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Familial Mediterranean fever (FMF) is an autosomal recessive hereditary disease characterized by recurrent attacks of fever, usually accompanied by sterile polyserositis. Although amyloidosis is the most common renal involvement, non-amyloid renal lesions, such as glomerulonephritis, have been described in patients with FMF. In this report, we present the first case of an FMF patient with heterozygous mutation of E148Q, mesangial proliferative glomerulonephritis, and no amyloidosis. While the association of mutation E148Q with renal involvement is still obscure, colchicine treatment is useful in mesangial proliferative glomerulonephritis with FMF.  相似文献   
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BACKGROUND: Moderate hyperhomocysteinemia is an independent risk factor for cardiovascular disease. Cyclosporine (CsA) has been suggested to interfere with folate-assisted remethylation of homocysteine, thus causing hyperhomocysteinemia. But, this issue is controversial. In this experimental study, we attempted to determine the association between CsA administration and total homocysteine levels. Working with rabbits that have normal creatinine levels, we obviated the misleading effects of renal functional variations, which are the most important confounding factors affecting total homocysteine level. METHODS: Male New Zealand rabbits fed a standard quantity of diet received 10 days of subcutaneous injections of 10 mg/kg per day CsA. After these loading doses, CsA (20 mg/kg) was administered subcutaneously three times a week for 20 days. After first 30 days, the rabbits were followed for another 30 days without CsA therapy. Plasma creatinine, BUN, and total homocysteine levels were measured on days 0, 10, 30, and 60. RESULTS: There were no significant changes in BUN results on days 0, 10, 30, and 60 (P > .05). There was a slight, but significant, increase in mean creatinine levels during CsA administration (P < .01). However, the mean creatinine levels remained in the normal ranges during the 60 days of study. No significant changes were observed in total homocysteine levels (P > .05) compared to baseline, 10-, 30-, and 60-day values. CONCLUSION: Our experimental research minimized confounding factors. It showed that CsA does not increase total homocysteine levels, confirming clinical studies that reported no association between CsA and total homocysteine.  相似文献   
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We conducted three prospective studies of Haemophilus influenzae in different groups of children. Pharyngeal swab samples were taken (i). from 1382 healthy infants and children between 0 and 10 years of age (group 1), attending well child clinics (n=438), day care centres (n=440) and elementary schools (n=504), and (ii). from 322 children aged 2-10 years (group 2), clinically diagnosed as having upper respiratory tract infection. Pharyngeal swab samples and sinus aspirates were obtained from 49 children between 2 and 9 years of age (group 3), clinically diagnosed as having sinusitis. H. influenzae was isolated in similar rates from 315 (22.7%) of children in group 1, 72 (22.3%) of children in group 2 and 12 (24.4%) of children in group 3. Serotype b comprised 7, 5.2 and 2% of all H. influenzae isolates for group 1, 2 and 3, respectively. Production of beta-lactamase was detected in 1.0% of H. influenzae type b isolates in group 1, 1.2 and 6.1% of all isolates in group 2 and 3, respectively. There were no beta-lactamase negative ampicillin-resistant strains.  相似文献   
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Background: Cardiovascular (CV) disease is a major cause of morbidity and mortality in patients with end-stage renal disease. In recent years, arterial stiffness has taken on great importance in the pathophysiology of CV diseases. The independent predictive value of arterial stiffness for CV events and for all-cause and CV mortality has been demonstrated in the general population and in hemodialysis patients. Our aim in this study was to determine the relationship of arterial stiffness with mortality and fatal and nonfatal CV events in peritoneal dialysis (PD) patients.♦ Methods: In this prospective observational cohort study with 2 years of follow-up, we studied a cohort of 156 PD patients with a mean follow-up of 19.2 ± 6.4 months. At baseline, echocardiography and standard clinical and biochemical analyses were performed in all patients and in 28 healthy subjects. Aortic stiffness index beta (ASIβ, a surrogate marker of arterial stiffness) was calculated as follows: ♦ Results: During the follow-up period, 25 of the patients (16.0%) died, and 10 of those deaths had CV causes. Nonfatal CV events occurred in 15 patients. The median ASIβ was greater in PD patients than in control subjects (4.2 vs. 3.5; interquartile range: 3.2 – 5.5 vs. 2.5 – 4.8; p = 0.028]. In the fully adjusted multivariate Cox regression analysis (co-variates: age, sex, albumin, hemoglobin, diabetes mellitus, comorbid CV disease, left ventricular mass index, residual glomerular filtration rate, dialysate-to-plasma ratio of creatinine, Kt/V urea, left ventricular ejection fraction, duration of dialysis, smoking), ASIβ independently predicted fatal and nonfatal CV events (hazard ratio: 1.239; 95% confidence interval: 1.103 to 1.392), but not all-cause mortality.♦ Conclusions: Our results provide the first direct evidence that arterial stiffness is an independent risk predictor of adverse CV outcome in PD patients.  相似文献   
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Background

The pathogenesis of post-transplantation erythrocytosis (PTE) is not well understood and appears to be multifactorial. Our hypothesis in this study was that several factors, including toxicity of calcineurin inhibitor, immunologic factors, and chronic allograft nephropathy, can trigger local tissue hypoxia in peritubular interstitium, which is where production of erythropoietin (EPO) takes place. This local interstitial tissue hypoxia can cause an increase in renal EPO production, which induces the development of PTE.

Methods

This cross-sectional study included 15 renal transplant recipients, in whom polycythemia developed after kidney transplantation, with elevated hematocrit level to >51%. Forty-eight age- and gender-matched renal transplant recipients with normal hematocrit level were included as the renal transplant control group. In addition, 13 age- and gender-matched healthy subjects were also included as the healthy control group. We used urine hypoxia-inducible factor-2 alpha (HIF-2α) levels to evaluate whether there is local tissue hypoxia in renal allograft. HIF-2α levels were measured by double antibody sandwich enzyme-linked immunosorbent assay (ELISA). Serum EPO and insulin-like growth factor-1 (IGF-1) levels were also measured.

Results

HIF-2α levels were significantly lower in the polycythemia group than the other two groups, but there was no significant difference between the healthy control group and the renal transplant control group with regard to HIF-2α levels. There was no significant difference among the 3 study groups in terms of levels of serum EPO and IGF-1.

Conclusion

Local tissue hypoxia in renal allograft does not seem to play an important role in the development of PTE.  相似文献   
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The aim of the current study was to determine the presence of styloid process elongation (SPE) detected on panoramic radiographs in patients with torus palatinus (TP). Between December 2005 and November 2007, a total of 149 patients with TP are investigated for routine dental examination in the outpatient clinic. Any patient who had disorders related to calcium and phosphorus metabolism was excluded. All medical data were obtained from the files. Twenty (15%) subjects demonstrated SPE at least one side. These patients consisted of 15 women (14.3% of all women) and five men (17.8% of all men). In our previous report performed in normal population at the same region, the prevalence had been found to be 7.7% in 698 dental patients. Although the number of the patients is different in both studies, there is a marked difference in terms of the SPE prevalence between the two reports. This prevalence difference might be related to concomitant disorder.  相似文献   
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