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

• Ano de defesa: 2006
• Autor(a) principal: Giovana Vignoli Cordeiro
• Tipo de documento: Dissertação
• Tipo de acesso: Acesso aberto
• Idioma: por
• Instituição de defesa: Universidade Federal de Minas Gerais; UFMG
• Palavras-chave em Português: Corticoterapia; Altura final; Deficiência de 21-hidroxilase; Tratamento precoce; Corticosteróides/uso terapêutico; Diagnóstico precoce; Hidrocortisona /uso terapêutico; Estatura; Resultado de tratamento; Adulto; Esteróide 21-hidroxilase; Hiperplasia supra-renal congênita/quimioterapia; Estudos retrospectivos; Fludrocortisona/uso terapêutico; Adolescente; Pediatria
• Link de acesso: http://hdl.handle.net/1843/ECJS-6Y6FQK

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.