Linked Life Data

9309559

Source:http://linkedlifedata.com/resource/pubmed/id/9309559

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
8
pubmed:dateCreated
1997-11-5
pubmed:abstractText
Randomised and controlled treatment studies of juvenile-onset myasthenia gravis have not been published. We therefore report our retrospective analysis of 79 patients with juvenile-onset myasthenia gravis observed for as long as 30 years. The mean age at onset was 13.7 years and median follow-up 7.7 years. The initial presentation was generalised disease in 90% and ocular disease in the remaining patients. Sixty-five patients (82%) were thymectomised. In 14 of these, treatment consisted of a combination of azathioprine (2-3 mg/kg), corticosteroids (prednisolone up to 60 mg for a maximum duration of 12 months with subsequent tapering) and acetylcholinesterase (AChE) inhibitors, and of azathioprine and AChE inhibitors in 27 patients. One patient received azathioprine and 22 AChE inhibitors only; in another no further medication was necessary. In the severely affected group (n = 16), plasmapheresis was performed additionally before thymectomy and continued for some time after the operation. Treatment was started between 1 and 14 months (mean 2.4 months) after the onset of myasthenic symptoms. No thymectomy was done in 14 patients, and immunosuppressive treatment and AChE inhibitors were given in 9 of these cases. One patient received azathioprine only; 4 patients received AChE inhibitors only. The histology of the thymus gland showed follicular hyperplasia in 89% of the 65 thymectomised patients and normal findings in the remainder. Remission occurred in 60% of patients who underwent thymectomy and in 29% of those who were not thymectomised. Hyperthyroidism (6 patients, 8%), diabetes mellitus (2 patients, 3%) and rheumatoid arthritis (2 patients, 3%) were the most frequent associated immune-mediated diseases. Epileptic seizures and neoplasia were coincident diseases in 2 (3%) and 3 (4%) patients, respectively. There were no deaths from thymectomy or from immunosupression. This open, retrospective analysis suggests that juvenile-onset myasthenia gravis can be treated satisfactorily in most patients by the use of thymectomy and/or immunosupressive medication.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Aug
pubmed:issn
0340-5354
pubmed:author
pubmed:issnType
Print
pubmed:volume
244
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
515-20
pubmed:dateRevised
2004-11-17
pubmed:meshHeading
pubmed-meshheading:9309559-Adolescent, pubmed-meshheading:9309559-Antibodies, pubmed-meshheading:9309559-Azathioprine, pubmed-meshheading:9309559-Child, pubmed-meshheading:9309559-Drug Combinations, pubmed-meshheading:9309559-Female, pubmed-meshheading:9309559-Follow-Up Studies, pubmed-meshheading:9309559-Glucocorticoids, pubmed-meshheading:9309559-Humans, pubmed-meshheading:9309559-Immunosuppressive Agents, pubmed-meshheading:9309559-Longitudinal Studies, pubmed-meshheading:9309559-Male, pubmed-meshheading:9309559-Myasthenia Gravis, pubmed-meshheading:9309559-Prednisolone, pubmed-meshheading:9309559-Receptors, Cholinergic, pubmed-meshheading:9309559-Retrospective Studies, pubmed-meshheading:9309559-Thymectomy, pubmed-meshheading:9309559-Thymus Gland, pubmed-meshheading:9309559-Treatment Outcome
pubmed:year
1997
pubmed:articleTitle
Outcome in juvenile-onset myasthenia gravis: a retrospective study with long-term follow-up of 79 patients.
pubmed:affiliation
Department of Neurology, Julius-Maximilians-University Würzburg, Germany.
pubmed:publicationType
Journal Article