Mildly elevated serum creatinine concentration correlates with the extent of coronary atherosclerosis

Mildly elevated serum creatinine concentration was proposed to be a marker for increased risk of cardiovascular disease mortality. The aim of our prospective study was to evaluate a possible association between serum creatinine concentration and extent of coronary atherosclerosis together with conventional risk factors for atherosclerosis. Serum creatinine concentration was measured in 40 male patients without overt renal or ischemic renal disease (mean age 53 +/- 7 years) with stable or unstable angina undergoing routine coronary arteriography. The extent of coronary atherosclerosis was assessed by Gensini score. In univariate linear regression analysis Gensini score significantly correlated with serum concentrations of apolipoprotein AII (r=-0.3242, P<0.05) and creatinine (r=+0.3194, P<0.05), but not with serum concentrations of lipids (total, low- and high-density lipoprotein cholesterol, triglycerides), other apolipoproteins (apo B, apo AI), lipoprotein(a), autoantibodies to oxidatively modified low-density lipoprotein or age, weight and status of smoking, diabetes or hypertension. Multivariate linear regression analysis revealed that elevated serum creatinine was associated with the extent of coronary atherosclerosis independently of conventional risk factors for atherosclerosis. Mildly elevated serum creatinine was probably the marker of generalised vascular disease denoting early nephrovasculopathy in correlation with established atherosclerotic risk factors.     

Transitional cell carcinoma involving the ureterovesical part of a transplanted ureter

Ureterovesical malignancy in the renal transplant recipient is an infrequent occurrence. We report a woman with a cadaver kidney recipient, transplanted 10 years ago, with transitional-cell carcinoma at the ureterovesical part of the transplanted ureter invading the bladder muscle around the orifice. Though aggressive surgery and chemotherapy of such patients is proposed, most patients die after an average of 16-17 months. In our patient we have done partial ureter and bladder resection in order to preserve kidney graft and bladder. We continued immunosuppressive treatment. The patient has survived 16 months and is symptom free. Our case suggests that nonaggressive operative treatment without chemotherapy in selected patients may provide comparable survival to patients with aggressive treatment, but with better quality of life.     

Xanthogranulomatous pyelonephritis in nonfunctioning transplanted kidney

Xanthogranulomatous pyelonephritis in native and allografted kidneys is a rare variant of severe chronic infection of the renal parenchyma. In a native kidney the diagnosis may sometimes be established by ultrasonography and computed tomography. In the renal allograft, the diagnosis could only be established by histologic evaluation of the transplant biopsy or nephrectomy. The reported case presents a febrile patient with a failing renal graft, in whom xanthogranulomatous pyelonephritis was established by histologic evaluation of transplantectomy specimens. Xanthogranulomatous pyelonephritis should therefore be included in the list of possible etiologies of the fever in patient with nonfunctioning transplanted kidney.     

Antineutrophil cytoplasmic autoantibodies-antigen specificity and associated diseases

Antineutrophil cytoplasmic antibodies (ANCA) are widely used as a useful diagnostic marker for small vessel vasculitides, although the test may occasionally be positive in various other conditions. The aim of this study was to assess ANCA in various clinical-pathological settings. ANCA were tested by indirect immunofluorescence and enzyme-linked immunosorbent assay and were found to be positive in 423 patients in the period from 1989-1999. Patients were grouped in accordance with their clinical-pathological setting as follows: 1. pauci-immune vasculitis confirmed by biopsy (n = 151), 2. clinically suspected vasculitis (n = 59), 3. inflammatory bowel diseases and autoimmune hepato-biliary disorders (n = 83), and 4. miscellaneous diseases (n = 130). The association of proteinase 3 ANCA with Wegener's granulomatosis (45/56) and myeloperoxidase ANCA with microscopic polyangiltis (45/54) and pauci-immune necrotising glomerulonephritis (24/28) was established. However, ANCA with other specificities were also shown to be present in these forms of vasculitides. ANCA, specific mostly for myeloperoxidase but also for other or unknown ANCA antigens, frequently revealing atypical immunofluorescence patterns, were characteristically found in other diseases. The titres of ANCA were significantly higher (p < 0.05) in patients with pauci-immune vasculitis than in those with clinically suspected vasculitis and other diseases. In conclusion, well standardised techniques for ANCA testing in conjunction with the clinical picture and histopathologic findings, if available, may significantly contribute to the diagnosis of small vessel vasculitides.     

Correlation between glomerular filtration and markers of the atherosclerotic process in young patients with myocardial infarction

In 41 young post myocardial infarction (MI) patients we investigated the relations between glomerular filtration rate (GFR) and markers of atherosclerosis: flow-mediated dilation of the brachial artery (FMD), intima-media thickness (IMT) of the common carotid arteries and high sensitive C-reactive protein (hsCRP). GFR was within normal values in all patients. Importantly, we found that GFR was significantly related to all three markers. Thus, our study supports the idea of the generalized nature of the atherosclerotic process and early involvement of the kidney.     

Sixteen novel mutations identified in COL4A3, COL4A4, and COL4A5 genes in Slovenian families with Alport syndrome and benign familial hematuria

Alport syndrome (ATS) and benign familial hematuria (BFH) are type IV collagen inherited disorders. Mutations in COL4A5 are generally believed to cause X-linked ATS, whereas mutations in COL4A3 and COL4A4 genes can be associated with the autosomal-recessive and -dominant type of ATS or BFH. In view of the wide spectrum of phenotypes, an exact diagnosis is sometimes difficult to achieve. This study involved screening each exon with boundary intronic sequences of COL4A3, COL4A4, and COL4A5 genes by optimized polymerase chain reaction-single-stranded conformational polymorphism analysis in 17 families with ATS and in 40 families diagnosed as having BFH. Twelve different mutations were found in the COL4A5 gene in ATS patients, comprising nine missense mutations, a splice site mutation, a mutation causing frameshift, and a nonsense mutation. One of the missense mutations (p.G624D) was present not only in one family with ATS but also in five families with suspected BFH. Three heterozygous mutations in the COL4A3 gene (two missense and one frameshift) and four heterozygous mutations in COL4A4 (two splice site, one in-frame deletion, and one missense) were identified in patients with BFH. Sixteen mutations are to the best of our knowledge new and private.