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Anthropometric, biochemical and hormonal profiles of the partially admixed pygmoid group in Rampasasa (Flores, Indonesia)
Journal of Pediatric Endocrinology and Metabolism ( IF 1.3 ) Pub Date : 2021-05-01 , DOI: 10.1515/jpem-2020-0526
Aman Pulungan 1 , Attika A Andarie 2 , Frida Soesanti 1 , Muhammad Ramdhani Yassien 1 , Christiaan de Bruin 3 , Andi Wijaya 4 , Agus Firmansyah 1 , Jan M Wit 3
Affiliation  

Objectives We performed a cross-sectional study on anthropometric and laboratory characteristics of inhabitants of Rampasasa (Flores, Indonesia). Adults were categorised according to ancestry into three groups: pygmoid (P/P, offspring of pygmoid parents, n=8), mixed pygmoid (P/N, offspring of pygmoid and non-pygmoid parents, n=12) and non-pygmoid (N/N, n=10). Children (n=28) were P/N. Methods Measurements included height, weight, sitting height, arm span, head circumference, haematological analysis and serum albumin, calcium, vitamin D, insulin-like growth factor-I (IGF-I) and IGF binding protein 3 (IGFBP-3). Pubertal stage and bone age was assessed in children. Anthropometric data were expressed as standard deviation score (SDS) for age. IGF-I, IGFBP-3 and IGF-I/IGFBP-3 ratio were expressed as SDS for age, bone age and pubertal stage. Results Mean height SDS showed a gradient from P/P (−4.0) via P/N (−3.2) to N/N (−2.3) (−3.4, −3.1 and −2.2 adjusted for age-associated shrinking). Sitting height and head circumference showed similar gradients. Serum IGF-I SDS was similar among groups (approximately −1 SDS). IGFBP-3 SDS tended toward a gradient from P/P (−1.9) via P/N (−1.5) to N/N (−1.1), but IGF-I/IGFBP-3 ratio was normal in all groups. In P/P and P/N, mean head circumference SDS was >2 SD greater than mean height SDS. Children showed a progressive growth failure and bone age delay, delayed female pubertal onset and an initial low serum IGF-I, normal IGFBP-3 and low IGF-I/IGFBP-3 ratio. Conclusions P/P showed proportionate short stature with relative macrocephaly and relatively low IGFBP-3; P/N presented an intermediate pattern. P/N children were progressively short, showed delayed skeletal maturation, delayed puberty in girls and low IGF-I and IGF-I/IGFBP-3.

中文翻译:

Rampasasa(弗洛雷斯,印度尼西亚)部分混合侏儒群的人体测量学、生化和激素特征

目标 我们对 Rampasasa(印度尼西亚弗洛雷斯)居民的人体测量学和实验室特征进行了横断面研究。成年人根据血统分为三组:侏儒(P/P,侏儒父母的后代,n=8),混合侏儒(P/N,侏儒和非侏儒父母的后代,n=12)和非侏儒(N/N,n=10)。儿童 (n=28) 是 P/N。方法测量包括身高、体重、坐高、臂展、头围、血液学分析和血清白蛋白、钙、维生素 D、胰岛素样生长因子-I (IGF-I) 和 IGF 结合蛋白 3 (IGFBP-3)。评估儿童的青春期阶段和骨龄。人体测量数据表示为年龄的标准偏差评分 (SDS)。IGF-I、IGFBP-3 和 IGF-I/IGFBP-3 比率表示为年龄、骨龄和青春期的 SDS。结果 平均身高 SDS 显示从 P/P (-4.0) 到 P/N (-3.2) 到 N/N (-2.3) 的梯度(-3.4、-3.1 和 -2.2 针对年龄相关的萎缩进行了调整)。坐高和头围显示出相似的梯度。组间血清 IGF-I SDS 相似(大约 -1 SDS)。IGFBP-3 SDS 趋向于从 P/P (-1.9) 到 P/N (-1.5) 到 N/N (-1.1) 的梯度,但 IGF-I/IGFBP-3 比率在所有组中均正常。在 P/P 和 P/N 中,平均头围 SDS 比平均身高 SDS 大 >2 SD。儿童表现出进行性生长障碍和骨龄延迟、女性青春期延迟和初始低血清 IGF-I、正常 IGFBP-3 和低 IGF-I/IGFBP-3 比率。结论 P/P 显示成比例的身材矮小,具有相对的大头畸形和相对较低的 IGFBP-3;P/N 呈现中间模式。P/N 儿童逐渐矮小,
更新日期:2021-05-05
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