcting fluid deficit or till urine ketones are 0, run NS without sugar. Insulin will correct acidosis. o Run 0.1 units/kg/hr. If sugars falling too rapidly, increase dextrose in fluids. o Once acidosis corrected and not nauseous, transition to subQ insulin Calculating subQ insulin: Always good to check with Endocrine, but total daily dose will be between 0.3-0.75 units/kg, prepubertal pts on lower end, pubertal pts on higher end o Approximately of daily dose in long acting lantus/levemir o Approximate Carb ratio based on age: Toddlers: 1:20, Elementary School: 1:15, Junior High school: 1:12, High school: 1:10 o Approximate CF based on age: Toddlers: 1 U for 100>200, Elementary School, Junior High and High school: 1 U for 50>150 o In hospital, prefer sugar to be slightly high as most pts will see drop in sugars at home o The older the patient, the tighter control should be Labs: Initially want chem 8, glucose and blood gas. o While on insulin drip, check glucose every hour. Once on subQ insulin, check pre-meals, 2 hours post, at midnight and 3 am o Some attendings like to follow chemistries, some blood gas and some both. How often depends on severity, often start every 1-2 hours, then can space out to 2-4 hours. o New diabetic labs: C peptide, Insulin level, IgA level, TSH, Thyroid antibodies, celiac screening, HgbA1C, Beta-Hydroxybutyric acid, Diabetes Autoimmune panel o Urine ketones initially and continue until 0 Complications of DKA: Cerebral edema-watch with neuro checks, dont want glucose to fall to rapidly, no more than 100/hour, avoid aggressive hydration, Insulin Formulations: Type Onset (hr) Peak Duration Lispro (Humalog) 0.25 hrs 1 hr 2-3 hrs Insulin aspart (Novolog) 0.25 hrs 1 hr 2-3 hrs Glulisine (Apidra) 0.25 hrs 1 hr 2-3 hrs Regular human insulin 0.5-1 2-4 hrs 4-6 hrs NPH human 0.5-1 4-6 hrs 8-16 hrs Insulin glargine (lantus) 0.5-1 hrs none 23-26 hrs Insulin detemir (levemir) 0.5-1 none 5.7-26 (dose dependent) DI

ENDOCRINOLOGY

Presentation: Polyuria and polydipsia. Can also have irritability, FTT and intermittent fevers Work-up: Obtain Chem-8 and serum osmolality, urine osmolality, specific gravity and glucose o Suspect DI if serum osmolality is >300 mOsm/kg and urine osmolality is <300 mOsm/kg. DI is unlikely if serum osmolality is <270 or urine osmolality is >600. Definitive test is water deprivation test, response to sub-q AVP Treatment: o Central: DDAVP o Nephrogenic: free water and salt restriction, can try thiazide diuretics o Monitor I and Os Strictly!!!

SIADH Presentation: Hyponatremia (euvolemic)-associated with CNS etiologies, pneumonia, surgery-T&A especially SIADH is a diagnosis of exclusion, need to rule out other causes of hyponatremia, Will see: Urine osmolality >100 and usually greater than plasma, Serum osmolality <280, serum NA < 135, urine Na > 30 Work-up: Chem-8, serum osmolality, urine osmolality, TSH (rule out thyroid dx), uric acid (decreased in SIADH). AM cortisol (rule out adrenal insufficiency) Treatment: Fluid restriction Short Stature-Ht less than 3%, decreasing growth velocity, ht percentile significantly below target range DDx: Constitutional/Familial Short stature vs Pathological process o Genetic (suspect if dysmorphic): Turners, Trisomy 21, Noonans syndrome, Prader-Willi, Russel-Silver o Endocrine (weight > height): Cushings, Hypothyroidism, growth hormone deficiency o Malnutrition/Chronic dx (height > weight): celiac dx, IBD, CF, cardiac dx, renal dx, immunodeficiency Work-up: Parental Heights, CBC, CMP, ESR, TSH, T4, UA, IGF-1, IGFBP-3, screen for celiac disease and get bone age. May want to karyotype girls o Midparental Height-Girls: (paternal ht + maternal ht -5 inches)/2 o Midparental Height- Boys: (paternal ht + maternal ht + 5 in)/2 Treatment: Treat underlying cause, do not treat if constitutional or familial. Growth hormone deficiency can be treated with replacement Precocious Puberty-signs of 2ndary sexual maturation before age 8 in girls, or 9 in boys DDx: o Central: Early activation of hypothalamic-pituitary-gonadal axis, usually sporadic, but need to rule out brain mass o Peripheral: GnRH independent-causes: CAH, adrenal tumors, McCune-Albright syndrome, gonadal tumors, hCG producing tumors, exogenous sex hormones, hypothyroidism Work-up: Bone age, estradiol, testosterone/DHEAS levels, basal or GnRH-stimulated LH levels, 17-OHP. o If suspect central cause-MRI. Consider pelvic ultrasound in girls to look for ovarian mass Adrenal Insufficiency/Congenital Adrenal Hyperplasia Acute adrenal crisis: Presents with emesis, diarrhea, dehydration, hypotension, metabolic acidosis and shock. Often will be hypoglycemic, hyponatremic and hyperkalemic

ENDOCRINOLOGY

Causes of adrenal insufficiency:Congenital Adrenal Hyperplasia, chronic glucocorticoid treatment, Addisons disease, surgery, radiation, tumor or congenital defects affecting hypothalamus or pituitary Congenital Adrenal Hyperplasia: disorder of cortisol synthesis, can see glucocorticoid deficiency, mineralcorticoid deficiency or excess, virilization or premature puberty in males, virilization or sexual infantilism in affected females o Most common defect: 21-Hydroxylase deficiency: see glucocorticoid deficiency, mineralcorticoid deficiency, ambiguous genitalia in females, post-natal virilization in males and females Diagnosis: AM Cortisol (suspect if level <5), ACTH stim test o If suspect CAD: 17-Hydroxyprogesterone levels, elevated testosterone in girls, elevated androstenedione in males in addition to cortisol and ACTH stim test Treatment: o Glucocorticoid Replacement: Hydrocortisone 9-15 mg/m2/day divided q8 hrs Families are told to triple dose if fever, vomiting, or diarrhea If hospitalized may need 50-100 mg/m2/day PO of hydrocortisone divided q6-8, or 25-50 mg/m2/day IV For severe illness or surgery may need 50-125 mg/m2/day IV Also mineralcorticoid replacement for CAH, primary adrenal insufficiency 0.05-0.1 mg for infants. 0.05-0.2 mg for children and adults

ENDOCRINOLOGY

FLUIDS, ELECTROLYTES, AND NUTRITION (Harriet Lane, NICU Quick Reference Cards) Dehydration

Maintenance Fluid Needs:

FEN
Fluid Basics: 1. Bolus Normal Saline (0.9%) at 20 ml/hr over 10-15 minutes max and then another 20 ml/hr if clinical exam not significantly improved. 2. If you have done 2-3 boluses and still no clinical improvement consider adrenal insufficiency, cardiac insufficiency or need for pressor support 3. Maintance fluids: Above rate rules (4-2-1) with D5 NS (0.9) with 20 mEq KCl/liter. Hold KCl if concerns of oliguira/anuria. If age <1 month, consider normal saline. 4. If you have not completely corrected the fluid deficit, maintenance fluids will be insufficient unless the child is taking liquids by mouth. If the child has increased metabolic rate, fever, stress, high RR, will need more maintenance fluids because of increased insensible losses. Oral Rehydration: 1. May be used without IV for mild to moderate dehydration

2. 3. 4. 5. 6.

Always use pedialyte do not use water, Gatorade, or juice If child not willing to drink volume, place NG tube. Goal is 50-100 ml/kg over 3-4 hours If <5 years, 5ml q5min then 10 q 10 etc If >5 years, 10 ml q5min

Neonatal Fluid Rules DOL #1: Use D10W at 80 ml/kg/day DOL #2: Use D10 NS at 100 ml/kg/day DOL #3: Use D10 NS with 20 KCl at 120 ml/kg/day DOL #4: Use D10 NS with 20 KCl at 140 ml/kg/day DOL #5: Use D10 NS with 20 KCl at 150 ml/kg/day

Formula Choices Type MBM & Standard Formulas Hydrolysate formula Elemental formula Premature formula Big kid formula Examples MBM, Enfamil, Similac Nutramagin, Alimentum, Gentlease, Pregestimil Neocate, Elecare, Nutramagin AA Enfamil premie, Neosure Peptamen Jr, Pediasure, Boost Notes 1 scoop for 2 oz. water = 20kcal/oz Enzymatically hydrolyzed proteins, less alergenic Fully hydrolyzed, expensive Typically 22-24 kcal/oz

Growth Age 1-6 months 6-12 mo. 1-3 years 4-6 years Age Week 1 Week 2 <3 months 3-6 Weight gain (g/day) May lose up to 10% of birth weight Regain birth weight & start weight gain 25-30 (30 g = 1 ounce) 10-25 Kcal/kg/day 100-108 (if CHD, 120130) FEN ~100 80-110 70-100

ELECTROLYTES Hypoglycemia Treatment

<10 kg 10 to 40 kg

2 ml/kg bolus D10W 2-4 ml D25W (if central line or emergent) OR 5-10 ml D10W OR 10-20 ml/kg D5 in NS. 1 amp of D50 diluted 1:1 in water

>40 kg

FEN

EKG progression in hyperkalemia: 1. Peaked T waves 2. Loss of P waves with QRS widening 3. ST depression with further QRS widening 4. Bradycardia, AV block, torsades

FEN

FEN

GASTROENTEROLOGY GERD DDx Physiologic reflux Incomplete obstruction MSPI ; Eosinophilic esophagitis Pyloric stenosis- very young toddlers Diagnostic Tests History and Exam Esophageal pH monitoring Esophageal impedance Response to Acid suppression UGI series- insensitive, non-specific. Treatment Lifestyle- left side sleeping, elevate headend Acid suppression: PPI, H2 blocker Prokinetic: erythromycin, metoclopramide FTT DDx Psychosocial Anatomic (cleft, hypotonia, GERD, pyloric stenosis) Malabsorption (MSPI, CF, celiac, IBD) Increased metabolic needs (infection, malignancy endocrine, cardiac, pulmonary, renal disease)

Work-up Food diary, social work Exam, UGI, swallow study Stool analysis, sweat test, celiac panel CBC, CMP, UA/UCx, bone age, TB, HIV, thyroid, growth hormone

Chronic Abdominal Pain in Children (Nelson) DISORDER NONORGANIC Functional abdominal Nonspecific pain, often pain periumbilical Irritable bowel syndrome Non-ulcer dyspepsia Hx and PE; tests as indicated CHARACTERISTICS KEY EVALUATIONS

Intermittent cramps,diarrhea, Hx and PE and constipation Peptic ulcerlike symptoms without abnormalities on evaluation of the upper GI tract Hx; esophagogastroduodenoscopy

GASTROINTESTINAL TRACT Chronic constipation Lactose intolerance/Excess fructose ingestion Parasite infection (especially Giardia) Crohn disease Peptic ulcer Hx of stool retention, evidence Hx and PE; plain x-ray of abdomen of constipation on examination Symptoms may be associated Trial of lactose-free diet; lactose breath with lactose ingestion; hydrogen test/ History of large intake of bloating, gas, cramps, and fruit juice, sorbitol sweetened gum diarrhea Bloating, gas, cramps, and diarrhea Refer to IBD section Burning or gnawing epigastric pain; worse on awakening or Esophagogastroduodenoscopy or upper before meals; relieved with GI contrast x-rays antacids Epigastric pain with substernal Esophagogastroduodenoscopy burning Stool evaluation for O&P; specific immunoassays for Giardia

GI

Esophagitis

DISORDER

CHARACTERISTICS

KEY EVALUATIONS Meckel scan or enteroclysis

Periumbilical or lower Meckel's diverticulum abdominal pain; may have blood in stool Recurrent intussusception

Paroxysmal severe cramping Identify intussusception during episode abdominal pain; blood may be or lead point in intestine between present in stool with episode episodes with contrast studies of GI tract PE, CT of abdominal wall

Internal, inguinal, or Dull abdomen or abdominal abdominal wall hernia wall pain Chronic appendicitis or appendiceal mucocele

Recurrent RLQ pain; often incorrectly diagnosed, may be Barium enema, CT rare cause of abdominal pain RUQ pain, might worsen with Ultrasound of gallbladder meals RUQ pain, mass ? elevated bilirubin Persistent boring pain, might radiate to back, vomiting Ultrasound or CT of RUQ Serum amylase and lipase ? serum trypsinogen; ultrasound or CT of pancreas

GALLBLADDER AND PANCREAS Cholelithiasis Choledochal cyst Pancreatitis

GENITOURINARY TRACT Urinary tract infection Hydronephrosis Urolithiasis Other genitourinary disorders Dull suprapubic pain, flank pain Unilateral abdominal or flank pain Progressive, severe pain; flank to inguinal region to testicle Urinalysis and urine culture; renal scan Ultrasound of kidneys Urinalysis, ultrasound, IVP, CT

Suprapubic or lower Ultrasound of kidneys and pelvis; abdominal pain; genitourinary gynecologic evaluation symptoms nausea, family Hx migraine Might have seizure prodrome Anemia Vague abdominal pain ? constipation Recurrent, severe crampy abdominal pain, occult blood in stool, characteristic rash, arthritis Swelling of face or airway, crampy pain Severe pain precipitated by drugs, fasting, or infections Hx EEG (can require >1 study, including sleep-deprived EEG) Hematologic evaluation Serum lead level

MISCELLANEOUS CAUSES Abdominal migraine Abdominal epilepsy Sickle cell crisis Lead poisoning Henoch-Sch?nlein purpura Angioneurotic edema Acute intermittent porphyria

Hx, PE, urinalysis Hx, PE, upper GI contrast x-rays, serum C1 esterase inhibitor Spot urine for porphyrins

GI

Differential Diagnosis of GI Bleeding in Children INFANT COMMON Bacterial enteritis Milk protein allergy Intussusception Bacterial enteritis Anal fissure Colonic polyps Bacterial enteritis Inflammatory bowel disease Peptic ulcer/gastritis CHILD ADOLESCENT

INFANT

CHILD Intussusception Peptic ulcer/gastritis Swallowed epistaxis Prolapse (traumatic) gastropathy secondary to emesis Mallory-Weiss syndrome Esophageal varices Esophagitis Meckel diverticulum Lymphonodular hyperplasia Henoch-Sch?nlein purpura Foreign body Hemangioma, arteriovenous malformation Sexual abuse Hemolytic-uremic syndrome Inflammatory bowel disease Coagulopathy Duplication cyst

