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Newborn Baby Boy with Hypoglycemia and Idiopathic Neonatal Hepatitis Moina Uddin, D.O Endorama 12/5/13 Chief Complaint

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Newborn Baby Boy with Hypoglycemia and Idiopathic Neonatal Hepatitis Moina Uddin, D.O Endorama 12/5/13 Chief Complaint Newborn baby boy with hypoglycemia and idiopathic neonatal hepatitis Moina Uddin, D.O Endorama 12/5/13 Chief Complaint • 11 day old male neonate with persistent hypoglycemia HPI • Born FT, AGA, via c‐section • Pregnancy complicated by maternal diabetes, asthma and HTN, GBS + status • Meconium‐stained amniotic fluid perinatal stress • APGARS = 91, 95 • Initially to General Nursery HPI • POC glucose = 20 at 1 hour of life (26 on repeat) • POC glucose = 41 s/p feed • Needed continuous IVF until DOL 5, with highest GIR of 9.6 ml/kg/min to maintain POC glucose 60s‐70s. HPI • Repeated episodes of hypoglycemia after GIR was weaned ENDOCRINE CONSULT Additional History • Family Hx – Mother with GDM – Father with seizure disorder from birth until 7 yo, some staring spells – Paternal great aunt with seizure disorder – Half‐brother died at age 3 yo from esophageal rupture • Social Hx – Will live at home with both parents and 2 siblings Review of Systems • Constitutional: Negative • Genitourinary: Negative for fever. + appropriate • Skin: Negative weight • Neurological: Negative • Endocrine: Hypoglycemia for seizures • HENT: Negative • Hematological: • Respiratory: Negative Polycythemia, now • Cardiovascular: Negative resolved • Gastrointestinal: • Psychiatry: No agitation Hyperbilirubinemia or increased somnolence Physical Examination • Vitals: Wt: 3.425 (~40%ile), • CV: RRR, no murmurs, CR < Lt: 52 cm (~55%ile) 3 secs, 2+ femoral pulses Temp: 36.6‐36.8 C, HR: 140‐ • Pulmonary: CTAB, good 181, RR: 32‐51, BP: 68‐ aeration bilaterally, 75/42‐44 intermittent tachypnea, no nasal flaring • General: Well appearing, good grimace • Abdomen: Soft, ND, NT, No HSM, +BS • HEENT: No facial deformities, no cleft • GU: Normal phallus length palate, no nystagmus (3.5 cm), testes descended bilaterally, • Skin: Plethoric, sloughing skin all over, non‐icteric • Neuro: Normal tone, Equal DTRs, Nonfocal exam Differential diagnosis and assessment for this 11 day‐old with persistent hypoglycemia? Differential Diagnosis of Hypoglycemia • Hyperinsulinism • Defects in ketone body • Hormonal deficiency synthesis/utilization • Defects in hepatic • Metabolic conditions glycogen • Drug induced storage/release • Other‐ sepsis • Defects of fatty acid oxidation and carnitine metabolism Assessment and Plan • Hyperinsulinism • Treat with Diazoxide • Send HI genetic testing panel Additional history Further testing • CMV and Toxoplasma titers negative • Enterovirus culture negative • Liver biopsy: consistent with idiopathic neonatal hepatitis. – Giant cell hepatitis, few acidophil bodies sign of resolved viral infection – Dx: Idiopathic Neonatal Hepatitis Assessment and Plan • 1. Hyperinsulinism‐ • Diazoxide, Endo F/u Unknown etiology • 2. Low GH? • Observation • 3. Idiopathic neonatal • Actigall, GI F/u hepatitis Follow‐up History • Seen in GI clinic with improving labs and Actigall stopped • Repeated episodes of hypoglycemia on Diaxozide 15 mg/kg/day and q3 hr feeds READMISSION Hospital Course • Diazoxide increased to 20 mg/kg/day • Diuril began empirically • Feeds spaced and BG monitored qac q3 q4 q6 q6‐8 • 10 hour safety fast (BG checked at +6 hr, +8 hr, +9 hr and +10 hr) – Lowest POC BG: 78 – Serum BG: 91, insulin <2, ketones 1.17, FFA 1.18 Hospital Course Hospital Course Clinical Questions • Is there a single mechanism to explain hyperinsulinemia and cholestasis in our patient? • What is the clinical course of neonatal cholestasis due to growth hormone deficiency? • What is the expected GH level following hypoglycemia in a neonate? Clinical Questions • Is there a single mechanism to explain hyperinsulinemia and cholestasis in our patient? NO. Clinical Questions • What is the clinical course of neonatal cholestasis due to growth hormone deficiency? Spray CH, McKiernan P, Waldron KE, Shaw N, Kierk J, Kelly DA. Investigation and outcome of neonatal hepatitis in infants with hypopituitarism. Acta Paediatr 2000 89: 951‐4 Spray CH, McKiernan P, Waldron KE, Shaw N, Kierk J, Kelly DA. Investigation and outcome of neonatal hepatitis in infants with hypopituitarism. Acta Paediatr 2000 89: 951‐4 Clinical Questions • What is the expected GH level following hypoglycemia in a neonate? Poor Specificity of Low Growth Hormone and Cortisol Levels During Fasting Hypoglycemia for the Diagnoses of Growth Hormone Deficiency and Adrenal Insufficiency Andrea Kelly, MDa,b, Randy Tang, RN, BSN, BSEa, Susan Becker, BSN, RNa, Charles A. Stanley, MDa, Pediatrics 2008;122;e522 Poor Specificity of Low Growth Hormone and Cortisol Levels During Fasting Hypoglycemia for the Diagnoses of Growth Hormone Deficiency and Adrenal Insufficiency Andrea Kelly, MDa,b, Randy Tang, RN, BSN, BSEa, Susan Becker, BSN, RNa, Charles A. Stanley, MDa, Pediatrics 2008;122;e522 Senniappan S, Hussain K. An Evaluation of Growth Hormone and IGF‐1 Responses in Neonates with Hyperinsulinaemic Hypoglycaemia . Int J of Endocrinol 2013; Article ID 638257 Senniappan S, Hussain K. An Evaluation of Growth Hormone and IGF‐1 Responses in Neonates with Hyperinsulinaemic Hypoglycaemia . Int J of Endocrinol 2013; Article ID 638257 References • Choo Kang LR, Sun CC, Counts DR. Cholestatsis and hypoglycaemia: manifestations of congenital anterior hypopituitarism. J Clin Endocrinol Metab 1996; 81: 2786–9 • Senniappan S, Hussain K. An Evaluation of Growth Hormone and IGF‐1 Responses in Neonates with Hyperinsulinaemic Hypoglycaemia . Int J of Endocrinol 2013; Article ID 638257 • Spray CH, McKiernan P, Waldron KE, Shaw N, Kierk J, Kelly DA. Investigation and outcome of neonatal hepatitis in infants with hypopituitarism. Acta Paediatr 2000 89: 951‐4 • Kelly A, Tang R, Becker S, Stanley CA Poor Specificity of Low Growth Hormone and Cortisol Levels During Fasting Hypoglycemia for the Diagnoses of Growth Hormone Deficiency and Adrenal Insufficiency. Pediatrics 2008;122;e522 .
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