Volume 5 Issue 6, June 2009

Cinacalcet is an effective treatment for secondary hyperparathyroidism in patients on dialysis. Until now, no randomized, placebo-controlled, long-term trial has tested this drug in individuals with chronic kidney disease who are not receiving dialysis.

An analysis of the US kidney transplant waiting list suggests that recording a potential recipient as 'inactive', during which time he or she cannot be offered a donor organ, is becoming increasingly common and is not in patients' best interests.

One of the early products of the Chronic Kidney Disease in Children (CKiD) study has been an updated equation for estimating GFR in children on the basis of demographic and laboratory parameters. This formula is comparable in form and performance to the MDRD equations for adults in clinical trials first published a decade ago.

Autosomal-dominant polycystic kidney disease is characterized by the development and expansion of cysts, which ultimately results in kidney failure. The rate of this expansion can now be quantified within a short period of time, which has implications for assessing the risk of renal failure in affected patients.

Pauci-immune crescentic glomerulonephritis is usually a manifestation of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. However, some patients with pauci-immune crescentic glomerulonephritis lack ANCA. This Review compares the prevalence, clinical manifestations, histopathology, and outcomes of ANCA-negative pauci-immune crescentic glomerulonephritis with that of ANCA-positive disease. The authors also discuss the possible pathogenesis of ANCA-negative pauci-immune crescentic glomerulonephritis, paying particular attention to the mechanisms and role of neutrophil activation.

Activation of local resident fibroblasts and generation of myofibroblasts via epithelial–mesenchymal transition are key processes in the development of the interstitial fibrosis that characterizes obstructive nephropathy. Authors from the University of Salamanca examine the molecular mechanisms of these processes, focusing mainly on data fromin vivomodels of unilateral ureteral obstruction. In the final section of the Review, they highlight possibilities for future therapeutic intervention to prevent or even reverse obstructive nephropathy.

Hemophagocytic syndrome (HPS), which was first described in 1939, is a heterogeneous disorder characterized by excessive activation and proliferation of non-malignant macrophages. HPS can cause acute kidney injury and nephrotic syndrome; in addition, renal transplant recipients are at risk of developing HPS as a result of their immunocompromised state. This Review summarizes the presentation, causes, pathophysiology and renal features of HPS for the benefit of the practicing nephrologist.

The podocytopathies—minimal-change nephropathy, focal segmental glomerulosclerosis, collapsing glomerulopathy and diffuse mesangial sclerosis—involve diverse types of injury to podocytes. Although the precise pathologic mechanisms are unclear, evidence has emerged linking the immune system with podocyte injury. Current immunosuppressive treatments have limited efficacy and exert considerable toxicity, but here, Marasà and Kopp discuss how new therapeutic monoclonal antibodies and other biologic therapies have the potential to define pathogenetic pathways and contribute to new therapies.

Fabry disease, a rare lysosomal storage disorder, predominantly affects males due to its X-linked inheritance pattern. Here, Abaterusso and colleagues describe a heterozygous female who developed severe Fabry disease-related renal disease with neither proteinuria nor microalbuminuria. This case highlights the importance of considering heterozygous females not merely as 'carriers' of Fabry disease, and emphasizes that the absence of microalbuminuria and proteinuria does not rule out chronic kidney disease in Fabry disease.

Alport syndrome is the most common inherited cause of renal failure after nephronophthisis and reflux nephropathy in children, and after polycystic kidney disease in adults, but often goes unrecognized. In this article, Savige and Colville describe the ocular features of Alport syndrome, and suggest that imaging of the central and peripheral retina might be a rapid, noninvasive and inexpensive test to aid diagnosis of Alport syndrome, particularly in males with early-onset renal failure.