Endocrine Abstracts

Introduction: Effective management of the transition from childhood to adulthood in congenital adrenal hyperplasia (CAH) can reduce the problems that may arise in the follow-up during adulthood.

Aim: Evaluation of the clinical characteristics and sociodemographic data of CAH patients transferred from the Pediatric Endocrinology Clinic to the Adult department of our hospital and the comparison of the 2 transition models carried out during this period was the primary aim of this study.

Methods: Thirty-six patients who have been transferred from the pediatric endocrinology clinic to the adult endocrinology clinic between 2001 and 2022 were included in the study. Clinical data were obtained from the hospital medical records. Transition was carried out by two models. While Model 1 included one visit, Model 2 included two visits with an interval of 4-6 months and with participation of pediatric and adult endocrinologists at ‘Transition Outpatient Clinic’. Sociodemographic characteristics, frequency and duration of follow-up, clinical features, comorbidities (glucose metabolism disorder, dyslipidemia, TARTs, adrenal crisis,\.), treatment modalities, doses and treatment compliance were evaluated and compared according to the transfer model.

Results: Eighteen patients were raised as female and the remaining of them raised as male. Karyotype analysis revealed 46,XX in 47.2% of the patients and 46,XY in 22.2% of them. The mean age±SD of cases at transition time was 20.1±1.8 years (range;17.5-24.2). Consanguineous marriage rate among the parents of the patients was 55.6%. The CAH subtypes were 21 hydroxylase deficiency (61.1%),11 beta hydroxylase deficiency (25%), 17 hydroxylase deficiency (11.1%), and non-classical CAH (2.8%). Of the 36 patients, 19 (52%) were transferred using Model 1 and 17 (47%) using Model 2. There was no difference in terms of comorbidity such as hypertension, glucose metabolism disorder, TARTs, adrenal crisis frequency between the transfer models and also between pediatric and adult follow-up period. Obesity frequency was 22.2% before transition and it was 35.5% after transition. However low bone mineral density was detected in 16.7%(n=4) patients before and 56.3%(n=9) patients after transition (p:0.0088). Before transition the percentage of patients receiving hydrocortisone(33.3%) as glucocorticoid option was higher than that in post-transition period (9.7%) (p:0.0018). The hydrocortisone equivalent dose of steroid therapy was also higher before the transition (18.5 ±15.9 mg/m²/day vs 9.9±4.7 mg/m²/day, p:0.002). Pregnancy rate of the cohort was 16.7%.

Conclusion: The two transition models evaluated in our study seem to have no difference in terms of comorbidities and follow-up parameters. It may be more appropriate to compare models in large size cohorts.

Keywords: Congenital adrenal hyperplasia, transition, pediatrics