Dehydration, renal vein thrombosis and hyperkalemic renal tubular acidosis in a newborn.
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Biomedical subjects
Publications and source records attributed to R J Glassock.
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BACKGROUND: Clinical researchers are beginning to use the immunosuppressive agent cyclosporine to treat a variety of immunologically mediated diseases. PURPOSE: To review the clinical experience with cyclosporine in the nephrotic syndrome and in lupus nephritis. SUMMARY: Most studies to date have been small or uncontrolled, or both. Nevertheless, cyclosporine appears to produce remissions in adults and children with the nephrotic syndrome with minimal histologic changes ("minimal-change disease"). It also appears to produce remissions in children with focal and segmental glomerulosclerosis; it is less effective in adults and in individuals with focal and segmental glomerulosclerosis that is resistant to steroids. It appears to reduce proteinuria and to slow the progression of renal insufficiency in membranous glomerulonephritis but has little beneficial effect in IgA nephropathy. Anecdotal experience in patients with lupus nephritis suggests cyclosporine produces an improvement in symptoms, a decrease in protein excretion, and improvement in renal function when other treatments have failed. Cyclosporine's pharmacokinetic properties vary widely with the age of the patient and the presence of concomitant diseases and drugs. Relapses are common after the drug is stopped, and nephrotoxicity is a real risk with prolonged treatment. CONCLUSIONS: Cyclosporine shows promise in treating immunologically mediated glomerular diseases, but larger randomized studies will be needed to define its role in this setting. Physicians should become more familiar with this drug as its indications increase.
In the early years after the first description of IgA nephropathy, a nihilistic attitude prevailed in therapy because the initial proposals were not convincing, being both empirical and uncontrolled. The situation now seems to be changing. Although the pathogenesis of IgA nephropathy is still not fully understood, a number of drugs--supposed to interfere with some disease mechanisms--have been tested with proper study design. As a consequence, the current therapeutic approach to IgA nephropathy is based on the results of large controlled trials of glucocorticoids, immunosuppressors, anticoagulants, ACE-inhibitors, fish oil, etc., provided that the patients are selected among those with a greater risk for progression. This therapeutic approach, though still empirical, is the mainstay at present. As we approach the new millennium, we are getting tantalizingly closer to understanding the pathogenesis of IgA nephropathy and to develop new effective drugs.
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