Bosio L, Beccaria L, Benzi F, Sanzari A, Chiumello G
Paediatric Department, Scientific Institute H San Raffaele, University of Milan, Italy.
J Pediatr Endocrinol Metab. 1999 Apr;12 Suppl 1:351-3.
Prader-Labhardt-Willi syndrome (PLWS) is a model to study GH secretion, body composition and consequences of GH therapy. Twenty-seven patients were studied by dual-energy X-ray absorptiometry (DXA) and were each compared to two age- and sex-matched controls (obese and normal weight). Fat mass (FM) was significantly greater in PLWS than in patients with simple obesity; lean body mass (LM) and bone mineral content (BMC) were significantly lower compared to both controls. The peculiar body composition of PLWS patients seems to be similar to that found in GH deficiency. In six PLWS children treated with GH, LM increased after 6 months (p<0.02) up to 12 months (p<0.03); FM decreased in 5/6 patients. Obese adult PLWS patients treated with GH for 6 months showed a reduction in adiposity; LM increased significantly only in the leg compartment. Abdominal CT scan did not show a significant reduction of intrabdominal fat area. In conclusion, GH therapy might improve final stature and exert a positive influence on body composition in patients with PLWS.
普拉德-拉巴哈特-威利综合征（PLWS）是研究生长激素（GH）分泌、身体成分及GH治疗后果的一个模型。对27例患者进行了双能X线吸收法（DXA）检查，并将每位患者与两名年龄和性别匹配的对照者（肥胖和正常体重）进行比较。PLWS患者的脂肪量（FM）显著高于单纯肥胖患者；与两名对照者相比，瘦体重（LM）和骨矿物质含量（BMC）均显著降低。PLWS患者独特的身体成分似乎与生长激素缺乏患者的相似。在6例接受GH治疗的PLWS儿童中，LM在6个月后增加（p<0.02），直至12个月时仍持续增加（p<0.03）；6例患者中有5例FM下降。接受GH治疗6个月的肥胖成年PLWS患者脂肪量减少；仅腿部的LM显著增加。腹部CT扫描未显示腹腔内脂肪面积有显著减少。总之，GH治疗可能会改善PLWS患者的最终身高，并对其身体成分产生积极影响。
