ESPE2024 Poster Category 3 Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology (20 abstracts)
Comparative Analysis of Growth and Hormonal Parameters in Pediatric Patients with 11-Beta-Hydroxylase Deficiency (11OHD) vs. 21-Hydroxylase Deficiency (21OHD)
Dina Diab 1 , Mohammed Alaa El-Din Thabet 2 , Iman Marzouq 3 , Dalia Elneely 4 , Ashraf Tawfeeq Soliman 5 , Ahmed Elawwa 1 & Shymaa Elsayed 1
1Pediatic Endocrinology and Diabetes Department, Alexandria University Children's Hospital, Alexandria, Egypt. 2Pediatric Department, Alexandria University Children's Hospital, Alexandria, Egypt. 3Genetic Department, Alexandria University Children's Hospital, Alexandria, Egypt. 4Clinical and Chemical Pathology Department, Alexandria University Children's Hospital, Alexandria, Egypt. 5Hamad University, Doha, Qatar
Background: Congenital Adrenal Hyperplasia (CAH) is characterized by various enzyme deficiencies, notably 11-Beta-Hydroxylase Deficiency (11OHD) and 21-Hydroxylase Deficiency (21OHD), each affecting growth and hormonal levels differently. This study aim ed to compare the anthropometric and hormonal profiles of pediatric patients affected by these two conditions.
Methods: The clinical records of pediatric patients diagnosed with 11OHD (n = 18) and 21OHD (n = 54) were retrospectively reviewed. Parameters such as Weight Standard deviation for age and sex (WtSd), Height Standard deviation for age and sex (HtSd), BMI Standard deviation for age and sex (BMIZ), Bone Age Standard Deviation, hydrocortisone dosage, and last measured levels of 17-hydroxyprogesterone (17OHP) and adrenocorticotropic hormone (ACTH) were analyzed for 11 patients with 11OHD and 55 with 21OHD.
Conclusion: The comparative analysis revealed that pediatric patients with 11OHD have a different growth and hormonal profile compared to those with 21OHD. Specifically, 11OHD patients are better off in terms of weight and BMI for their age, despite requiring a lower dose of hydrocortisone. However, they exhibit higher variability in bone age and ACTH levels, suggesting that clinical management should be tailored to address these differences.
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