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Myopathy with mitochondrial alterations in patients with primary biliary cirrhosis and antimitochondrial antibodies

✍ Scribed by John Varga; Terry Heiman-Patterson; Santiago Muñoz; Lori A. Love

Publisher: John Wiley and Sons
Year: 1993
Tongue: English
Weight: 785 KB
Volume: 36
Category: Article
ISSN: 0004-3591
DOI: 10.1002/art.1780361020

No coin nor oath required. For personal study only.

✦ Synopsis

To describe a syndrome of severe progressive myopathy, cardiomyopathy, and gastrointestinal dysmotility in 2 patients with asymptomatic primary biliary cirrhosis (PBC) and circulating anti-mitochondria1 autoantibodies, and to review pertinent literature concerning this syndrome.

Methods. Clinical, elec troph ysiologic , serologic, and pathologic studies of the 2 affected patients were conducted.

Results. Skeletal muscle involvement was manifested by progressive weakness of the proximal muscles, marked diaphragmatic dysfunction with consequent hypoventilation and respiratory failure, and moderately elevated levels of muscle-associated enzymes. Serum from both patients contained antimitochondrial antibodies that reacted with components of the mitochondrial keto acid dehydrogenase enzyme complex. Results of electromyography were consistent with a myopathic process. The microscopic and ultrastructural changes in

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