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Eleven pediatric patients with nephrosis and focal segmental glomerulosclerosis were treated with long-term (8-38 months) ciclosporin A in combination with steroids. All had abnormal height-velocity curves and multiple hospitalizations for complications of nephrosis. Eight patients attained remission with a dramatic improvement in growth and decrease in necessity for hospitalization for therapy of nephrosis complications, while maintaining adequate renal function. Three nonresponders developed end-stage renal disease. Long-term ciclosporin A therapy may be of benefit in steroid-resistant nephrosis in childhood.

Subacute sclerosing panencephalitis (SSPE) is a progressive neurologic disease associated with chronic measles infection. We have assessed renal function parameters and renal biopsy details in 14 patients with SSPE. All had normal renal function. However, 9 had microscopic hematuria. 9 patients had focal segmental mesangial proliferation and 5 had capillary wall thickening with deposits visible by light microscopy. 13 patients had glomerular C3 deposition and 4 had IgG deposits. SSPE is a disease caused by chronic viral infection, in which nephritogenic complex formation may occur.

Many previous series reporting the construction of Brescia-Cimino fistulae in children describe a high rate of immediate and late patency failure. Using microsurgery, we have constructed 23 Brescia-Cimino fistulae in 23 children, 10 weighing less than 20 kg and 13 weighing more than 20 kg. An immediate patency rate of 100% was achieved in both weight groups with a late patency rate of 90%-100%, in those under and over 20 kg, respectively. We recommend using microsurgery for all such angioaccess procedures.

Constipation is associated with and may be an etiologic factor in recurrent urinary tract infection (UTI) in girls. To determine if an increased incidence of urinary infection occurs in constipating illness, we retrospectively analyzed UTI incidence in 146 patients with Hirschsprung's disease. An increased incidence pre- and postoperatively was noted.

Two children had polytetrafluoroethylene (PTFE) arteriovenous thigh grafts for hemodialysis access. In one patient, after renal transplantation, a steal syndrome, which prevented renal graft function, developed. The renal transplant functioned promptly when the vascular graft was ligated. In the second case, acute thrombosis of the PTFE graft resulted in transient hypertensive encephalopathy. PTFE thigh grafts, because of the diversion of a large percentage of cardiac output through them, may have dramatic and dangerous consequences in transplanted children.

10 children underwent cadaveric renal transplantation between the ages of 8.0 and 12.5 years for uremia secondary to infantile cystinosis. 6 children are doing well 6-62 months after-transplantation. 3 of the 4 other recipients required a second graft and eventually died of uremia or fulminant viral encephalitis, the other lost her first graft due to accelerated acute rejection and is now on maintenance hemodialysis. No further systemic complications of cystinosis have been observed in the patients with functioning grafts. Our experience confirms that kidney transplantation is the treatment of choice for uremic children with infantile cystinosis.

The effect of hemodialysis on myocardial function in 10 pediatric chronic hemodialysis patients was assessed utilizing echocardiography. Parameters of cardiac function were measured prior to and immediately after dialysis. A significant decrease in cardiac output was observed post-dialysis. Unlike that observed in adult patients a real improvement in myocardial function as indicated by an increase in the mean velocity of circumferential fibre shortening was evident post-dialysis.

An 11-year-old girl who presented with pneumonia, pleural effusion, hematuria, and red blood cell casts is described. Mycoplasma pneumoniae complement fixation titers were elevated. The pneumonia resolved following therapy with tetracycline. Light microscopy of renal biopsy tissue revealed an acute glomerulonephritis; immunofluorescent studies showed deposits of IgG, C3 and mycoplasma antigen along the glomerular capillary walls and in the mesangium; subendothelial and subepithelial deposits were seen by electron microscopy. This is the first report of acute glomerulonephritis associated with M. pneumoniae infection with evidence of mycoplasma-induced immune complex nephritis.

Blood flow rates were measured using an isotopic dilution method in the main drainage segments of Brescia-Cimino arteriovenous fistulas in 9 pediatric patients who had undergone successful renal transplantation, 3 patients on hemodialysis and 2 patients with end-stage renal disease. Blood flow rates varied from 68 to 882 ml/min. The possibility that such high flow rates may have deleterious effects on limb growth, cosmetic appearance and cardiac function without any benefit to the patient is discussed.

Based on clinical and experimental data, a cardiomyopathic syndrome has been attributed to chronic hypokalemia. Analysis of the published data indicates the presence of numerous other complicating factors which might have compromised cardiac function. Echocardiographic studies on 5 children with lifelong (Bartter's syndrome, 3 cases; congenital renal alkalosis, 1 case) or prolonged (primary hyperaldosteronism, 1 case) hypokalemia did not reveal any abnormalities of myocardial performance, thus questioning the premise that hypokalemia causes cardiomyopathy.