摘要:

Crohn disease and familial Mediterranean fever (FMF) are inflammatory diseases characterized by abdominal pain and fever. The concurrence of the 2 diseases (FMF-CD) may pose a challenge to diagnosis and treatment. We undertook the present study to determine the prevalence of Crohn disease in FMF and to characterize FMF-CD patients clinically and genetically. Using a computerized search, the patients of our FMF clinic were screened for a concomitant diagnosis of Crohn disease. Patients and their medical records were thoroughly examined, and their DNA was genotyped for mutations in the MEFV gene. Control groups of ethnically and sex-matched patients suffering from each of the diseases alone, either Crohn disease or FMF, were used for comparison. We identified 7 patients with concomitant Crohn disease and FMF, which is more than the expected prevalence in the general population (p = 0.03). Crohn disease presented at a significantly later age in the FMF-CD group (40.6 ± 10.0 yr versus 26.2 ± 11.4 yr; p < 0.004). Disease severity and other characteristics of Crohn disease were comparable to the Crohn disease control group. Contrary to the FMF control group patients, FMF in FMF-CD patients was characterized by a higher attack frequency (p < 0.05) and increased prevalence of amyloidosis (p < 0.02). The overall severity score was similar in both groups. In conclusion, Crohn disease appears to be more prevalent in FMF and presents later than in patients without FMF. FMF in this group of patients shows a higher attack frequency and is more often complicated by amyloidosis.

ISSN:0025-7974    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of primary pulmonary hypertension characterized by thin-walled microvessels infiltrating the peribronchial and perivascular interstitium, the lung parenchyma, and the pleura. These proliferating microvessels are prone to bleeding, resulting in accumulation of hemosiderin-laden macrophages in alveolar spaces. Here we report 2 cases of PCH with pulmonary hypertension, 1 of them associated with mechanical intravascular hemolysis, a feature previously reported in other hemangiomatous diseases, but not in PCH. Case 2 was diagnosed by pulmonary biopsy; to our knowledge the patient is the second adult to be treated with interferon α-2a.Review of the literature identified 35 patients with PCH and pulmonary hypertension. The prognosis is poor and median survival was 3 years from the first clinical manifestation. Dyspnea and right heart failure are the most common findings of the disease. Hemoptysis, pleural effusion, acropachy, and signs of pulmonary capillary hypertension are less common. Chest X-ray or computed tomography scan usually shows evidence of interstitial infiltrates, pulmonary nodules, or pleural effusion. Hemodynamic features include normal wedge pressures. Radiologic and hemodynamic findings are undifferentiated from those of pulmonary veno-occlusive disease but differ from other causes of primary pulmonary hypertension.Epoprostenol therapy, considered the treatment of choice in patients with primary pulmonary hypertension, may produce pulmonary edema and is contraindicated in patients with PCH. Regression of lesions was reported in 1 patient treated with interferon therapy and 2 other patients stabilized, including our second patient. PCH was treated successfully by lung transplantation in 5 cases. Early recognition of PCH in patients with suspected primary pulmonary hypertension is possible based on clinical and radiologic characteristics. Diagnosis by pulmonary biopsy is essential for allowing appropriate treatment.

ISSN:0025-7974    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

The incidence of fungal infections has been increasing for the last 3 decades, especially among neutropenic, cancer, and critically ill patients. These infections are associated with high mortality rates. We retrospectively reviewed medical charts of adult patients with fungemia from 1989 to 2000 at our institution. The characteristics of the population groups served by the hospital were described. Of 328 patients with fungemia, we reviewed 315 (96%) medical records, and focused on those with candidemia (n = 294). The species distribution in patients with candidemia showed that the most commonly identified species wereCandida albicans(66%), followed byC. glabrata(17%), andC. parapsilosis(6%).The incidence of candidemia ranged from 0.2 to 0.46 per 10,000 patient-days with the highest incidence in 1993 and the lowest in 1997. Although most studies show an increased incidence of candidemia, we observed a reduction over the study period. Furthermore, we observed no shift fromC. albicansto non-albicansCandidaspecies despite a significant increase in the use of fluconazole. The overall mortality among patients with candidemia was 44%, with the highest rate in patients over 65 years (52%). Factors independently associated with higher mortality were patient age greater than 65 years, intensive care unit admission, and underlying cancer.

ISSN:0025-7974    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Patients with suspected non-ST-segment elevation acute coronary syndromes (NSTEACS) constitute a heterogeneous population with variable outcomes. Risk stratification in this population of patients is difficult due to the complexity in patient risk profile. We conducted this study to characterize the value of clinical and electrocardiographic variables for risk stratification in an unselected population of consecutive patients with NSTEACS on admission. Thirty-five clinical and electrocardiographic variables at presentation in the emergency room of 18 hospitals were prospectively analyzed in 4,115 patients with NSTEACS and related with the outcomes at 90 days. We also developed a risk score using the variables found to be independent predictors of ischemic events to facilitate risk stratification.Cardiovascular mortality was 4.3% and the rate for the outcome of either cardiovascular death or nonfatal myocardial infarction was 6.9%. The only independent predictors of mortality were age, diabetes, peripheral vascular disease, postinfarction angina, Killip class ≥2, ST-segment depression, and elevation of cardiac markers. A risk profile using the variables found to be independent predictors of events was calculated for cardiovascular mortality and for the combination of either death or nonfatal myocardial infarction. Event rates increased significantly in all subgroups of patients based on the number of independent risk factors as the risk score increased. Using these factors, 90-day mortality ranged from as low as 0.4% in patients with no risk factors to 21.1% for those with more than 4 risk factors.In conclusion, simple clinical and electrocardiographic data obtained at hospital admission allow an accurate risk stratification of patients with NSTEACS. In the PEPA registry, simple variables easy to obtain at admission appear to be a valuable tool in discerning between patients at very low and very high risk according to the cluster of factors for each patient. The risk score developed was obtained from an unselected population, representative of the whole spectrum of patients with NSTEACS, allowing identification of patients at different risks for adverse outcomes, and, therefore, permitting optimization of therapy.

