Medicine
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Kawasaki disease (KD) is an acute multisystemic vasculitis occurring predominantly in children and rarely in adults. Diagnosis is made clinically using diagnostic guidelines; no specific test is available. "Incomplete" KD is a more recent concept, which refers to patients with fever lasting > or =5 days and 2 or 3 clinical criteria (rash, conjunctivitis, oral mucosal changes, changes of extremities, adenopathy), without reasonable explanation for the illness. To describe the clinical and laboratory features of classical (or "complete") KD, and incomplete KD in adults, we report 10 cases of adult KD, including 6 patients who fulfilled the criteria for incomplete KD, diagnosed either at presentation (n = 4) or retrospectively (n = 2). ⋯ Seven patients received intravenous immunoglobulins (IVIG), after a mean delay of 12.5 days, which appeared to shorten the course of the disease. This relatively large series of adult KD highlights the existence of incomplete KD in adults and suggests that the algorithm proposed by a multidisciplinary committee of experts to diagnose incomplete KD in children could be useful in adults. Further studies are needed to determinate whether prompt IVIG may avoid artery sequelae in adult patients with complete or incomplete KD.
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Thoracic endometriosis (TE) is a rare disorder affecting women during their reproductive years. The etiopathogenesis of this disease is not well understood; the prevailing opinion is based on analysis obtained from case reports and small case series. A 1996 review of TE was not able to address the association between clinical presentation and thoracic pathology due to a paucity of thoracoscopic findings in these earlier cases. ⋯ Hemoptysis occurring in younger subjects may be the earliest manifestation of parenchymal lung involvement in TE. Diaphragmatic defects do not increase the risk for pneumothorax. Hemothorax reflects an increased burden of pleural implants in TE.
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Multicenter Study
Use of thalidomide for severe recurrent aphthous stomatitis: a multicenter cohort analysis.
Severe recurrent aphthous stomatitis (SRAS) is a rare, disabling disorder of unknown etiology. Thalidomide is an effective second-line therapy for SRAS, but is suppressive rather than curative, and adverse events limit its use. Few reports describe the efficacy, tolerance, and safety of thalidomide, and how it is actually used as long-term (maintenance) therapy for SRAS. ⋯ Despite its retrospective nature, this detailed study provides novel information on the different ways thalidomide is used as SRAS maintenance therapy in a large and unselected cohort of patients. Low-dose maintenance regimens appear to be widely used, effective, and relatively well tolerated. These observations suggest the value of undertaking a randomized trial to assess various maintenance regimens.
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Although cardiac troponin I (cTnI) elevation in patients presenting to the hospital with supraventricular tachycardia (SVT) is well recognized, the prevalence, predictors, and prognostic significance of cTnI elevation associated with SVT presentation are not known. We screened records of all patients presenting to 2 hospitals over a 4-year period with the diagnosis of SVT confirmed by 12-lead electrocardiogram, and who had at least 1 measured cTnI level and at least 1 year of follow-up after discharge. The primary endpoint was the occurrence of 1 of the following outcomes: death, myocardial infarction, or cardiovascular rehospitalization. ⋯ After multivariable adjustment, the presence of elevated cTnI with SVT was associated with increased risk of the primary endpoint of death, myocardial infarction, or cardiovascular rehospitalization (hazard ratio [HR], 3.67; 95% CI, 1.22-11.1; p = 0.02). Mild elevation of cTnI is common in patients presenting to the hospital with SVT, and is associated with increased risk of future cardiovascular events. Further study is needed to determine the mechanisms of SVT-related cTnI elevation and its association with elevated cardiovascular risk.