Elizabeth Schierholz, MSN, NNP ❍ Section Editor

Case of the Month

Congenital Hyperinsulinemia With Grade 4 Intraventricular Hemorrhage A Case Report With a 2-Year Follow-up Jahanara Begum-Hasan, MD, PhD

ABSTRACT Massive intraventricular hemorrhage associated with hypoglycemia has rarely been reported. We present a late preterm baby girl with severe hypoglycemia after birth. Despite high glucose infusion rates, her glucose levels remained in the 30s to 50s (mg/dL) during the first week of life with a brief period of normoglycemia. On day 2, her computed tomography scan of head showed extensive intraventricular hemorrhage with intraparenchymal extension, which was reported as unexpected for an infant born close to term. She was diagnosed with congenital hyperinsulinemia. Her glucose levels normalized on diazoxide therapy started on day 6. In the absence of any other clear cause, the massive brain hemorrhage was thought to be secondary to prolonged severe hypoglycemia the infant suffered. She remains in global developmental delay at her age at 26 months. This case highlights the importance of early referral of neonatal hypoglycemia for prompt diagnosis and management to avoid the consequences of prolonged low blood glucose. Key Words: diazoxide, hypoglycemia, intracranial bleeding, seizure

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ypoglycemia is a common metabolic problem in the nursery and rarely requires endocrine consultation. Although many clinical effects of neonatal hypoglycemia have been reported,1-3 massive intraventricular hemorrhage (IVH) in late preterm or term babies with persistently low blood glucose has rarely been documented in the literature. Intraventricular hemorrhage is more commonly described in preterm neonates.4,5 The differential diagnosis of neonatal hypoglycemia is very broad.6 Reaching a definitive diagnosis can be challenging, particularly in cases of congenital hyperinsulinemia

Author Affiliation: Department of Pediatrics, Carilion Clinic Children’s Hospital, and Virginia Tech Carilion School of Medicine, Roanoke, Virginia. The author declares no conflict of interest. Correspondence: Jahanara Begum-Hasan, MD, PhD, Virginia Tech Carilion School of Medicine, 102 Highland Ave, Ste 301, Roanoke, VA 24013 (jxbegumhasan@ carilionclinic.org). Copyright © 2014 by The National Association of Neonatal Nurses DOI: 10.1097/ANC.0000000000000137 392

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(CH).7,8 The delay in diagnosis and management can have long-term adverse consequences. A case of neonatal hypoglycemia associated with grade 4 IVH is reported here. The purpose of this report is to increase awareness of an atypical presentation of severe hypoglycemia and to emphasize the importance of prompt diagnosis and management of neonatal hypoglycemia to avoid significant morbidity and mortality.

CASE REPORT T. K. is a baby girl who was born vaginally at the gestational age of 36 weeks and 5 days to a healthy 27-year-old mother with no history of gestational diabetes. Other than the precipitous labor (

Congenital hyperinsulinemia with grade 4 intraventricular hemorrhage: a case report with a 2-year follow-up.

Massive intraventricular hemorrhage associated with hypoglycemia has rarely been reported. We present a late preterm baby girl with severe hypoglycemi...
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