Kidney international
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Kidney international · Sep 2004
Case ReportsTwo novel ADAMTS13 gene mutations in thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome (TTP/HUS).
Thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) are now considered to be variants of one single syndrome called thrombotic thrombocytopenic purpura/hemolytic-uremic syndrome (TTP/HUS). Key features are thrombocytopenia, hemolytic anemia, and subsequently impaired function of different organs, especially the kidneys and the central nervous system (CNS). One possible reason is the deficiency of von Willebrand factor-cleaving protease (vWF-CP) resulting in persistence of uncleaved, ultralarge von Willebrand factor multimers (ULvWFM). ⋯ Enteropathogenic E. coli- and Shiga-like toxin-negative patients who present with hemolytic or thrombocytopenic episodes and HUS like symptoms should be tested for vWF-CP deficiency and other noninfectious reasons for TTP/HUS since plasma substitution possibly provides an efficient therapeutic option for this subgroup of patients.
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Kidney international · Sep 2004
Epidemiologic data of renal diseases from a single unit in China: analysis based on 13,519 renal biopsies.
Renal biopsy specimens of 13,519 cases were collected during the period of January 1979 to December 2002 from the Research Institute of Nephrology, Nanjing University School of Medicine, Nanjing, China. Analysis of the data of this group of patients showed that primary glomerulonephritis (GN) remained the most important and prevalent renal disease in China. The ratio of primary to secondary GN was 2.75:1. ⋯ Membranous nephropathy was 9.89% and minimal change disease was 0.93%, remarkably less. The most prevalent etiology of secondary GN was systemic lupus erythrematosus (SLE) (54.3%) followed by Henoch-Schönlein purpura (20.3%), diabetic nephropathy (6.6%), systemic vasculitides (4.0%), and amyloidosis (2.2%). Based on the study of biopsy materials obtained from 607 cases manifesting chronic renal failure, IgAN was identified as the most frequent cause (26.69%) of chronic renal failure.
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Kidney international · Aug 2004
Clinical TrialThe urine-blood PCO gradient as a diagnostic index of H(+)-ATPase defect distal renal tubular acidosis.
Urine pH during acidemia and urine PCO2 upon alkalization both may be useful to indicate H+ secretion from collecting ducts. The urine anion gap has been used to detect urinary NH4+ for differential diagnosis of hyperchloremic metabolic acidosis. We have previously demonstrated that the lack of normal H(+)-ATPase may underlie secretory defect distal renal tubular acidosis (dRTA). In this study we evaluated the diagnostic value of the urine-blood (U-B) PCO2 in H(+)-ATPase defect dRTA, and compared it with that of urine pH and urine anion gap during acidemia. ⋯ The U-B PCO2 during NaHCO3 loading is an excellent diagnostic index of H(+)-ATPase defect dRTA.
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Kidney international · Aug 2004
EPO and alpha-MSH prevent ischemia/reperfusion-induced down-regulation of AQPs and sodium transporters in rat kidney.
Ischemia-induced acute renal failure (ARF) is known to be associated with significant impairment of urinary concentrating ability and down-regulation of renal aquaporins (AQPs) and sodium transporters in rats. We tested whether treatment with erythropoietin (EPO) or alpha-melanocyte-stimulating hormone (alpha-MSH) in combination with EPO reduces the renal ischemia/reperfusion (I/R) injury and prevents the down-regulation of renal AQPs and major sodium transporters. ⋯ EPO and/or alpha-MSH treatment significantly prevent I/R-induced injuries such as urinary-concentrating defects and down-regulation of renal AQPs and sodium transporters.
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Kidney international · Aug 2004
ReviewEndothelial injury and dysfunction: role in the extension phase of acute renal failure.
The pathophysiology of ischemic acute renal failure (ARF) involves a complex interplay between renal hemodynamics, tubular and endothelial cell injury, and inflammatory processes. A growing body of evidence supports the contribution of altered renal vascular function, especially at the microvascular level, in initiating and subsequently extending the initial tubular injury. The extension phase of ischemic ARF involves continued reduction in renal perfusion, ongoing hypoxia, and inflammatory processes that occur during reperfusion and contribute to continued tubular cell injury. ⋯ Vascular congestion, edema formation, diminished blood flow, and infiltration of inflammatory cells have been documented in the corticomedullary junction of the kidney. However, linking their genesis to microvascular endothelial injury and dysfunction has been difficult. New diagnostic and therapeutic approaches to ischemic ARF must incorporate these finding to devise early recognition strategies and therapeutic approaches.