PAEDIATRICS CASE DISCUSSION –

KETOTIC V.S. NON-KETOTIC

HYPOGLYCAEMIA OF CHILDHOOD
DR. C. CATLYN
APRIL 2018
CASE SUMMARY

 Patient – S.W.
 Age – 4 years
 D.O.B. – 27/5/2013
 Historian – Mother
 P.C. –
 H/o hypoglycemic episodes associated with seizures throughout infancy
 Elective admission for fasting tests & hypoglycemia work-up
HPC

 4 year old female child, no known chronic illnesses

 H/o multiple seizure episodes 2o hypoglycaemia from 2 years of age associated with lethargy requiring multiple
hospital admissions
 H/o low blood sugar readings requiring several hospital admissions
 Initially was presumed to be 2o to poor feeding during neonatal period, ? Inborn error of metabolism
 Nil reported seizure activity during neonatal period
 Admission to May Pen Hospital x 2Τ52 for low blood sugars (? reading) at age 2
 Pt was noted to be sleeping, non-arousable & unresponsive, with eye-rolling, frothing at mouth & shaking of entire body
 Blood sugars fluctuated throughout course of hospital stay until control was eventually achieved
HPC CONT’D

 Hospitalized at Cornwall Regional Hospital (CRH) at age 3 for ‘chest infection’

 Presented with fever, runny nose, cough
 x 3Τ7 into treatment, seizure occurred lasting from 5am – 11am (status)
 Afebrile
 Informed by doctors that patient was hyponatraemic, and seizures resolved onced Na was corrected

 At 4 years old, 3 separate Admissions at CRH for seizures

 Home glucose monitoring had GMR readings < 1.0 at this time
 Each admission was approximately x 1Τ52
 Noted that low GMR readings tended to occur with seizures and occur in the early hours of the morning – 2AM
 Mother would feed the patient q3hrly but patient was noted to still go hypoglycaemic
HPC CONT’D

 Most recent admission was in March 2018 at CRH,

 Treated x 1Τ52 for ‘chest infection’ – presented with fever, cough, nasal stuffiness at night & decreased appetitite
 x 13Τ7 post discharge (x 3Τ7 prior to presentation), patient reported feeling hungry at 2 AM after having dinner and snack &
juice at 9pm. She was given sausage and juice before going back to sleep at 4 AM
 Mother attempted to wake pt at 8 AM but was found to be unresponsive
 GMR readings were ‘1 point something’ and was not conscious to take anything orally
 Taken to CRH and give IV fluids, recovered fully after ~6 hours monitoring and subsequently discharged for UHWI
Endocrine clinic the day of presentation
 Admitted from endocrine clinic for work-up of hypoglycemia via fasting studies / critical samples
HISTORY CONT’D

 PMH – as per HPC

 PSH – Nil  Immunizations

 DH – Nil  BCG – x 4Τ52

 Allergies  OPV/IPV – x 4Τ52, x 3Τ12, x 6Τ12, x 9Τ12, x4 𝑦𝑒𝑎𝑟𝑠

 Penicillin – generalized fine bumps to body and swelling  DPT/DT – x 4Τ52, x 3Τ12, x 6Τ12, x 9Τ12, x4 𝑦𝑒𝑎𝑟𝑠
to throat that hinders breathing  Hep B, Hib – x 4Τ52, x 3Τ12, x 6Τ12, x 9Τ12,
 MMR – x 12Τ12, x 18Τ12
ANTENATAL & BIRTH HX

 Urine Pregnancy (hCG) test

 LSCS 20 Breech @ GA x 41Τ40
 Maternal age – 33 yr old
G4P2 +1 – Spontaneous Miscarriage x 6Τ52  Apgar 71 min, 95 min

 F/u a May Pen Hospital, then CRH from 7 months  BW 3.71 kg (~ 25th centile); Length 52 cm (25th – 50th
onwards centile)

 Booking Parameters  HC 37 cm (25th – 50th centile)

 Maternal Blood Gp A+  Neonatal hypoglycemia requiring NICU admission x 17Τ7

 HIV – Neg,VDRL – Neg, Sickle – Neg
 USS normal throughout pregnancy
 Hospitalized on multiple occasions for hyperemesis
gravidarum, tx w/ steroids
 H/o UTI,Yeast infection – both fully treated
 Gestational DMO, PIHO
– tx with dextrose infusion
 Presumed to be 2o to poor feeding at the time, ? Inborn
error of metabolism
 Sepsis screen at time was negative; Abx ✔️
 NNJO
 Supp. O2 via face mask shortly after birth- ?duration
GROWTH & DEVELOPMENT

 Language
 Gross Motor
 Started talking @ <2 years
 Hops ✔️, Skips ✔️, Jumps over low obstacles✔️
 Knows colours ✔️
 Walking @ < 1 year
 Says song from memory ✔️
 Asks questions ✔️
 Visual  Writes a few letters from her name ✔️
 Copies a Square ✔️
 Buttons clothing ✔️  Social / Adaptive
 Dresses self but sometimes puts on clothes  Shares toys ✔️
wrong-sided or back to front  Plays well with other children ✔️

 Attends Montego Bay Infant School

 Nil concerns with respect to behavior but reported that she does not like to eat much at school
HABITS AND BEHAVIOUR

 Tendency to pick at thumb nails

 Nails have gotten progressively thickened over time
 Noted by mother in January 2018
NUTRITIONAL HISTORY