ISSN:0025-7974    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

We report 12 cases of Whipple disease in patients with prominent neurologic symptoms, along with 122 cases of Whipple disease with nervous system involvement reported in the literature. We analyzed the clinical signs and results of additional examinations in 2 groups: the first group included patients with predominantly but not exclusively neurologic signs, and the second included patients with clinically isolated neurologic presentation of the disease. Whipple disease is a multisystemic infectious disease due toTropheryma whippeliithat may present with prominent or isolated symptoms of either the central or the peripheral nervous system. Recent reports stress the importance of polymerase chain reaction (PCR) analysis of cerebrospinal fluid, magnetic resonance imaging (MRI) during follow-up, and prolonged antibiotic therapy with drugs able to cross the blood-brain barrier.Cerebrospinal fluid should be analyzed repeatedly during follow-up, and treatment should be discontinued only when the results of PCR assay performed on cerebrospinal fluid are negative. Other examinations to be done include searching for gastrointestinal tract involvement with multiple duodenal biopsies and searching for systemic involvement with lymph node biopsies, which should be analyzed with light microscopy, electron microscopy, and PCR. When all examinations are negative, if Whipple disease is suspected and a lesion is found on brain MRI, a stereotactic cerebral biopsy should be performed. Treating Whipple disease with long-term trimethoprim-sulfamethoxazole is usually effective, but the use of third-generation cephalosporins in case of incomplete response deserves further attention.

ISSN:0025-7974    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Lemierre syndrome (postanginal septicemia) is caused by an acute oropharyngeal infection with secondary septic thrombophlebitis of the internal jugular vein and frequent metastatic infections. A high degree of clinical suspicion is necessary for diagnosis.Fusobacterium necrophorumis the usual etiologic agent. The disease progresses in several steps. The first stage is the primary infection, which is usually a pharyngitis (87.1% of cases). This is followed by local invasion of the lateral pharyngeal space and IJV septic thrombophlebitis (documented in 71.5% of cases), and finally, the occurrence of metastatic complications (present in 90% of cases at the time of diagnosis). A sore throat is the most common symptom during the primary infection (82.5% of cases). During invasion of the lateral pharyngeal space and IJV septic thrombophlebitis, a swollen and/or tender neck is the most common finding (52.2% of patients) and should be considered a red flag in patients with current or recent pharyngitis. The most common site of metastatic infection is the lungs (79.8% of cases). In contrast to the preantibiotic era, cavitating pneumonia and septic arthritis are now uncommon. Most patients (82.5%) had fever at some stage during the course of the disease. Gastrointestinal complaints such as abdominal pain, nausea, and vomiting were common (49.5% of cases). An elevated white blood cell count occurred in 75.2% of cases. Hyperbilirubinemia with slight elevation of liver enzyme levels occurred in one-third of patients, but frank jaundice was uncommon, in contrast to its high frequency reported in the preantibiotic era. We conclude that, most likely as a consequence of widespread antibiotic use for pharyngeal infections, the typical course of the disease has changed since Lemierre’s original description. The typical triad in our series was: pharyngitis, a tender/swollen neck, and noncavitating pulmonary infiltrates. The previous classical description of severe sepsis with cavitating pneumonia and septic arthritis was not commonly seen in our review. Mortality was low in our series (6.4%), but significant morbidity occurred, which was likely preventable by early diagnosis and treatment. The pathophysiology, natural history, diagnostic methods for internal jugular vein thrombosis, and management are discussed.

ISSN:0025-7974    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Strategies for preventing central venous catheter (CVC)-related bloodstream infection are most likely to be effective if guided by an understanding of the risk factors associated with these infections. In this critical review of published studies of risk factors for CVC-related bloodstream infection that were prospective and used multivariable techniques of data analysis or that were randomized trials of a preventive measure, a significantly increased risk of catheter-related bloodstream infection was associated with inexperience of the operator and nurse-to-patient ratio in the intensive care unit, catheter insertion with less than maximal sterile barriers, placement of a CVC in the internal jugular or femoral vein rather than subclavian vein, placement in an old site by guidewire exchange, heavy colonization of the insertion site or contamination of a catheter hub, and duration of CVC placement > 7 days. Prospective studies or randomized trials of control measures focusing on these risk factors have been shown to reduce risk significantly: formal training in CVC insertion and care, use of maximal sterile barriers at insertion, use of chlorhexidine rather than povidone-iodine for cutaneous antisepsis, applying a topical anti-infective cream or ointment or a chlorhexidine-impregnated dressing to the insertion site, and the use of novel catheters with an anti-infective surface or a contaminationresistant hub.Better prospective studies of sufficient size to address all potential risk factors, including insertion site and hub colonization, insertion technique, and details of follow-up care, would enhance our understanding of the pathogenesis of CVC-related bloodstream infection and guide efforts to develop more effective strategies for prevention.

ISSN:0025-7974    

出版商:OVID    

年代:2002

数据来源: OVID