Characteristics of intestinal pseudo-obstruction in patients with mitochondrial diseases

World Journal of Gastroenterology : WJG
Yusuke SekinoAtsushi Nakajima

Abstract

To reveal the frequency, characteristics and prognosis of chronic intestinal pseudo-obstruction (CIP) in mitochondrial disease patients. 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. 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 C...Continue Reading

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Apr 10, 2016·Developmental Biology·Allan M GoldsteinRoberto De Giorgio
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Related Concepts

Intestinal Diseases
Visceral Myopathy
Retrospective Studies
Comorbidity
Mitochondrial Encephalomyopathies
Melas Syndrome
Chronic Progressive External Ophthalmoplegia
Oxidative Phosphorylation Deficiencies
Acidosis, Lactic
Amaranth Dye

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