ADOLESCENT Prolapse (traumatic) gastropathy secondary to emesis Mallory-Weiss syndrome Colonic polyps Anal fissure

Swallowed maternal blood Anal fissure Lymphonodular hyperplasia

RARE

Volvulus Necrotizing enterocolitis Meckel diverticulum Stress ulcer, gastritis Coagulation disorder (hemorrhagic disease of newborn) Esophagitis

Hemorrhoids Esophageal varices Esophagitis Pill ulcer Telangiectasia-angiodysplasia Graft versus host disease Duplication cyst

Refeeding syndrome can complicate the acute nutritional rehabilitation of children who are undernourished from any cause .The hallmark of refeeding syndrome is the development of severe hypophosphatemia after the cellular uptake of phosphate during the 1st week of starting to refeed. Serum phosphate levels of 0.5 mmol/L can produce weakness, rhabdomyolysis, neutrophil dysfunction, cardiorespiratory failure, arrhythmias, seizures, altered level of consciousness, or sudden death. Phosphate levels should be monitored during refeeding, and if they are low, phosphate should be administered during refeeding to treat severe hypophosphatemia Inflammatory Bowel Disease (IBD)[8,9] 1 Classification/types: a Crohn's disease: Transmural inflammatory process affecting any segment of the GI tract from mouth to anus in discontinuous fashion. Abdominal pain, weight loss, and diarrhea. Other symptoms include lethargy, anorexia, fever, nausea, vomiting, growth retardation, malnutrition, delayed puberty, psychiatric symptoms, arthropathy, and erythema nodosum[9] b Ulcerative colitis (UC): Chronic relapsing inflammatory disease of the colon and rectum. Symptoms (present for at least 2 weeks) include gross or occult rectal bleeding, diarrhea, abdominal pain with or around time of defecation. Exclusion of enteric pathogens (e.g., Salmonella, Shigella, Yersinia, Campylobacter, Escherichia coli 0157:H7, C. difficile) is necessary.[10] Weight loss, anorexia, lethargy are less common 2 Evaluation: a Complete history and physical exam including family history, exposure to infectious agents or antibiotic treatment, assessment of hydration and nutritional status, signs of peritoneal inflammation, signs of systemic chronic disease. Stomatitis, perianal skin tags, fissures, fistulas are suggestive of Crohn's Disease. Presence of fever, orthostasis, tachycardia, abdominal tenderness, distension, or masses suggests moderate to severe disease and need for hospitalization b Laboratory assessment: CBC, ESR, CRP, serum urea and creatinine, serum albumin, liver function tests. IBD is associated with decreased hemoglobin and albumin, rise in platelet count, ESR, CRP (although children with UC may have normal hemoglobin, platelets, and ESR). Anti-neutrophil cytoplasmic antibodies (ANCA) may be elevated in UC. Diagnostic endoscopy is typically used to make diagnosis 3 Management: a First-line therapy: Corticosteroids, 5-aminosalicylates, antibiotics

GI

b c

Second-line: Immunosuppression :azathioprine, methotrexate Crohn's, cyclosporine, tacrolimus and anti-tumor necrosis factor (TNF) monoclonal antibodies Surgical intervention: only after medical management has failed in both.

Pancreatitis 1 Presentation: Sudden onset of abdominal pain associated with rise of pancreatic digestive enzymes in serum or urine with or without radiographic changes in the pancreas. Etiologies: trauma, multisystem disease, drugs, infections, idiopathic, and congenital anomalies. 2 Diagnosis/evaluation: a Clinical signs/symptoms: Abdominal pain (sudden or gradual, most commonly epigastric), anorexia, nausea, vomiting, tachycardia, fever, hypotension, guarding/rebound tenderness/decreased bowel sounds, sonographic or radiologic evidence of pancreatic inflammation. Gray-Turner and Cullen's sign are rare in children b Laboratory findings: (1) Elevated lipase and amylase: 3 times above normal limit (but no correlation with disease severity); lipase is more sensitive and specific for acute pancreatitis but normalizes more slowly so it may be preferable to follow amylase after establishment of diagnosis [8] (2) Additional findings: Leukocytosis, hyperglycemia, glucosuria, hypocalcemia, hyperbilirubinemia 3 Management: a Pancreatic rest: Nasogastric decompression, analgesia, IV fluid hydration, and oral intake restriction. Enteral feeding via naso-jejunal tube may be used for nutrition. b Antibiotics are reserved for only the most severe cases (Nelson, Harriet Lane)

GI

HEMATOLOGY/ONCOLOGY Blood Product Transfusions *Think of labs youd want before transfusion, (i.e. Coombs). Order now or draw and hold. Irradiation: prevents GVHD in neonates, oncology patients, ALC <500. Nebraska blood is leukoreduced. CMV negative: when CMV negative/unknown and neonate or oncology or HIV. Leukoreduced: when receiving regular blood transfusions, Hx severe reactions to blood products, potential transplant candidates, CMV neg is unavailable. PRBCs: Type: Patients blood incubated with antibodies to common blood group antigens. Cross-match: Patients serum mixed with donor PRBCs to insure compatibility. Uncross-matched PRBCs: Usually O neg, used only in emergencies. Transfuse when Hb <7-8; higher for infants <4 mo, patients with cardiopulmonary disease. 1 unit ~300 mL. Dose based on initial Hb. Monitor for CHF symptoms. Hb 8-10: 10 mL/kg over 3 hours. Hb 5-8: 10 mL/kg over 3-4 hours. Hb 3-5: 5 mL/kg over 3 hours, repeat after 2-3 hours break. Hb <3: 3 mL/kg over 3 hours, repeat after 2-3 hours break. Platelets: Transfuse when: plt <50 and bleeding or invasive procedure. plt <20 and marrow failure with hemorrhagic risk factors. plt <10 and marrow failure without hemorrhagic risk factors. Higher parameters for infants <4 mo 1 unit ~300-600 mL. Dose: 10 mL/kg Volume-reduced: 50% volume reduction; longer prep time and significant loss of platelets and plasma/factors. Peak platelets 30 min after infusion, expect increase by 30,000 per unit. FFP: Replaces all clotting factors, antithrombin III, protein C, protein S 1 unit ~250 mL, dose 10-15 mL/kg, will raise clotting factors by 1%. Cryoprecipitate: Replaces factors VIII, XIII, fibrinogen, vWF. 1 unit ~10-15 mL, dose 1-2 units/10 kg, 1 unit contains 200 mg fibrinogen. Transfusion reactions Acute hemolytic: mismatched ABO, Rh, JKa antigens. Fever, flank pain, hemoglobinuria, DIC. Management: stop transfusion. ABCs. NS 1.5-2x maint (dextrose may aggravate hemolysis). Support BP and renal function. Delayed hemolytic: anamnestic response to previously incompatible minor blood group antigen, commonly Rh or Kidd antigens. Jaundice, back pain, falling Hb, newly positive Ab screen, spherocytes on smear. Management: identify incompatible antigens to prevent future reactions. Extended crossmatch call and discuss with bloodbank. Anaphylactic: commonly IgA deficient patients exposed to IgA via transfusion. Management: stop transfusion. ABCs. Fluids to support BP. Epinephrine, antihistamines prn. Transfusion-related acute lung injury (TRALI): transfused anti-HLA antibodies destroying host granulocytes. Within 6 hours: dyspnea, hypotension, fever, tachycardia, noncardiogenic pulmonary edema. Management: supportive, mechanical ventilation if needed, consult PICU. Transfusion associated circulatory overload (TACO): within 6 hours: tachypnea, orthopnea, hypoxemia, tachycardia, hypertension. Elevated CVP, pulmonary wedge pressure, BNP (normal in TRALI). Management: supportive O2, diuretics, phlebotomy if needed. Febrile: transfused granulocytes destroyed by host or transfused cytokines causing fevers during or after transfusion. Management: stop transfusion, evaluate for infection, acetaminophen. Infection: CMV, EBV, HIV, bacterial. Management: cultures, viral titers, antibiotics if indicated. Mechanical hemolysis: schistocytes, negative antibodies. (Dr. Harper, UpToDate, Nelson) HEM / ONC

Anemia

Age-Specific Blood Cell Indices Age Hb Hct Retic Preterm 13423-10 15 47 Term 16.5 51 3-7 1 mo 13.9 44 0.11.7 6 mo-2 yr 12 36 0.72.3 2-6 yr 12.5 37 0.51.0 6-12 yr 13.5 40 0.51.0 12-18 yr M 14.5 43 0.51.0 12-18 yr F 14 41 0.51.0

MCV 118120 108 101 78 81 86 88 90

WBC Platelets 4.4 250-290 18.1 10.8 10.6 8.5 8.1 7.8 7.8 290 150-350 150-350 150-350 150-350 150-350 150-350 (Harriet Lane)

HEM / ONC

Anemia in a newborn

(Nelson)

HEM / ONC

Classification of anemia Retic Microcytic Low Iron def Lead poisoning Chronic disease Aluminum toxicity Copper def Protein malnutrition

Normocytic Chronic disease RBC aplasia (TEC, infection, druginduced) Malignancy JIA Endocrinopathies Renal failure

Macrocytic Folate, B12 def Aplastic anemia Congenital bone marrow dysfunction (Diamond-Blackfan or Fanconi) Drug-induced Trisomy 21 Hypothyroidism

Normal Thalassemia trait Sideroblastic anemia High Thalassemia syndromes Hemoglobin C disorders

Acute bleeding Hypersplenism Dyserythropoietic anemia II Antibody-mediated hemolysis Dyserythropoietic anemia I, Hypersplenism III Microangiopathy Active hemolysis (HUS, TTP, DIC, KasabachMerritt) Membranopathies (spherocytosis, elliptocytosis) Enzyme disorders (G6PD, pyruvate disorders) Hemoglobinopathies

Iron deficiency: Decreased ferritin (can be falsely elevated with inflammation or infection), low serum iron, elevated TIBC, low MCV, high RDW, elevated transferring receptor level, low reticulocyte Hb content Mentzer Index: MCV/RBC >13.5 suggests Fe def, <11.5 suggests thalassemia minor. RDW >13.5% suggests Fe def. Iron therapy results in increased retic count 2-3 days, increased Hct 1-4 weeks, iron stores repleted 3 months. Hemolytic anemia Increased retic count and corrected retic count (% reticulocytes x patient Hct/normal Hct). Increased aspartate aminotransferase, LDH (intracellular enzymes), decreased haptoglobin. Direct Coombs: Antibody or complement on patient RBCs. Indirect Coombs: Free autoantibody in patients serum after RBC antibody binding sites are saturated. Spherocytosis by osmotic fragility test; G6PD by assay, Heinz body preps; hemoglobinopathies by Heinz body preps. Physiologic anemia of infancy: Hb 9-11 btw 8-12 wks full-term and 3-6 wks preterm. (Nelson)

HEM / ONC

Thrombocytopenia

Shaded boxes for conditions common in neonatal period (Nelson) HEM / ONC

Coagulation (Harriet Lane)

Hemophilia Questions to ask: type and severity of hemophilia? Presence of inhibitor antibody? Factor concentrate normally used and time of last treatment? Primary hematologist name? Factor VIII replacements: Advate, Xyntha, Recombinate, Helixate, Kogenate FS Dose: % desired rise in activity x kg x 0.5 Factor IX replacements: Mononine, Alphanine SD, Benefix Dose: % desired rise in activity x kg x 1.4 Severe bleeding (head, neck, thorax, pelvis): 1. Give factor BEFORE anything else. Factor VIII: 50 units/kg IV bolus, then 4 units/kg/hr continuous IV Factor IX: 100 units/kg IV bolus, then 5 units/kg/hr continuous IV Inhibitor patients: use one or the other NovoSeven RT: 90 mcg/kg IV bolus q2 x3, or megadose 270 mcg/kg IV bolus q6 FEIBA: 50-100 units/kg IV q6 2. STAT call hem/onc. Admit to PICU if unstable vitals, altered mental status. 3. CT without contrast of injured area AFTER factor given 4. STAT neurosurgery consult if suspected or proven intracranial hemorrhage. Surgery consult if appropriate. 5. Type and cross-match >2-3 units PRBCs. Pre- and post-op care: Mild hemophilia A: DDAVP pre-op, aminocaproic acid IV or PO (100 mg/kg/dose), Factor VIII if needed. Moderate or severe hemophilia: Factor bolus to raise factor to 100%, then continuous infusion, aminocaproic acid if needed. Check factor level at 24 hours or sooner if Hx inhibitor or rapid clearance. For low levels, bolus to 100% correction, recheck at 2 hours. von Willebrand Disease (Dr. Harper) Prothrombotic States Congenital Deficiency of anticoagulants: AT-III, protein C, protein S, plasminogen

HEM / ONC

 Resistance to cofactor proteolysis: Factor V Leiden High levels of procoagulants: prothrombin 20210 mutation, elevated Factor VIII Damage to endothelium: homocystinemia Acquired Obstruction to flow: indwelling lines, pregnancy, polycythemia/dehydration Immobilization Injury: trauma, surgery, exercise Inflammation: IBD, vasculitis, infection, Behcet syndrome Hypercoagulability: pregnancy, malignancy, antiphospholipid syndrome, nephrotic syndrome, oral contraceptives, L-asparaginase, elevated Factor VIII Rare other entities Congenital: dysfibrinogenemia Acquired: paroxysmal nocturnal hemoglobinuria, thrombocythemia, vascular grafts (Nelson) Sickle Cell Disease Vaso-occlusive crisis Differential: VOC, pneumonia, osteomyelitis, other infection, intestinal obstruction. Check sats, temp, imaging. Management: Fluids: NS bolus, then 1.5-2x maintenance, 1-1.5x maintenance after 24-48 hours to avoid over-hydration (pulmonary edema increases risk of ACS) Pain ladder: with incentive spirometry, supplemental oxygen, and ambulation to decrease ACS risk. 1. NSAID (combo) (acetaminophen +/- ibuprofen) 2. NSAID + oral narcotic (Lortab) 3. NSAID + IV bolus narcotic (ketorolac + morphine) 4. NSAID + PCA (acetaminophen + morphine or hydromorphone) PCA results in good pain control with less narcotic use and quicker release from hospital compared to continuous infusion. Dose mg/kg/hr (IV continuous infusion dose): basal rate. hourly total of PCA doses with 10 min lockout time Splenic sequestration: vaso-occlusion of spenic venules. Can trap RBC, WBC, platelets, plasma. Obstruction can be relieved with improved hydration status and blood components returned to circulation. Dx: splenomegaly (absolute or relative), sudden drop Hb >2 gm/dl with reticulocytosis, thrombocytopenia, leukopenia, hypotension/hypovolemic shock. Management 1. IV access, 1 if stable, 2 if unstable 2. Hydration: D5NS at 1.5x maintenance + boluses to maintain BP prn. 3. Labs: CBC, retic, type and crossmatch, chem14, LDH. 4. Careful measurement of spleen. 5. Review records to see if this is repeat event. 6. Call hem/onc and surgery. Surgical indications: first event with hemodynamic instability and pancytopenia, or second event even if stable. 7. Once vitals stable, transfuse gingerly. Trapped deoxygenated RBCs can return to circulation. Avoid Hb >10 to prevent hyperviscosity. Aplastic crisis: Rapid fall Hb with low retic, normal platelet, normal WBC. May see pancytopenia in immunocompromised. Sickle RBC lifespan 10 days (vs 120 days in normal). Dx: fever, slapped cheek rash, arthropathy, flu-like sx. Management: CBC, retic, Parvovirus B19 PCR,IgG, IgM, Type and Possible. IV access. Contact/droplet isolation, no pregnant caregivers. Course: normal sicklers resolve 2-3 wks (antibodies formed). Immunocompromised patients take months, may need IVIG. Stroke Dx: hemiparesis, dysphagia, gait disturbance, altered LOC. Management: CBC, retic, Chem14, LDH. Type and Cross-match for Double Volume Exchange Transfusion (call blood bank and Red Cross Medical Director). Admit to PICU. STAT call hem/onc on call, STAT consult Peds Neurology/Stroke team.

