Background: Data on congenital isolated growth hormone deficiency (cIGHD), mostly due to consanguinity, treated in childhood and followed into adult age is very rare and on few patients.
Aim: To assess the clinical and social characteristics of adults with cIGHD who were treated in childhood and followed thereafter.
Subjects: Thirty nine patients with cIGHD from our clinic were followed into adult age (mean age 30.7±13.3). All were treated by hGH in childhood. Starting age was 7±4.2 years and duration was 218 years. Out of the cohort of 39 patients, ascertained detailed data was found for 32 patients.
Methods: Data was collected from medical records of our endocrine clinics. The study was approved by the Hospital Ethics committee.
Results: Mean (±S.D.) height for the males is 160.2±10.6 cm, for the females 146.4±5.4 cm. Twenty two patients have an education of high school or higher and 2 are in special institutions. Most are employed in manual labor. All have full sexual development and 14 are married. After cessation of GH treatment and with advancing age all have progressive increase in adiposity to the degree of obesity as revealed by high skinfold thickness and missed by BMI. Twelve patients suffer from hyperlipidemia, four developed diabetes mellitus, and five have cardiovascular diseases. One patient died. None developed cancer.
Conclusion: Patients with congenital IGHD who do not receive early and regular replacement treatment are prone to lag in achieving normal height and suffer from educational and vocational handicaps. They are protected from cancer.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology