title: Characteristics of intestinal pseudo-obstruction in patients with mitochondrial diseases.
abstract: OBJECTIVE
  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 diagnosed 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.
  
  
  CONCLUSIONS
  Patients with CIP tend to have disorders that are suspected to be related to mitochondrial diseases at younger ages than are patients without CIP.
authors.affiliation: Gastroenterology Division, Yokohama City University School of Medicine, Yokohama 236-0004, Japan.
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Sekino
authors.forename: Masahiko
authors.initials: M
authors.lastname: Inamori
authors.forename: Eiji
authors.initials: E
authors.lastname: Yamada
authors.forename: Hidenori
authors.initials: H
authors.lastname: Ohkubo
authors.forename: Eiji
authors.initials: E
authors.lastname: Sakai
authors.forename: Takuma
authors.initials: T
authors.lastname: Higurashi
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Iida
authors.forename: Kunihiro
authors.initials: K
authors.lastname: Hosono
authors.forename: Hiroki
authors.initials: H
authors.lastname: Endo
authors.forename: Takashi
authors.initials: T
authors.lastname: Nonaka
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Takahashi
authors.forename: Tomoko
authors.initials: T
authors.lastname: Koide
authors.forename: Yasunobu
authors.initials: Y
authors.lastname: Abe
authors.forename: Eiji
authors.initials: E
authors.lastname: Gotoh
authors.forename: Shigeru
authors.initials: S
authors.lastname: Koyano
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Kuroiwa
authors.forename: Shin
authors.initials: S
authors.lastname: Maeda
authors.forename: Atsushi
authors.initials: A
authors.lastname: Nakajima
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002908:Chronic Disease; D015897:Comorbidity; D005260:Female; D006801:Humans; D007410:Intestinal Diseases / Q000175:diagnosis* / Q000453:epidemiology* / Q000401:mortality; D007418:Intestinal Pseudo-Obstruction / Q000175:diagnosis* / Q000453:epidemiology* / Q000401:mortality; D017241:MELAS Syndrome / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D028361:Mitochondrial Diseases / Q000453:epidemiology*; D017237:Mitochondrial Encephalomyopathies / Q000453:epidemiology; D017246:Ophthalmoplegia, Chronic Progressive External / Q000453:epidemiology; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D055815:Young Adult