HEM / ONC

o Imaging: CT vs MRI/MRA/MRV. Contrast may aggravate sickling, safe when Hb S <30% o Transfusion: Exchange (better, manual or mechanized) vs simple. Optimally 2 large bore lumen CVL (phoresis catheters), but can do 2 large bore IVs. Will need long-term transfusions. PT, OT, rehab. (Dr. Harper) Pediatric malignancies Pertinent work-up History: weight loss, fatigue, fevers, night sweats, bleeding/bruising, pain Exam: petechiae/bruising, mass, lymph nodes, respiratory distress, hepatosplenomegaly Initial work-up: CBC with smear read by pathologist, CMP, Mg, Phos, LDH, uric acid, CRP, ESR, CXR, other imaging (Harriet Lane) ALL Standard risk: Age 1-10 yrs, WBC <50,000 High risk: Age >10 yrs, WBC >50,000 Very high risk:t(9;22), hypodiploidy Higher risk: Infant ALL (age <1 year) (Nelson) Oncologic emergencies Fever and neutropenia o ANC = WBC x neut% x 100, include bands. Neutropenia for oncology patients = ANC <500 o Goal to have abx in within 1 hour of fever onset o Initial evaluation: vitals, last chemo, last ANC, infection-related symptoms Infected sites will have pain in absence of redness, swelling. o Labs: CBC, blood culture Only if wont delay Abx: BMP/CMP, CXR, UA/culture depending on symptoms o Treatment: broad-spectrum IV abx Cefepime 50 mg/kg/dose IV q8hrs Add vancomycin if skin source or recent high dose cytarabine (risk strep viridans) Add gentamicin if shaking chills or hypotension Observe patient at least 1 hour after abx; can decompensate quickly. Tumor Lysis Syndrome: hyperkalemia, hyperuricemia, hyperphosphatemia o More common in Burkitts lymphoma, lymphoblastic lymphoma, ALL o Management: BMP, Phos, Uric acid, q6-12 hrs Hydration 1.5-2x maintenance, no K in fluid. Goal UOP 2-3 ml/kg/hr, mannitol diuresis if needed Hyperkalemia: Ca2+, NaHCO3, kayexalate, lasix, insulin/glucose, albuterol. Hyperuricemia: Allopurinol or rasburicase Hyperphosphatemia: Low phosphate diet, phos binder (sevelamer); CaCO3 (Tums) if hypocalcemic Hyperleukocytosis: WBC >100,000: hyperviscosity, leukostasis, microthrombi o More common in ALL, AML, esp T-cell ALL and infant leukemia o Pulmonary leukostasis: hypoxia, dyspnea o Brain hemorrhage or clot: headache, altered mental status, blurred vision o Priapism, dactylitis (less common) o Management IV hydration No RBCs unless critically anemic Transfuse plt if <20,000 to prevent hemorrhage Leukophoresis: temporarily decreases WBC Mediastinal mass o Superior mediastinal syndrome: compression of trachea, cough, dyspnea, orthopnea, wheezing, stridor. o Superior vena cava syndrome: compression of SVC, headache, lethargy, vision changes, facial edema or cyanosis, plethora, distended head/neck veins

HEM / ONC

o Management: frequent vitals, avoid supine, avoid sedation/intubation Non-invasive diagnostic testing: blood, bone marrow aspirate, thoracentesis, peripheral node biopsy Corticosteroids, radiation Acute abdomen o Classic physical findings may by masked due to neutropenia, steroids o Pain is principal symptom o DDx: appendicitis, typhlitis (necrotizing colitis of cecum), pancreatitis (asparaginase), constipation/ileus (vincristine) o Management Appendicitis: appendectomy Typhlitis: NPO, abx, supportive care; surgery if free air or hemorrhage Pancreatitis: bowel rest, abx, surgical drainage of abscess if indicated Constipation/ileus: No enemas. Golytely, colace, senna, etc. Increased ICP: stat call neurosurgery, elevate head of bed 30 degrees, no hypotonic fluids, dexamethasone, mannitol, intubation/hyperventilation, ventriculostomy per neurosurgery. Spinal cord compression: metastatic neuroblastoma, Ewing sarcoma, germ cell tumors, CNS tumors o Back pain, progressive weakness, paresis, sensory abnormalities, constipation or incontinence. o Management: Spine MRI, dexamethasone 1-2 mg/kg, radiation therapy, surgical decompression and laminectomy, chemotherapy Other neurological emergencies o PRES: posterior reversible leukoencephalopathy syndrome; caused by chemo and high BP o Ifosfamide: confusion, seizure-like activity o Vincristine or high-dose cyclophosphamide: SIADH o Asparaginase: hemorrhage or thrombotic stroke o Cranial radiation: somnolence syndrome 6-8 weeks later o IT chemo: chemical arachnoiditis, spinal headaches last 24-48 hrs. o Methotrexate neurotoxicity: due to folate depletion; hx IT MTX in last 4-8 days Seizure or stroke-like symptoms; weakness can be asymmetric Treatment: IV leukovorin, usually self-resolve 24-72 hrs. (Dr. Lowas)

Chemotherapeutic agents (M = myelosuppression, N = nausea, V = vomiting) Drug Mechanism Adverse reactions Comments Methotrexate Folic acid M, mucositis, Monitor levels with highantagonist dermatitis, osteopenia, dose MTX or with renal with high-dose: renal failure. Leucovorin rescue. and CNS toxicity 6-Mercaptopurine Purine analog M, hepatic necrosis, Allopurinol increases mucositis toxicity Cytarabine Pyrimidine Fever, rash, myalgias, analog CNS dysfunction Cyclophosphamide, Alkylates M, N, V, hemorrhagic Mesna to prevent ifosfamide guanine cystitis, pulmonary hemorrhagic cystitis; fibrosis, SIADH, cyclophosphamide less bladder cancer, effective in liver anaphylaxis dysfunction; ifosfamide can have CNS dysfunction. Doxorubicin, Intercalates M, N, V, Dexrazoxane reduces risk daunorubicin, DNA cardiomyopathy, red of cardiotoxicity idarubicin urine, tissue necrosis on extravasation Dactinomycin Inhibits DNA M, N, V, tissue transcription necrosis on extravasation Bleomycin Cleaves DNA N, V, pneumonitis,

HEM / ONC

strands Vincristine Vinblastine Inhibits microtubule formation Depletes Lasparaginase

PEG-asparaginase

Prednisone and dexamethasone

Lymphatic cell lysis

Carboplatin and cisplatin

Inhibits DNA synthesis

Etoposide

Topoisomerase inhibitor

stomatitis, Raynaud, pulmonary fibrosis, dermatitis Local cellulitis if given peripherally, peripheral neuropathy, constipation, jaw pain, SIADH Allergic reaction, pancreatitis, hyperglycemia, platelet dysfunction and coagulopathy, encephalopathy Hypertension, hyperglycemia, mood swings, Cushing syndrome, cataracts, myopathy, osteoporosis M, N, V, renal dysfunction, ototoxicity, tetany, neurotoxicity, HUS, anaphylaxis M, N, V, secondary leukemia

IV only, must not be allowed to extravasate; fatal if given intrathecally

Aminoglycosides may increase nephrotoxicity

(Nelson)

HEM / ONC

ID

INFECTIOUS DISEASES

ID

ID

Fever without a Localizing Source

FIGURE 17-2 Algorithm for the management of a previously healthy infant 90 days of age with a fever without localizing signs. This algorithm is a suggested, but not exhaustive, approach to management. hpf, High-power field. (Modified from Baraff LJ: Management of fever without source in infants and children. Ann Emerg Med 2000;36[6]:602614; and Baraff LJ: Management of infants and young children with fever without source. Pediatr Ann 2008;37(10):673679.) Fever without Localizing Source: Evaluation and Management Guidelines

Age 90 days: See Figure 17-2. Due to the greater risk of serious bacterial infections in young infants with fever, a conservative approach is warranted a 28 days with fever should be hospitalized for full evaluation b Infants >28 days who are well-appearing and meet low-risk criteria may potentially be managed as outpatients if reliable follow-up and monitoring is assured

ID

ID

Age >90 days: Traditionally, similar risk stratification strategies had been advocated and utilized to aid in evaluation and management of febrile children >90 days. However, the marked decline in invasive infections due to Haemophilus influenzae type B and Streptococcus pneumoniae since the introduction of conjugate vaccines has significantly reduced the likelihood of serious bacterial infection in a well-appearing child within this age group a If ill-appearing without source of infection identified, consider admission and empiric antimicrobial therapy b c If source of infection identified, treat accordingly If well-appearing and without foci of infection, many experts now advocate urinalysis + urine culture as the only routine diagnostic test, if reliable follow-up and monitoring is assured, including all females and uncircumcised males <2 years, all circumcised males <6 months, and all children with known genitourinary tract abnormalities[5,6]

Treatment NICU: use Ampicillin and Gentamicin Childrens: use Acyclovir, Ampicillin and Cefotaxime

Common pediatric infections: guidelines for initial management (Harriet Lane) Infectious Syndrome Usual Etiology Suggested Empirical Therapy Suggested Length of Therapy/Comments

Neonatal bacterial infections Duration depends on extent of disease: 10 days for bacteremia, 14 days for uncomplicated meningitis, up to 34 weeks for complicated Ampicillin + infection aminoglycoside (especially Early onset disease (<7 if meningitis; usually days old): Respiratory gentamicin) distress, apnea, shock, Alternative: Cefotaxime pneumonia, and less often, meningitis. Late-onset disease (1 week3 months): Bacteremia, meningitis, osteomyelitis, septic arthritis, and cellulitis

GBS infections

GBS

Chlamydial infections Conjunctivitis (ophthalmia neonatorum)

Chlamydia trachomatis Erythromycin 50 mg/kg/day 14 days PO/IV divided QID 5 days Alternative: Azithromycin Exudative conjunctivitis, Regular saline irrigation onset 310 days. Topical treatment ineffective All infants should receive silver nitrate,

ID

Infectious Syndrome

Usual Etiology

Suggested Empirical Therapy

Suggested Length of Therapy/Comments tetracycline, or erythromycin ointment instilled into each eye within 1 hr of birth NOTE: Association between PO erythromycin and pyloric stenosis has been reported in infants <6 weeks

Pneumonia

14 days 5 days Presents at 219 weeks Erythromycin 50 mg/kg/day of life with staccato PO/IV divided QID cough, tachypnea, rales, Alternative: Azithromycin bilateral infiltrates and hyperinflation on CXR, usually afebrile Infants born to mothers with active gonococcal infection should be empirically treated with a single dose of ceftriaxone Single dose 7 days Exudate conjunctivitis, onset 24 days. Admit for evaluation and treatment of possible disseminated disease All infants should receive silver nitrate, tetracycline, or erythromycin ointment instilled into each eye within 1 hr of birth

Gonococcal infections

Neisseria gonorrhoeae

Conjunctivitis (ophthalmia neonatorum)

Ceftriaxone 2550 mg/kg (max 125 mg) IV/IM Alternative: Cefotaxime Regular saline irrigation

Sepsis, arthritis, scalp abscess Meningitis S. aureus, GBS, gram-negative bacilli, N. gonorrhoeae (see above)

Ceftriaxone 2550 mg/kg 7 days IV/IM q24hr or cefotaxime Ceftriaxone or cefotaxime 14 days

Septic arthritis

(Cefotaxime or gentamicin) + (nafcillin or oxacillin) Consider vancomycin Drainage essential instead of PCNs if MRSA prevalent

Meningitis GBS, E. coli, L. monocytogenes Ampicillin + (cefotaxime and/or gentamicin) Add gentamicin if gramnegative organisms 1421 days for GBS and Listeria 21 days for gram negative organisms (Cefotaxime plus aminoglycoside)

Neonate <1 mo

Pneumonia Neonatal E. coli, GBS, Ampicillin and Streptococcus gentamicin, ? cefotaxime spp., S. aureus, L. Vancomycin if MRSA 1021 days, depending on disease severity Blood cultures indicated.

