characteristics of intestinal pseudo-obstruction in patients with mitochondrial diseases

查看全文 作　　者：Yusuke [1]Sekino;Masahiko [1]Inamori;Eiji [1]Yamada;Hidenori [1]Ohkubo;Eiji [1]Sakai;Takuma [1]Higurashi;Hiroshi [1]Iida;Kunihiro [1]Hosono;Hiroki [1]Endo;Takashi [1]Nonaka;Hirokazu [1]Takahashi;Tomoko [1]Koide;Yasunobu [1]Abe;Eiji [2]Gotoh;Shigeru [3]Koyano;Yoshiyuki [3]Kuroiwa;Shin [1]Maeda;Atsushi [1]Nakajima

机构地区：[1]Gastroenterology Division, Yokohama City University School of Medicine, Yokohama 236-0004, Japan;[2]Department of Medical Education, Yokohama City University School of Medicine, Yokohama 236-0004, Japan;[3]Department of Neurology, Yokohama City University School of Medicine, Yokohama 236-0004, Japan

出　　处：《World Journal of Gastroenterology》索引2012年第18卷第33期,共6页

基　　金：Health and Labour Sciences Research Grants for Research on Intractable Diseases, awarded to Nakajima A, from the Ministry of Health, Labour and Welfare of Japan 摘　　要：AIM: To reveal the frequency, characteristics and prognosis of chronic intestinal pseudo-obstruction (CIP) in mitochondrial disease patients. METHODS: Between January 2000 and December 2010, 31 patients (13 males and 18 females) were di-agnosed with mitochondrial diseases at our hospital. We conducted a retrospective review of the patients' sex, subclass of mitochondrial disease, age at onset of mitochondrial disease, frequency of CIP and the age at its onset, and the duration of survival. The age at onset or at the first diagnosis of the disorder that led to the clinical suspicion of mitochondrial disease was also examined. RESULTS: Twenty patients were sub-classified with mitochondrial encephalopathy with lactic acidosis and stroke-like episodes (MELAS), 8 with chronic progressive external ophthalmoplegia (CPEO), and 3 with myoclonus epilepsy associated with ragged-red fibers (MERRF). Nine patients were diagnosed with CIP, 8 of the 20 (40.0%) patients with MELAS, 0 of the 8 (0.0%) patients with CPEO, and 1 of the 3 (33.3%) patients with MERRF. The median age (range) at the diagnosis and the median age at onset of mitochondrial disease were 40 (17-69) and 25 (12-63) years in patients with CIP, and 49 (17-81) and 40 (11-71) years in patients without CIP. During the survey period, 5 patients (4 patients with MELAS and 1 with CPEO) died. The cause of death was cardiomyopathy in 2 patients with MELAS, cerebral infarction in 1 patient with MELAS, epilepsy and aspiration pneumonia in 1 patient with MELAS, and multiple metastases from gastric cancer and aspiration pneumonia in 1 patient with CPEO. CONCLUSION: Patients with CIP tend to have disorders that are suspected to be related to mitochondrial diseases at younger ages than are patients without CIP. 关键词：线粒体疾病肠梗阻患者假性死亡原因 CIP 心肌病生存时间

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characteristics of intestinal pseudo-obstruction in patients with mitochondrial diseases

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