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Fifteen black children with juvenile myasthenia gravis presented to our institution over a 10-year period at ages ranging from 18 months to 7 years, 4 males and 11 females. Twelve presented with progressive, generalised weakness and 3 had bulbar manifestations. Ocular signs were absent in 2 patients. All 15 patients were placed on pyridostigmine bromide (Mestinon) and 1 also required steroids and plasmapheresis. Medical therapy only was instituted in 5 patients, of whom 2 came to surgery, 2 died, and 1 was lost to follow-up. Thymectomy was performed in 12 children (including the 2 with failed medical therapy) via a median sternotomy with no mortality or morbidity related to the operation. The timing of surgery was less than 3 months in 75% of the children. Thymic hyperplasia was noted in 10 glands and 2 were reported as normal. Of the children in the operative group, 83% are on minimal medication or in total remission. Better results were noted in young patients with early thymectomy and diseased glands.

Type

Journal article

Journal

Pediatr Surg Int

Publication Date

21/03/1997

Volume

12

Pages

113 - 115