IgA nephropathy and Henoch-Schönlein purpura nephritis.
OBJECTIVE
IgA nephropathy and Henoch-Schönlein purpura nephritis are common glomerular disorders in pediatrics that can potentially progress to end-stage renal disease in some patients. This review summarizes our current understanding of the pathogenesis of these closely related conditions and discusses the rationale for development of diagnostic tests and prognostic markers. The review also presents the best data for long-term outcome, clinical markers of prognosis, and the results of randomized controlled trials.


RESULTS
Our understanding of the defective galactosylation of O-linked glycans in the hinge region of human IgA1 and its role in the pathogenesis of IgA nephropathy and Henoch-Schönlein purpura nephritis has evolved over the past decade. This review discusses studies that suggest that demonstration of galactose-deficient IgA1 in the serum may become an important diagnostic tool for these conditions. Proteomic techniques for development of biomarkers for diagnosis and prognosis show promise. Although data from randomized controlled trials have failed to support the use of immunosuppressive agents in pediatric IgA nephropathy and Henoch-Schönlein purpura nephritis, recent data indicate that angiotensin converting enzyme inhibitor therapy is indicated for reduction of proteinuria.


CONCLUSIONS
Childhood IgA nephropathy and Henoch-Schönlein purpura nephritis have the potential for serious morbidity, either during childhood or later in adulthood. In the future clinical tests will be used for noninvasive diagnosis and as markers for judging response to treatment, particularly in those individuals at highest risk for eventual progression to end-stage renal disease.
Sanders
John T
JT
Children's Foundation Research Center at Le Bonheur Children's Medical Center, Memphis, Tennessee, USA.
Wyatt
Robert J
RJ
D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D015415:Biomarkers; D015984:Causality; D002648:Child; D005922:Glomerulonephritis, IGA / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D006801:Humans; D011695:IgA Vasculitis / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D007676:Kidney Failure, Chronic / Q000453:epidemiology; D011237:Predictive Value of Tests; D011379:Prognosis; D016032:Randomized Controlled Trials as Topic; D016019:Survival Analysis; D016896:Treatment Outcome
D000806:Angiotensin-Converting Enzyme Inhibitors; D015415:Biomarkers