Medicine
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Multicenter Study
Long-term follow-up in 128 patients with primary antiphospholipid syndrome: do they develop lupus?
We retrospectively studied a large cohort of patients with primary antiphospholipid syndrome (APS) from 4 different referral centers to analyze the clinical and serologic features and, specifically, to determine the number of patients going on to develop systemic lupus erythematosus (SLE) or other autoimmune disease after long-term follow-up. The study included 128 unselected patients with primary APS who fulfilled the Sapporo International Criteria from 4 different tertiary hospitals in the United Kingdom, Mexico, and Spain. The patients had attended the referral centers between January 1987 and July 2001. ⋯ The current study confirms that progression from primary APS to SLE or lupus-like disease is unusual, even after a long follow-up. Only 3 patients developed anti-dsDNA antibodies. The presence of a positive Coombs test might be a marker for the development of SLE in patients with primary APS.
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Bacteremic pneumococcal pneumonia (BPP) is an important disease that should be frequently re-evaluated due to changes in demographics and recommended treatment. We conducted a prospective study from 2000 to 2002 in adults aged 17 years and over who presented to any of 6 hospitals and 1 freestanding emergency room in Edmonton, Alberta, with signs and symptoms compatible with pneumonia, a chest radiograph interpreted as pneumonia by the attending physician, and a positive blood culture for Streptococcus pneumoniae. We identified 129 patients with BPP, for an overall incidence of 9.7/100,000 person years. ⋯ Of the S. pneumoniae isolates, 12.5% were not susceptible to penicillin. The overall rate of BPP appears to be decreasing, although the rate is markedly increased in certain populations, which now should be targeted for vaccination. We identified 3 subsets of patients with BPP according to the site of care (ambulatory, ward, and ICU), with different outcomes.
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Pituitary apoplexy is an ill-defined clinical entity. Some authors include hypoxic pituitary infarction, even in the absence of tumor after hemorrhagic delivery, whereas others apply this term strictly to hemorrhage within a pituitary adenoma. We conducted the present study to establish the prevalence, clinical characteristics, and outcome of pituitary apoplexy, defined as an endocrine crisis characterized by acute intense headache, with or without altered consciousness, rapid development of visual or motor ocular disorders, and pituitary failure, associated with a large pituitary adenoma. ⋯ These data show that early surgical decompression prevents persistent neuro-ophthalmic deficit, but does not prevent persistent pituitary insufficiency. Moreover, published data indicate that the efficacy of surgery for the relief of neuro-ophthalmic symptoms decreases with increasing syndrome duration. Our data confirm that apoplexy occurs most often as the inaugural manifestation of pituitary macroadenoma, and suggest a recent increase of cases of apoplexy in our area.
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Comparative Study
Comparison of 3 clinical models for predicting the probability of pulmonary embolism.
Two clinical models have been described to predict the probability of pulmonary embolism: the Canadian (or Wells) model, and the Geneva model. A third model has been developed recently at our institution (the Pisa model). We compared the performance of the 3 models in 215 consecutive patients with suspected pulmonary embolism. ⋯ Among patients with pulmonary embolism, there was a strong, positive relation between clinical probability predicted by the Pisa model and the extent of pulmonary embolization. The Pisa model proved more accurate than the 2 other models. It may be useful to physicians in defining precisely the pretest probability of pulmonary embolism.
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Multicenter Study
Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study.
Familial Mediterranean fever (FMF) is an autosomal recessive disease that is prevalent among eastern Mediterranean populations, mainly non-Ashkenazi Jews, Armenians, Turks, and Arabs. Since a large proportion of all the FMF patients in the world live in Turkey, the Turkish FMF Study Group (FMF-TR) was founded to develop a patient registry database and analyze demographic, clinical, and genetic features. The cohort was composed of 2838 patients (mean age, 23.0 +/- 13.33 yr; range, 2-87 yr), with a male:female ratio of 1.2:1. ⋯ There was a high rate of a past diagnosis of acute rheumatic fever, which suggested a possible misdiagnosis in children with FMF presenting with recurrent arthritis. To our knowledge, this is the largest series of patients with FMF reported from 1 country. We describe the features of the disease in the Turkish population and show that amyloidosis is still a substantial problem.