Background: Cushing syndrome is very rare in childhood and adolescence and often occurs with iatrogenic causes. The major cause of endogenous Cushing syndrome is Cushings disease, which results due to excessive ACTH secretion from pituitary cells (corticotroph adenoma).
Objective and hypothesis: Cushing syndrome cases, which presented with diabetic ketoacidosis (DKA) in adulthood have been rarely reported. However, to our knowledge, there is no report of a pediatric case of Cushings disease presenting with DKA.
Methods: A 16-year-old girl suffering from polyuria and polydipsia for the last 2 weeks, was admitted with menstrual irregularity and oral moniliasis. Due to her hyperglycemia (venous glucose 556 mg/dl), ketonuria, glucosuria, and metabolic acidosis on arterial blood gas, she was diagnosed with DKA.
Results: Her weight was 95 kg (+2.89 S.D.), height was 156 cm (−1.03 S.D.), and BMI was 36.98 kg/m2 (+3.30 S.D.), with a normal blood pressure. On physical examination; bilateral exophthalmos, oral moniliasis, acanthosis nigricans, moon face, central obesity and striae were detected. Her HbA1c level was 10.4%, C-peptide level was 11.4 ng/ml (0.97.1 ng/ml) and diabetes autoantibodies were negative. Her serum cortisol levels at 0800 and 2300 h were high (respectively 31.7 and 26.3 μg/dl), ACTH level was 46.7 pg/ml, and 24-h urinary free cortisol excretion level was 151 μg/m2 per day (n<70 μg/m2 per day). Based on the results of overnight and low-dose dexamethasone suppression tests, which both failed to suppress endogenous cortisol secretion, the patient was diagnosed with Cushing syndrome. In high-dose dexamethasone suppression test, free urinary cortisol excretion rate was suppressed by 80%, and a pituitary MRI revealed a 4 mm microadenoma thus she was diagnosed as Cushings disease.
Conclusion: Although very rare in childhood, Cushing syndrome should be kept in mind in pediatric patients with obesity, acanthosis nigricans, moniliasis and striae. Diabetic ketoacidosis may be the presenting clinical picture in patients with impaired glucose metabolism.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology