Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Myasthenia gravis is considered to be an autoimmune disease in which several factors reciprocally influence the clinical type and course. We investigated the relative importance of the following factors: anti-acetylcholine receptor antibody (AChR Ab), HLA, age at onset, autoimmunity, thymic abnormality, duration of treatment, change in AChR Ab titer and immunosuppressive therapy. The pretreatment-AChR Ab titer and HLA were shown to significantly influence the clinical type. On the other hand, the age at onset significantly influenced the clinical course. The finding that with an onset at less than 5-year-old there was a tendency for a good prognosis suggests an association between the immaturity of the muscle and immune systems, and the clinical course.
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PMID:Factors influencing the clinical type and course of myasthenia gravis. 162 31

To the best of our knowledge this is the seventh case report of monozygotic twins both affected by myasthenia gravis (MG). The monozygotism was proven by sex identity, blood group and HLA determinations ('O' Rh+, A2, A19.2, B40, CW3). One paternal aunt was also affected and the three cases have high titles of anti-acetylcholine receptor antibodies and anti-striated muscle antibodies, which indicate an acquired form of MG. After several myasthenic crises the twins are now doing well with corticosteroid therapy. The paternal aunt has a more benign form of MG, with ocular and limb involvement (grade IIa of Osserman) and was submitted to thymectomy. The authors discuss the use of corticosteroid for the infantile form of MG instead of thymectomy, based on the immaturity of the immune system in the young age. The use of other immunosuppressive drugs is not advisable because of potential hazardous development of neoplasms.
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PMID:[Familial myasthenia gravis: a case report in identical twins]. 268 10

Double labelled lymphocytes were prevalent in the thymus of a 32-year-old woman with myasthenia gravis. The simultaneous presence of both E and C3b surface receptors was demonstrated. Focal paranuclear acid phosphatase activity of the cells supported their T cell origin. Histological analysis revealed a hyperplastic thymus with germinal centres in one lobe and an unusual transition to "thymoma-like" diffuse sheets of thymocytes and large thymic epithelial cells in the other lobe. Immunological studies of isolated cells from the "thymoma-like" region revealed a high percentage of E rosettes (96%), EAC rosettes (87%) and IgG-EA rosettes (60%). The large number of "active" rosettes (94%) indicated high avidity of receptors for sheep red blood cells. The postoperative accumulation of acid phosphatase positive T cells with C3b surface receptors in the peripheral blood was striking. These characteristics suggest that this cell population represents an activated T subset (TG) with a certain degree of immaturity similar to a particular stage of foetal T cell development (prothymocytes).
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PMID:Double labelled T suppressor lymphocytes in the thymus of a patient with myasthenia gravis: morphological and immunological characteristics. 646 69