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Revue Maghrebine de Pediatrie [La]. 2010; 20 (2): 91-98
em Francês | IMEMR | ID: emr-133613

RESUMO

Juvenile myasthenia gravis is a rare disease in childhood. It can be dangerous by the possibility of respiratory failure which is the dread-ful complication in myasthenia gravis. We studied 8 childs with myasthenia gravis [there were a familial form with 2 sisters], followed at the Pediatrics departments of Sahloul and Farhat Hached University Hospitals, during 23 years [since 1982 to 2004]. All patients, were with the generalized form, moderate or severe disease. The median age at diagnosis moment were 8 years and a half. The anticholinesterase test was positive in all cases, EMG with repetitive nerve stimulations showed a decremental response in 4 patients. Seropositivity to acetylcholine receptor antibodies was found in 5 patients. Treatment of juvenile myasthenia gravis was based on anticholinesterase drugs. Steroids and immunosuppressive medications were associated in the severe form. Thymectomy was indicated in the thymic abnormalities form. The follow-up of our patients found a complete remission in 4 cases, an improvement in 3 cases and a death in one case by myasthenic crisis

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