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Kokyu to Junkan Volume 53, Issue 10 （October 2005）

呼吸と循環 53巻10号（2005年10月発行）

Japanese English

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著明な肺高血圧を伴ったimmotile cilia syndromeの1例 A Case of Immotile Cilia Syndrome with Marked Pulmonary Hypertension 福田理子 ¹ , 赤柴恒人 ¹ , 大地康司 ¹ , 川原誠司 ¹ , 桂一仁 ¹ , 赤星俊樹 ¹ , 高橋典明 ¹ , 斉藤修 ¹ , 馬島徹 ¹ , 堀江孝至 ¹ Riko Fukuda ¹ , Tsuneto Akashiba ¹ , Yasushi Ohchi ¹ , Seiji Kawahara ¹ , Kazuto Katsura ¹ , Toshiki Akahoshi ¹ , Noriaki Takahashi ¹ , Osamu Saito ¹ , Toru Majima ¹ , Takashi Horie ¹ ¹日本大学医学部呼吸器内科 ¹Department of Respiratory Medicine, Nihon University School of Medicine キーワード：原発性線毛機能不全症候群 , 呼吸不全 , 肺高血圧症 , immotile cilia syndrome , respiratory failure , pulmonary hypertension Keyword: 原発性線毛機能不全症候群 , 呼吸不全 , 肺高血圧症 , immotile cilia syndrome , respiratory failure , pulmonary hypertension pp.1093-1097

発行日 2005年10月1日 Published Date 2005/10/1

DOI https://doi.org/10.11477/mf.1404100120

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Abstract 文献概要
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要旨　患者は48歳の男性で，気管支喘息として治療されていたが，著明な呼吸不全状態となって当科に入院した．著しい閉塞性換気障害，肺高血圧症が認められ，気道閉塞の可逆性が軽度であったため気管支喘息以外の閉塞性肺疾患が疑われた．男性不妊，副鼻腔炎を認めたため，immotile cilia syndrome(ICS)を疑い気管支鏡検査を行い，各気管支に大量のmucoid impactionを認めた．気管支粘膜生検の電顕像で，dynein armの欠損，中心鞘構造の消失などICSに特徴的な所見が認められ，また，精子の運動が全く認められないことからICSと診断した．

Summary

　We encountered a case of immotile cilia syndrome(ICS) with severe respiratory failure and pulmonary hypertension. A 48-year-old male who had been treated as having bronchial asthma by his local doctor was referred to our hospital with marked respiratory failure. Chest X-P and CT revealed diffuse nodular shadows in both lung fields and marked enlargement of the pulmonary trunks. Although spirometry showed severe obstructive ventilatory impairment, complete reversibility of airway obstruction was not attained after inhalation of bronchodilator. Since the patient along with his brothers suffered from male sterility and also had chronic sinusitis, ICS was suspected. On bronchofiberscopy, a lot of mucoid impaction was detected in each bronchi, so bronchial biopsy was performed for pathological diagnosis. By electron microscopic observation of bronchial mucous epithelium, dynein arm defects were found. Additionally, as his sperm was immotile, the diagnosis of ICS was confirmed.