American journal of nephrology
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It is widely believed that the most common morphological lesion in children with the idiopathic nephrotic syndrome who manifest a frequently relapsing steroid-responsive course is the minimal-change lesion. However, there are no prospective renal biopsy studies in such patients to substantiate this assertion. We performed a renal biopsy in all children with early frequently relapsing steroid-responsive nephrotic syndrome during the years 1980-1984. ⋯ Long-term follow-up revealed that 10 patients have remained protein free, 4 have persistent proteinuria despite cyclophosphamide therapy, 1 had progressed to end-stage renal disease, and 1 is lost to follow-up. On the basis of these findings, we recommend that all children with nephrotic syndrome and an early frequently relapsing steroid-responsive course undergo a prompt renal biopsy. Such patients constitute a high-risk group with a spectrum of renal histopathological lesions characterized by an unpredictable response to therapy and an unfavorable prognosis.
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In 10 patients with the nephrotic syndrome (NS) and edema persisting despite a NaCl-poor diet, the effect of a single infusion of hyperoncotic albumin (75 g) on NaCl excretion was studied. 6 patients had minimal lesions, and 2 patients were studied twice. On half of the occasions the glomerular filtration rate was reduced. Blood volume (BV), calculated from plasma volume and hematocrit, was slightly elevated before infusion, and increased to 136 and 120% of normal at 4 and 20 h after it, respectively. ⋯ Distal fractional NaCl reabsorption was also elevated before (93.0 +/- 6.4%), but unaltered after infusion (93.0 +/- 5.6%). Thus, after marked expansion of BV and suppression of PRA and PA, sodium excretion remained low despite the present edema. The results indicate that in many patients with the NS, including minimal lesion NS, intravascular hypovolemia is not the sole cause of sodium retention.
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Pericarditis with or without effusion as a complication of end-stage renal disease responds to the institution of chronic dialytic therapy. The management of pericardial effusion which has its onset after some time of chronic dialytic therapy is less well established. Since a surgical pericardial drainage procedure is often performed on an emergency basis in some patients with pericardial effusion treated with maintenance dialysis, it would be advantageous to be able to predict which patients would subsequently require an operation which then could be performed electively. ⋯ Our analysis of 22 patients with pericardial effusion demonstrates that those with a large effusion are best treated by elective pericardial drainage using a subxiphoid approach with instillation of triamcinolone hexacetonide into the pericardial sac. Those patients with a small or moderate effusion can be subjected to a trail of nonsteroid anti-inflammatory drugs and/or intensive dialysis. Evidence of an enlarging effusion should prompt surgical drainage before hypotension or tamponade require the patient to undergo an emergency operation.