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This document provides information on neonatal jaundice and hyperbilirubinemia. It discusses the causes, pathophysiology, clinical presentation, diagnostics including lab tests, imaging, and liver biopsy. Lab tests outlined include total and conjugated bilirubin levels as well as liver enzymes. Imaging options like ultrasound and scintigraphy are described. The importance of considering the history including prenatal and perinatal factors is also highlighted.
This document provides information on neonatal jaundice and hyperbilirubinemia. It discusses the causes, pathophysiology, clinical presentation, diagnostics including lab tests, imaging, and liver biopsy. Lab tests outlined include total and conjugated bilirubin levels as well as liver enzymes. Imaging options like ultrasound and scintigraphy are described. The importance of considering the history including prenatal and perinatal factors is also highlighted.
This document provides information on neonatal jaundice and hyperbilirubinemia. It discusses the causes, pathophysiology, clinical presentation, diagnostics including lab tests, imaging, and liver biopsy. Lab tests outlined include total and conjugated bilirubin levels as well as liver enzymes. Imaging options like ultrasound and scintigraphy are described. The importance of considering the history including prenatal and perinatal factors is also highlighted.
NEONATAL JAUNDICE ▫ Primary complication is neurotoxicity of UCB across
Yellow discoloration of the skin and sclerae due to the BBB (mostly in the basal ganglia, pons, & elevated serum bilirubin (2-2.5 mg/dL); physical evidence cerebellum), known as kernicterus of jaundice usually seen in levels of 5-10mg/dL CONJUGATED HYPERBILIRUBINEMIA Severity of jaundice: o Sclera icterus (3mg/dL) ▫ Can be caused by: o Face (5mg/dL) a. Hepatocellular dysfunction o Mid-abdomen (15mg/dL) b. Biliary obstruction o Soles (20mg/dL) c. Abnormal excretion of bile acids / bilirubin
REVIEW: NORMAL BILIRUBIN FORMATION DIAGNOSTICS
LAB TESTS ▫ Formed from the degradation of heme-containing BILIRUBIN compounds (eg. Hgb) Total serum bilirubin should be fractionated to 1 Microsomal heme oxygenase catabolizes heme differentiate between conjugated and unconjugated to biliverdin hyperbilirubinemia 2 Reduction of biliverdin to bilirubin by biliverdin Hemolysis can interfere with assays, resulting in a falsely reductase producing unconjugated bilirubin elevated conjugated fraction (UCB) AMINOTRANSFERASES 3 Binding of UCB to albumin Aspartate aminotransferase (AST) and Alanine 4 Bilirubin uptake by hepatocytes aminotransferase (ALT) used as arkers for hepatocelular 5 Conjugation of bilirubin (CB) w/ glucuronic acid by injury bilirubin uridine diphosphate glucoronosyltransferase Increased ALT level is more suggestive of liver disease (UDPGT) Markedly elevated levels (>5 to 10-fold) of both AST and ALT can be due to: 6 Excretion of CB from the hepatocyte through ▫ Hepatitis, hepatotoxicity, ischemia, or cannaliculi (biliary tree) to the duodenum genetic/metabolic liver disorders 7 Bacterial hydrolysis converts CB to urobilinogen Elevation of AST in excess of ALT suggests extrahepatic 8 Reabsorption via: Excretion via: source of injury ▫ enterohepatic ▫ Renal excretion of circulation urobilinogen ALKALINE PHOSPHATASE ▫ - glucoronidase ▫ Conversion of Enzyme found in bile ducts, bone, intestine, placenta, & hydrolizes CB to urobilinogen to tumors UCB and stercobilin and Elevations occur with hepatobiliary disease but also in reabsorbed via excreted in feces normal growth, healing fractures, vit. D deficiency, bone enterohepatic disease, pregnancy and malignancy circulation (in Fractionation of alkaline phosphatise isoenzymes can neonates) help determine its site of origin In evalusation of hyperbilirubinemia: ▫ UCB is lipophilic, cannot be excreted by the kidneys, ▫ Alp > 3x normal indicates cholestasis and can easily cross cell membranes and the blood ▫ Milder elevation suggestive of hepatocellular disease brain barrier Y-GLUTAMYLTRANSFERASE (GGT) ▫ CB is a