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普拉德-威利综合征患者睡眠相关呼吸障碍与生长激素治疗时期的关系

Sleep-related breathing disorders in patients with Prader-Willi syndrome depending on the period of growth hormone treatment.

作者信息

Lecka-Ambroziak Agnieszka, Jędrzejczak Małgorzata, Wysocka-Mincewicz Marta, Szalecki Mieczysław

机构信息

Klinika Endokrynologii i Diabetologii, Instytut Pomnik-Centrum Zdrowia Dziecka, 04-730 Warszawa.

出版信息

Endokrynol Pol. 2017;68(6):676-681. doi: 10.5603/EP.a2017.0057. Epub 2017 Oct 12.

Abstract

INTRODUCTION

Sleep-related breathing disorders (SRBD) are commonly present in patients with Prader-Willi syndrome (PWS). Recombinant human growth hormone (rhGH) treatment is reported to improve breathing function in PWS, but the findings are not explicit.

MATERIAL AND METHODS

Screening polysomnography- polygraphy (PSG), assessing nasal respiratory flow, respiratory effort, and blood oxygen saturation, was used. Group 1 - before rhGH therapy (n = 11, mean age 3.0 years); PSG was repeated after the start of rhGH therapy in a mean time of 0.9 years in six patients (Group 1a). Group 2 - on rhGH treatment, for a mean time of four years (n = 17, mean age 8.8 years). Group 3 - without rhGH therapy due to severe obesity (n = 8, mean age 13.1 years).

RESULTS

Group 1 - mean apnoea-hypopnoea index (AHI) was 10.2, oxygen desaturation index (ODI)- 36.3, Group 1a- AHI 12.0, ODI 60.9, Group 2-AHI 9.0, ODI 25.1, Group 3- AHI 8.2, ODI 22.0. ODI was significantly higher in Group 1a than in the other groups (p < 0.005), but not strictly related to SRBD. The results in Group 2 did not differ significantly from those of Group 1.

CONCLUSIONS

Our study proves the high frequency of SRBD among PWS patients, with worsening of ODI after short-term rhGH therapy.

摘要

引言

普拉德-威利综合征(PWS)患者中常见睡眠相关呼吸障碍(SRBD)。据报道,重组人生长激素(rhGH)治疗可改善PWS患者的呼吸功能,但结果并不明确。

材料与方法

采用筛选多导睡眠图-多导仪(PSG),评估鼻呼吸气流、呼吸努力和血氧饱和度。第1组 - rhGH治疗前(n = 11,平均年龄3.0岁);6名患者在rhGH治疗开始后平均0.9年重复进行PSG检查(第1a组)。第2组 - 接受rhGH治疗,平均时间为4年(n = 17,平均年龄8.8岁)。第3组 - 因严重肥胖未接受rhGH治疗(n = 8,平均年龄13.1岁)。

结果

第1组 - 平均呼吸暂停低通气指数(AHI)为10.2,氧饱和度下降指数(ODI)为36.3;第1a组 - AHI为12.0,ODI为60.9;第2组 - AHI为9.0,ODI为25.1;第3组 - AHI为8.2,ODI为22.0。第1a组的ODI显著高于其他组(p < 0.005),但与SRBD并非严格相关。第2组的结果与第1组无显著差异。

结论

我们的研究证明PWS患者中SRBD的发生率很高,短期rhGH治疗后ODI会恶化。

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