Endocrine Abstracts

Introduction: Diabetes mellitus is the most common chronic disease in children, with type 1 diabetes accounting for 80% of diabetic patients under the age of 19 years. Though hyperglycemia in pediatric patients with diabetes mellitus commonly occurs due to poor adherence to insulin therapy, other etiologies should be considered when hyperglycemia is refractory and additional systemic signs and symptoms are present. We discuss a case of a female patient presenting with signs and symptoms of elevated cortisol levels several years after being diagnosed with T1DM.

Case Presentation: We present the case of a 19-year-old woman diagnosed with type 1 diabetes mellitus and celiac disease at 3 years and 9 months. Come to our consultation after follow-up in Pediatric Endocrinology for follow-up of type 1 DM. Screening for Cushing’s syndrome is requested due to reports of increased insulin needs, weight gain, rounder face and menstrual alterations since 2020. On examination, attention is drawn to facial plethora, a full moon face, vinous red striae on the abdomen and accumulation of retrocervical and supraclavicular fat. A functionality study was performed (Suppression with 1 mg of Dexamethasone, urinary free cortisol, salivary cortisol, cortisol rhythm, strong suppression with Dexamethasone and CRH test) suggestive of ACTH-dependent syndrome. A subsequent brain MRI showed pituitary gland with an increase in size of 9.9×8.6 mm in the sagittal plane suggestive of pituitary adenoma. Normal appearing pituitary stalk and optic chiasm. Medical treatment with ketoconazole was initiated with improvement in glycemic control and normalization of urinary free cortisol until complete transsphenoidal resection of the adenoma was performed. Following resection her long-acting insulin requirement decreased by 50%. At his three-month postoperative follow-up, the patient’s striae and facial edema had improved significantly and her weight decreased by 9 kg.

Conclusions: We described the case of a 19-year-old Spanish female, with poorly controlled T1DM and Cushingoid features, who was found to have a functioning pituitary macroadenoma. An ACTH-secreting pituitary adenoma is an extremely rare phenomenon in such young patients, but it should be considered in patients presenting with refractory hyperglycemia and other signs and symptoms of Cushing disease.