polar, water soluble compound More specific for biliary tract disease compared HYPERBILIRUBINEMIA aminotransferases Can result from alteration of any step in the process of GGT elevations are inducible by alcohol & certain drugs bilirubin formation (phenytoin & Phenobarbital) Classified either as Conjugated (direct) or Unconjugated Can be elevated in chronic pulmonary disease, liver (indirect) depending on CB concentration in serum failure, or DM Conjugated hyperbilirubinemia exists when more than Helpful in confirming that an elevated Alp is a result of 20% of the total bilirubin or >2mg/dL is conjugated liver disease and in differentiating familial cholestatic syndromes UNCONJUGATED HYPERBILIRUBINEMIA ▫ Can be caused by: BILE ACIDS a. Increased production of bilirubin Sensitive measure of cholestatic disease b. Decreased delivery to the liver Levels are generally very high in primary cholestasis & c. Decreased hepatic uptake biliary obstruction d. Decreased conjugation Mildly increased (>2x normal) in hepatocelLular disease e. Increased enterohepatic circulation of bilirubin ALBUMIN LIVER BIOPSY Produced in the liver; reflecting hepatic synthetic In which specific pattern of injury (eg. Paucity of bile function ducts / bile duct proliferation) Used in monitoring progression of chronic liver disease Specific markers of disease may be identified (inclusions nad in discriminating acute illness vs. Chronic disorder in 1-antitrypsin deficiency) or measured (metabolic Hypoalbuminemia may be secondary to nephritic enzyme activity) syndrome or protein-losing enteropathy HISTORY PROTHROMBIN TIME Note age at onset and duration of jaundice Best marker of hepatic synthetic function (since clotting ▫ Onset after 24 hr of age factors are produced in the liver) ▫ Not persisting beyond 14 days of age (1 mo if breast- If PT is prolonged, document response to parenteral fed) administration of vit K (Vit K deficiency) Acholic stools Consider disseminated intravascular coagulation & ▫ Indicate obstruction of the biliary tree; can also be thrombosis of a major blood vessel as alternative causes seen in severe hepatocellular injury for prolonged PT Delayed passage of meconium (within 24-48 hrs) may be IMAGING 2° to cystic fibrosis or Hirschsprung disease ULTRASONOGRAPHY Assess prenatal and perinatal history Useful, non-invasive, rel. Inexpensive for evaluation of ▫ Maternal infections (Syphilis, Toxoplasmosis, CMV, liver disease Hep B, Enterovirus, HSV, HIV) that can cause Provides info on size & consistency of liver, spleen, and cholestatic liver disease in neonate anatomic abnormalities of the biliary tree, gallstone & ▫ Premature infants are more prone to higher level of hepatic masses bilirubinemia and prolonged hyperbilirubinemia Dilated intrahepatic ducts – indicate extrahepatic ▫ Symptoms of constipation, hypotonia & hypothermia obstruction could indicate Hypothyroidism Doppler UTZ – demonstrate dynamic flow in hepatic ▫ Hx of repeated affected neonates could indicate blood vessels & portal vein (Portal HTN) Alloimune hepatitis Delayed feeding SCINTIGRAPHY Breast-feeding associated w/ inc levels og UCB and Aid in diagnosis of biliary atresia longer duration of jaundice Administration of Phenobarbital (5mg/kg/day) for 5 days Vomiting before the study may inc bile flow, increasing diagnostic ▫ (+) lethargy and poor feeding is suggestive of accuracy metabolic disorder COMPUTED TOMOGRAPHY ▫ Can also be a symptom of intestinal obstruction Useful in identifying mass lesions within the liver (malrotation / volvulus) CT + contrast used to define nature of liver tumors PHYSICAL EXAMINATION CT angiography to define anatomy of portal and hepatic Jaundice spreads in a cephalopedal direction circulation Pallor may indicate hemolytic disease MAGNETIC RESONANCE Petechiae suggestive of thrombocytopenia, possible Can demonstrate storage of heavy metals such as iron sepsis, congenital infection, or severe haemolytic disease (Neonatal iron storage disease) Dysmorphic features: MR cholangiopancreatography (MRCP) is rel non-invasive ▫ Microcephaly + jaundice is associated