zebras of neonatal hypoglycemia 06.09.2014
DESCRIPTION
Pediatric morning report at Primary Children's HospitalTRANSCRIPT
Morning ReportJune 2014Johanna Viau
Direct admit… PCP call 6 day old with hiccup like movements
and concerns for low sugars. Looks well now, feeding well, no fevers.
Birth History Pregnancy:
Late prenatal care, labs reassuring Delivery:
GBS positive, adequately treated NSVD, no complications BW: 3.3kg (AGA @ 39 wks)
Nursery course: Feeding: breast + formula supplementation Mild jaundice, Bili on DOL 2 < LL Discharged on DOL 2
DOL 3: PCP’s office Mom’s questions: hiccups on L side Feeding well, every 2 hours (breast + formula) Physical exam:
Wt: 3.15kg (95% birth weight) Normal vital signs and physical exam L side twitching x 20 sec, not suppressible
Bedside labs: Bili 12, glucose 38 Fed immediately, Sent to OSH for workup/tx via
EMS
DOL 3-5: OSH Admitted for workup/treatment Workup…. Normal per report Discharged on DOL 5
No clear diagnosis Glucoses in the 30-50’s range, better
after feeding Required IVF but feeding well on
discharge day Follow up w PCP the next day for a
glucose check
DOL 6: PCP office Chief complaint: hiccup like movements
last night, similar to the ones before you sent us to the hospital
Wt: 3.3kg (100% of BW, 58th %ile) Lt: 47cm (18th %ile) OFC 34cm (20th %ile) Vital Signs and physical exam: normal Feeding well Sent to PCH as direct admit
Admit Res pager:The direct admit has arrived Vital signs: T 36.5. HR 120x. RR 40x. BP
90/72. Sats 93% 30 second physical exam:
No acute distress, well hydrated No dysmorphic features Heart and lungs: normal Abd: Soft, without hepatosplenomegaly Neuro: somnolent, responding to
touch. +moro, +rooting, +suck. Moving all extremities equally and symmetrically.
Bedside glucose: 17 mg/dL
Differential diagnosisNEWBORN- LGA (hyperinsulinism)- SGA (no stores)- Prematurity- Infant of diabetic
mother
GASTRO/NUT- Poor feeding- Diarrhea- Vomiting- Malabsorption
ID- Sepsis- Meningitis- NEC- UTI
NEURO- Seizures- Asphyxia (depletion of
glycogen)- Midline
malformations(pituitary)
METAB/GENETICS- Inborn errors- Beckwith-Wiedemann- Hyperinsulinism- Adrenal insufficiency- Hypothyrodism- Hypopituitarism
OTHER- Maternal meds:
terbutaline, sulfonylureas
- Drugs- Toxins- Tumor- Muchausen
Outside hospital Workup Septic Workup:
CBC: WBC 8.2. HGB 21. PLT 140
CSF: WBC 4. RBC 222. Glu 14. Prot 84
UA: normal Blood, Urine &
CSF: negative @ 48h
Glucoses: 30-50’s
Neuro Workup: Head CT: normal EEG: mild
generalized cerebral dysfunction which could be due to medications, post-ictal state, metabolic or underlying structural
Started on phenobarbital
Critical Labs results CMP: Na 143, K 5.2, Cl 107, CO2 22, AG 14, glucose
22, BUN 4, Cr 0.57, Ca 10.3, protein 6.0, albumin 3.4, bili 9.2, alk phos 209, ALT 14, AST 36
Cortisol 6.5 (1-22) Insulin 10.6 (6.0-27.0) Beta-hydroxybutyrate 0.2 (0.0-0.6) Lactic Acid 3.8 (0.5-2.2) Ammonia 50 (56-92) Growth Hormone 7.84 (0.1-6.2) Urine ketones negative Reducing substances negative carnitine free 15 (15-55); total 19 (21-83 free fatty acids 0.49 (0.43-1.37)
The zebras of Neonatal HypoglycemiaAka: “something metabolic”
Glucose homeostasis in the newborn
Pregnancy
0 – 8-12 hours
> 12 hours
Passive diffusion of glucose Fetal plasma glucose = 70-80% of
maternal Insulin doesn’t cross the placenta
Fetus produces his own insulin
Birth clamping of umbilical cord Stress hormones: catecholamines,
glucagon, cortisol, growth hormone Insulin secretion
Pregnancy
0 – 8-12 hours > 12 hours
Glucose > 40 mg/dL (controversial) Feeds: ± Glucose depends on GLUCOGENOLYSIS
Adequate stores (glycogenesis) Prematurity, SGA, IUGR, etc. GSD 0
Enzymes for glucogenolysis GSD 1 GSD 3
Pregnancy 0 – 8-12 hours
> 12 hours
KetonesLactateHepatomegaly ?
Glucose > 50mg/dL (controversial) Glycogen stores depleted Glucose depends on
Feeds Galactose intolerance
Gluconeogenesis Amino acids metabolism Fatty acid metabolism
Pregnancy 0 – 8-12h > 12 hours
KetonesAcidosisAmmonia
Lactic acidKetonesAmmonia
Critical Labs results Cortisol 6.5 (1-22) Insulin 10.6 (6.0-27.0) Growth Hormone 7.84 (0.1-6.2) Ketones, Beta-hydroxybutyrate: negative Lactic Acid 3.8 (0.5-2.2) Ammonia 50 (56-92) Reducing substances negative carnitine free 15 (15-55); total 19 (21-83 free fatty acids 0.49 (0.43-1.37)
Congenital Hyperinsulinism
#1 cause of persistent hypoglycemia Several diseases High insulin despite hypoglycemia
Increased glucose utilization Even if feeds are going well
High GIR’s needed Multiple mutations Channelopathies Islet cell hyperplasia Focal vs diffuse Transient vs permanent
Treatment Supportive Diasoxide Pancreatectomy
Take home points: No obvious perinatal risk factors
gestational diabetes, perinatal asphyxia, infections, prematurity, SGA, LGA
Dysmorphic features Severe hypoglycemia
GIR > 8-10mg/kg/min Glucose Infusion Rate calculator
http://www-users.med.cornell.edu/~spon/picu/calc/glucinfr.htm Persistent hypoglycemia
Despite feeds or more than a few hours after birth
When to consider the zebras
Take home points:Overview of the zebrasPregnancy/
Birth 4-12 hours > 12 hours
The “horses” or associated to perinatal risk factors
Glycogen storesGlycogenesisGlycogenolysis
FeedsAmino acidsFatty acids
Low cortisol, low GH, high insulin
References See Wai Chan. Neonatal Hypoglycemia.
UptoDate. Sperling, Mark. Differential diagnosis
and management of Neonatal Hypoglycemia. Pediatr Clin N Am 51 (2004) 703 – 723