Symptoms of respiratory involvement are frequently present but overlooked by patients with Myotonic Dystrophy type 1 (DM1). A respiratory symptom checklist was designed to test whether a DM-specifically designed checklist to detect symptoms of respiratory involvement (The Respicheck Questionnaire) could help patients be more aware of their respiratory problems, if any, and help clinicians in identifying potential candidates for intervention. The Respicheck questionnaire was administered to 58 consecutive adult-onset patients with genetically determined DM1 who did not complain of respiratory involvement per history at enrollment. Based on respiratory function test results patients were divided into 3 groups: A, (n = 17) having no signs of respiratory involvement; B (n = 13), patients having borderline results on respiratory assessments and having no need for respiratory intervention; C, (n = 28) patients having respiratory impairment requiring intervention. Respiratory test results and Respicheck scores were analyzed. Respicheck total score and subscales correlated positively with global respiratory impairment. Respicheck appears to be able to discriminate between patients having a higher level of respiratory dysfunction from those having a lower risk of respiratory involvement. This might allow to better target efforts and resources in respiratory management in DM1.

中文翻译：

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使用 RESPICHECK 问卷筛查 1 型肌强直性营养不良呼吸系统受累的早期症状

呼吸系统受累的症状经常出现，但被 1 型肌强直性营养不良 (DM1) 患者忽视。呼吸系统症状检查表旨在测试专门为 DM 设计的检查表以检测呼吸系统受累症状（Respicheck 问卷）是否可以帮助患者更加了解他们的呼吸系统问题（如果有的话），并帮助临床医生确定潜在的干预对象。对 58 名连续成年发病的遗传性 DM1 患者进行了 Respicheck 问卷调查，这些患者在入组时没有抱怨呼吸系统受累。根据呼吸功能检查结果将患者分为 3 组： A，（n = 17）没有呼吸系统受累的迹象；B (n = 13), 呼吸系统评估结果处于临界值且无需呼吸干预的患者；C，（n = 28）需要干预的呼吸障碍患者。分析了呼吸测试结果和 Respicheck 分数。Respicheck 总分和分量表与整体呼吸障碍呈正相关。Respicheck 似乎能够区分呼吸功能障碍程度较高的患者和呼吸系统受累风险较低的患者。这可能允许在 DM1 中更好地针对呼吸管理的努力和资源。Respicheck 总分和分量表与整体呼吸障碍呈正相关。Respicheck 似乎能够区分呼吸功能障碍程度较高的患者和呼吸系统受累风险较低的患者。这可能允许在 DM1 中更好地针对呼吸管理的努力和资源。Respicheck 总分和分量表与整体呼吸障碍呈正相关。Respicheck 似乎能够区分呼吸功能障碍程度较高的患者和呼吸系统受累风险较低的患者。这可能允许在 DM1 中更好地针对呼吸管理的努力和资源。