裘馨氏肌肉失養症患者呼吸狀況的偵測

Predicting Respiratory status in Patients with Duchenne Muscular Dystrophy

洪榮斌(Rong-Bin Hong) ； 王亭貴(Tyng-Guey Wang) ； 盧志輝(Chih-Whei Lu) ； 賴金鑫(Jin-Shin Lai) ； 連倚南(I-Nan Lien)

《中華民國復健醫學會雜誌》 25卷2期 (1997/12) Pp. 97-104

https://doi.org/10.6315/JRMA.199712.0097

摘要

由裘馨氏肌肉失養症之自然病史可知，患者約於18-19歲時會因呼吸衰竭而死亡，但患者通常在疾病末期前早已有呼吸功能異常的現象產生，而早期偵測其呼吸功能異常則有助於早期治療，以延續其生命。本研究採橫斷式研究，30名患者參與本研究，其中19名接受肺功能檢查及四肢功能評估；而有10名患者同時接受夜眠呼吸監控。研究結果顯示在接受肺功能檢查的患者中有42% (8/19)有肺功能異常，這些有肺功能異常患者均失去行走能力；在接受夜眠呼吸監控者中有40% (4/10)有夜眠呼吸異常。肺活量小於預測值50%之患者，有較大機會產生夜眠呼吸異常 (75% vs 14%)，但在統計學上並無顯著意義。我們建議裘馨氏肌肉失養症患者，在逐漸失去行走能力時，便應至醫院接受肺功能評估，當肺活量小於預測值之50%時，則應接受夜眠呼吸監控，以便早期發現及早期治療。

關鍵字

裘馨氏肌肉失養症 ； 夜眠呼吸監控 ； 肺功能檢查 ； 運動功能

並列摘要

The life expectancy of individuals with untreated Duchenne muscular dystrophy(DMD) is around 18-19 years. The cause of their death is mostly from respiratory complications. Early detection of respiratory function will provide the opportunity of early management and prolong their life span.
This study included 30 patients with DMD in a period of three years and took a cross-section design. All the data were obtained by telephone interview with the patients or their family. Nineteen of 30 patients accepted functional evaluation in Brooke’s grading scale and pulmonary function test(PFT). Ten of these 19 patients received nocturnal breathing monitor including O2 saturation and end-tidal CO2.. The result showed that 8 of 19(42%) patients who received PFT had impaired respiratory function. all the patients with impaired pulmonary function lost their ambulation ability simultaneously. Four of 10 patients(40%) with nocturnal breathing monitor had nocturnal oxygen desaturation (NOD). The patients whose vital capacity below 50% of prediction got a more chance to develop NOD, but there was no significant difference in statistics.
It is suggested that DMD patients should have a routine pulmonary function test once they progressively lost their ambulation ability. Nocturnal breathing monitor should be performed in DMD patients when their vital capacity was below 50% of predicted value for early detection of NOD and early management.

並列關鍵字

duchenne muscular dystrophy ； sleep breathing monitor ； pulmonary function test ； motor function