影响膈肌吸气运动、膈肌增厚及其对杜氏肌营养不良症呼吸受限模式预测作用的因素。

Determinants of diaphragm inspiratory motion, diaphragm thickening, and its performance for predicting respiratory restrictive pattern in Duchenne muscular dystrophy.

机构信息

Service de Réanimation médico-chirurgicale et Pole ventilation à domicile, CHU Raymond Poincaré, APHP, Université Paris Saclay/UFR Sciences de la santé-Université de Versailles Saint Quentin en Yvelines, Garches, France.

APHP, Nord/Est/Ile-de-France Neuromuscular reference center, Institut de Myologie, Hôpital Pitié-Salpêtrière, Paris, France.

出版信息

Muscle Nerve. 2022 Jan;65(1):89-95. doi: 10.1002/mus.27432. Epub 2021 Oct 25.

DOI:10.1002/mus.27432

PMID:34618930

Abstract

INTRODUCTION/AIMS: Respiratory status is a key determinant of prognosis in patients with Duchenne muscular dystrophy (DMD). We aimed to evaluate the determinants of diaphragm ultrasound and its performance in predicting restrictive respiratory patterns in DMD.

METHODS

This was a retrospective study of DMD patients followed in our center and admitted for an annual checkup from 2015 to 2018. We included DMD patients who underwent diaphragm ultrasound and pulmonary functional tests.

RESULTS

This study included 74 patients with DMD. The right diaphragm thickening fraction (TF) was significantly associated with age (P = .001), Walton score (P = .012), inspiratory capacity (IC) (P = .004), upright forced vital capacity (FVC) (P < .0001), supine FVC (P = .038), and maximal inspiratory pressure (MIP) (P = .002). Right diaphragm excursion was significantly associated with age (P < .0001), steroid use (P = .008), history of spinal fusion (P < .0001), body mass index (BMI) (P = .002), Walton score (P < .0001), IC (P < .0001), upright FVC (P < .0001), supine FVC (P < .0001), and MIP (P < .0001). A right diaphragm TF >28% and a right diaphragm excursion>25.4 mm were associated with an FVC >50% with, respectively, an area under the curve (AUC) of 0.95 (P = .001) and 0.93 (P < .001). A left diaphragm TF >26.8% and a left diaphragm excursion >21.5 mm were associated with an FVC >50% with, respectively, an AUC of 0.95 (P = .011) and 0.97 (P < .001).

DISCUSSION

Diaphragm excursion and diaphragm TF can predict restrictive pulmonary insufficiency in DMD.

摘要

介绍/目的：呼吸状况是杜氏肌营养不良症（DMD）患者预后的关键决定因素。我们旨在评估膈肌超声的决定因素及其在预测 DMD 限制性呼吸模式中的表现。

方法

这是一项回顾性研究，纳入了 2015 年至 2018 年在我们中心接受年度检查并入院的 DMD 患者。我们纳入了接受膈肌超声和肺功能检查的 DMD 患者。

结果

本研究纳入了 74 例 DMD 患者。右侧膈肌增厚分数（TF）与年龄（P =.001）、沃尔顿评分（P =.012）、吸气量（IC）（P =.004）、直立用力肺活量（FVC）（P <.0001）、仰卧 FVC（P =.038）和最大吸气压力（MIP）（P =.002）显著相关。右侧膈肌活动度与年龄（P <.0001）、类固醇使用（P =.008）、脊柱融合史（P <.0001）、体重指数（BMI）（P =.002）、沃尔顿评分（P <.0001）、IC（P <.0001）、直立 FVC（P <.0001）、仰卧 FVC（P <.0001）和 MIP（P <.0001）显著相关。右侧膈肌 TF >28%和右侧膈肌活动度>25.4 mm 与 FVC >50%相关，曲线下面积（AUC）分别为 0.95（P =.001）和 0.93（P <.001）。左侧膈肌 TF >26.8%和左侧膈肌活动度>21.5 mm 与 FVC >50%相关，AUC 分别为 0.95（P =.011）和 0.97（P <.001）。