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Background.?Hyper-IgD and periodic fever syndrome (HIDS) is an autosomal recessively inherited disorder characterized by recurrent episodes of fever and inflammation. Unlike other chronic inflammatory conditions, amyloidosis is very rare in HIDS. For deposition of amyloid of the AA type, high concentrations of SAA are a prerequisite, together with certain SAA1 gene polymorphisms. The SAA1.1 genotype predisposes for amyloidosis, while SAA1.5 genotype exerts a protective effect.Aim of the study.?To determine if SAA concentrations and SAA1 gene polymorphisms could explain the virtual absence of amyloidosis in HIDS patients.Methods.?We measured SAA and CRP concentrations in serum of 20 HIDS patients during an attack and during the asymptomatic phase. Genotype of SAA1 gene was determined in 60 HIDS patients.Results.?SAA serum concentrations during attacks were very high (median 205?mg/l; range 75–520?mg/l, normal?<?3.1?mg/l). During attack-free periods 45% of patients still had elevated SAA concentrations. The distribution of the genotype of SAA1 gene in HIDS was similar to healthy controls (SAA1.1 0.41 vs. 0.50 p?=?0.32).Conclusion.?Patients with HIDS have high SAA during attacks and show sub-clinical inflammation when asymptomatic. The low incidence of amyloidosis cannot be explained by a predominance of non amyloidogenic SAA related genotypes. 相似文献
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Hepatic parenchymal gas was demonstrated by computed tomography in a boy who had sustained severe blunt trauma to the abdomen 12 hours earlier. There was no clinical evidence of infection. Although previous reports have suggested that hepatic parenchymal gas indicates the presence of infection, such gas may also be a manifestation of severe blunt trauma without infection. 相似文献
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DA O'Sullivan VE Torres PA Gabow SN Thibodeau BF King EJ Bergstralh 《American journal of kidney diseases》1998,32(6):976-983
Recent experiments in cultured cyst epithelial cells from kidneys of patients with autosomal dominant polycystic kidney disease (ADPKD) have shown that the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) is present in the apical surface of these cells and mediates chloride (Cl-) and fluid secretion in vitro. To determine whether the presence of CF with the expression of mutated CFTR proteins modifies cyst formation in ADPKD, we studied a large family with both inherited diseases. ADPKD in this family is linked to PKD1. The family is composed of 26 members; 11 members with ADPKD, 4 members with CF, and 2 members with both diseases. Renal volumes measured by computerized tomography (CT), calculated creatinine clearances, and other clinical parameters in the family members with ADPKD and CF were compared with those in the family members with ADPKD alone, as well as to a large population of patients with ADPKD. The patients with CF and ADPKD, but not the CF heterozygote carriers with ADPKD, had less severe polycystic kidney and liver disease, as indicated by normal renal function; smaller renal volume, even when corrected for height and body surface area; and the absence of hypertension and liver cysts. These observations suggest that the coexistence of CF may reduce the severity of ADPKD. 相似文献
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We examined differences in approaches to HIV-related infection control practices in two university teaching hospitals in the United States and Nigeria. Health care workers (n = 202 in Nigeria and 186 in the USA) responded to a previously validated measure of infection control practices. There were significant differences in the estimated probability of treating a person with HIV disease (higher in USA), and a greater probability of peer ridicule as a way of enforcing group norms on infection control in Nigeria. Peer enforcement of norms was significantly lower in the USA. In both countries, more precautions would be taken if it was known that the patient was HIV infected. Infection control practices were more likely to be followed in the USA compared with Nigeria if they were praised for this activity, if appropriate facilities (sharps containers, gloves, etc.) were nearby, and if they felt that infection control procedures were effective. These data point to the importance of normative social pressures in Nigeria and of knowing the patient is HIV infected in the USA and feeling that infection control procedures are effective ways of avoiding occupational HIV infection. The role of normative pressures and assumptions about HIV infection status as well as cues and availability of facilities for infection control appear to differ between these health care workers in Nigeria and the USA. 相似文献
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P. M. King J. M. Blazeby† P. Ewings R. J. Longman R. M. Kipling‡ P. J. Franks†† J. P. Sheffield§ L. B. Evans M. Soulsby S. H. Bulley¶ R. H. Kennedy 《Colorectal disease》2006,8(6):506-513
OBJECTIVE: Optimizing peri-operative care using an enhanced recovery programme improves short-term outcomes following colonic resection. This study compared a prospective group of patients undergoing resection of colorectal cancer within an enhanced recovery programme, with a prospectively studied historic cohort receiving conventional care. PATIENTS AND METHODS: Sixty patients underwent elective resection within an enhanced recovery programme (ERP). This incorporated pre-operative counselling, epidural analgesia, early feeding and mobilization. Clinical outcomes were compared with 86 prospectively studied historic control patients receiving conventional care (CC). All patients completed EORTC QLQ-C30, QLQ-CR38 and health economics questionnaires up to three months after surgery. RESULTS: Baseline clinical data were similar in both groups. Postoperative hospital stay was significantly reduced in the ERP, with patients staying 49% as long as those in the CC group including convalescent hospital stay (95% CI 39% to 61%P < 0.001). There were no differences in the number of complications, readmissions or re-operations. There were no significant differences in quality of life or health economic outcomes. CONCLUSION: Patients undergoing colorectal resection within an ERP stay in hospital half as long as those receiving conventional care, with no increased morbidity, deterioration in quality of life or increased cost. 相似文献
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Three, six-month-old dogs were infected with 2,000 eggs ofToxocara canis. Three weeks after infection 15 immature adult parasites were found in the intestine of the dog which had been treated with high doses of corticosteroids (2 ml of Opticortenol S given every four days) while no parasites were found in the intestine of the dog treated with repeated injections of 0.3 ml Opticortenol S or in the untreated control dog. The increased susceptibility to infection withT. canis in the dog treated with high doses of corticosteroids was associated with a decrease in in vitro lymphocyte responsiveness to the mitogens concanavalin A and pokeweed mitogen and with a complete suppression of the eosinophilia that was seen 10 days after infection in the untreated control dog. 相似文献