Morning

Report
June 2014
Johanna 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: PCPs office
Moms 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 30-50s 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, 58
th
%ile)
Lt: 47cm (18
th
%ile)
OFC 34cm (20
th
%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.

Differential diagnosis
NEWBORN
- 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: 30-50s
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
Hypoglycemia
Aka: 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 doesnt 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
Ketones
Lactate
Hepatomegaly ?
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
Ketones
Acidosis
Ammonia
Lactic acid
Ketones
Ammonia
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 GIRs 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 zebras
Pregnancy/Birth 4-12 hours > 12 hours
The horses or
associated to
perinatal risk
factors
Glycogen stores
Glycogenesis
Glycogenolysis
Feeds
Amino acids
Fatty 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