ID

Infectious Syndrome

Usual Etiology

Suggested Empirical Therapy

Suggested Length of Therapy/Comments Effusions should be drained and gram stain of fluid obtained 710 days (longer for some pathogens) Occult bacteremia with susceptible S. pneumoniae may be treated with amoxicillin if afebrile, well, and without focal complications

monocytogenes, C. prevalent trachomatis

Bacteremia (outpatient)

S. pneumoniae, GAS, Neisseria meningitidis, E. coli, Salmonella spp.

Ceftriaxone or cefotaxime

Metronidazole 500 mg PO BID Polymicrobial, often Alternatives: 7 days Bacterial vaginosis Gardnerella Metronidazole gel 0.75% 5 days vaginalis 5 g intravaginally daily or 7 days clindamycin cream 2% 5 g intravaginally daily Bites PO: Amoxicillin/clavulanic acid Streptococcus Alternative: Clindamycin + spp., S. aureus, S. (3rd-generation epidermidis, oral cephalosporin or anaerobes, TMP/SMX) Eikenella IV: Ampicillin/sulbactam corrodens, Alternative: TMP/SMX + Haemophilus spp. (clindamycin or cefoxitin or meropenem) Add Pasteurella multocida, Capnocytophaga, Same Corynebacterium spp., Neisseria spp. S. aureus, CONS, enteric gram negative bacilli, Candida spp; Immunocompromised patients: Add Pseudomonas, Corynebacterium spp. Immunocompetent: Vancomycin + 3rdgeneration cephalosporin or aminoglycoside Immunocompromised: Vancomycin + (ceftazidime or cefepime or piperacillin/tazobactam or aminoglycoside) PO: Cephalexin Alternative: Clindamycin if PCN-allergic or MRSA common IV: Oxacillin Alternative: Clindamycin or vancomycin if MRSA common 57 days Cleaning, irrigation, and d?bridement most important Assess tetanus immunization status, risk of hepatitis B and HIV Antibiotic prophylaxis routinely used for human bites 710 days Assess tetanus immunization status, risk of rabies Antibiotic prophylaxis for all cat bites and selected dog bites

Human

Dog/cat

Catheter-related bloodstream infections

Consider catheter removal and fungal coverage as clinically indicated

Cellulitis

GAS, S. aureus (MSSA or MRSA)

3 days after acute inflammation resolves (usually 710 days) TMP/SMX not active against GAS See Figure 17-5 for management of skin and soft tissue infections caused by communityacquired S. Aureus

ID

Infectious Syndrome Conjunctivitis (Suppurative, nonneonatal)

Usual Etiology

Suggested Empirical Therapy

Suggested Length of Therapy/Comments 5 days Ointments preferred for infants or young children and eyedrops for older children and adolescents

S. pneumoniae, H. Ophthalmic: Erythromycin, influenzae bacitracin/polymyxin B, or (nontypeable) polymyxin B/TMP Moraxella S. pneumoniae, H. influenzae, S. aureus, S. pyogenes, P. aeruginosa, CONS

Dacrocystitis

Initial conservative management: warm Consider ophthalmologic compresses. evaluation to relieve PO: Nafcillin/oxacillin or obstruction cephalexin or clindamycin Consider dental evaluation for surgical drainage

Dental abscesses Gastroenteritis Community

Oral flora, including Clindamycin or anaerobes amoxicillin/clavulanic acid

Viruses Antibiotic therapy strongly discouraged because of possible increased risk for hemolytic-uremic syndrome occurring in patients with E. coli 0157:H7 treated with antibiotics[12] Azithromycin or ciprofloxacin for traveler's diarrhea Cefotaxime or ceftriaxone Alternative: Azithromycin Ampicillin, amoxicillin, or TMP/SMX for susceptible strains

Primary treatment: Fluid and electrolyte replacement

Acquired

E. coli

Salmonella spp.

1014 days for infants <3 mo, bacteremia, toxicity, hemoglobinopathy, or immunosuppressed. Antibiotics generally not indicated otherwise 5 days for dysentery, immunosuppressed, or to prevent spread in mild disease. Oral cephalosporins not useful. Amoxicillin or TMP/SMX can be used if susceptible, but not recommended for empiric treatment due to high rates of resistance Usually no antibiotic therapy is recommended except with bacteremia, extraintestinal infections, or immunocompromised hosts 57 days. Shortens duration of fecal

Shigella spp.

Ceftriaxone (IV), azithromycin, or fluoroquinolone

Yersinia spp.

TMP/SMX, aminoglycosides, cefotaxime, flouroquinolone, or tetracycline (8 yr) Azithromycin or erythromycin

Campylobacter spp.

ID

Infectious Syndrome

Usual Etiology

Suggested Empirical Therapy

Suggested Length of Therapy/Comments excretion 7 days Stop the precipitating antibiotic therapy as soon as possible Community organisms unlikely after 72 hr of hospitalization Treatment: 5 days, greatest benefit when initiated within 48 hours of symptom onset Chemoprophylaxis: 5 10 days. Recommendations vary yearly based on characteristics and resistance patterns of circulating strains. See http://www.cdc.gov/flu/ for most up-to-date recommendations The FDA granted an Emergency Use Authorization for the use of oseltamivir in children <1 yr in 2009. However, none of the antivirals have been licensed for general use in children <1 yr

Nosocomial

Metronidazole Clostridium difficile Oral vancomycin for severe infection

Influenza

Influenza A Influenza B

Neuraminidase inhibitors: Oseltamivir (1 yr) or Zanamivir (7 yr for treatment, 5 yr for chemoprophylaxis); Adamantine class: Rimantadine (>13 yr for treatment, >1 yr for prophylaxis) or Amantadine (>1 yr) for influenza A (no activity against influenza B) if circulating strain susceptible (novel H1N1 resistant)

Lymphadenitis

Viruses, GAS, M. tuberculosis, S. aureus, anaerobes, atypical mycobacteria, Actinomyces, Bartonella henselae (cat scratch disease)

PO: Amoxicillin/clavulanic acid or clindamycin if MRSA prevalent Alternative: Cephalexin or dicloxacillin IV: Oxacillin or nafcillin or clindamycin if MRSA present Alternative: Cefazolin If PCN-allergic: Cefdinir, cefuroxime, or vancomycin Ampicillin/sulbactam or clindamycin Alternative: Cefuroxime for PCN allergy

Surgical excision with M. tuberculosis Needle aspiration with B. henselae

S. pneumoniae, S. pyogenes, S. Mastoiditis (acute) aureus, H. influenzae (nontypeable)

10 days Surgical management required, definitive therapy should be guided by culture obtained at surgery

Meningitis (nonneonatal) Infants >1 mo and children

Duration depends on organism Cefotaxime or ceftriaxone, See Red Book 2009[9] S. pneumoniae, N. + vancomycin (resistant S. for chemoprophylaxis meningitidis, H. Pneumoniae) recommendations for influenzae, For severe PCN allergy, contacts of neonatal consider chloramphenicol meningococcal and Hib pathogens + vancomycin disease Dexamethasone use, except for H. influenzae

ID

ID

Infectious Syndrome

Usual Etiology

Suggested Empirical Therapy

Suggested Length of Therapy/Comments uncertain

Orbital cellulitis

Ampicillin/sulbactam; or (cefotaxime or ceftriaxone) S. pneumoniae, H. AND (clindamycin or influenzae oxacillin) (nontypeable), Alternative: Cefuroxime Moraxella PCN allergy: (vancomycin catarrhalis, S. or clindamycin) + rifampin, aureus, GAS OR aztreonam Consider vancomycin if concern for MRSA

10 days Recommend ophthalmologic consultation, CT to evaluate intracranial extension, cavernous thrombosis

Osteomyelitis Uncomplicated S. aureus, GAS, Streptococcus spp., Kingella Oxacillin or nafcillin or clindamycin Alternative: Vancomycin 46 wk Clindamycin ineffective as monotherapy for Kingella infections

Foot puncture Sickle cell disease

Add ceftazidime or antiAdd Pseudomonas pseudomonal PCN and aeruginosa aminoglycoside Add Salmonella spp. Add cefotaxime or ceftriaxone

10 days (57 days for Not severe: High dose healthy children >6 yr) amoxicillin (80 Analgesia for otalgia 90 mg/kg/day) 10 days Severe (moderate-severe If >6 mo, not severe, S. pneumoniae, H. otalgia or fever >39?C): and uncertain diagnosis, influenzae Otitis media (acute) Amoxicillin/clavulanic acid or >2 yr and not severe, (nontypeable), M. Alternative for PCN allergy: observation (watchful catarrhalis, viruses cefuroxime, cefdinir, or waiting) may be azithromycin employed with Ceftriaxone IM if cannot reassessment at 4872 take PO hours Single dose Persistent otitis media (after 3 days): Amoxicillin/clavulanic acid, cefuroxime, or ceftriaxone IM daily Otitis externa (uncomplicated) Consider tympanocentesis 3 days (for IM Ceftriaxone)

710 days Eardrops: Polymyxin Pseudomonas, Analgesics for pain B/neomycin/hydrocortisone Enterobacteriaceae Consider wick for or ciprofloxacin or , Proteus spp, fungi moderate to severe TMP/SMX cases S. aureus most common. Also: oral flora, gramnegative rods, viruses (including mumps, HIV, EBV), or noninfectious causes Sialogogues, local heat, gentle massage of the gland from posterior to PO: Clindamycin or anterior, and hydration nafcillin/oxacillin/dicloxacilli provide symptomatic n or amoxicillin/clavulanic relief. Surgical drainage acid may be required. Consider HIV test if chronic parotitis 1014 days If secondary to local trauma and not

Parotitis

Periorbital cellulitis GAS and Ampicillin/sulbactam or (preseptal) Streptococcus spp., amoxicillin/clavulanic acid S. aureus, H. or clindamycin + 3rd-

Infectious Syndrome

Usual Etiology

Suggested Empirical Therapy

Suggested Length of Therapy/Comments

influenzae (nontypeable), M. catarrhalis GAS, Group C and G streptococci, Arcanobacterium haemolyticum, Mycoplasma, viruses (including coxsackievirus, other enteroviruses, Ebstein-Barr virus in infectious mononucleosis)

generation cephalosporin Alternative: Cefuroxime or associated sinusitis, fluoroquinolone treat as staph/strep Consider vancomycin, cellulitis TMP/SMX, or clindamycin if concern for MRSA

Pharyngitis

10 days, regardless of promptness of recovery PCN V or amoxicillin (to prevent acute Benzathine PCN G ? 1 for rheumatic fever in GAS GAS infection). TMP/SMX not Alternative: Clindamycin, effective macrolide or cephalexin Supportive treatment only for viral pharyngitis

Pneumonia (nonneonatal) Erythromycin Alternative: Azithromycin Add cefotaxime if febrile, ill-appearing Consider adding clindamycin or vancomycin C. trachomatis, S. in severe infections pneumoniae, B. Outpatient: Amoxicillin pertussis, S. (high-dose), ? azithromycin aureus, viruses Alternative: Clindamycin S. pneumoniae, Inpatient: (ceftriaxone or Mycoplasma, C. cefotaxime) + azithromycin pneumoniae, GAS, Alternative: (clindamycin or S. aureus, viruses, vancomycin) + influenza azithromycin Vancomycin or clindamycin if severe Illness or features suggestive of S. aureus (pleural effusion, cavitation) 10 days For pertussis, azithromycin if <1 mo of age. Treatment during catarrhal stage may provide clinical benefit; during paroxysmal stage, treatment is to limit spread of disease. Chemoprophylaxis for close contacts 710 days See influenza section for treatment guidelines Atypical organisms are more likely with older age of the child

4 wk3 mo Infant/child (3 mo 5 yr):

>5 yr

Outpatient: Azithromycin ? amoxicillin Alternative: Azithromycin ? S. pneumoniae, clindamycin Mycoplasma, C. Inpatient: (cetriaxone or 710 days pneumoniae, GAS, cefotaxime) + azithromycin See influenza section for S. aureus, M. Vancomycin or clindamycin treatment guidelines tuberculosis, if severe Illness or features viruses, influenza suggestive of S. aureus (pleural effusion, cavitation) Staphylococcus, Streptococcus spp.; enteric or genitourinary gramnegative organisms Consider suction irrigation to wound initially. Deep tissue culture of wound may direct treatment. Removal of instrumentation may be necessary

Postspinal fusion

Vancomycin + piperacillin/tazobactam Consider adding gentamicin ? rifampin

ID

ID

Infectious Syndrome

Usual Etiology

Suggested Empirical Therapy

Suggested Length of Therapy/Comments 34 wk IV Aspiration of affected joint recommended. Therapy should be guided by gram stain and culture, if available May switch to PO after response

Septic arthritis (non-neonatal) Cefotaxime + (nafcillin, oxacillin, or clindamycin) S. aureus, GAS, S. Add clindamycin or pneumoniae, vancomycin if MRSA Kingella, prevalent Haemophilus spp. Alternative: Vancomycin + cefotaxime Nafcillin, oxacillin, or S. aureus, GAS, clindamycin Streptococcus spp. Alternative: Vancomycin Add N. gonorrhoeae Add ceftriaxone See sexually transmitted infections

<5 yr

>5 yr Adolescent

Sexually transmitted infections Chlamydia infections C. trachomatis Uncomplicated urethritis, endocervicitis, or proctitis Epididymitis Infection in pregnancy (may require second course due to low efficacy) Azithromycin 1 g PO or doxycycline 100 mg PO BID (>8 yr) Alternative: Erythromycin PO QID (preferred for <6 mo) or ofloxacin or levofloxacin PO

Single dose 7 days 7 days

Doxycycline 10 mg PO BID 10 days Azithromycin 1 g PO or Amoxicillin PO TID Alternative: Erythromycin base PO QID Single dose 7 days 7 days Fluoroquinolones are no longer recommended for presumptive treatment of gonorrhea. *All gonorrheal infections should be treated presumptively for Chlamydia as detailed.

