Myoglobinemia in Children with Progressive Muscular Dystrophy
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概要
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Counterimmunoelectrophoresis for the detection of serum myoglobin was descrived.Among several methods this technique was preferable for clinical use because of its handiness, inexpensive ness and time saving. This also offers a sensitivity sufficient for the diagnosis of muscle damage in a variety of diseases.<BR>Out of 13 patients with congenital muscular dystrophy myoglobin was proven in four, and nine of 13 cases of Duchenne dystrophy showed myoglobinemia. Myoglobinemia was detected in 14 patients of 15 (93%), whose serum CPK levels were more than 2500 units, but in only one of 11 (9%) with lower CPK levels. All the myoglobine mic patients were less than 5 years of age in congenital muscular dystrophy and less than 9 years of age in Duchenne dystrophy.<BR>Myoglobinemia was closely related to the age of the patients, the clinical stages of the diseases and serum CPK levels. The myoglobin release into the circulation may be caused by the severity of muscle fibers degeneration as well as by the enhanced permeability of the muscle membrane. In Duchenne dystrophy myoglobinemia was found during the ages between 5 and 8, when probably the degeneration of muscle was still active. The degeneration of muscle in the congenital type, however, seems to occur in much younger ages.<BR>Although we failed to detect myoglobinuria in several myoglobinemic patients examined, muscle exercise or infectious diseases may have caused a release of myoglobin into the urine. Careful urinalysis is advisable to detect urinary myoglobin which may lead to renal failure inpatients with positive myoglobinemia and CPK value of more than 2500 units.
- 一般社団法人 日本小児神経学会の論文
一般社団法人 日本小児神経学会 | 論文
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