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Abnormalities of immunoglobulin free light chains (FLCs) are frequently present in patients with monoclonal gammopathies and can cause kidney disease. The recent introduction of highly sensitive immunoassays that measure FLCs to levels below those present in normal individuals has provided a new tool for diagnosis and management in this setting. In this article, Hutchison and colleagues review the biology of FLC production in health and disease, and the utility of FLC immunoassays in the assessment of monoclonal gammopathies in kidney disease.
The heart and kidney can mutually influence each other's function so that dysfunction in one organ can result in dysfunction in the other. In this Review, Khaled Shamseddin and Patrick Parfrey describe the mechanisms involved in this two-way interaction, with reference to the classification of the so-called cardiorenal syndromes introduced in 2008 by Claudio Ronco and colleagues.
Kidney Disease: Improving Global Outcomes (KDIGO) is an independent organization that aims to improve care and outcomes for patients with kidney disease worldwide through the development and dissemination of clinical practice guidelines. In this Review, the current co-chairs of KDIGO discuss the structure, methodology and activities of KDIGO and describe how KDIGO is trying to meet the multiple challenges of guideline development.
The incidence and mortality of sepsis and the associated development of acute kidney injury (AKI) remain high, despite intense research into potential treatment strategies. Inducible nitric oxide synthase—which is constitutively expressed in the kidney but is not expressed in many other organs—has known importance in the pathogenesis of sepsis-induced AKI in humans. In this article, Heemskerk and colleagues discuss the selective inhibition of iNOS as a potential novel treatment for sepsis-induced AKI.
Two observational studies report opposite effects of glitazones on clinical outcomes in patients with ESRD. Given the limited reliability of such studies in the assessment of moderate effects of treatment, however, findings in these articles should prompt the generation of hypotheses rather than dictate changes in clinical practice.
Use of ABO-incompatible renal transplants from living donors has proven a viable and practical transplantation strategy. The protocols devised through the Johns Hopkins Incompatible Kidney Transplant Program could result in the most rapid escalation of access to organs in the modern era of transplantation.
Two recent reports in the American Journal of Transplantation focus on the maternal and fetal outcomes of pregnancies in kidney donors and provide tantalizing, if somewhat worrisome, observations. The findings also leave us with several important unanswered questions.
Although ALMS, the largest prospective, randomized, controlled study comparing mycophenolate mofetil to intravenous cyclophosphamide for the initial treatment of severe lupus nephritis, failed to achieve its primary end point of mycophenolate superiority, mycophenolate plus corticosteroids has become the accepted standard of care. Are we really beyond cyclophosphamide for severe lupus nephritis?
Identifying patients at risk of end-stage renal disease relying only on measurement of both glomerular filtration rate and albuminuria could greatly decrease the number of patients flagged for renal surveillance without increasing the risk of overlooking high-risk individuals.
Defining the dose of a new renoprotective drug with the optimal benefit-to-risk ratio is an important consideration for drug developers and physicians. Have we learned from past experiences?
Calcific uremic arteriolopathy (CUA), a condition associated with high mortality, is most common among patients on dialysis. In this opinion article, Georg Schlieper and his colleagues discuss the evidence on the efficacy of administering sodium thiosulfate in the treatment of CUA. Given the lack of large clinical trials, the authors also evaluate the consultation of internet-accessible CUA case registries as a strategy to inform the treatment of this disease and to design future studies.
In this Review, Schurman and Scheinman detail the clinical and genetic features of syndromes with a defined genetic basis that are characterized by the concomitant presence of abnormalities of the central nervous system and kidneys. The authors focus their attention on the oculocerebrorenal syndrome of Lowe and on ciliopathies—particularly Joubert syndrome and Bardet–Biedl syndrome.