Hyperlipidemia in uremic children: response to peritoneal dialysis and hemodialysis.
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Biomedical subjects
Publications and source records attributed to R J Cunningham.
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Occasional patients with nephrotic syndrome and minimal histologic change demonstrate glomerular deposition of small amounts of immunoglobulin and complement. Some consider this a disease distinct from MCNS. To investigate the clinical importance of immune deposits and mesangial hypercellularity in the initial biopsy, the clinical records, follow-up data, and renal biopsies of 68 patients (ages 6 months to 16 years) with MCNS by light microscopy were reviewed. Among 68 patients followed a mean of 6.2 years, eight of 25 patients with immune deposits on initial renal biopsy were steroid nonresponsive. Only one of 43 patients without immune deposits was steroid nonresponsive (P = 0.00005). Of 44 patients with normal mesangial cellularity, 31 experienced fewer than three relapses a year, whereas of 15 patients with mesangial hypercellularity, only six experienced fewer than three relapses a year (P = 0.035). The data suggest that immune deposits and increased mesangial cellularity in children with NS and minimal light microscopic change may predict the clinical course.
Urinary kallikrein, as a reflection of the intrarenal kallikrein, is distinct from plasma kallikrein. The kallikrein-kinin system, activated via antigen-antibody complexes, has been implicated in the pathophysiology of acute transplant rejection. We studied urinary excretion of sodium, potassium, protein, and kallikrein following renal transplantation in nine patients, In four patients who took steroids daily and were followed from the time of transplantation (without rejection episodes), urinary kallikrein remained stable (3.7 +/- 0.5 EU/24 hr, x +/- SEM). Since sodium-retaining steroids influence urinary kallikrein excretion, two patients were studied 2 and 3 months post-transplantation while receiving alternate-day prednisone. Values from urinary kallikrein in these patients did not differ from those in patients taking steroids daily (3.7 +/- 0.5 versus 2.7 +/- 0.3 EU/24 hr). In three patients experiencing acute rejection episodes, urinary kallikrein excretion rose markedly (24 to 260 EU/24 hr) and remained elevated until the patients became oliguric. Furthermore, this rise occurred 1-3 days before the clinical diagnosis of rejection was made. For the group as a whole there was no significant correlation between urinary kallikrein excretion, the excretion of sodium or potassium, or urine volume. Although the number of patients studied is small, the increases observed in urinary kallikrein suggest that activation of the intrarenal kallikrein-kinin system is associated with acute transplant rejection.
The usual clinical course of focal segmental glomerulosclerosis is marked by progressive decrease in renal functional and sclerosis on biopsy over a period of months to years. We report a variant exemplified by 2 children who developed chronic renal failure within 12 weeks of the onset of nephrotic syndrome; repeat renal biopsies demonstrated extensive extracapillary glomerular proliferation and crescent formation. Both were males, less than 5 years of age, presenting with nephrotic syndrome resistant to steroid therapy and normal renal function. Renal biopsies done after 8 weeks of therapy, demonstrated findings compatible with focal segmental glomerulosclerosis. Within 12 weeks of onset of nephrotic syndrome, both patients experienced a decrease in renal function requiring dialysis. Repeat renal biopsies revealed extensive extracapillary glomerular proliferation with crescent formation. These patients represent a variant of focal segmental glomerulosclerosis characterized by rapid progression to renal failure with extensive extracapillary glomerular proliferation and crescent formation.
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The clinical course and outcome of rapidly progressive glomerulonephritis (RPGN) of variable etiology are not well defined in children. The present investigation reports on the clinical characteristics, the course and outcome, as well as the results of treatment of 13 children with apparent postinfectious RPGN. Three of 7 patients with documented streptococcal RPGN and 3 of 6 patients with RPGN of nonstreptococcal etiology progressed to chronic renal failure. In some patients, anticoagulant and antiplatelet therapy appear to have improved survival. The severity of crescent formation, not the presumable etiology, appears to be a reliable prognosticator.
Night calls attended by one doctor during five consecutive years (1973 to 1977) in a single-handed rural practice, were analysed and divided into two categories: (a) reasonable; (b) unreasonable. Half the night calls were genuine emergencies but 17 per cent were quite unnecessary.
Plasma immunoreactive prostaglandin A (iPGA) values were determined for 14 premature infants before and after 48 hours of phototherapy and in a control group of six age-matched premature infants. At 4 to 8 days of life, the infants who had received phototherapy for 48 hours had significantly lower iPGA values compared with controls. At 2 to 3 days of life, four infants with a clinically apparent patent ductus arteriosus were found to have significantly elevated iPGA values compared with controls. After 48 hours of phototherapy, these infants likewise had a significant decrease in iPGA values compared with controls; in all four infants, the ductus closed spontaneously. Phototherapy is an effective method for decreasing plasma iPGA values in premature infants.
The plasma kallikrein-kinin system, a potent vasodilator, has been implicated in causing the protein loss of the idiopathic nephrotic syndrome. However, the kidney possesses a kallikrein-kinin system separate from the plasma system. Thus, urinary kallikrein may reflect more accurately intrarenal events. Using a radiochemical esterolytic assay we measured the urinary kallikrein excretion in a patient with a minimal lesion nephrotic syndrome during relapse. Protein excretion was initially elevated (8.1 plus or minus 2.0 gm. per 24 hours) as was urinary kallikrein excretion (96.4 plus or minus 46.6 EU per 24 hours). After initiation of steroid therapy protein and kallikrein excretion decreased significantly (p less than 0.05). During the entire study kallikrein excretion was significantly correlated with protein excretion (r equals 0.89, p less than 0.01). It is tempting to speculate that activation of the intrarenal kallikrein-kinin system participates in the protein loss characteristic of the nephrotic syndrome.
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This report concerns two patients with Bartter's syndrome who were treated with propranolol, spironolactone, and potassium supplements. When ibuprofen was added to this regimen, potassium supplements were no longer required. In both patients, plasma renin activity decreased, plasma volumes increased, and a "catch-up" in linear growth ensued. This report confirms others that indicate prostaglandin synthetase inhibitors are a useful adjunct in the therapy of Bartter's syndrome.
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Urinary prostaglandins were measured by radioimmunoassay in neonates (31-35 weeks gestational age), children (4-15 years) and adults (24-36 years). Neonates showed significantly lower levels of both iPGE and iPGA compared to children (p less than 0.01) and adults (p less than 0.01). Children also had significantly lower levels than adults (p less than 0.01). Since urinary prostaglandins reflect intrarenal levels of prostaglandins, the results support other studies that suggest prostaglandins may play a role in controlling renal blood flow in infants.
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Hemodialysis and hyperlipidemia have been associated in both adults and children. The present study indicates hyperlipidemia in uremic children treated with peritoneal dialysis and implies that the cardiovascular risk felt to exist with hemodialysis also exists in peritoneal dialysis. Thirty-eight children with chronic renal insufficiency or end-stage renal disease were followed serially under varying conditions of medical management, hemodialysis, peritoneal dialysis, and transplantation. Serum triglyceride concentrations in patients on peritoneal dialysis were not significantly different from those in patients on hemodialysis, but both were significantly higher (P less than 0.01) than concentrations in patients on medical management and transplantation.