Gonorrheal infections

N. gonorrhoeae

Prepubertal children <100 lb (45 kg) Uncomplicated urethritis, vulvovaginitis, proctitis, or pharyngitis Disseminated gonococcal infections (e.g. arthritis-dermatitis syndrome) Meningitis or endocarditis Ceftriaxone 125 mg IM Alternative: Cefixime 8 mg/kg (max 400 mg) PO or spectinomycin 40 mg/kg (max 2 g) IM AND* azithromycin 20 mg/kg (max 1 g) PO or erythromycin PO QID Ceftriaxone 50 mg/kg/day (max 1 gm) IV/IM Q24H AND* erythromycin PO QID Single dose Single dose Single dose Single dose 14 days

7 days 14 days

Ceftriaxone 50 mg/kg/day Meningitis: 1014 days (max 2 g/day) IV/IM Q12H Endocarditis: 28 days

Infectious Syndrome

Usual Etiology

Suggested Empirical Therapy AND* erythromycin PO QID

Suggested Length of Therapy/Comments

Conjunctivitis Patients >100 lb (45 kg) and >8 years Uncomplicated urethritis, endocervicitis, proctitis, or pharyngitis Disseminated gonococcal infections (e.g. arthritis-dermatitis syndrome) Meningitis or endocarditis

Ceftriaxone 50 mg/kg (max Single dose 125 mg) IM

Ceftriaxone 125 mg IM Alternative: (Cefixime or cefpodoxime 400 mg PO Single dose or spectinomycin 2 g IM) Single dose AND* (azithromycin 1 g PO 7 days or doxycycline 100 mg PO BID) Ceftriaxone 1 gm IV/IM Single dose q24hr AND* (azithromycin Single dose 1 g PO or doxycycline 7 days 100 mg PO BID) Ceftriaxone 12 g IV/IM q12hr AND* (azithromycin 1 g PO or doxycycline 100 mg PO BID) Meningitis: 1014 days Single dose 7 days Endocarditis: 28 days

Conjunctivitis

Ceftriaxone 1 gm IM AND* Single dose (azithromycin 1 g PO or Single dose doxycycline 100 mg PO 7 days BID) Ceftriaxone 250 mg IM Single dose AND* doxycycline 100 mg 10 days PO BID Trichomonas vaginalis Metronidazole 2 g PO Single dose (adult dosing) Switch to oral therapy 24 hours after clinical improvement, to complete 14 days of treatment with clindamycin QID or doxycycline BID Fluoroquinolones may be considered only if parenteral cephalosporin therapy is not feasible AND community prevalence and individual risk of gonorrhea are low

Epididymitis Trichomoniasis

Parenteral regimens: Regimen A C. trachomatis, N. (Cefotetan 2 g IV q12hr or gonorrhoeae, lower cefoxitin 2 g IV q6hr) AND genital tract flora: doxycycline 100 mg IV/PO H. influenzae, q12hr Pelvic inflammatory gram-negative Regimen B disease (PID) rods, anaerobes, Clindamycin 900 mg IV Streptococcus q8hr AND gentamicin agalactiae, 2 mg/kg loading dose, then Mycoplasma spp. 1.5 mg/kg IV q8hr maintenance, or single daily dosing Oral Regimen: (Ceftriaxone 250 mg IM or other 3rd-generation cephalosporin) or (cefoxitin 2 g IM + probenecid 1 g PO) AND doxycycline 100 mg PO BID ? metronidazole 500 mg PO BID

Single dose Single dose each 14 days 14 days

ID

ID

ID

Infectious Syndrome Syphilis (not congenital, age >1 mo)

Usual Etiology Treponema pallidum

Suggested Empirical Therapy

Suggested Length of Therapy/Comments NOTE: Must exclude asymptomatic neurosyphilis in children

Primary, secondary, or early latent syphilis (<1 yr duration)

Benzathine PCN G 50,000 units/kg (max 2.4 ? 106 units) IM Single dose Alternatives if >8 yr: 14 days Tetracycline 500 mg PO 14 days QID or Doxycycline 100 mg PO BID Benzathine PCN G 50,000 units/kg (max 2.4 ? 106 units) IM Q1week 3 weeks Alternatives if >8 yr: 28 days Tetracycline 500 mg PO 28 days QID or Doxycycline 100 mg PO BID Aqueous crystalline PCN G 50,000 units/kg/dose (max 4 ? 106 units/dose) IV q4 6 hr (200,000300,000 units/kg/day, max 24 ? 106 units/day) Alternatives: Procaine PCN G 2.4 ? 106 units IM daily AND probenecid 500 mg PO q6hr May be followed by benzathine PCN G 50,000 units/kg/dose (maximum 2.4 ? 106 units) IM q week

Late syphilis (>1 yr duration), tertiary syphilis

Neurosyphilis

1014 days If PCN-allergic, PCN desensitization required 1014 days 1014 days 3 weeks

Sinusitis 1014 days Add clavulanic acid if severe, risk factors for resistance (day care, Amoxicillin (high dose) recent antibiotics), or 8090 mg/kg/day divided S. pneumoniae, H. fails to respond in 48 BID influenzae, M. 72 hr. If severe and fails Alternative: Cefpodoxime, catarrhalis to respond, consider cefuroxime, cefdinir, imaging and/or azithromycin, or rifampin drainage. Parenteral therapy generally reserved for complicated sinusitis Amoxicillin/clavulanic acid, cefpodoxime, cefuroxime, or cefdinir Alternatives: (ceftriaxone 21 days, or 7 days after or cefotaxime) AND resolution of symptoms (clindamycin or vancomycin) or fluoroquinolone Cefepime or Consider surgical

Acute

Chronic

Add S. aureus, anaerobes (to acute pathogens)

Seriously ill child or Add

Infectious Syndrome immunocompromised UTI

Usual Etiology Pseudomonas, gram negatives, Mucor, Rhizopus, Aspergillus

Suggested Empirical Therapy piperacillin/tazobactam ? amphotericin B

Suggested Length of Therapy/Comments intervention

Cystitis

E. coli, Enterobacteriaceae , Proteus spp., S. saprophyticus, Enterococcus spp. E. coli, Enterobacteriaceae , Proteus spp., Enterococcus spp. Add Pseudomonas, resistant gram negatives

PO: TMP/SMX, cefixime IV: (cefotaxime or ceftriaxone) OR (ampicillin and gentamicin) Alternatives: Nitrofurantoin OR ciprofloxacin

714 days Consider phenazopyridine for comfort (urine and other secretions may turn red) For severe PCN allergy or resistant organisms

Pyelonephritis

714 days Ceftriaxone OR (ampicillin Parenteral until afebrile + gentamicin) for 24 hr. Consider Alternative: Cefixime or outpatient treatment in ciprofloxacin selected patients Piperacillin/tazobactam or 1421 days ceftazidime Vancomycin + (cefotaxime or ceftriaxone) Add aminoglycoside if culture suggests Enterobacteriaceae Consider adding rifampin 1021 days, depending on organism and response Shunt removal or revision is often required for successful treatment

Abnormal host/urinary tract

S. epidermidis, S. aureus, Ventriculoperitone Enterobacteriaceae al shunt, infected , Propionibacterium acnes

Recommendations adapted from American Academy of Pediatrics. Pickering LK, Baker CJ, Kimberlin DW, Long SS (eds): Red book: 2009 Report of the Committee on Infectious Diseases, 28th ed. Elk Grove Village, IL, American Academy of Pediatrics, 2009; and McMillan JA, Siberry GK, Dick JD, et al: The Harriet Lane handbook of pediatric antimicrobial therapy. Philadelphia, PA, Mosby Elsevier, 2009. CONS, Coagulase-negative staphylococcus; CXR, chest x-ray; FDA, Food and Drug Administration; GAS, group A streptococcus; GBS, group B streptococcus; IM, intramuscular; IV, intravenous; MSSA, methicillin-sensitive Staphylococcus aureus; MRSA, methicillin-resistant Staphylococcus aureus; PCN, penicillin; PO, by mouth; TMP/SMX, trimethoprim/sulfamethoxazole; UTI, urinary tract infection.

CHILDRENS HOSPITAL & MEDICAL CENTER ANTIMICROBIALS SUSCEPTIBILITY SURVEY Adapted from Drugs and Bugs webpage

STAPHYLOCOCCUS AUREUS METHOD: DISK DIFFUSION METHOD (KIRBY-BAUER METHOD)

ID

Antibiotic

NAFCILLIN 53 VANCOMYC IN** CLINDAMYC IN (raw data) CLINDAMYC IN (with inducible) ERYTHROM YCIN PENICILLIN* TRIMETH/S ULFA RIFAMPIN 38 7 99+ 99+ 39 7 99+ 99+ 41 7 99+ 100 41 9 99 99 65 13 100 100 63 14 100 100 72 13 99+ 100 65 16 99 99 8 0 99+ 99+ 10 0 99+ 99+ 10 0 99+ 100 7 0 100 99 100 54 100 50 100 58 100 100 100 100 100 100 100 100 100 0 100 0 100 0 100 0 100

ID
TOTAL 2010 2009 2008 2007 93 89 89 88 97 82 80 78 76 82 ANTIBIOTIC NAFCILLIN VANCOMYCIN** CLINDAMYCIN ERYTHROMYCIN PENICILLIN RIFAMPIN 2010 34 100 60 24 12 99 2009 29 100 67 22 6 97

% SUSCEPTIBLE

NON MRSA

MRSA

2010

2009

2008

2007

2010

2009

2008

2007

96

98

95

88

81

79

78

89

84

79

82

77

73

71

* 1ST CHOICE FOR NON-PENICILLINASE PRODUCING S. AUREUS **1ST CHOICE FOR METHICILLIN-RESISTANT S. AUREUS MRSA

STAPHYLOCOCCUS SPECIES (COAGULASE NEGATIVE) METHOD: DISK DIFFUSION METHOD (KIRBY-BAUER METHOD) %SUSCEPTIBLE 2008 30 100 59 24 6 99 2007 32 100 63 27 10 98 2006 29 100 56 24 10 99 2005 25 100 54 24 6 98 2004 24 100 59 20 7 96

ID

**1ST CHOICE FOR METHICILLIN-RESISTANT STAPH SP (COAGULASE NEGATIVE) STREPTOCOCCUS PNEUMONIAE METHOD: disc diffusion or E test on MH Agar supplemented with 5% sheep blood

ANTIBIOTIC 2010 ERYTHROMYCIN* VANCOMYCIN* CLINDAMYCIN* LEVOFLOXACIN* TRIMETH/SULFA* 45 99 70 97 48 2009 37 100 59 96 44

%SUSCEPTIBLE 2008 33 100 51 99 37 2007 45 100 69 99 50 2006 25 100 55 99 44 2005 36 100 64 100 44 2004 39 100 72 100 37

* MODIFIED KIRBY BAUER TEST ENTEROCOCCUS METHOD: Microdilution MIC (MICROSCAN) % SUSCEPTIBLE ANTIBIOTIC AMPICILLIN* VANCOMYCIN* NITROFURANTOIN** GENTAMICIN HLAR* (High Level Aminoglycoside Resistance) Synergy Possible 86 85 85 89 90 95 89 93 2010 95 99 96 2009 93 96 95 2008 97 98 93 2007 91 91 91 2006 97 99 97 2005 99 99 100 2004 93 98+ 100 2003 93 99 98

+ Two VRE Isolates tested in 2004 (same patient). * FOR SEVERE INFECTIONS, INCLUDE GENTAMICIN ** FOR UNCOMPLICATED URINARY TRACT INFECTIONS ESCHERICHIA COLI

METHOD: DISK DIFFUSION METHOD (KIRBY-BAUER METHOD) % SUSCEPTIBLE ANTIBIOTIC CEFOTAXIME GENTAMICIN TOBRAMYCIN 2010 97 92 88+ 2009 99 94 94 2008 99 96 97 2007 98 97 93 2006 100 98 98 2005 98 98 95 2004 99 97 98

ID

CEFAZOLIN CEFOXITIN CIPROFLOXACIN AMPICILLIN TRIMETH/SULFA NITROFURANTOIN* MEROPENEM AMPICILLIN / SULBACTAM CEFEPIME PIPERACILLIN/ TAZOBACTAM

83 97 95 47 73 97 100

84 96 96 44 71 97 100

84 -96 41 73 97 100

86 97 97 41 75 97 100

89 99 -49 79 99 100

87 98 -48 75 98 100

89 99 -54 77 98 100

ID

71 98+

71 99

71 100

77 99

83 99

79 100

82 100

97+

94

97

97

92

95

90

*FOR URINE ISOLATES ONLY Cefotaxime susceptibility predicts susceptibility to Ceftriaxone. Ampicillin/Sulbactam (Unasyn) susceptibility predicts Amoxicillin/Clavulanic Acid (Augmentin) susceptibility. + 59 Isolates tested, non-urinary tract. KLEBSIELLA SPECIES (INCLUDES KLEBSIELLA PNEUMONIAE AND KLEBSIELLA OXYTOCA) METHOD: DISK DIFFUSION METHOD (KIRBY-BAUER METHOD) % Susceptible Antibiotic CEFAZOLIN CEFOXITIN CEFOTAXIME GENTAMICIN TOBRAMYCIN TRIMETH/SULFA NITROFURANTOIN* MEROPENEM CEFEPIME PIPERACILLIN TAZOBACTAM 2010 79 98 84 89 83 78 38 100 97 92 2009 87 93 97 99 98 79 31 99 98 83 Kp 2008 92 -100 96 90 77 48 100 100 88 2007 88 86 98 97 98 86 42 98 100 94 2010 24 92 98 96 97 96 83 100 100 77 2009 53 94 96 98 97 88 94 100 94 94 Ko 2008 38 -92 98 90 90 100 100 100 82 2007 91 85 91 94 89 87 85 100 100 94

*FOR URINE ISOLATES ONLY

PSEUDOMONAS AERUGINOSA METHOD: DISK DIFFUSION METHOD (KIRBY-BAUER METHOD)

% SUSCEPTIBLE ANTIBIOTIC 2010 Non-CF TOBRAMYCIN GENTAMICIN AMIKACIN CEFTAZIDIME MEROPENEM PIPERACILLIN/ TAZOBACTAM CEFEPIME CIPROFLOXACIN 93 90 91 93 90 89 94 91 91 95 91 89 91 92 93 92 92 90 91 92 91 92 92 88 85 70 85 91 91 2010 ALL 82 67 81 91 91 2009 NonCF 88 84 95 91 94 2009 ALL 87 80 90 93 95 2008 Non-CF 97 87 94 90 93 2008 ALL 92 82 89 91 94 2007 Non-CF 96 91 96 90 94 2007 ALL 92 87 91 91 94

Helpful links: www.idsociety.org www.aidsinfo.nih.gov/

METABOLISM Hyperammonemia Can be seen in urea cycle defects, organic acidemias, fatty acid oxidation defects, and liver dysfunction, rarely mitochondrial disorders Work-up: plasma amino acids, urine organic acids and liver function tests METABOLISM Metabolic Acidosis

Hypoglycemia

METABOLISM
Algorithms from: Rizzo WB, Kaplowitz PB. Metabolic and Endocrine Disorders. In Pellock JM and Myer EC, eds. Neurologic Emergencies in Infancy and Childhood, 2nd ed. Butterworth-Heinemann Publ, Boston, pp 310-343, 1993.

