Siemensma Elbrich P C, de Lind van Wijngaarden Roderick F A, Otten Barto J, de Jong Frank H, Hokken-Koelega Anita C S
Dutch Growth Research Foundation, RotterdamDepartment of Pediatrics, Division of Endocrinology, Erasmus University Medical Center Rotterdam/Sophia's Children's Hospital, RotterdamDepartment of Pediatric Endocrinology, Radboud University Medical Center, NijmegenDepartment of Internal Medicine, Erasmus University Medical Center, Rotterdam, the Netherlands.
Clin Endocrinol (Oxf). 2011 Jul;75(1):83-9. doi: 10.1111/j.1365-2265.2011.03989.x.
Premature pubarche (PP) is reported in children with Prader-Willi Syndrome (PWS). Pubarche is preceded by adrenarche - an increase in serum levels of adrenal androgens, most specifically dehydroepiandrosterone sulphate (DHEAS).
To assess DHEAS levels, the age at and progression of pubarche and the prevalence of PP in children with PWS.
DESIGN/PATIENTS: In the Dutch PWS Cohort Study, 120 children (6 months-17 years) are prospectively followed. Their age at onset of pubarche and various pubic hair stages and prevalence of PP were determined. Serum DHEAS levels were assessed in 97 children.
Median serum DHEAS levels were significantly higher in children with PWS than in healthy age-matched controls at ages 3-6 years (girls: P = 0·004 and boys: P = 0·010) and 6-10 years (girls: P = 0·045 and boys: P = 0·001). Age and gender significantly influenced DHEAS levels in children with PWS. The median [P10-P90] age at onset of pubarche in children with PWS was significantly younger than in healthy peers, 9·04[6·75-11·84] years in PWS girls (P < 0·0001) and 10·31 [8·65-12·29] years in PWS boys (P = 0·003). The prevalence of PP in children with PWS was 30·0% in girls and 16·1% in boys.
Compared to healthy children, children with PWS have significantly higher DHEAS levels from 3 to 10 years of age. They are younger at onset of pubarche and have a higher prevalence of premature pubarche. DHEAS levels in PWS are influenced by age and gender. Our findings indicate earlier maturation of the zona reticularis of the adrenal glands in children with PWS.
普拉德-威利综合征（PWS）患儿中报道有青春期阴毛早现（PP）。青春期阴毛早现之前会出现肾上腺功能初现——血清肾上腺雄激素水平升高，最显著的是硫酸脱氢表雄酮（DHEAS）。
评估PWS患儿的DHEAS水平、青春期阴毛早现的年龄及进展情况以及PP的患病率。
设计/患者：在荷兰PWS队列研究中，对120名儿童（6个月至17岁）进行前瞻性随访。确定了他们青春期阴毛早现的年龄、不同的阴毛阶段以及PP的患病率。对97名儿童评估了血清DHEAS水平。
在3至6岁（女孩：P = 0.004，男孩：P = 0.010）以及6至10岁（女孩：P = 0.045，男孩：P = 0.001）时，PWS患儿的血清DHEAS水平中位数显著高于年龄匹配的健康对照。年龄和性别对PWS患儿的DHEAS水平有显著影响。PWS患儿青春期阴毛早现的中位年龄[第10百分位数-第90百分位数]显著低于健康同龄人，PWS女孩为9.04[6.75 - 11.84]岁（P < 0.0001），PWS男孩为10.31[8.65 - 12.29]岁（P = 0.003）。PWS患儿中PP的患病率女孩为30.0%，男孩为16.1%。
与健康儿童相比，PWS患儿在3至10岁时DHEAS水平显著更高。他们青春期阴毛早现的年龄更小，青春期阴毛早现的患病率更高。PWS中的DHEAS水平受年龄和性别的影响。我们的研究结果表明PWS患儿肾上腺网状带成熟较早。
