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首页> 外文期刊>American journal of medical genetics, Part A >Growth hormone therapy and scoliosis in patients with Prader-Willi syndrome.
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Growth hormone therapy and scoliosis in patients with Prader-Willi syndrome.

机译:Prader-Willi综合征患者的生长激素治疗和脊柱侧弯。

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Growth hormone (GH) therapy for short stature in patients with Prader-Willi syndrome (PWS) has started worldwide, and various favorable effects have been reported. However, the possibility of progression of scoliosis arises as a new problem of the GH therapy. In this study, we analyzed whether 72 patients who have been followed up in our hospital have such a problem. They included 46 males and 26 females (41 patients with the GH therapy and 31 without it) aged from one to 49 years. Consequently, 33 (45.8%) of 72 patients had scoliosis with the Cobb angle of >10 degrees. Twenty (48.8%) of forty-one patients who received a GH therapy and 13 (41.9%) of 31 patients without the therapy had scoliosis, the frequency of scoliosis between the two groups showing no statistical difference (P = 0.56). Height velocity of scoliotic and non-scoliotic patients during the first year of the therapy was 8.59 +/- 1.92 and 10.70 +/- 2.54 cm, respectively, showing a significant difference (P < 0.001). This shows that accelerated height velocity may not induce scoliosis. Comparison of starting age of a GH treatment revealed that non-scoliotic patients received the therapy earlier than scoliotic patients (P = 0.021). Among 20 scoliotic patients who received the GH therapy, the degree of scoliosis progressed during the therapy in six patients, improved in three and fluctuated in one. Many patients showed progression of scoliosis with age irrespective of the use of GH, and some patients improved their scoliosis during the GH therapy. These findings showed that a GH therapy increases height velocity of PWS patients but does not necessarily develop scoliosis, and early start of the therapy may not be an exacerbating factor of scoliosis.
机译:在世界范围内已经开始对患有Prader-Willi综合征(PWS)的患者矮身材的生长激素(GH)疗法进行治疗,并且已经报道了各种有益的作用。但是,作为GH治疗的新问题,出现了脊柱侧弯发展的可能性。在这项研究中,我们分析了在我院接受随访的72名患者是否存在此类问题。他们包括46例男性和26例女性(41例接受GH治疗的患者和31例未接受GH治疗的患者),年龄从1岁到49岁。因此,72例患者中有33例(45.8%)的脊柱侧弯的Cobb角> 10度。接受GH治疗的41例患者中有20例(48.8%)和未经治疗的31例患者中有13例(41.9%)出现脊柱侧弯,两组之间的脊柱侧弯频率无统计学差异(P = 0.56)。在治疗的第一年,脊柱侧凸和非脊柱侧凸患者的身高速度分别为8.59 +/- 1.92和10.70 +/- 2.54 cm,显示出显着性差异(P <0.001)。这表明加速的身高速度可能不会诱发脊柱侧弯。比较GH治疗的起始年龄,发现非脊柱侧凸患者比脊柱侧凸患者更早接受该疗法(P = 0.021)。在接受GH治疗的20例脊柱侧弯患者中,有6例在治疗过程中出现了脊柱侧弯的进展,有3例得到改善,有1例出现了波动。无论是否使用GH,许多患者的年龄都显示出脊柱侧弯的进展,并且一些患者在GH治疗期间改善了脊柱侧弯。这些发现表明,GH疗法可以增加PWS患者的身高速度,但不一定会发展为脊柱侧弯,并且早期开始治疗可能不会加剧脊柱侧弯。

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