with that helps visualize abnormalities of the intrahepatic & congenital viral infections extrahepatic biliary tree and the pancreatic duct system ▫ Zellweger syndrome o Narrow cranium, prominent forehead, ENDOSCOPIC RETROGRADE hypertelorism, epicanthal folds, large CHOLANGIOPANCREATOGRAPHY (ERCP) fontanel Performed for evaluation of biliary anatomy ▫ Alagille syndrome Both diagnostic and therapeutic for common duct stones o Triangular face w/ broad forehead, and strictures hypertelorism, deep-set eyes, long nose, PERCUTANEOUS TRANSHEPATIC CHOLANGIOGRAPHY pointed mandible Both diagnostic & therapeutic; alternative to ERCP Ophthalmologic findings Done under UTZ guidance, where needle passes through ▫ (+) cataracts – galactosemia and rubella liver to biliary tree where contrast is injected ▫ Chorioretinitis – congenital infections Contraindicated in marked ascites or irreversible ▫ Nystagmus w/ hypoplasia of optic nerve – coagulopathy hypopituitarism w/ septo-optic dysplasia Complications include bleeding, pneumothorax, Presence of heart murmurs caused by underlying infection, and bile leakage congenital heart diseases associated with: ▫ Alagile syndrome UNCONJUGATED HYPERBILIRUBINEMIA ▫ Trisomies Hematologic evaluation must be performed to identify ▫ Syndromic forms of Biliary atresia (Polysplenia cause of hyperbilirubinemia syndrome) ▫ CBC with examination of smear Note: can also be a result of hepatic ischemia/congestion ▫ Reticulocyte count due to hyperbilirubinemia ▫ Direct Coombs test Microphallus can be associated with septo-optic ▫ Blood typing dysplasia and hypopituitaism POLYCYTHEMIA DIFFERENTIAL DIAGNOSIS Hct >65% by venipuncture Important to differentiate between physiologic and Results from increased bilirubin production due to pathologic jaundice increased RBC mass Total and fractionated bilirubin measurement should be Associated with maternal DM, maternal-fetal / twin-twin performed if assessment of physiologic jaundice is transfusion, intrauterine hypoxemia, endocrine questionable disorders, and delayed cord clamping PHYSIOLOGIC JAUNDICE HEMOLYTIC DISORDERS PATHOYSIOLOGY In patients w/ reticulocytosis, unconjugated Increased bilirubin production due to normal increased hyperbilirubinemia and inc nucleated RBC count w/ low- neonatal red blood cell mass and decreased lifespan of normal Hct RBC (80 days) ISOIMMUNE HEMOLYTIC DISEASE Lower albumin concentration and binding capacity (dec Maternal IgG to infant’s erythrocytes cross the placenta albumin binding) resulting in lowerUCB transport to the resulting in RBC destruction liver Bilirubin uptake in the 1st week of life is defective (dec Rh INCOMPATIBILITY Glutathione S-transferase B) presents with pallor, hepatosplenomegaly and rapidly Impaired conjugation due to dec activity of UDPGT developing jaundice Inc enterohepatic circulation of UCB due to: occurs when and Rh-negative mother gives birth to a Rh- ▫ Inc concentration of -glucoronidase positive infant ▫ Dec intestinal bacterial flora → diminished diagnosis is confirmed by: urobilinogen formation ▫ Rh-positive infant ▫ (+) direct Coombs test result MANIFESTATION ▫ Maternal antibody coating infant’s RBC Peak bilirubin level < 13 mg/dL on 3rd -5th day of life Administration of anti-D gamma globulin (RhoGAM) after Decrease to normal by 2 weeks of age delivery to Rh-negative women reduce incidence of Rh Conjugated fraction of < 20% sensitization and erythroblastosis fetalis BREAST MILK JAUNDICE Management depending on degree of hemolysis may be Unconjugated hyperbilirubinemia of > 13 mg/dL postnatal phototherapy and/or exchange transfusion
Early / Breast-feeding Late / Breast-milk ABO INCOMPATIBILITY
