ABSTRACT
OBJECTIVE: To evaluate the efficacy of cyclosporine (CyA) monotherapy in steroid resistant (SRNS) and steroid dependent (SDNS) nephrotic syndrome in children. DESIGN: A retrospective study. SETTING: Tertiary kidney care center for children at Bangalore. METHODS: Forty-one children with SDNS and SRNS with normal renal functions were treated with CyA at a dose of 6 mg/kg/day initially and maintained at 3 to 4 mg/kg/day if remission was sustained. The dosage was adjusted according to the CyA blood levels in non-responders. RESULTS: The median age of patients was 93 months (range 48-936) months. Thirteen children had minimal change disease (MCNS), 10 had mesangial proliferative glomerulonephritis (GN). Ten had membrano-proliferative (GN) (MPGN) and 8 had focal segmental glomerulosclerosis (FSGS). Median age at onset of disease and median time for CyA usage from disease onset was 22 months and 16 months respectively. Median duration of CyA therapy was 24 months (range 6-72) months. The data was analyzed to determine significance of variables on the outcome. Median follow up was 71 months (range 20-205) months. Eleven children were CyA resistant. Of the remaining 30 who were CyA responders, 22 (73.33%) were CyA dependent. Seven children developed chronic renal failure (CRF). CONCLUSIONS: The predictors for CyA non-responsiveness were steroid resistance, non MCNS on biopsy and longer duration between onset of nephrotic syndrome and CyA usage, irrespective of the age of onset of the disease. There was a higher incidence of CyA dependence among young responders. Patients with CyA resistance are at high risk for significant infections and CRF.
Subject(s)
Cyclosporine/therapeutic use , Drug Resistance , Nephrotic Syndrome/drug therapy , Case-Control Studies , Child , Child, Preschool , Dose-Response Relationship, Drug , Drug Administration Schedule , Female , Follow-Up Studies , Humans , Kidney Function Tests , Logistic Models , Male , Nephrotic Syndrome/diagnosis , Nephrotic Syndrome/mortality , Probability , Prospective Studies , Reference Values , Risk Assessment , Severity of Illness Index , Steroids/therapeutic use , Treatment OutcomeABSTRACT
A retrospective analysis was done on 47 pediatric renal transplants performed over last 16 years at Bangalore, Karnataka. The median age and weight of the recipients at transplantation were 120 months and 21 kg respectively; male to female ratio was 30 to 17. Twenty two children had underlying glomerular disease and 23 had tubulointerstitial disease. Preemptive transplantation was done in 33.3% of patients, 57.2% received hemodialysis and 9.5% received peritoneal dialysis prior to transplantation. The mean duration of dialysis was 2.6 months. The most common source of donor organ was the mother. Immunosuppression medications included cyclosporine, azathioprine, and corticosteroids. Graft survival at 1 year, 5 years, and 10 years was 80%, 45.8% and 37.5% respectively. Renal transplantation is the most optimal way to manage children with ESRD with satisfactory long term results.
Subject(s)
Kidney Failure, Chronic/surgery , Kidney Transplantation/methods , Transplantation Immunology , Cadaver , Child, Preschool , Female , Graft Rejection , Graft Survival , Humans , India , Kidney Failure, Chronic/diagnosis , Kidney Failure, Chronic/mortality , Kidney Transplantation/immunology , Kidney Transplantation/mortality , Living Donors , Male , Postoperative Complications/epidemiology , Prognosis , Retrospective Studies , Risk Assessment , Survival RateABSTRACT
Antiphospholipid antibody syndrome (APS) is characterized by recurrent thrombosis with the presence of circulating antiphospholipid antibodies. A diagnosis of APS requires the presence of at least one clinical and one laboratory criteria (detection of aCL IgG or IgM antibodies or the presence of lupus anticoagulant on two or more consecutive occasions 6 weeks apart). A severe, rapidly progressive form characterized by clinical involvement of at least three different organ systems with histopathological evidence of small and large vessel occlusion is termed catastrophic antiphospholipid syndrome. Early recognition of APS is crucial since aggressive management can result in a favorable outcome. We present the case of a 12-year-old boy who presented with a devastating illness with multiple thrombotic episodes and rapidly progressive renal failure.
Subject(s)
Acute Kidney Injury/etiology , Antiphospholipid Syndrome/complications , Antiphospholipid Syndrome/diagnosis , Thrombosis/etiology , Acute Kidney Injury/physiopathology , Catastrophic Illness , Child , Disease Progression , Humans , Male , Time FactorsABSTRACT
Snakebite is not an uncommon cause of acute renal failure (ARF) in developing countries. We report a12-year-old boy who presented with oliguric ARF following snakebite. He had pallor, icterus, generalized edema, hypertension, and was oliguric. Investigations revealed severe azotemia, microangiopathic hemolytic anemia, thrombocytopenia, prolonged coagulation parameters, and raised fibrin degradation products, suggesting disseminated intravascular coagulation as the cause of ARF. The patient improved with antisnake venom, dialysis, and other supportive treatment.