Evaluation of growth, laboratorial control and corticotherapy in a sample of patients with the classical form of 21-hydroxylase deficiency

Author(s)

Clarissa Cerchi A. Ramos ^[1] ; Lucas Ricci Bento ^[2] ; Ezequiel Moreira Gonçalves ^[3] ; Maricilda Palandi de Mello ^[4] ; Maria Tereza M. Baptista ^[5] ; Sofia Helena V. de Lemos-Marini ^[6] ; Gil Guerra-Júnior ^[7]

Affiliation

^[1] Universidade Estadual de Campinas. Faculdade de Ciências Médicas - Brasil

^[2] Universidade Estadual de Campinas. Faculdade de Ciências Médicas - Brasil

^[3] FCM - Brasil

^[4] Centro de Biologia Molecular e Engenharia Genética da Unicamp - Brasil

^[5] FCM. Departamento de Clínica Médica - Brasil

^[6] Unicamp. Departamento de Pediatria - Brasil

^[7] Unicamp. FCM. Departamento de Pediatria - Brasil

• Document type: Journal article
• Source: Revista Paulista de Pediatria; v. 25, n. 4, p. 317-323, 2007-12-00.

Abstract

OBJECTIVE: To verify the growth pattern of patients with congenital adrenal hyperplasia (CAH) due to classical 21hydroxylase (21-OH) deficiency in relation to hormonal control and use of corticoid during the treatment. METHODS: Retrospective analysis of data from 45 patients. The growth pattern was analyzed according to height gain or not, using the difference between height Z score (for bone age) at the last visit in relation to the height Z score (for chronological age) in the first visit. Concentrations of 17-OH progesterone (17-OHP), androstenedione and rennin were evaluated, and the patients were considered well-controlled when 50% or more of the dosages were normal. Corticoid therapy was analyzed according to type and dose. RESULTS: The age at last visit ranged from 2.8 to 26.6 years (12.6+5.8 years), 31 were females, 30 with salt wasting form; 62% were considered well-controlled for 17-OHP, 75% for androstenedione and 78% for renin. Hidrocortisone was used in 41 patients (20.2+2.6 mg/m²/day) and 40 in association with 9a-fludrocortisone. There were 14 patients with height gain, 20 with maintenance and 11 with loss. Height gain was associated with salt wasting patients (p=0.01) and with patients well-controlled for 17OHP (p=0,0005) and androstenedione (p=0,02). CONCLUSIONS: In this sample of patients with CAH due to classical 21-OH deficiency, better height gain was associated with a good control of 17-OHP and androstenedione and with salt wasting clinical form of the disease. (AU)

• FAPESP's process: 06/01978-0 - Classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency: effect of hyperandrogenism in female proportion and composition
• Grantee: Ezequiel Moreira Gonçalves
• Support Opportunities: Scholarships in Brazil - Master