Jaundice Jaundice Common in infants with blood type A or B born to a type o Occurs within the first o Occurs after the 1st O mother 5 days of life week of life Hemolysis develops in 50% of sensitized infants with a o In infants not feeding o Peaks between 2nd & bilirubin level > 10mg/dL adequately; may be 3rd week of life (10-20 Diagnosis: dehydrated / mg/dL) ▫ Blood smear: (+) anemia, reticulocytosis, & malnourished o May be due to spherocytosis ▫ Inhibition of ▫ Weakly-positive direct Coombs UDPGT activity ▫ (+) indirect Coombs test ▫ Inc enterohepatic ERYTHROCYTE DEFECTS circulation of UCB MEMBRANE DEFECTS Kernicterus as a rare complication associated w/ a family Hx of hemolysis, transfusions, cholecystectomy, or splenectomy MANAGEMENT Hemolysis results from fragility of RBC Rooming-in and frequent feeding dec risk of breast- Presents with: feeding jaundice ▫ Anemia, jaundice, and splenomegaly If bilirubin exceeds 20mg/dL in breast-fed infant ▫ Blood smear: spherocytosis . elliptocytosis Discontinue breast-feeding for 24hrs ▫ (-) Coombs test Phototherapy Exchange transfusion ENZYME DEFECTS A. G6PD deficiency ▫ Oxytocin, excess vit K in preterm infants, Most common antibiotics, phenol disinfectants and herbal Manifests as neonatal jaundice on day 2 or 3 after remedies birth; or in later childhood (jaundice associated with THERAPY FOR UNCONJUGATED HYPERBILIRUBINEMIA acute haemolytic crisis) Depends on degree of elevation of bilirubin (> 20-30 B. Pyruvate kinase deficiency mg/dL in healthy, term infants w/o evidenc of hemolysis) Enzymatic deficiency in the glycolytic pathway Mostly autosomal recessive mode of transmission PHOTOTHERAPY Produces reduction of bilirubin by 1-2mg/dL in 4-6 hours FAMILIAL DISORDERS OF BILIRUBIN METABOLISM Photoisomerization and photodegradation of GILBERT SYNDROME unconjugated bilirubin to more water-soluble forms; to Heterogenous group of disorders be excreted in bile & urine 50% dec in UDPGT activity from defect in gene Possible complications: retinal damage, diarrhea, & responsible for this enzyme dehydration Dec in hepatocyte bilirubin uptake Begun at levels below exchange transfusion (~5mg/dL) or Generally asymptomatic until 2nd or 3rd decade of life during prep for exchange transfusion All lab tests are normal except for inc indirect bilirubin level EXCHANGE TRANSFUSION Diagnosis is clinical;confirmed by 2 to 3-fold rise in UCB Indicated for severe hyperbilirubinemia during 24hr fast Indications: ▫ Term infants with evidence of hemolysis and CRIGLER-NAJJAR SYNDROME (Arias Syndrome) bilirubin >25-30mg/dL Rare autosomal recessive condition caused by mutation ▫ Non-responsive to phototherapy of gene coding for UDPGT ▫ (+) signs of kernicterus: A. TYPE I High-pitched cry, gaze paralysis, fever, ▫ Marked hyperbilirubinemia (20-40mg/dL) in lethargy,& opisthotonic posture neonatal period of a healthy infant ▫ Untreated individuals develop kernicterus CONJUGATED HYPERBILIRUBINEMIA ▫ Lab results show Important to evaluate infant for potentially treatable o (-) CB in bile or serum problems o Colorless bile HYPOPROTHROMBINEMIA o (-) dec in serum UCB during Phenobarbital ▫ Characterized by prolonged PT administration ▫ Initial administration of IV vit K to avoid spontaneous ▫ Treatment via exchange transfusion, intensive hemorrhage phototherapy, and liver transplant ▫ Managed with oral fat-soluble vitamin B. TYPE II supplementation until cholestasis resolves ▫ Onset at birth with <5% normal UDPGT activity HYPOGLYCEMIA ▫ Bile contains bilirubin monogluronides ▫ Associated with severe hepatic dysfunction, ▫ Bilirubin level of 8-25mg/dL metabolic disorders, and hypopituitarism ▫ Responds to Phenobarbital administration ▫ Serum glucose level is measured before feeding LUCEY-DRISCOLL SYNDROME ▫ Managed with frequent feeding, continuous feeding, Transient familial neonatal hyperbilirubinemia or IV dextrose infusions Appears in the first few days of life and resolves by 2- HYPERAMMONEMIA 3weeks of age ▫ Present in sever liver dysfunction and metabolic liver Results from inhibition of UDPGT by substance found in disorders both infant & maternal serum ▫ Checked in infants presenting with lethargy or Bilirubin level can rise until 60mg/dL (resulting in change in mental status kernicterus) Other labs included in evaluation: Treated with exchange transfusion ▫ Aminotransferases, GGT, and Alkaline phosphatise ▫ CBC OTHER CONSIDERATIONS When Hct is normal, no evidence of hemolysis / OBSTRUCTIVE/ANATMOIC ABNORMALITIES consumptive process BILIARY ATRESIA o GI