CASE PRESENTATION: He was born full term and appropriate for gestational age. He was referred to endocrinology at 3.5 years of age for short stature with a height SDS of -2.48.
IGF-1 51.1 ng/mL and
IGFBP-3 1.2 ng/mL were low. GH stimulation test noted baseline and peak GH of 0.1 ng/mL. MRI brain showed hypoplastic adenohypophysis, aplastic pituitary stalk, and ectopic neurohypophysis. There had been difficulty with adherence to daily GH over the following 9 years. BMI trajectory rose above 180% of the 95th percentile. By age 12, A1c was 6.6%.
Metformin was started and increased to 1,000 mg twice daily. Subsequent A1c was 6.0%. Due to poor compliance with daily GH, at 12 years and 9 months, he was initiated on 22 mg (0.25 mg/kg/week) of weekly
lonapegsomatropin-tcgd to improve compliance. The day after his first injection, he developed non-bloody, non-bilious
emesis. He denied
headaches and endorsed
polyuria. Due to concern for increased intracranial pressure, he was sent to the emergency department; however, ophthalmologic exam was negative. Initial serum
glucose was 500 mg/dL, then 336 mg/dL after 1-L
normal saline.
Hemoglobin A1c was 5.7%, urine
glucose 3+ mg/dL, and urine
ketones 2+ mg/dL. Venous pH of 7.379 and
bicarbonate of 20.6 mmol/L ruled out
diabetic ketoacidosis.
Metformin was held during the hospitalization.
Hyperglycemia rapidly improved with transient
insulin administration. He received one dose of
glargine 20 units. He was initiated on
lispro carb ratio of 1:8 and correction factor 1:15 for target
glucose 150 mg/dL. By day four, glucoses were below 100 mg/dL;
lispro was discontinued, and he was discharged home. Weekly GH was discontinued with plans to resume daily GH
therapy in several months.
CONCLUSION: