الفهرس | Only 14 pages are availabe for public view |
Abstract Introduction: Congenital adrenal hyperplasia (CAH) encompasses a group of inherited autosomal recessive diseases affecting adrenal steroid synthesis. The impaired cortisol secretion causes ACTH levels to rise and stimulate adrenocortical hormone secretion, resulting in adrenal hyperplasia, and increased production. Aim of the Work: To evaluate echo-cardio graphic functions in infants with congenital adrenal hyperplasia at baseline before starting glucocorticoid therapy. Patients and Methods: This is a prospective case-control study that included thirteen patients diagnosed as having congenital adrenal hyperplasia and are following-up regularly at the Pediatric Endocrinology Clinic Children Hospital, Ain-Shams University during the period from November 2014 to April 2016. Results: This case-control study was conducted on thirteen congenital adrenal hyperplasia pediatric patients due to 21-OHase deficiency. All patients were diagnosed based on clinical and laboratory basis and are following-up regularly at the Pediatric Endocrinology Clinic, Children’s Hospital, Ain-Shams University. They included 8(61.5%) females (46 XX) and 5 (38.5%) males (46XY), their ages ranged between 0.05 –0.7years, with a median (IQR) of 0.14 (0.08 – 0.35) years. Patients were compared with 13 healthy age- and sex-matched controls for evaluation of echocardiographic measurements. Conclusion: Blood pressure levels are substantially low in CAH patients at base line before starting glucocorticoid therapy. Patients with classical CAH had an increased risk for developing myocardial dysfunction and subclinical atherosclerosis. Recommendations: Measuring blood pressure regularly with a careful anthropometric examination in the clinical setting for patients with congenital adrenal hyperplasia (CAH) are mandatory points for good control of these patients. |