ESPE Abstracts (2019) 92 P2-78

ESPE2019 Poster Category 2 Diabetes and Insulin (43 abstracts)

A Case of an Infant with Congenital Hyperinsulinism Complicated by Diabetic Ketoacidosis during Treatment

Chaeri Yoo , Seonkyeong Rhie , Eun-Gyong Yoo & Mo Kyung Jung


CHA Bundang Medical Center, Gyeonggi-do, Korea, Republic of


Background: Congenital hyperinsulinism (CHI) is a disorder causing persistent hypoglycemia due to oversecretion of insulin. Diazoxide, a KATP channel opener in pancreatic beta cells is the treatment of choice, however, the glucose level should be monitored carefully. We report here a case of an infant girl with CHI who was complicated by diabetic ketoacidosis (DKA) during acute febrile illness.

Case Report: A 15–month-old girl visited emergency room with recurrent seizures. She had a history of recurrent afebrile seizures since 4 months of age. She had no specific birth history or family history. On physical examination, her weight was 10.5kg (50-75 percentile) and height was 78.5cm (50-75 percentile). She also showed developmental delay, however, her tandem mass screening test, thyroid function test, and brain MRI were all normal. Hypoglycemia (serum glucose < 30 mg/dL) was noted, but serum insulin (8.36 µU/mL) and C-peptide (1.97 ng/mL) levels were inappropriately high. On glucagon stimulation test, blood glucose level increased from 38 mg/dL to 76 mg/dL, and serum insulin level was 7.6 and 4.4 µU/mL before and after glucagon stimulation, respectively. Abdominal ultrasonography was normal. Genetic study including ABCC8, KCNJ11, GLUD1, HNF4A, GCK, HADH, and UCP2 was all negative. Diazoxide (3mg/kg/day) was started under the diagnosis of CHI, and her self-monitored blood glucose level was well-controlled on follow up visit after discharge. However, after 4 weeks, she visited emergency center again with 2 days of fever and hyperglycemia. The serum glucose was 398 mg/dL with pH 7.293, HCO3 7.9 mmol/L, and positive serum ketone, which was consistent with DKA. Continuous intravenous insulin infusion was initiated with the rate of 0.05 IU/hr/kg. After recovery from DKA, her glucose level was well-controlled with the same dosage of diazoxide. She is 24 months old at present, and her developmental milestone has also been dramatically improved after diazoxide treatment.

Volume 92

58th Annual ESPE

Vienna, Austria
19 Sep 2019 - 21 Sep 2019

European Society for Paediatric Endocrinology 

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