Endocrine Abstracts

JOINT221

Background: The most common causes of persistent hypoglycemia Hyperinsulinism (PHHI) are genetic or congenital abnormalities in the regulation of insulin secretion, deficiencies in growth hormone and/or cortisol, or abnormalities in the metabolism of glucose, glycogen, and fatty acids. Congenital hyperinsulinism’s prevalence: 1 in 2700 in some consanguineous populations; 1 in 28,000 to 50,000 live births in the general population. According to our committee’s report, it can be difficult to identify neonates with PH issues because they can resemble transitional hypoglycemia within the first 48 hours. Furthermore, the first few months are crucial since 25% to 50% of children with congenital hyperinsulinism have developmental difficulties. For infants and young children, hypoglycemia is defined as blood glucose levels below 60 mg/dl/lthroughout the first 72 hours of life and beyond.

Case: This report aims to present a case of diffused type PHHI that was effectively treated with a near pancreatectomy and later identified with adrenal insufficiency. K, a 4-month-old girl, weight 5.9 kg, length 56 cm, was sent to Soetomo Hospital because she had hypoglycemia and frequent seizures since 3 days old. According to the family history, no member of family with diabetes. Ketone levels were 0.1 mmol/l, C.peptide levels were 3.3 ng/ml (1.1-4.4), lactate levels were 2.77 mmol/l(0.4-2.0), insulin levels were 16.8 μU/ml (4.04-23.46), FT4 levels were 1.63 ng/dl (0.9-1.75), TSH levels were 5.032 μIU/ml (0.64-6.27), GH levels were 1.35 ng/ml (0.14-6.27), cortisol levels were 1.8 μg/ml (45.5-208.2), taken when blood glucose 33 mg/dl. We evaluated a patient with wasting, adrenal insufficiency, and recurrent hypoglicemia. Due to the lack of diazoxide in Indonesia, the patient was treated with oral nifedipine (~2 mg/kg/day), SC octreotide (~5 mg/kg/day), and oral prednisone (~6 mg/kg/day), along with a glucose infusion rate up to 12 (formula milk added to IV fluids via a central line). After a month, the treatment failed, and we referred to pediatric surgery for pancreatectomy. Preoperative labs were normal, and abdominal CT showed no mass. We want to conduct a genetic test to determine the type of hypoglycemia, but the family refuses. Seven days post-pancreatectomy, the patient was ready for discharge but had morning hypoglycemia. We prescribed nifedipine 1 mg/kg/day orally to stabilize. Early recognition and treatment are crucial for preventing these sequelae.

Conclussion: Patients PHHI who cannot control their hypoglycemia with medication may need a near-total pancreatectomy; nevertheless, this could lead to diabetes mellitus or recurrent hypoglycemia after surgery.