Obstruction Result of a progressive inflammatory process leading to ▫ vomiting, abdominal distention, delayed obliteration of the lumen of the extraheptic duct passage of meconium Leading indication for liver transplant in pediatric ▫ investigate via imaging population o Hypothyroidism / Hypopituitarism Manifestation: ▫ Look at thyroxine and TSH levels ▫ Icteric at birth and develops jaundice at 2-6 weeks of o Drug administration age ▫ Jaundice, dark urine, and acholic stools Triangular face w/ broad forehead, Types: hypertelorism, deep-set eyes, long nose, A. Embryonic / Fetal Form pointed mandible ▫ There is no jaundice-free period ▫ Vertebral arch defects ▫ Associated with cardiac defects, polysplenia, butterfly vertebrae, hemivertebrae, decreased malrotation and situs inversus interpedicular distance B. Perinatal Form ▫ Posterior embryotoxon (ocular) ▫ There may be jaundice-free interval after ▫ Cardiac defects resolution of normal physiologi jaundice peripheral pulmonic stenosis to complex CHDs ▫ No associated anomalies ▫ renal anomalies, pancreatic insufficiency, and growth Diagnostics: retardation may be present ▫ Conjugated hyperbilirubinemia, acholic stools and ▫ pruritus develops by 4-6 mos of age elevated GGT suggests prompt evaluation ▫ Xanthomas appear in association with markedly ▫ Ultrasound: elevated cholesterol to exclude other treatable anatomic Autosomal dominant transmission abnormalities (eg. Choledocal cyst) Diagnosis is confirmed by liver biopsy Gallbladder is absent or collapsed TREATABLE INFECTIONS ▫ Liver biopsy BACTERIAL INFECTION Periportal edema & fibrosis, bile duct Blood and urine cultures should be obtained proliferations, and bile duct plugs May present with poor feeding, lethargy, vomiting, temp ▫ Hepatobiliary scintigraphy instability, apnea, bradycardia, or shock Demonstrates uptake of tracer but no E. Coli most common etiologic agent – excretion into the duodenum hyperbilirubinemia caused by endotoxin-mediated Requires pretreatment with Phenobarbital canalicular dysfunction (due to delay in diagnosis) Other gram-negative causes: Listeria, Staphylococcus, Inconclusive in hepatic dysfunction and Streptococcus Management: Surgery o Surgery HERPES SIMPLEX ▫ Should be performed before 2 mos. of age Manifests at 7-14 days of age with lethargy, por feeding, ▫ Kasai Procedure a vesicular rash, jaundice, hepatomegaly, temp portoenterostomy procedure in which porta instability, encephalitis, and coagulopathy hepatis is transacted, and a loop of intestine Diagnosis by identification of virus in skin lesions through is brought up to drain the bile ducts ▫ Direct fluorescent antibody staining complication: Bacterial ascending ▫ Polymerase chain rxn of HSV in blood & CSF cholangitis Treatment with IV Acyclovir ▫ Liver Transplant ENTEROVIRUS o Supplementation with MCT oil and fat-soluble Manifests within 1-7 days after birth with similar vitamins presentation w/ HSV except for macular rash Portal HTN w/ splenomegaly, esophageal varices, and Diagnosis by polymerase chain reaction or viral culture ascitis can develop overtime Treatment by IV immunoglobulin and Pleconaril CHOLEDOCAL CYST CYTOMEGALOVIRUS Presentation: 90% are asymptomatic at birth ▫ In neonates: conjugated hyperbilirubinemia w/ Can manifest within the first 24 hrs after birth w/ IUGR, jaundice, vomiting, acholic stools and hepatomegaly conjugated hyperbilirubinemia, haemolytic anemia, ▫ In older children: jaundice, abdominal pain, and RUQ thrombocytopenic purpura, and hepatosplenomegaly mass Diagnosis by urine culture Diagnosis by UTZ and confirmed by MR cholangiography Treatment via Ganiciclovir and CMV immunoglobulin or intraoperatice cholangiography Treatment by surgical excision HEPATITIS B Seen in mothers who are seropositive for hep B e antigen ALGILLE SYNDROME or have acquired acute infection in the last trimester Abnormal development of multiple organs related to Perinatal infection can be prevented with hep B immune defective JAG-1/NOTCH-2 signaling globulin and vaccination Clinical features: ▫ Marked reduction in number of interlobar bile ducts