NEONATOLOGY (NICU Reference Cards) Neonatal Resuscitation

NEONATOLOGY

Newborn Assessment Apgars: Assessed at 1 and 5 minutes. May be repeated at 5-minute intervals for infants with 5-minute scores <7 Score 0 1 2 Heart rate Respiratory effort Muscle tone Reflex irritability (nose suction) Color Absent <100 bpm >100 bpm Good Active motion Cough or sneeze Completely pink

Absent, irregular Slow, crying Limp No response Blue, pale Some flexion of extremities Grimace Acrocyanosis

Data from Apgar V: Proposal for a new method of evaluation of the newborn infant. Anesth Analg 1953;32:260. Newborn Nursery Void by 12 hours of age and pass stool by 48 hours Hypoglycemia (>40mg/dl): at risk infants: infants of diabetic mothers, low-birthweight infants (<2,500g), SGA , LGA , hypothermia, and sepsis. Hearing screen Newborn screen: congenital adrenal hyperplasia, congential primary hypothyroidism, CF, biotinidase deficiency, hemoglobinopathies, fatty acid conditions, amino acid, organic acid conditions and vitamin metabolismconditions Exam Age Genitalia Fontanel Pulses, murmur eval Palate, frenulum hips (ortolani/Barlow) Clavicle Activity/tone Umbilical cord extremities (Erbs/Klumpkes, digits) Care of the well newborn, Pediatrics in review Vol. 27 No. 3 March, 2006, P 89-98

NEONATOLOGY

Ballard Gestational Age Estimation Most accurate at 12-20 hours of life. Gestational age is based on the sum of the neuromuscular and physical maturity

NEONATOLOGY

Modified from Ballard JL et al: New Ballard Score, expanded to include extremely premature
infants. J Pediatr 1991;119:417423

Neonatal Hyperbilirubinemia Algorithm for management of jaundice in the newborn nursery: for infants >35 weeks Before discharge all newborns should be assessed for the risk of developing jaundice F/U after discharge should established to assess the patients well-being and jaundice Breastfeeding: dehydration and poor caloric intake associated with inadequate BF may contribute to hyperbilirubinemia recommend increasing frequency of nursing

NEONATOLOGY

Infant discharged Should be seen by >24 hours 72 hours 24-47.9 hours 96 hours 48-72 hours 120 hours Risk stratification for newborns 2840 g and 36 or greater weeks or 2000 g and 35 or greater weeks gestation

NEONATOLOGY Risk Factors for Severe Hyperbilirubinemia in Infants of 35 or More Weeks Gestation Major risk factors Predischarge TSB or TcB level in the high-risk zone (Fig 2)25,31 Jaundice observed in the first 24 h30 Blood group incompatibility with positive direct antiglobulin test, other known hemolytic disease (eg, G6PD deficiency), elevated ETCO c Gestational age 3536 wk39,40 Previous sibling received phototherapy40,41 Cephalohematoma or significant bruising 39 Exclusive breastfeeding, particularly if nursing is not going well and weight loss is excessive39,40 East Asian race39* Minor risk factors Predischarge TSB or TcB level in the high intermediate-risk zone 25,31

Gestational age 3738 wk39,40 Jaundice observed before discharge40 Previous sibling with jaundice40,41 Macrosomic infant of a diabetic mother42,43 Maternal age 25 y39 Male gender39,40 Decreased risk (these factors are associated with decreased risk of significant jaundice, listed in order of decreasing importance) TSB or TcB level in the low-risk zone (Fig 2)25,31 Gestational age 41 wk39 Exclusive bottle feeding39,40 Black race38* Guidelines for phototherapy in hospitalized infants of 35 or more weeks gestation.

NEONATOLOGY

Guidelines for exchange transfusion \

NEONATOLOGY

Differential diagnosis of neonatal hyperbilirubinemia

NEONATOLOGY

Nelson Textbook of Pediatrics, 19th ed., Fig 96-7

ALTE: Apparent life threatening event Events that are witnessed by and are frightening to the observer. The episode includes some combination of obstructive or central apnea, color change, change in muscle tone , choking, or gagging. Work up: History and physical to guide the work up and treatment Depending on the clinical picture: CXR, cardiorespiratory monitoring, sleep study/pneumogram, EEG, UGI/barium swallow If no diagnosis is suggested by the initial presentation, may require broader testing. Including: screening for GER, UA/culture, brain neuroimaging, pneumogram DDx Gastroenterologic 33% Neurologic 15% Respiratory 11% GERD Seizure RSV Sandifers Central apnea Pertussis Gastroenteritis Meningitis/encephalitis Aspiration Surgical abdomen Hydrocephalus Respiratory tract infection Dysphagia Brain tumor Foreign body Esophageal dysfunction Otolaryngologic 4% Cardiovascular 1% Other Laryngomalacia Congenital heart disease Idiopathic apnea of infancy Subglotic stenosis Cardiomyopathy 23% Laryngeal stenosis Cardiac arrhythmias Anemia Obstructive sleep apnea Myocarditis Child maltreatment Breath-holding Choking Drug/toxin reaction Munchhausen by proxy Harriet Lane, 19th ed., p. 591-592

NEONATOLOGY

NEONATOLOGY

GA at birth:

Estimated weights for GA:

23 weeks 27 weeks 31 weeks 33 weeks 35 weeks 37 weeks 40 weeks

500 grams 1000 grams 1500 grams 2000 grams 2500 grams 3000 grams 3500 grams

ETT Insertion Estimate Chart: 6+ kg= length of insertion in cm Size Insertion length Tube size 500-1000 grams NEONATOLOGY 1000-1500 grams 1500-2000 grams 2000-2500 grams 2500-3000 grams 3000-3500 grams 3500-4500 grams 6.5 to 7 cm 7 to 7.5 cm 7.5 to 8 cm 8 to 8.5 cm 8.5 to 9 cm 9 to 9.5 cm 9.5 to 10.5 cm 2.5 mm 3.0 mm 3.0 mm 3.5 mm 3.5 mm 3.5 mm 3.5 or 4.0 mm

Various UAC/UVC placement calculations UAC (cm) GA= cm 3 x kg + 9= cm (Length (cm)/ 3) + 1= cm Desired level: T6-T10 or L3-L4 1/3 GA= cm

UVC (cm) 0.5 x UAC depth + 1= cm length of UAC depth + 1= cm Desired level: T8

Artery runs on left side of spine Vein runs on right side of spine Use 3.5 french for <1.5 kg. Use 5 fr for >1.5kg. Emergent palcement of UVC: 2-5 cm or just until blood return Newborn screen: On admit and at 48-72 hours. Nutrition: DOL #1: start IVF at 80mL/kg/day DOL #2: advance to 100 mL/kg/day DOL #3: advance to 120 mL/kg/day DOL #4: advance to 140 mL/kg/day DOL #5: advance to 150 mL/kg/day Trophic feedings are beneficial for infants <1500 grams. By 12 hours of life, consider small volumes of human milk or premature formula at 10-20 mL/kg/day for 3-7 days as a prime for the GI tract. TPN: initiate starter TPN on infant <1800 grams at 80 ml/kg/day IL: initiate IL on DOL #1 at 2 grams/kg/day. Advance to max of 3.5 grams/kg/day over 3-4 days. Keep triglycerides<300 Protein: initial protein 3.5 grams/kg/day. Advance step wise to 4/5 grams/kg/day. In infants <800 grams continue TPN until on full volume fortified enteral feedings. Enteral Calorie Goals: 100-110 cal/kg/day (term) 120-130 cal/kg/day (preterm) 70-80 cal/kg/day (term) 80-90 cal/kg/day (preterm)

Parenteral Calorie Goals:

Vitamin/Mineral supplementation:

Vitamin D: 400 IU po no later than when infant is at 50% enteral volume. Can be initiated with first feeds. Probiotics (LB): initiate when infant is on 2 mL/hr on infants <34 weeks gestational age. Discontinue when CGA is 34 weeks. Ventilator Management: Initial settings CPAP Biphasic mode

4-6 cm H2O (if unsuccessful change to BiPhasic Rate: 10 High Pressure (PIP) 6-8 cm H2O Low Pressure (PEEP) 4-6 cm H2O - start at 6/4 for infants <1000 grams -start at pressures 8/5 for infants >1000 grams -maintain high pressure 2-3cm H2O above low pressure PIP: 20-24 (HFOV if PIP>25) PEEP: 4-5 Rate: 40-60 IT: 0.35 MAP: 12-15 IT: 0.33 Frequency (Hertz): 10 Delta P: adjust to obtain adequate chest vibration (25-30)

NEONATOLOGY

Conventional ventilation

HFOV

HFOV Management To: increase oxygenation: increase FiO2 or MAP decrease oxygenation: decrease FiO2 or MAP To: increase pCO2: decrease amplitude or increase hertz decrease pCO2: increase amplitude or decrease hertz Intraventricular Hemorrhage Grade Mortality

Hydrocephalus

Location

% with neurological complications 10% 50% 50%

I II III

0 0 10%

0 0 20%

Isolated germinal matrix GM hemorrhage into ventricles GM hemorrhage with ventricular dilation

GM hemorrhage 90% with extension into parenchyma All infants <32 weeks: initial on DOL# 3. Repeat on DOL #7-10 and again at 1 month. Retinopathy of Prematurity (ROP) Stage 1 Demarcation line separates avascular from vascularized retina Stage 2 Stage 3 Stage 4 Stage 5 Plus disease Ridge forms along demarcation line Extraretinal fibrovascular proliferation tissue forms on ridge Partial retinal detachment Retinal detachment Tortuosity and enforgement of blood vessels near the optic disk; may be present at any stage

IV

50-60%

65-100%%

Guidelines for the Use of Phototherapy in preterm infant <1 week age

Weight (gram) 500-1000 1000-1500 1500-2500 >2500

Phototherapy 5-7 7-10 10-15 >15

Consider exchange 12-15 15-18 18-20 >20

NEONATOLOGY

NEUROLOGY

PRIMARY CARE & DEVELOPMENT CDC Growth Charts Source:http://www.cdc.gov/growthcharts

______________________________________________________________________

Immunization Schedules- Child, Adolescent, and Catch-Up

*Source: http://www.cdc.gov/vaccines

Contraindications to vaccines- Any child with moderate to severe illness should have vaccines postponed. DTaP-anaphylactic reaction, encephalopathy within 7 days of administration (to subsequent doses of pertussis), progressive neurologic disorder including infantile spasms or uncontrolled epilepsy (pertussis component), some contain latex so not for anaphylactic reaction to latex *Take precaution with next dose if temp >105 within 48 hrs of vaccine, hypotonichyporesponsive episode within 48 hrs, seizure within 3 days, persistent inconsolable crying >3 hrs within 48 hrs of vaccine HIB- None Polio- None Prevnar- Hypersensitivity to vaccine component Rotateq- Combined immunodeficiency, history of intussusception, moderate to severe gastroenteritis Rotarix- Hx of intussusception, allergy to latex Hepatitis A- Hx of severe reaction to prior dose

Hepatitis B- hypersensitivity to yeast MMR- Pregnancy, congenital immunodeficiencies, long-term immunosuppressive therapy, hematologic/solid tumors Varicella- immunocompromised, pregnancy, moderate to severe illness, previous anaphylaxis to neomycin/gelatin, on high dose steroids Meningococcal- None HPV- None InfluenzaInactivated: Chicken or egg allergy Flumist: Age <2 years, History of anaphylactic reaction to egg or chicken protein, gentamicin, gelatin, or arginine , history of Guillain-Barr syndrome, asthma, recurrent wheezing in children younger than five years, chronic pulmonary, cardiovascular (excluding systemic hypertension), renal, hepatic, neurologic, hematologic, or metabolic (including diabetes mellitus) disorders, pregnancy, known or suspected immunodeficiency or immunosuppressive therapy, receipt of other live virus vaccine within the previous four weeks (other live virus vaccines can be administered on the same day), residents of chronic care facilities Adverse Reactions- ALL vaccine can cause fever, redness/tenderness at injection site and anaphylaxis DTaP- entire limb swelling, previously mentioned precautions above HIB- None other than local reactions Polio- None for inactivated vaccine Prevnar- Increased risk of febrile seizure when given with influenza vaccine (still low risk overall), rare thrombocytopenia Rotateq/Rotarix- Diarrhea/vomiting Hepatitis A- Headaches Hepatitis B- None other than local rxn MMR- Fever (6-12 days out from date given), thrombocytopenia/ITP (in 6 weeks after given, should not receive subsequent dose), rash, joint complaints (7-21 days out), febrile seizures (8-14 days out), hypersensitivity reactions (to neomycin/gelatin NOT egg) Varicella- rash, increased risk of seizure with MMRV vs. separate vaccines Meningococcal- Increase risk of Guillain-Barre HPV- syncope, venous thromboembolism, NO increased risk of Guillain-Barre InfluenzaInactivated: ? increased risk for Guillain-Barre, red eyes, respiratory symptoms Flumist: nasal congestion, low grade fever ***Difference between DTaP and Tdap- DTaP is given for children 6 weeks to 6 years of age, Tdap is given as booster for older children and adults. Tdap contains lower amounts of diphtheria toxoid and pertussis antigens than DTaP and cannot be used for primary immunization *Source: UpToDate Standard Immunizations for Children and Adolescents ______________________________________________________________________ Obesity in Children: At increased risk for type 2 diabetes mellitus, hypertension, cardiovascular disease. Screen for family hx of these diseases. Overweight BMI: 85-95% Obese BMI: >95% Important ROS: delayed development (genetic syndrome), short stature (genetic syndrome), headaches (pseudotumor cerebri), snoring, daytime sleepiness (sleep apnea), abd pain, hip pain/knee pain/limp, oligomenorrhea or amenorrhea (PCOS, Prader-Willi), urinary frequency, nocturia, polydipsia/polyuria, binge eating/purging, insomnia or anhedonia (depression) Screening: Fasting lipid profile (overweight children included regardless of family history or risk factors). Children with a positive family hx of dyslipidemia/premature CVD should be screened no earlier than 2 yrs or later than 10 yrs old. If the levels are acceptable repeat testing at HCM visit in 3-5 yrs. Also check fasting blood sugar (plus/minus HgbA1C), and ALT (for fatty liver disease). Monitor blood pressure. Healthful diet: low fat dairy products, even for children age 12 months-2 years who are overweight/obese or who have a family history of obesity, dyslipidemia, or CVD For high triglyceride/low HDL: weight management is key with diet and exercise For patients 8 yrs old with LDL 190, OR 160 with fam hx early CVD or 2 other risk factors (obesity, HTN, cigarette smoking), OR 130 if diabetes present: Pharmacological management recommended to goal of ideally 110. Options include bile acid binding resins (poor compliance with many GI complaints), niacin (not routinely recommended in children), statins (few small studies