 Breakfast
 Macaronic & Cheese, nuggets, sausage, sweet corn, plaintain
 Snack  Dietary Habits
 Cereal, juice, fruits e.g. ripe banana, naspberry, star apples,  Noted to be a picky eater by mother
grapes
 Alternates particular food choices every other week or
 Lunch so
 Rice & Peas, chicken, soup, cup cake, hot dog  Note to eat small portions, for instance will eat 6
spoonfuls and state that she is full
 Snack (after school)
 Has been referred to a dietician after each hospital
 1 bowl cereal admission but has not been found to be impactful
 Lacto 1+ (5 scoops) & Nestum (3 scoops) 4 – 5 ounces,
Chips
 Dinner
 Chicken, dumplings, banana, rice, fish
FAMILY HX

 HTNO, DMO, Thyroid ProblemsO, Heart

ProblemsO, EpilepsyO, CancerO
 Asthma – paternal cousins
 Eczema – paternal cousins
 Sickle Cell Disease - paternal cousins
SOCIAL HX

 Lives with mother & two half siblings in 4 bedroom concrete home with modern amenities
 Father works in Turks and Caicos but supports financially and visits twice per year
 PestsO, PetsO
 Garbage disposal every 3 days
SUMMARY
PHYSICAL EXAMINATION

 PR 110 bpm
 RR 22 / min
 SpO2 100% on Room Air  Weight 11.1 kg (< 3rd centile)

 T 36.3 oC  Height 63cm (<3rd centile)

 BP 78Τ
36
 BMI 27.97 kg/m2
 Using 5th centile for age:
 50th 88Τ50
 90th 101Τ64
 95th 105Τ68
 99th 112Τ76
PHYSICAL EXAMINATION CONT’D

General:
Resp
 Female infant sitting in mother’s lap, nil CPD
 RR 2, NFO, TTO, ICRO, SCRO
 AI, AC, AF, MM – pink + moist
 Chest Expansion equal and adequate
 Nil Lymphadenopathy
 PN resonant
 Hydration status: Normal Skin Turgor, Adequate salivary
pool, Cap. Refill < 2sec  BS- bronchovesicular throughout, Normal intensity
 CrepsO, RhonciO

CVS Abdomen
 Pulse 110, normal volume, regular rhythm, R-R  Soft, non-tender
synchrony  MassesO
 Apex - 4th LICS MCL, PSHO, ThrillsO, P2O  OrganomegalyO
 HS – S1 ✔️, S2 ✔️, S3O, S4O, MO  BS ✔️ - Normal
PHYSICAL EXAMINATION CONT’D

CNS
 Awake, Alert, cooperative with exam Motor
 Nil abnormal movements  Tone normal, ?bulk

 CN I – not assessed  Power at least 4Τ5

 CN II – PERTL, unable to visualize fundus  Reflexes 2+ throughout

 CN III, IV,VI – Normal ROM, NystagmusO, PtosisO  Down-ging plantars

 CN V – normal massester & temporalis  ClonusO

 CN VII – nil facial asymmetry, normal furrowing

Skin
 CN VIII – grossly intact
 Hypopigmented patches to left knee, inner left thigh +
 CN IX, X – uvula central, gag intact right lower limb ranging from 3-5 cm in diameter
 CN XI – normal shrug, Sternocleidomastoid ✔️,  PruriticO, ScalingO
Trapezius ✔️  Mother reports present from birth
 CNXII – tongue central, nil deviation, nil fasciculation
PHYSICAL EXAMINATION CONT’D

 Ear
 Bilaterally Tympanic Membrane visualized & Normal
 ErythemaO,Air-fluid levelO, Discharge

 Nose
 No abnormalities detected

 Tonsils
 Grade 1, ErythemaO,ExudateO
ASSESSMENT & DIFFERENTIAL DIAGNOSIS

1. Hypoglycemia
 ? Ketotic vs. Non-Ketotic Hypoglycemia of Childhood

2. Undernutrition / Failure to Thrive

PLAN – INVESTIGATIONS

 Admit to Ward  At 6 AM, Fasting glucose

 Vitals q4hrly  Then at 8 AM, RBG, Insulin, Glucagon, C-peptide,
Growth Hormone, U&Es, Cortisol, Urine ketones,
 For 8 AM & 8 PM cortisol IGF binding protein, Serum Lactate, Urine Organic
 For controlled fasting protocol to commence at Acid (ideally, but not available locally)
10pm and appropriately-timed critical samples  Repeat serum and urine testing @ 12pm & 4pm
 Ensure IVA  If becomes symptomatic of hypoglycemic (lethargy /
 Rescue Dextrose to be present at bedside @ all seizures)
times  Stop fast, Do critical samples
 GMRs q2-4hrly  Push 2mls 1g 10% DW
 Do urine ketones 2 hours after
COURSE THROUGHOUT ADMISSION
DISCHARGE & FOLLOW-UP
DISCUSSION
GLUCONEOGENIC, GYLCOLYTIC, AND GYCOGEN SYNTHESIS
PATHWAYS
DISCUSSION
LABORATORY FINDINGS IN IDIOPATHIC KETOTIC HYPOGLYCEMIA

 Elevated FFA, and ketones

 Ketonuria
 Low plasma alanine concentration – Decreased alanine on quantitative plasma amino acids
 Normal blood lactate & pyruvate levels
 Appropriately suppressed plasma insulin levels (≤2 microM/mL [15 pmol/L])
 Increased counterregulatory hormones – GH, cortisol
 Normal thyroxine (excludes hypopituitarism)
 Glycemic response to glucagon (0.03mg/kg IM or intravenous (IV); maximum 1 mg) is normal in the fed state, but
blunted at the time of hypoglycemia
 Negative urine reducing substances.
REFERENCES