Abstract

1. Jocelyn K. Lambuth, MSc, MD* 2. Hector F. Lantigua, MD† 3. Monaliza S. Evangelista, MD‡ 4. Amanda Tchakarov, MD§ 5. Avni Shah, MD† 1. *McGovern Medical School, University of Texas Health Science Center at Houston, Houston, TX 2. †Division of Endocrinology, Department of Pediatrics, 3. ‡Division of Community and General Pediatrics, Department of Pediatrics, and 4. §Department of Pathology and Laboratory Medicine, McGovern Medical School at the University of Texas Health Science Center at Houston, Houston, TX A 17-year-old girl presents with recurrent episodes of sleepiness. Her mother brought her to the emergency department after she was difficult to awaken from sleep. She has had recurrent symptoms of fatigue, difficult arousability, tremulousness, and diaphoresis for 5 months and experiences mild symptoms daily, which are alleviated by eating. In addition, she has had recent weight loss of 4 lb (1.8 kg), headache, and irregular menstruation. The remainder of her medical and family history is normal. On hospital admission her vital signs are normal. Growth parameters include weight of 103.4 lb (46.9 kg), height of 60 in (152 cm), and BMI of 20.3 (BMI Z -score, –0.3); physical examination findings are normal, and no unusual skin findings are seen. Her initial serum glucose level is 24 mg/dL (1.33 mmol/L), and dextrose-containing intravenous fluids are started. Pediatric endocrinology is consulted to aid in investigation and management. On further questioning, she reports a previous evaluation in another hospital for these episodes. During her admission she is found to have a fingerstick glucose level of 21 mg/dL (1.17 mmol/L) and receives 2 ampules of dextrose 50% due to recurrent hypoglycemia. She is admitted to the PICU for further monitoring. During a hypoglycemic event, her serum glucose level was 41 mg/dL (2.28 mmol/L), β-hydroxybutyrate level, 2.1 mg/dL; C-peptide level, 3.6 ng/mL; and serum insulin level, 16.6 …

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