We evaluated renal functional reserve (RFR) in 36 patients aged 5-21 years, who had recovered from an acute episode of poststreptococcal glomerulonephritis (PSGN) 1-16 years previously, without apparent sequelae, as evidenced by normal serum creatinine, blood pressure, and urinary sediment. The control group consisted of 12 children aged 2-12 years with recurrent urinary tract infections or nocturnal enuresis, without active infection or anatomical anomalies. The basal creatinine clearance was similar in the PSGN and control groups: 140.0 +/- 27.4 ml/ min per 1.73 m2 and 142.9 +/- 15.5 ml/min per 1.73 m2, respectively. The RFR in the PSGN group was significantly reduced compared with that of the control group: 18.6 +/- 12.9 ml/min per 1.73 m2 and 41.1 +/- 25.3 ml/min per 1.73 m2, respectively (P < 0.02). In 7 PSGN patients (19.4%), no RFR was found. In 69% of patients who had recovered from PSGN more than 10 years before the protein loading tests, a significantly reduced RFR (less than 10% of baseline) was found. The same degree of reduction in RFR was found in only 26% of patients who had suffered from PSGN less than 10 years ago.
Summary:A 4.6 kg infant with Wiskott-Aldrich syndrome received an accidental overdose of busulfan during preparation for allogeneic stem cell transplantation. Pharmacokinetic analysis of plasma busulfan levels alerted staff to the dosing error. Hemodialysis was immediately performed and resulted in accelerated clearance of busulfan. There were no acute neurologic and hepatic side-effects of the busulfan overdose, and despite 2 months of cough accompanied by rales, the patient is now free of pulmonary symptoms. Stable partial donor chimerism occurred after transplantation. At present, the patient is thriving and infection-free 12 months after transplantation, although his platelet count remains at the lower limit of normal. Bone Marrow Transplantation (2001) 27, 551-553. Keywords: busulfan; hemodialysis; overdose; WiskottAldrich syndrome Busulfan is a potent immunosuppressant and antileukemia agent that has been used in the context of allogeneic stem cell transplantation for nearly 20 years. It has a very narrow therapeutic index; acute toxicity may be related to absorption and disposition of the drug and its metabolites. The absorption of busulfan is highly variable, particularly in infants and small children. Measurement of plasma busulfan levels is used to monitor its disposition, to ensure therapeutic drug levels, and may prevent excess drug toxicity, particularly to the hepatic vasculature, that is associated with high plasma busulfan levels.1 The exact role of pharmacokinetic monitoring on the prevention of busulfan-related toxicities in children is uncertain. 2,3We describe a case of inadvertent busulfan overdose in an infant, that occurred during preparation for allogeneic bone marrow transplantation, and the subsequent removal of busulfan by hemodialysis. Our results suggest that hemodialysis can be used to enhance clearance of busulfan. Case reportA 4.6 kg male infant with Wiskott-Aldrich syndrome (WAS) was referred for bone marrow transplant (BMT) from his HLA-identical sister. Diagnosis was suggested by the family history and confirmed by the presence of thrombocytopenia and mutation of the WASP protein. The planned preparative regimen for BMT was busulfan 4 mg/kg p.o. as crushed pills in divided doses daily for 4 days (total dose,16 mg/kg), followed by cyclophosphamide 50 mg/kg daily for 4 days. Phenytoin anti-convulsant prophylaxis was administered. Plasma busulfan levels were drawn at 0, 15, 30, 60, 90, 120, 180, 240, 300 and 360 min after the first dose of the drug. Additional busulfan levels were obtained as indicated later in the text. The pharmacokinetic analysis that followed busulfan dose No. 1, revealed a dosing error. The patient had already received three doses of 4 mg/kg busulfan at intervals of 6 h instead of the planned dose of 1 mg/kg. Because of the patient's age, and because nearly 4 h had passed since the last busulfan dose, nasogastric lavage was not performed and activated charcoal was not administered.The patient was heavily sedated and No. 4 and No. 3 French single-lumen cathete...
A 9.5-year-old girl, whose early symptoms were polyuria and growth retardation, is described. During the progression of her disease, hyperkalaemia developed out of proportion to the degree of renal insufficiency. Her fractional excretion of sodium increased from 3.3% to 35%, and her fractional excretion of potassium decreased from 55% to 22%. The plasma aldosterone level and plasma renin activity (PRA) were very high--290 ng/ml and 100 ng/dl per hour, respectively (normal range for this age 2.6-20.8 ng/ml and 1.2-2.7 ng/ml per hour, respectively). In an attempt to reduce these hormone levels, an acute and sustained saline load, captopril and peritoneal dialysis were used. Only the sustained saline load normalized the PRA, and only peritoneal dialysis sufficiently suppressed the plasma aldosterone level. Successful renal transplantation normalized both plasma aldosterone and PRA. This girl presents the unusual occurrence of pseudohypoaldosteronism type I, during the course of familial juvenile nephronophthisis.
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