favorable, but are teratogenic), cholesterol-absorption inhibitors (many GI complaints), fibrates (have not been studies well in children) *Source: Lipid Screening and Cardiovascular Health in Childhood Pediatrics, UpToDate Clinical evaluation of the obese child and adolescent ______________________________________________________________________ DDx Vaginal Discharge: bacterial vaginosis, vulvovaginal candidiasis, trichomoniasis, gonorrhea, Chlamydia, foreign body with secondary infection, physiological leukorrhea Testing: Nucleic acid amplification (NAAT) on urine for GC/Chlamydia, clue cells on wet mount for BV, motile trichomonads on wet mount Common Treatments: Gonorrhea: Ceftriaxone IM once Chlamydia: Azithromycin or Doxycycline (increased resistance) Bacterial vaginosis or trichomoniasis: Metronzidazole Pelvic Inflammatory Disease: acute infection of upper reproductive tract, can include uterus, fallopian tubes, ovaries or neighboring pelvic organs resulting in peritonitis, tubo-ovarian abscess, or perihepatitis (Fitz-Hugh Curtis Syndrome) Causes: Neisseria gonorrhea, Chlamydia trachomatis, vaginal flora (anaerobes and gram negatives) Presentation: lower abd pain which worsens with sex, abnormal uterine bleeding, new vaginal discharge, fever, chills Exam: diffuse tenderness more so in lower quadrants, rebound tenderness, decreased bowel sounds, purulent endocervical discharge, cervical motion tenderness, adnexal tenderness CDC guidelines for empiric treatment: cervical motion tenderness, uterine/adenxal tenderness in presence of lower abd or pelvic pain, oral temp >101, abnormal cervical/vaginal mucopurulent discharge, presence of WBCs on microscopy of vaginal secretions, elevated ESR/CRP Inpatient treatment: Cefoxitin or Cefotetan + Doxycycline OR Clindamycin + Gentamicin (NO use of fluoroquinolones recommended due to resistance!!) Outpatient treatment: Ceftriaxone + Doxycycline, can also add Metronidazole is feel at risk Duration of treatment: Typically 14 days per CDC recommendations *Source: UpToDate Pelvic Inflammatory Disease, Vaginitis ______________________________________________________________________ Psychiatry Psychosis- disturbance in the perception of reality, evidenced by hallucinations (false sensory perceptions), delusions (false beliefs that are firmly held despite evidence to the contrary), or thought disorganization (loose associations, nonsensical speech, or bizarre behavior) Conversion Disorder- Symptoms or deficits of voluntary motor or sensory function that suggest a neurological or general medical condition and are associated with psychological factors. Ex.) paralysis, aphonia, blindness, deafness, pseudoseizures Depression- At least 5 symptoms of following: depressed or irritable mood, markedly diminished interest or pleasure in almost all activities, change in appetite/weight, insomnia/hypersomnia, psychomotor agitation/retardation, fatigue or loss of energy, feelings of worthlessness or guilt, impaired concentration, recurring thoughts of death or suicide present for at least two weeks Bipolar Disorder- Any episode of mania or hypomania in addition to a depressive episode, can have rapid cycling, places adolescents at particular risk for suicide Attention Deficit Hyperactivity Disorder- Hyperactivity, impulsivity, and inattention. Must be present in more than 1 setting, > 6 months, present before age 7 yrs, must impair function in academic, social, or occupational activities, must be excessive for the developmental level of the child, other mental disorders cannot account for the symptoms Oppositional Defiant Disorder- Is a pattern of disobedient, hostile, and defiant behavior toward authority figures. Examples: actively does not follow adults' requests, angry and resentful of others, argues with adults, blames others for own mistakes, has few or no friends or has lost friends, is in constant trouble in school, loses temper, spiteful or seeks revenge, touchy or easily annoyed. Symptoms must be present for at least 6 months. Conduct Disorder- is a disorder of childhood and adolescence that involves long-term (chronic) behavior problems, such as: defiant or impulsive behavior (cruelty to animals, forcing sexual activity, fighting, truancy), drug use, criminal activity (vandalism, arson). *Source: pToDate Overview of psychosis, Somatization, Depression in adolescents ADHD in children and adolescents, http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0002504/ Child Abuse Reporting in Nebraska

**State law requires any person who has a reasonable suspicion that a child has been physically or sexually abused or neglected report it promptly to the Nebraska Department of Health and Human Services. People who have duty to report: Physicians, social workers, teacher, mental health professionals, child care providers, medical examiners/coroners, law enforcement officers. All reports are CONFIDENTIAL. When to report: When you suspect or have reason to believe a child has been abused or neglected. Phone Number: 1800-652-1999 Nebraska, 1800-362-2178 Iowa Omaha Police Dept. Youth Services Division (402) 444-5636 Who Can Take Custody: Law enforcement or the court **In Iowa a physician can take emergency custody of a child How Admit Usually Works When Pertains to Abuse: Perform admission as you normally would (history from parent or medical documents available). Consult Dr. Haney with the Childrens Advocacy Team. She will review the case and interview the parents if available. She will suggest any additional labs/studies and make her best clinical judgement as to whether the history and injuries are consistent or whether the injuries are likely the result of abuse. She works closely with social work, child protective services and law enforcement. Law enforcement and CPS then picks up their end and decides whether/what charges can be filed and take protective custody of the child. Dr. Haney is then occasionally asked to testify in court. Suspected Abuse Workup SCM Order Sets: *All order sets have consult CAT (Child Advocacy Team) included. CAT Suspicious Broken Bone(s) CAT Suspicious Bruising CAT-Suspected Traumatic Brain Injury Failure to Thrive Project Harmony Mission: Project Harmony exists to provide effective, immediate and sensitive support to children who are victims or suspected victims of abuse and neglect, and their non-offending family members. A child-centered facility, Project Harmony assembles a multidisciplinary team composed of law enforcement, investigative, social service, medical and referral professionals dedicated to protecting children and prosecuting offenders. The purpose of the center is to enhance the communication among professionals by co-housing in one facility: Project Harmony, the Omaha Police Department-Child Victim/ Sexual Assault Unit, and Nebraska Health and Human ServicesChild Protection Services. Medical examinations: Dr. Haney and her staff will provide a medical examinations as needed after forensic interviews. They will also provide examinations when an interview is not warranted or if the case is not accepted for investigation. Forensic Interviews: When requested by law enforcement or Child Protective Services, a child interview specialist will conduct a video-taped forensic interview with the child. One of the goals of this interview is to reduce the number of time the child must tell the story of his/her abuse. Reducing the number of interviews reduces the trauma of the child. Medical Director: Dr. Suzanne Haney Address: 11949 Q Street, Omaha, NE 68137 Phone: (402) 595-1326 *Source: Child Abuse Reporting Requirements, State of Nebraska, Nebraska DHHS Website, Project Harmony website, SCM order sets

PULMONOLOGY (UpToDate, Harriet Lane, NICU Red Cards) -Normal Respiratory Rates in Children: Age (yrs) Resp Rate (breaths/min) 0-1 24-38 1-3 22-30 4-6 20-24 7-9 18-24 10-14 16-22 14-18 14-20 -Inspection: evaluate for chest wall abnormalities (barrel chest, pectus excavatum/carinatum), symmetry, accessory muscle use, cyanosis of lips/skin/nails, clubbing -Auscultation: Crackles/rales: heard in inspiration; bronchiolitis, pulmonary edema, pneumonia Wheezes: asthma, bronchiolitis, foreign body Rhonchi: continuous, low-pitched; pneumonia, cystic fibrosis Stridor: high-pitched, harsh, blowing sound usually during inspiration; croup, laryngomalacia, subglottic stenosis, allergic reaction, vocal cord dysfunction -Pulse Oximetry (SpO2): indirect measurement of arterial O2 saturation estimated by light absorption characteristics of oxygenated and deoxygenated hemoglobin through the skin in peripheral blood. Limitation: measures O2 sat and not O2 delivery to tissues; a marginally low saturation may be more significant in an anemic patient due to their reduced O2-carrying capacity -Blood Gases: 1. Arterial Blood Gas (ABG): most accurate way to assess oxygenation (PaO2), ventilation (PaCO2), and acid base status (pH and HCO3) 2. Venous Blood Gas (VBG): PvCO2 averages 6-8mm Hg higher than PaCO2, and venous pH is slightly lower than arterial pH. Measurement strongly affected by local circulatory and metabolic environment 3. Capillary Blood Gas (CBG): correlation with ABG best for pH, moderate for PCO2, and worst for PO2 -Analysis of Acid-Base Disturbances pH CO2 HCO3 Metabolic nl Acidosis Respiratory nl Acidosis Metabolic nl Alkalosis Respiratory Alkalosis nl BE nl Compensation Treatment pCO2 Give NaHCO3 HCO3 Improve Ventilation pCO2 Stop diuretics; acetate in TPN HCO3 Wean Ventilator

nl

-Pulmonary Function Tests (PFTs): provide objective and reproducible measurements to airway function and lung volumes; used to characterize disease, assess severity, and follow response to therapy 1. PEFR (Peak Expiratory Flow Rate): used to follow the course of asthma; predicted average peak based on height 2. Spirometry: useful to characterize patterns of airway obstruction (for children >6 yrs) **See Figure 24-3, 24-5, Harriet Lane** -Obstructive lung disease: asthma, cystic fibrosis -Restrictive lung disease: interstitial fibrosis, scoliosis, neuromuscular disease -Hypoxia: Differential Diagnosis includes:

1. Hypoventilation (normal Aa gradient): CNS depression (drug overdose), muscular weakness (myasthenia gravis, diaphragmatic paralysis, muscular dystrophy), poor chest wall elasticity (flail chest, kyphoscoliosis) 2. VQ Mismatch (increased Aa gradient; O2 helps): obstructive lung disease (asthma, COPD, pneumonia), pulmonary vascular disease, interstitial lung disease 3. Right-to-left shunting (increased Aa gradient; O2 doesnt help): intracardiac shunts, anatomic bypassing of alveolae (pulmonary AVM, hepatopulmonary syndrome), physiologic shunts (ARDS, atelectasis, pneumonia, PE) -ALTE (Apparent Life Threatening Event): Events that are frightening to the observer, and include some combination of obstructive or central apnea, color change (usually cyanosis and/or pallor), a marked change in muscle tone (usually extreme limpness), choking, or gagging. In some cases, the observer fears the infant has died or would have died without significant intervention 1. DDx: GI (33%), Idiopathic Apnea of Infancy (23%), Neuro (15%), Otolaryngolic (4%), Cardiovascular (1%), Metabolic/endocrine, infectious, other (** see box 24-1, Harriet Lane**) 2. Work-up: guided by clinical story and physical exam. Consider CBC (infection or anemia), serum bicarb (acid/base status), blood glucose (hypoglycemia), EKG (QT interval) -Bronchiolitis: lower respiratory tract infection common in infants; characterized by acute inflammation, edema, and necrosis of airway epithelium leading to increased mucus production and bronchospasm 1. Clinical Manifestations: variable and dynamic course ranging from transient apnea and mucus plugging to progressive lower airway disease. CXR: hyperinflation and patchy atelectasis. MC cause: RSV (also: parainfluenza, adenovirus, mycoplasma, human metapneumovirus). 2. RSV: worst symptoms 3-5 days after onset 3. Treatment: Supportive. Suctioning. Can do trial of bronchodilators and continue only if respond (<30%). Hold feeds for tachypnea. 4. Clinical Pearls: 1- In infants younger than 3 months with RSV, there is an appreciable incidence of co-nfection with UTI and/or acute otitis media. 2- Infants hospitalized for bronchiolitis are more likely to have recurrent wheezing. 3- Ensure RSV immunoprophylaxis for high-risk infants (Synagis) -Cystic Fibrosis: autosomal recessive disorder; mutation in cystic fibrosis transmembrane conductance regulator (CFTR) gene changes the function of the chloride channel that usually resides within mucosal epithelial cells in the arways, pancreatic ducts, biliary tree, intestive, vas deference, and sweat glands. Most patients have chronic progressive obstructive pulmonary disease, pancreatic exocrine insufficiency withprotein and fat malabsorption, and abnormally high sweat electrolyte concentrations 1. Clinical Manifestations: **See Table 24-6, Harriet Lane** 2. Diagnosis: Gold standard is sweat chloride test Also part of most Newborn Screens (confirmatory test is sweat chloride) 3. MC mutation: Delta F508 (70%) 4. Pulmonary Therapies: Airway clearance therapy to mobilize airway secretions and facilitate expectoration (percussion, vest therapy); Aerosolized medication to increase mucociliary clearance (dornase alfa, hypertonic saline nebs); Chronic antibiotics (pseudomonas common; consider tobramycin and/or chronic macrolide therapy) 5. Common Complications: pancreatic disease (pancreatic enzyme replacement therapy prior to meals to improve digestion and intestinal absorption of dietary protein and fat; nutritional supplementation to keep BMI >50%); intestinal (meconium ileus, distal intestinal obstruction syndrome, rectal prolapse); infertility (males: most all lack vas deferens; women may have trouble getting pregnant due to mucus-associated obstruction of cervix); decreased life expectancy (survival improving; median predicted survival age is >37 yrs) -Apnea: complete lack of oronasal airflow lasting 2 respiratory cycles 1. Central: lack of airflow with lack of effort (infection, apnea of prematurity, meds, arrhythmias, seizure, GERD, central hypoventilation syndromes, primary neuro [Chiari malformation, brainstem lesions], metabolic syndromes [Leighs] 2. Obstructive: lack of airflow despite continued abdominal/thoracic effort (adenotonsillar hypertrophy, atopic disease, macroglossia, Pierre-Robin [microglossia, glossoptosis, cleft soft palate], craniofacial hypoplastic syndromes, post-pharyngeal muscle incoordination, laryngospasm, obstructive airway anomalies [tracheomalacia, web, vascular ring], secretions/plugging, obesity/weight of chest wall, neuromuscular weakness 3. Mixed: central and obstructive components (obesity hypoventilation syndrome; chronic obstruction can blunt central drive leading to a mixed picture)

-Stridor: High-pitched, musical sound made when breathing caused by oscillation of a narrowed airway 1. Diagnosis: largely made by history and physical. Extrathoracic: more pronounced during inspiration. Consider imaging, scope 2. Management: Dependent upon cause -Epiglottitis: Epiglottitis is inflammation of the epiglottis and adjacent supraglottic structures that without treatment, can progress to life-threatening airway obstruction 1. Diagnosis: visualization of the epiglottis or demonstration of epiglottal swelling on lateral neck radiographs. Cultures of the blood and/or surface of the epiglottis identify the microbial pathogen 2. Management: manage airway (consider artificial airway early), empiric antibiotics based on most likely organism (3rd generation cephalosporin (ceftriaxone or cefotaxime) + anti-staph agent effective against MRSA (vanc or clinda) 3. MC organisms: Haemophilus influenza type b (HIB- vaccine preventable), PCN-resistant strep pneumo, beta-hemophytic strep, staph aureus including MRSA -Croup (laryngotracheitis): respiratory illness resulting from inflammation of the larynx and subglottic airway characterized by inspiratory stridor, barky cough, and hoarseness. Typically occurs in children aged 6 mo to 3 years (up to 6 yrs); caused most commonly by parainfluenza virus 1. Diagnosis: clinical 2. Management: dexamethasone (0.6 mg/kg, maximum of 10 mg) by the least invasive route possible: oral if oral intake is tolerated, intravenous if IV access has been established, IM if oral intake is not tolerated and IV access has not been established. Racemic epinephrine is administered as 0.05 mL/kg per dose (maximum of 0.5 mL) of a 2.25 percent solution diluted to 3 mL total volume with normal saline. It is given via nebulizer over 15 minutes -Ventilators 1. SIMV: Pressure controlled Set: PIP, PEEP, RR, PS, FiO2. Varies: TV Advantage: easy to wean Disadvantage: set PIP can give large TV variation in lungs if changing complance 2. HFOV: High Frequency Oscillator Set: Amplitude, Hz, MAP Advantage: small volumes at high frequency; improves oxygenation when conventional vents fail Vent changes to alter blood gases: Desired Effect Rate PIP Increase PaCO2 Decrease PaCO2 Increase PaO2 Decrease PaO2 PEEP IT FiO2 MAP AMP Hz (if chronic) (if acute)

