| Distal myopathy in nephropathic cystinosis. | |
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MedLine Citation:
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PMID: 10654328 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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In long-standing nephropathic cystinosis complications are observed in various organs. Distal myopathy was first described in detail in 1994. The prevalence was calculated to be 24%. We studied seven patients with nephropathic cystinosis with neurophysiological techniques. Only two patients complained of a distal muscle weakness but all showed signs of myopathy on electromyography, which was more pronounced in the distal muscles. Motor and sensory nerve conduction parameters were within normal ranges. One patient with the juvenile form of nephropathic cystinosis also had myopathy. We conclude that distal myopathy can be detected in nephropathic cystinosis even in the absence of clinically overt muscle weakness. Cystine-depleting therapy with cysteamine is recommended for all patients with cystinosis, even after renal transplantation, and the effect on the myopathy should be studied. |
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Authors:
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U Vester; M Schubert; G Offner; J Brodehl |
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Publication Detail:
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Type: Clinical Trial; Journal Article |
Journal Detail:
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Title: Pediatric nephrology (Berlin, Germany) Volume: 14 ISSN: 0931-041X ISO Abbreviation: Pediatr. Nephrol. Publication Date: 2000 Jan |
Date Detail:
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Created Date: 2000-02-17 Completed Date: 2000-02-17 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 8708728 Medline TA: Pediatr Nephrol Country: GERMANY |
Other Details:
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Languages: eng Pagination: 36-8 Citation Subset: IM |
Affiliation:
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Kinderklinik der Universität (GHS), Essen, Germany. udo.vester@uni-essen.de |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Child Cysteamine / therapeutic use Cystinosis / complications*, physiopathology Electromyography Female Hand / physiopathology Humans Kidney Failure, Chronic / complications*, physiopathology Male Muscle Weakness / etiology, physiopathology Muscular Diseases / etiology*, physiopathology Neural Conduction / physiology |
| Chemical | |
Reg. No./Substance:
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60-23-1/Cysteamine |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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