Nakamura Yutaka, Murakami Nobuyuki, Iida Takahiro, Asano Satoshi, Ozeki Satoru, Nagai Toshiro
Department of Orthopaedic Surgery, Dokkyo Medical University Koshigaya Hospital, 2-1-50 Minami-Koshigaya, Koshigaya, Saitama, 343-8555, Japan,
J Orthop Sci. 2014 Nov;19(6):877-82. doi: 10.1007/s00776-014-0641-0. Epub 2014 Sep 26.
Patients with Prader-Willi syndrome (PWS) have fragile bones. Osteoporosis is a major concern in scoliosis surgery. Our aim was to investigate bone mineral density (BMD) in PWS patients and to verify the efficacy of and scoliosis deterioration with growth hormone (GH) administration for osteoporosis.
We followed 148 PWS patients who underwent lumbar spine (L2-4) BMD testing. Sixty-four patients had scoliosis, and 84 were non-scoliosis patients. Patients were treated with GH (0.245 mg/kg/week) until they reached a skeletal age of 17 years for males and 15 years for females. We also evaluated the effect of GH treatment on BMD in 101 patients (60 males, 41 females) undergoing BMD testing more than twice. The mean patient age was 5.4 years. The mean duration of GH administration was 54 months.
Mean lumbar BMD was 0.567 g/cm(2). Fifty patients (33.8%) had osteoporosis and 41 (27.7%) had osteopenia. There was no significant difference in mean BMD between patients with scoliosis (0.598 g/cm(2)) and without scoliosis (0.548 g/cm(2)). GH treatment caused a significant increase in Z score (pre-GH: mean -2.28 vs. post-GH: mean -1.53, P < 0.001). There was no statistical difference in the prevalence of scoliosis between the GH treatment group (45/112, 40.1%) and non-treatment group (19/36, 52.8%).
Among patients with PWS, 61.5% had low BMDs. GH administration significantly improved the lumbar BMD (Z score). There were no statistically significant differences in the prevalence of scoliosis among patients who received GH treatment compared to patients who did not.
普拉德-威利综合征（PWS）患者骨骼脆弱。骨质疏松是脊柱侧弯手术中的一个主要问题。我们的目的是研究PWS患者的骨密度（BMD），并验证生长激素（GH）治疗骨质疏松的疗效以及对脊柱侧弯恶化的影响。
我们对148例接受腰椎（L2-4）骨密度检测的PWS患者进行了随访。其中64例患者患有脊柱侧弯，84例为非脊柱侧弯患者。患者接受GH治疗（0.245mg/kg/周），直至男性达到骨骼年龄17岁，女性达到骨骼年龄15岁。我们还评估了GH治疗对101例（60例男性，41例女性）接受两次以上骨密度检测患者骨密度的影响。患者平均年龄为5.4岁。GH平均给药时间为54个月。
腰椎平均骨密度为0.567g/cm²。50例患者（33.8%）患有骨质疏松，41例患者（27.7%）患有骨质减少。脊柱侧弯患者的平均骨密度（0.598g/cm²）与无脊柱侧弯患者的平均骨密度（0.548g/cm²）之间无显著差异。GH治疗使Z值显著增加（GH治疗前：平均-2.28 vs GH治疗后：平均-1.53，P<0.001）。GH治疗组（45/112，40.1%）和非治疗组（19/36，52.8%）之间脊柱侧弯的患病率无统计学差异。
在PWS患者中，61.5%的患者骨密度较低。GH治疗显著改善了腰椎骨密度（Z值）。接受GH治疗的患者与未接受治疗的患者相比，脊柱侧弯的患病率无统计学显著差异。