SYPHILIS (most important) Presents with jaundice, fever, diffuse macular-papular ▫ Unusual facies rash, hepatosplenomegaly, edema, anemia, and periostitis in severely infected infants Diagnosis via o Nontreponemal serologic tests on cord blood Most common inherited cause of neonatal cholestasis o Confirmation from infant serum showing Manifests in early infancy with prolonged conjugated ▫ (+) IgM for syphilis hyperbilirubinemia, failure to thrive, acholic stools, ▫ (+) Immunoglobulin G fluorescent treponemal hepatoegaly, and possible ascitis antibody Diagnosis is by serum phenotyping (ZZ) Treated with IV Penicillin for 10-14 days Treatment is supportive TOXOPLASMOSIS CYSTIC FIBROSIS IgM titers should be obtained or histologic examination Incidence is increased among infants w/ meconium ileus of placenta should be done Diagnosis is confirmed with: Mostly asymptomatic; in severe infection may present o Sweat chloride test with hydrocephaly/microcephaly, intracranial o Detecting abnormal gene calcifications, chorioretinitis, aseptic meningitis, jaundice, HYPOTHYROIDISM & HYPOPITUITARISM purpura, and hepatomegaly May manifest with hypoglycaemia, microphallus, Postnatal treatment: jaundice, and signs of hypothyroidism ▫ Pyrimethamine and Sulfadiazine Wandering nystagmus seen in Hypopituitarism ▫ Folinic acid (to prevent folate deficiency) associated w/ septo-optic dysplasia TREATABLE METABOLC DISORDERS Resolution by treatment of underlying endocrine GALACTOSEMIA disorder Life-threatening disorder PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS Autosomal recessive disorder with deficiency of Related to defective transport of bile acids galactose-1-phosphate uridyltransferase Finding suggestive by: ▫ needed for conversion of galactose to glucose ▫ presence of cholestasis in absence of physical ▫ accumulation is hepatotoxic damage to bile ducts once lactose is introduced in diet, infant presents with ▫ normal GGT except for PFIC type 3 variant vomiting, diarrhea, jaundice, hepatomegaly, & cataracts autosomal recesive disorder with 3 types (refer to often presents with E.coli sepsis at 1st wk of life appendix) Laboratory evaluation: ▫ Elevated AST & ALT IDIOPATHIC NEONATAL HEPATITIS ▫ Prolonged PT Diagnosis of exclusion; common in premature / SGA ▫ Hemolytic anemia infants ▫ Aminoaciduria Hepatobiliary scintigraphy demonstrates delayed uptake, ▫ (+) galactose in urine but there is usually excretion into the duodenum unless Diagnosis by confirmed deficiency of enzyme in the hepatitis is severe erythrocytes and leukocytes Treatment is supportive Transfusion may cause false-negative results TREATMENT FOR CHOLESTASIS Treatment by eliminating galactose in diet Malabsorption of fats and fat-soluble vitamins occur as a HEREDITARY FRUCTOSE INTOLERANCE (Fructosemia) result of dec concentration of bile salts in intestinal lumen Uncommon Affected infants should be given formulas containing Manifests w/ hepatic failure when exposed to medium-chain triglycerides (MCTs) fructose/sucrose in formula, juice, fruit, or medication Caloric intake excess of 150 kcal/kg/day to maintain Treatment by removal of fructose, sucrose, and sorbitol growth from diet Proper monitoring of growth ▫ Anthropometric evaluation of skinfold and mid-arm TYROSINEMIA circumference Diagnosed by serum amino acid levels and urine organic Supplemental vit A, D, E, K to prevent visual problems, levels rickets, neuropathy, and coagulopathy Elevated urinary succinylacetone (pathognomonic) Ascitis can be managed w/ Na restriction and diuretics Treatment by dietary restriction of phenylalanine, methionine, & tyrosine and use of 2-(2-nitro-4- trifluoromethylbenzoyl)-1,3-cyclohexanedione DISRODERS OF BILE ACID METABOLISM Suggested by conjugated hyperbilirubinemia w/ low- normal GGT, and low-normal total bile acid levels Detected by bile acid FAB-MS urinalysis Treatment with oral cholic acid supplementation 1-ANTITRYPSIN DEFICIENCY s PEDIA 250
NEONATAL JAUNDICE APPENDIX
Figure 1: Diagnostic Approach to Neonates/Infants with Hyperbilirubinemia
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