Congenital adrenal hyperplasia due to 21-hydroxylase deficiency: final Height in 27 patients with the classical form

Autor: S. H. V. Lemos-Marini, André Moreno Morcillo, Gil Guerra-Júnior, Andréa Trevas Maciel-Guerra, Maria Tereza Matias Baptista, Luciana de Oliveira Silva
Jazyk: portugalština
Rok vydání: 2005
Předmět:
Zdroj: Arquivos Brasileiros de Endocrinologia & Metabologia v.49 n.6 2005
Arquivos Brasileiros de Endocrinologia & Metabologia
Sociedade Brasileira de Endocrinologia e Metabologia (SBEM)
instacron:SBEM
Arquivos Brasileiros de Endocrinologia & Metabologia, Volume: 49, Issue: 6, Pages: 902-907, Published: DEC 2005
Popis: OBJETIVO: Determinar a altura final (AF) de pacientes com hiperplasia supra-renal congênita e investigar fatores de melhor prognóstico de altura. METODOLOGIA: Estudamos 13 pacientes perdedores de sal (PS) e 14 virilizantes simples (VS). AF e altura-alvo (AA) foram transformadas em escores z. Os dados foram analisados de acordo com sexo, variante clínica, idade do início do tratamento e duração do tratamento até AF. RESULTADOS: O zAF (n= 27) foi -1,57 ± 1,01. Houve diferença entre AF (-1,50 ± 1,03) e AA (-0,78 ± 0,84) (n= 25, p< 0,001). Não houve diferença quanto a sexo, variante e início do tratamento, embora PS e pacientes com terapia precoce tivessem tendência a melhor AF; houve diferença (p= 0,018) entre a estatura de pacientes que atingiram a AF com menos de 5 anos de tratamento (-2,49 ± 1,03) em relação àqueles tratados por mais de 10 anos (-1,21 ± 0,88). CONCLUSÃO: Houve comprometimento na AF, e melhor prognóstico parece depender principalmente de diagnóstico e tratamento precoces. OBJECTIVE: To determine final height (FH) in congenital adrenal hyperplasia (CAH) patients and investigate conditions allowing better height outcome. METHODOLOGY: 13 salt-wasting (SW) and 14 simple virilizing (SV) patients were studied. FH and target height (TH) were transformed into standard deviation score (z). Data were analyzed according to sex, clinical form, age at treatment onset and length of treatment before attaining FH. RESULTS: zFH (n= 27) was -1.57 ± 1.01. FH (-1.50 ± 1.03) was below TH (-0.78 ± 0.84) (n= 25, p< 0.001). FH has not differed according to sex, clinical form and age at therapy onset although there was a trend towards better FH in SW patients and in early treated cases; there was significant difference (p= 0.018) between patients treated for less than 5 years (-2.49 ± 1.03) and those accompanied longer than 10 years (-1.21 ± 0.88) before attaining FH. CONCLUSIONS: There was a FH impairment and adult height improvement seems to depend mainly on early diagnosis and treatment.
Databáze: OpenAIRE