-Extracorporeal Membrane Oxygenation (ECMO): mechanical cardiopulmonary support used most often intraoperatively to facilitate cardiac surgery but can be delivered in a more prolonged fashion in an ICU 1. Two types: VA (venoarterial) and VV (venovenous). Both provide respiratory support but only VA provides hemoynamic support

RHEUMATOLOGY (Dr. Reinhardt) Characteristics of arthritis: Joint swelling, limping, morning stiffness, with or without pain Pain is very rarely only symptom without associated swelling Worse in the morning, improves with activity Physical exam: Muscle atrophy, asymmetric limb length, decreased ROM = synovitis Night pain: *Not consistent with arthritis; worry about malignancy If ever in question: Do MRI with contrast, and synovium will light up if inflamed Oligoarthritis (< 5 joints) 1-3 y/o Knees > ankles > wrists Asymmetric ESR/CRP: normal CBC: normal Polyarthritis RF (-) (5 or > joints) 6-7 y/o Knees > ankles > wrists > small joints Asymmetric ESR/CRP: normal or slightly elevated CBC: normal or mild anemia, leukocytosis, high platelets 20-40% positive in low titer (1:40 1:320) Less than oligoarthritis Polyarthritis RF (+) (5 or > joints) 9-12 y/o Small joints > knees > ankles > wrist Symmetric ESR/CRP: elevated CBC: leukocytosis, high platelets, anemia Systemic JIA All ages; usually 1-5 y/o Knees > ankles > wrists > small joints, TMJ, cervical spine Symmetric ESR/CRP: very elevated CBC: leukocytosis, high platelets, anemia Ferritin: very high (1000s) Typically dont check Rare

Ages Joints

Symmetry Labs

ANA

70-80% positive in low titer (1:40 1:320) Highest; especially if ANA + (50-70%). ANA(-): screen q6mo ANA(+): screen q3mo Joint swelling, morning stiffness, limping, pain less common

Uveitis risk

75% positive in low titer (1:40 1:320), but lower risk of uveitis Much less than oligoarthritis

Presentation

Joint swelling, morning stiffness, limping, pain less common

Treatment

Naproxen/Celebre x + PT Injections MTX

Naproxen/Celebre x + PT MTX TNFs

Similar to adult RA; symmetric small joint swelling, morning stiffness, limping, rheumatoid nodules (forearm, elbow), joint erosions Naproxen/Celebre x + MTX upon initial diagnosis TNF inhibitors

Arthritis + Fever (2wks) + 1 of following: Rash, LAD, Serositis, HSM. Fever: Quotidian; evening hrs Rash: Salmon pink, transient IV steroids + Naproxen IL-1 inhibitors (Anakinra)

JIA Pearls If ANA (+): slit lamp exam q3-4mo x 4yrs, If ANA (-): slit lamp exam q6mo x 4yrs Need to screen for uveitis because asymptomatic (chronic anterior uveitis) If suspect systemic JIA, reconsider if they dont have thrombocytosis SoJIA diagnosis: Quotidian fever x 2wks + arthritis + of following: Rash, LAD, HSM, Serositis SoJIA rash: Salmon-pink (macular or urticarial), prominent during febrile episodes, induced by heat or stress RF(+) polyarthritis: Need 2 RF (+) 3 months apart for diagnosis 2 types of Oligoarthritis: Persistent (still have <5 joints @6mo), Extended (initially <5 joints, then @6mo 5 or > joints)

Macrophage Activation Syndrome Most frequently associated with Systemic JIA (5-8%) Defective NK cell function & low perforin expression => inability to suppress lymphocyte activation => Cytokine storm Sudden onset of sustained fever, HSM, LAD, elevated transaminases, purpura, mucosal bleeding Labs: Pancytopenia (especially platelets), WBC count decreasing, ESR dropping, elevated triglycerides, hyponatremia, very high Ferritin (10s of thousands), prolonged PT/PTT, D-Dimer positive Tx: IV steroids (30mg/kg/dose) SLE Diagnosis (4/11) Malar rash Discoid rash Treatment Plaquinel, topical steroids, sunblock, tacrolimus Plaquinel, topical steroids, sunblock, tacrolimus Antibodies Labs ANA (95%) AntidsDNA (60%) C3/C4 decreased What to check Common Sx during flares Anti-dsDNA (rising Headaches titer) (refractory to meds) C3/C4 Polyarthralgias (decreasing)

Photosensitivity Sunblock

Anti-smith (25-40%) Anti-RNP SSA/SSB

Oral/Nasal ulcers (painless) CNS Dx (Seizures, psychosis) Arthritis (pain out of proportion to swelling) Serositis Renal Dx (protein, blood, HTN, elevated Cr) Positive ANA Cytopenias (low platelets, low lymphocytes, coombs + anemia Anti-dsDNA, anti-Sm, APLA

Plaquinel Pulse IV steroids, MMF, Cytoxan

CBC: low platelets, Coombs (+), hemolytic anemia, low lymphocytes Ur Prot/Cr Ur Prot/Cr ratio: ratio: elevated (>0.2) elevated (>0.2) ESR: ESR (increasing) elevated CRP: normal

Low grade fevers Fatigue Raynauds phenomenon

CBC (lymphopenia, hemolytic anemia, thrombocytopenia )

Naproxen, MTX

Alopecia

Symptomatic Tx Pulse IV steroids, Cytoxan, MMF, Enalapril ANA Pulse IV steroids if severe, MMF, Rituximab for resistant low platelets Low dose aspirin if APLA +, Heparin/Coumadin if clots

Vasculitic rashes LAD

HSM

SLE Pearls Everyone treated with Plaquinel, sun protection If ANA negative, should reconsider diagnosis If anti-Smith positive, you have SLE ANA needs to be sent for titer; >1:640 is considered significant

15-30% of normal population will have positive ANA in low titer (1:40-1:320), so it is a bad screening tool There are 3 APLA: Anti-cardiolipin, Lupus anticoagulant, Beta 2 glycoprotein APLA Signs/Sx: Livedo reticularis, thrombosis, chorea, transverse myelitis, recurrent miscarriages Infection is biggest cause of mortality (look for elevations in ESR/CRP w/o evidence of elevated dsDNA titer, or decrease in C3/C4) dsDNA titers increase with disease activity, C3/C4 levels decrease with disease activity

Neonatal Lupus Most common cause of congenital idiopathic heart block Manifested by fetal bradycardia Nearly all will need pacemaker Caused by maternal SSA antibodies (5% risk for neonatal lupus if mom SSA positive) 20% risk for subsequent pregnancies if prior child with neonatal lupus Rash (50%) within days to weeks after birth, can be induced by phototherapy for jaundice, resolves by 6 mo Benign joint hypermobility syndrome Very common: 20% of females, 10% of males Ages: 3-8 y/o Pain typically in the evening or at night; behind knees or calves Pain responsive to massage, heat, NSAIDS No swelling, or morning stiffness Diagnosis: Beighton score: 4/10 (Bilaterally) Thumb to forearm 5th MCP parallel to forearm >10 degree hyperextension of elbows >10 degree hyperextension knees Palms to floor with knees straight Dermatomyositis 4-10 y/o white females; No association with malignancy in children Weakness, rash, myalgias Proximal muscle weakness (symmetric); Neck, abdomen, hip flexors Positive Gowers sign, difficulty climbing stairs, difficulty arising from sitting/lying down Rashes: o Malar rash; no sparing of nasal labial folds o Heliotrope: purple discoloration of eyelids o Gottrons papules: over knuckles o Gottrons sign: scaly rash over elbows & knees Nail bed capillary changes (Tortuosity, dilation, capillary dropout) Soft tissue calcifications can develop in skin a few years into course of disease Labs: Check all muscle enzymes; CK, Aldolase, LDH, AST, ALT Many will only have elevation of 1 or 2 of them Other means of diagnosis: STIR MRI of quads (has basically replaced EMG) Treatment: Steroids (IV pulse during flares, PO otherwise), MTX, IVIG for resistant cases Acute Rheumatic Fever Jones Criteria (2 major or 1 major + 2 minor) Major: Joints, Heart, Nodules, Erythema Marginatum, Syndham chorea Minor: Prolonged PR interval, Fevers, Arthralgias, Elevated ESR/CRP Group A Strep caused; only pharyngitis, not skin infections Typically 5-15 y/o, rare <4 y/o Arthritis: migratory (hrs to days in 1 joint), large joints, red/hot/very painful Extremely responsive to NSAIDS Appears 14-21 days after strep infection Carditis: Mitral & aortic valve involved o Endocarditis > Myocarditis > Pericarditis (Inside => Outside)

Nodules: Extensor surfaces, last for 2-3 weeks, usually indicate carditis is also present Chorea: late finding (2-6 months) Tx: PCN x 10 days, Prednisone for carditis Prophylaxis: PCN or erythromycin; Lifelong if carditis is present, until 18-21 y/o if no carditis

Post-Streptococcal Reactive Arthritis Arthritis in non-migratory Typically asymmetric oligoarthritis of lower extremity Symptoms appear within 10 days of infection (Rheumatic fever is 14-21 days) Response to NSAIDS not as dramatic as rheumatic fever Treatment: PCN x 10 days, NSAIDS Vasculitis Large vessel Medium vessel Small vessel Takayasu arteritis Giant cell arteritis (extremely rare in kids) Kawasaki disease Polyarteritis nodosa HSP Wegeners granulomatosis (ANCA associated vasculitis) Microscopic polyangiitis (ANCA associated vasculitis) SLE Churg-Strauss syndrome (very rare in kids) Cryoglobulinemia Urticarial vasculitis Behcet disease Primary CNS vasculitis

Other

Vasculitis Pearls Most common by far are HSP, Kawasaki disease Small vessel vasculitis manifested in Lung, Kidney, Skin Medium vessel vasculitis typically with deeper skin lesions: ulceration, nodules HSP: typically 3-10 y/o (95% < 10 y/o) Diagnosis: o Palpable purpura (100%); Starts on lower extremities and moves cephalad o Arthritis/Arthralgias (80%) o Abd pain (60%); Intussusception (Ileal-ileal) **Need to do U/S not enema because of location. o Renal disease (40-50%) Glomerulonephritis (Protein in urine is what to watch for; microscopic hematuria very common) Late finding: 95% develop within the first 3 months F/U: UA & BP checked weekly until symptoms resolve completely then: Every 2 wks for next 3 months Monthly for subsequent 3 months Yearly therafter Labs: o CBC: r/o leukemia, ITP; Platelets typically elevated o Predominantly IgA deposition (50% have elevated IgA levels, but not routinely checked) Treatment: o Supportive; no need to hospitalize; NSAIDS, Bowel rest o Steroids only if: Severe abd pain, intussuception, renal disease, refractory arthritis Anti-dsDNA Anti-SSA/SSB Anti-Smith Anti-RNP Anti-Scl-70 SLE SLE, Neonatal lupus, Sjogren SLE Mixed Connective Tissue Dx, SLE, Scleroderma Systemic scleroderma

Anti-centromere Anti-Jo1 Anti-SRP Anti-Mi-2 c-ANCA (Proteinase-3 or PR3) p-ANCA (Myeloperoxidase or MPO) ANA RF HLA-B27

CREST Dermatomyositis, Polymyositis w/ lung disease Dermatomyositis (severe cardiac disease) Dermatomyositis (better prognosis) Wegener granulomatosis Microscopic polyangiitis, Churg-Strauss, SLE Ulcerative colitis SLE, JIA, Dermatomyositis, Systemic scleroderma, localized scleroderma JIA, SLE Enthesitis-related arthritis, psoriatic arthritis, IBD, reactive arthritis, ankylosing spondylitis

Lab Pearls Never send RF in children unless symmetric polyarthritis acting like adult RA Only send ANA if: 1. Suspect SLE or overlap syndrome 2. See arthritis on exam, & screening for uveitis risk ANA typically significant if titer >1:640 Raynaud Phenomenon Vascular spasm leading to triphasic color sequence (White Blue Red) Well-demarcated areas of color change Typically affects fingers & toes, but can also affect tip of nose, ears Triggers: Cold, stress, caffeine, smoking May be primary or secondary o Secondary causes: Scleroderma, MCTD, SLE, Dermatomyositis, Cryoglobulinemia o Predictors for secondary: Positive ANA, abnormal nail bed capillary findings Treatment: Preventative (Warm mittens, socks, boots, avoiding cold), Low dose Nifedipine => Cialis Autoinflammatory Fever Syndromes (Periodic fever syndromes) Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis (PFAPA) o Most common fever syndrome in children in North America o No known genetic association o Typically starts at <5 y/o & resolves within 5 years o High fevers occur every 4-6 wks o Fevers last up to 5 days o Have associated aphthous ulcers, pharyngitis, and cervical adenitis o Treatment: Single dose of PO steroids aborts the fever (may decrease time between attacks) o Also cimetidine, and last resort is tonsillectomy (only if severe because they grow out of it) TNF-Receptor Associated Periodic Syndrome (TRAPS) o Autosomal dominant o Distinguished by long attacks (3-4 wks at a time) o Migrating maculopapular rash spreading from trunk to extremities o Myalgias, arthalgias associated with rash o Severe abdominal pain Others o Familial Mediterranean Fever o Hyper IgD Syndrome o Neonatal Onset Multisystem Inflammatory Disease (NOMID) o Muckle Wells Syndrome

TRAUMA Code Protocol Respond to all overhead/paged code 4 MSOAP o Monitor: Attach cardiac to patient. o Suction: Wall suction with Yankauer attached o Oxygen: Start non-breather mask with 100% O2 for all patients except interstage HLHS. When in doubt, oxygenate. o Access: Establish reliable IV access (large bore, multiple sites). If cannot establish access within 1-2 minutes, immediately place IO with EZ IO o Personnel: Need MD for following positions: Airway: In conjunction with RT, in charge of positioning, bagging, prepping endotracheal intubation supplies, and intubating Cardiac: In charge of getting backboard, monitoring pulse, and providing CPR Drugs: In charge of ordering all drugs positioned at drug cart. Use emergency drug sheet at bedside and order dose and volume. Have multiple doses of epi drawn up in advance as well as RSI drugs: For RSI, give drugs in the following order: 1. Atropine 0.01 mg/kg 2. Midazolam 0.1 mg/kg 3. Rocuronium 1.0 mg/kg Follow PALS guidelines