Medicine
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Hypothenar hammer syndrome (HHS) is an uncommon form of secondary Raynaud phenomenon, occurring mainly in subjects who use the hypothenar part of the hand as a hammer; the hook of the hamate strikes the superficial palmar branch of the ulnar artery in the Guyon space, leading to occlusion and/or aneurysm of the ulnar artery. In patients with HHS, such injuries of the palmar ulnar artery may lead to severe vascular insufficiency in the hand with occlusion of digital artery. To date, only a few series have analyzed the long-term outcome of patients with HHS. ⋯ Awareness of HHS is required to increase suspicion of the disorder so that further exposure to risk factors like repetitive hypothenar trauma can be avoided for these patients; this is of great importance for their overall prognosis. We found favorable outcomes in most patients after conservative measures were initiated; therefore we suggest that surgery may be undertaken in the subgroup of patients who exhibit partial improvement while receiving conservative therapy. Finally, because we observed recurrence of HHS in 27.7% of patients, we note that HHS patients require close follow-up, including both regular and systematic physical vascular examination.
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Enterococci are the third leading cause of infectious endocarditis, and despite advances in diagnosis and treatment, the mortality of enterococcal endocarditis has not changed in recent decades. Although variables such as advanced age, cardiac failure, and brain emboli have been recognized as risk factors for mortality, cooperative multi-institutional studies have not assessed the role of other variables, such as nosocomial acquisition of infection, the presence of comorbidities, or the changing antimicrobial susceptibility of enterococci, as factors determining prognosis. We conducted the current study to determine the risk factors for mortality in patients with enterococcal endocarditis in a single institution. ⋯ In the multivariate analysis, only nosocomial acquisition increased the risk of mortality. In patients with enterococcal endocarditis, nosocomial acquisition of infection is an important factor determining outcome. As the incidence of bacteremia and the population of elderly people at risk continue to grow, the hazard of acquiring nosocomial enterococcal endocarditis may increase; hence, major emphasis must be put on prevention.
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We conducted a retrospective review to assess outcomes of therapy in patients with newly diagnosed Wegener granulomatosis (WG) using methotrexate (MTX) for mild to moderate disease and short-term treatment with cyclophosphamide (CYC) followed by MTX for severe disease. Patients with WG were included if their initial plan of therapy and subsequent care were directly supervised by the Cleveland Clinic Center for Vasculitis Care and Research. Severe disease (immediately life-threatening or involving critical organs) was initially treated with CYC and glucocorticoids. ⋯ In patients with WG, a strategy that limits or avoids CYC therapy produced a frequency of remission comparable to that achieved with conventional CYC protocols, excellent survival, and avoidance of long-term CYC toxicity. However, relapses were common and incremental permanent morbidity occurred in most patients. While not a goal of therapy, when treatment produced marked lymphopenia, prolonged remissions were more likely.
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Primary Sjögren syndrome (pSS) is a fairly common autoimmune disease with glandular and extraglandular manifestations. Pulmonary involvement mainly corresponds to small airways and interstitial lung disease. Pulmonary arterial hypertension (PAH) is rare: to our knowledge, only 32 cases have been reported in pSS patients to date. ⋯ These data suggest that systemic vasculopathy, B-cell activation, and autoimmunity could play a role in the pathophysiology of pSS-associated PAH. In conclusion, this report underlines the rarity and severity of PAH in pSS patients. The best therapeutic regimen remains to be defined but should include standard PAH therapy and/or immunosuppressants.
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Comparative Study
Molecular diagnosis of infective endocarditis by real-time broad-range polymerase chain reaction (PCR) and sequencing directly from heart valve tissue.
Traditionally, infective endocarditis (IE) has been microbiologically diagnosed by blood cultures or serology. However, conventional microbiologic methods do not always provide an etiologic diagnosis. We conducted the current study to evaluate the usefulness of a universal real-time polymerase chain reaction (PCR) of the 16S rRNA gene followed by sequencing for the diagnosis of IE in explanted heart valve tissue (HV) as part of the routine of a clinical microbiology laboratory, and to compare it with conventional culture of blood or HV. ⋯ Our universal PCR method has proved to be more sensitive, specific, and rapid than conventional culture methods, and should therefore be included as a new major Duke criterion for the diagnosis of IE. According to the results of the current study, this technique should be used to supplement blood and HV culture. Conventional HV cultures are frequently responsible for false-positive and false-negative results, and are not always useful to establish the etiology of IE.