青春期延迟
医学
人体测量学
性早熟
人口
内分泌学
初潮
超重
身材矮小
内科学
肾上腺素
发病年龄
肥胖
骨龄
年轻人
儿科
激素
疾病
环境卫生
作者
Raíssa Rezende,Renata Maria de Noronha,Ana Keselman,Elisangela P S Quedas,Naiara Castelo Branco Dantas,Nathalia Liberatoscioli Menezes De Andrade,Débora Romeo Bertola,Alexsandra C. Malaquias,Alexander A L Jorge
摘要
Pubertal delay is described as one of the clinical features in Noonan Syndrome (NS) and it may be one of the factors causing short adult height in those patients. The present study aims at characterizing pubertal development in NS and identifying pubertal delay predictors.We analyzed 133 individuals with a molecular diagnosis of NS and clinical puberty evaluation. We characterized delayed puberty as pubertal onset after 12 years in girls and 13.5 years in boys, according to parameters of the Brazilian population. To investigate its predictors, we correlated the age at onset of puberty with several characteristics and genotype in a multilevel regression model. For comprehending pubertal development in NS, we assessed age and anthropometric measures at each Tanner stage and adult age.The mean age at puberty onset for girls was 11.9±1.9 years and for boys, 12.5±1.7 years, significantly later than the Brazilian population (p=0.025; p<0.001). Girls (49.1%) presented delayed puberty more frequently than boys (27.9%, p=0.031). BMI SDS and IGF1 SDS at puberty onset significantly predicted later puberty entry. Height gain from the onset of puberty to adult height was lower in children with pubertal delay.Pubertal delay is characteristically found in children with NS, more frequently in females. The low weight of patients with NS could modulate the age of puberty, just as the increase in overweight/obesity in the general population has shown an effect on reducing the age of onset of puberty.
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