Avaliação da altura final de pacientes com Hiperplasia adrenal congênita por deficiência de 21 hidroxilase

Abstract

The aim of this study was to evaluate the final height (FH) and associated factors of 31 children with classical congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency who were regularly followed at the Hospital das Clínicas of the Federal University of Minas Gerais. The median age at the begining of the treatment was 2 years and 3 months and they were followed-up for 10.28 ± 4.2 years until they reached their FH. Twenty six patients were female and 17 presented the salt-wasting form. Twenty six received daily oral hydrocortisone acetate (Hc) in a median dose of 13.7mg/m². We calculated the Z score for height related to chronological age (HAZ), bone age (BA) and target height according to the NCHS data. The initial HAZ was 0.12 ± 2.6 and for the FH (FHZ) 0.64 ± 1.45, without significant difference according to sex, treatment compliance and clinical forms. Patients with known target heights (n=20) showed FHZ of -0.62 ± 1.39, with no diference to Z score for the target height (THZ) which was -1.25 ± 0.75 (p=0.06). Patients who had wrist Rx in their first year of treatment (n=15) showed advanced BA. The Z score for BA (BAZ) was 2.04 ± 1.31, lower than the HAZ of 0.69 ± 2.57 (p=0.00025). Morever this their FHZ score was -0.92 ± 1.62, significantly higher than the BAZ at the beginning of the treatment (p=0.02). Children who were treated in their first two years of life (n=17) showed catch-up growth during follow-up. They presented HAZ of 1.38 ± 2.3 at 2 years of CA and the FHZ of 0.341 ± 1.26 (p = 36 0.04). Children who received a mean Hc dose lower than 15 mg/m²/d achived better FH results (FHZ = -0.19 ± 1.45) as compared to those who used higher doses more than 15 mg/m²/d (FHZ =1.45 ± 1.18), p=0.018. There was a moderate but significant negative correlation between the Hc dose and the FH (r=-0.51; p<0.05). Patients with a poor hormonal control (17-OH progesterone and androstenedione levels 6 times higher than the reference values) showed no FH differences when compared to those with a satisfactory control. We conclude that CAH patients achieved a satisfactory FH, higher than their familiar target and that early treatment as well as lower Hc doses allowed for the better results concerning the final stature.