Background: Diagnosis and treatment of Cushings disease in children are challenging.
Objective and hypotheses: Cabergolin is a long acting dopamine receptor agonist used for the treatment of patients with recurrent Cushings disease.
Method: year-old female patient admitted to the hospital because of short stature, amenorrhea, facial and body hair growth, rapid weight gain, hair loss and excessive acne.
Results: Her birth weight was 3650 g, neuromotor development was normal. At the time of diagnosis her weight was 50 kg (−0.7 SDS), height 138 cm (−4.0 SDS), BMI 26.2 kg/m2 (+1.7 SDS) and she was hypertensive. She was Tanner stage 5 with Cushingoid appearance. She was diagnosed as Cushings disease with laboratory and clinical findings. MRI detected no pituitary adenoma. ACTH hypersecretion and lateralization to the left was found by petrosal sinus sampling. Endoscopic transnasal left hemi-hypophysectomy was performed. The operation was considered to have failed due to excessive bleeding in the pituitary. Although postoperatively hypercortisolemia persisted clinical findings for the first three months after surgery did not progress and the patient lost weight, markers of insulin resistance regressed. On the 6th month of her operation her following, clinical and laboratory evidences showed to relapse and a second transnasal left hemi-hypophysectomy was performed. Although diurnal cortisol rhythm was damaged after the second operation 24-hour urinary free cortisol was 70 μg/day (normal). 15 months after second surgery Cushings disease relapsed clinically and biochemically. Because of the mortality and morbidity risk of the third operation. Cabergoline 1 mg/week was initiated. The patient responded to cabergoline treatment with normal urinary free cortisol and improve clinical findings on the 9th month of treatment.
Conclusion: Cabergoline is effective in the control of cortisol secretion in the treatment of recurrent Cushings disease.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology