PubMed · 5342236
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E Rajka. [Autoallergy-autosensitization].. https://pubmed.ncbi.nlm.nih.gov/5342236/
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A consistent, planned approach to thymectomy for patients with myasthenia gravis has been used at the University of North Carolina since 1977. This involves a sternotomy, with excision of the entire thymus and adjacent fatty tissues from the thyroid to the diaphragm and laterally to 1 cm from each phrenic nerve. Between 1977 and 1993, 100 consecutive patients were treated in this manner with no mortality. Eight-four percent of patients were extubated in the operating room or within 1 hour of surgery, and no patients experienced postoperative respiratory difficulty. After a mean follow-up of 65 months, 78% of all patients improved by at least one modified Osserman classification, and 69% of patients with preoperative class I, II, or III disease (maximal preoperative severity) are in pharmacological remission. We conclude that transsternal thymectomy is associated with minimal morbidity and no mortality, and results in long-term improvement in symptoms for patients with myasthenia gravis.
Transcervical thymectomy is appropriate for managing carefully selected patients with myasthenia gravis due to its noninvasive nature, good cosmetic results, and favorable long-term outcomes. Contraindications to its use include the presence of a thymoma and advanced age. In optimally prepared patients, the operative complication rate is negligible and the average length of hospital stay is 1 to 2 days. The ultimate results of therapy often are not evident for several years postoperatively, indicating that comprehensive preoperative and postoperative treatment by a qualified neurologist is essential in optimizing outcomes. A meta-analysis of long-term results shows that 90% of patients who undergo the operation are improved, 80% become asymptomatic, and 50% achieve a complete remission.
Chronic granulomatous disease (CGD) is an inherited disorder of phagocyte function in which defective superoxide production results in deficient microbicidal activity. CGD patients suffer from recurrent, life-threatening infections, and nearly half develop chronic gastrointestinal (GI) complications (colitis, gastric outlet obstruction, or perirectal abscess) and/or autoimmune/rheumatologic disorders (AIDs). To identify genetic modifiers of disease severity, we studied a cohort of 129 CGD patients, in whom seven candidate genes (myeloperoxidase [MPO], mannose binding lectin [MBL], Fcgamma receptors IIa, IIIa, IIIb, TNF-alpha, and IL-1 receptor antagonist), each containing a physiologically relevant polymorphism predicted to influence the host inflammatory response, were selected for analysis. Genotypes of MPO (P = 0.003) and FcgammaRIIIb (P = 0.007) were strongly associated with an increased risk for GI complications, while an FcgammaRIIa (P = 0.05) genotype was suggestive for an association. Patients with all three associated genotypes had the highest risk for GI complications (P < 0.0001). The risk of AIDs was strongly associated with variant alleles of MBL (P = 0.01) and weakly associated with an FcgammaRIIa genotype (P = 0.04). Patients with variant forms of both MBL and FcgammaRIIa had the highest risk of developing an AID (P = 0.003).