Endocrine Abstracts

Background: Hypopituitarism is a condition caused by deficiency in one or multiple pituitary hormones. The disease is associated with various metabolic disorders and decreased quality of life that are particularly marked in patients with childhood onset of the disorder. There are manyfactors influencing the metabolic status of patients such as different treatment modalities (surgery, radiotherapy, chemotherapy) and current supplementation (e.g. steroid or testosterone/estradiol substitution)which should be taken into consideration. This specific group of patients requires an extensive care because of increased risk of metabolic abnormalities.

Aim: To present the metabolic characteristic of patients with childhood onset of hypopituitarism on the basis of long term observation in the pediatric/adult endocrinology departments of our university.

Methods: We analyzed retrospectively current complete data of 35/75 patients with childhood onset of pituitary dysfunctions (13 W/22 M). 28/75 patients with PROP-1 mutation were excluded from this study due to the separate analysis. Etiologically, congenital malformations were found in 13/35 of patients; 10/31 were treated for craniopharyngioma, 3/35 for histiocytosis, 2/35 for germinoma. The mean age of diagnosis was 9.18 years (s.d. 4.8).

Results: 29/35 of patients presented with somatotropic axis deficiency. Deficits in gonadal, thyroid and adrenal hormones were detected in 26/35, 25/35 and 18/35 of patients, respectively. Body weight abnormalities were found in 24/35 of patients (obesity in 9/35 of patients, overweight in 10/35 of cases and underweight in 5/35 of them). Extreme values were observed in patients operated for craniopharyngioma (cachectic patient with BMI 16,9 and obese one with BMI 46,5). Systolic and diastolic blood pressure were higher in 6/35 and 8/35 cases, respectively. 18/35 of patients were diagnosed with various lipid disturbances (among them 15/25 of patients with central hypothyroidism and 15/29 with somatotropic pituitary dysfunction). Kidney function was preserved in all patients. 8/35 of patients had at least one liver enzyme elevated. Vitamin D deficit was found in 12/35 of cases. Oral glucose tolerance test (performed in 26/35 of patients) revealed diabetes in 1 case, impaired glucose tolerance in 3 patients (all of them had secondary hypoadrenalism and were treated with hydrocortisone) and confirmed insulin resistance (IR) in 14/26. The highest incidence of IR were observed in patients with history of craniopharyngioma (6/7) and histiocytosis (2/2).

Conclusions: Majority of patients with childhood onset of hypopituitarism characterizes with at least one metabolic dysfunction. Proper management of hormonal abnormalities and early administration of treatment might preserve these patients from serious consequences.