William R. Griswold

Treatment of steroid-resistant focal segmental glomerulosclerosis with pulse methylprednisolone and alkylating agents

In children, steroid-resistant nephrotic syndrome due to focal segmental glomerulosclerosis (FSGS) is frequently a progressive condition resulting in end-stage renal disease. There have been no reports of effective treatment for this condition. For the past several years, the Pediatric Nephrology services at the University of California, San Diego and Stanford University Schools of Medicine have treated these patients with a protocol involving infusions of high doses of methylprednisolone, often in combination with oral alkylating agents. Twenty-three children have been treated in this manner with a follow-up of 46±5 months. Twelve of these children are in complete remission. Six have minimal to moderate proteinuria. Four children remain nephrotic. Each of these children has a normal glomerular filtration rate. One child developed chronic renal failure and subsequently died while on dialysis. These results appear significantly better than previous series of children with FSGS. A controlled, multi-center trial of this protocol has been proposed.

Peritoneal dialysis for acute renal failure in children.

Fifty infants and children with acute renal failure were treated with acute peritoneal dialysis between 1987 and 1990. The patients were dialyzed using either a catheter introduced percutaneously over a guide-wire (n=40) or a Tenckhoff catheter (n=10). The cause of the acute renal failure was primary renal disease in 17 children, cardiac disease in 19, and trauma/sepsis in 14. Peritoneal dialysis succeeded in controlling metabolic abnormalities, improving fluid balance, and relieving the complications of uremia. The procedure had few major complications. Overall mortality was 50%, reflecting the serious nature of the underlying diseases. We conclude that acute peritoneal dialysis is a safe and effective treatment in most pediatric patients with acute renal failure. Our series of patients treated with acute peritoneal dialysis serves as a basis of comparison for the evaluation of new modalities of therapy in childhood acute renal failure.

Theoretical analysis of the sensitivity of the solid phase antibody assay (ELISA)

The sensitivity of the solid phase ELISA antibody assay was analyzed from a theoretical perspective. Results show that the investigator may choose either affinity dependent or affinity independent performance by adjusting the assay conditions. High antigen concentration (concn) and small interepitope distance favor affinity independence, while low antigen concn and large interepitope distance favor affinity dependent assay behavior. The maximum sensitivity will occur in the affinity independent region. Antibody titers obtained under affinity independent conditions will reflect true antibody concn, but measurements performed under affinity dependent conditions will underestimate antibody concn.

Follow-up of infants with obstructive uropathy detected in utero and treated surgically postnatally

Thirty-four infants with obstructive uropathy detected by prenatal ultrasonography at 34 +/- 0.56 weeks (SE) had surgery during the first 2 years of life. The growth and renal function of the 25 children after being followed for over 1 year are described. Twelve children had unilateral disease, and 13 had bilateral disease. All 12 children with unilateral disease grew at or above the fifth percentile and had normal renal function (glomerular filtration rate, 101 +/- 7 mL/min/1.73 m2). Thirteen children with bilateral disease were followed for 26.1 +/- 3.4 months. Growth was good: 10 of the 13 grew at or above the fifth percentile. The serum creatinine was 0.7 +/- 0.2 mg/dL, and the glomerular filtration rate was 91 +/- 10 mL/min/1.73 m2. One child required chronic dialysis. The prenatal diagnosis of urinary tract anomalies followed by early intervention may improve the long-term outcome of children with obstructive uropathy.

Pediatric renal transplantation in Laurence-Moon-Biedl syndrome.

Two cases of renal transplantation in pediatric patients with Laurence-Moon-Biedl syndrome are reported. Immunosuppressive therapy consisted of cyclosporine, prednisone and azathioprine. Renal function has been good but both patients developed morbid obesity.

A computer program for calculating antibody affinity constants

A program is presented for the calculation of antibody affinity constants which avoids the limitations of graphical plotting of titration data using the Sips or Scatchard equations.

Calculation of monoclonal antibody affinity constants directly from antibody dilution curves

Abstract A method for estimating the affinity constants of monoclonal antibody directly from antibody dilution curves is described.

Congenital megalourethra associated with prune belly syndrome.

Dilation of the penile urethra is described in a male infant with the classic triad of the prune belly syndrome. This dilated penile urethra was not accompanied by obstruction of the urethra proximal or distal to the dilation. Dilation of the penile urethra is a rare feature of the prune belly syndrome and may be part of the same developmental abnormality that affects the remainder of the urinary collecting system.

Interference with creatinine concentration measurement by high dose furosemide infusion.

Three patients had unmeasurable serum creatinine concentrations using a colorimetric method while receiving high doses of furosemide. The present study shows that enzymatic methods of measuring the serum creatinine concentration should be used in patients receiving high doses of furosemide.

Pulmonary hemorrhage in children with glomerulonephritis.

Pulmonary hemorrhage may occur in patients with immune-mediated glomerulonephritis. This association can be seen in a variety of disorders including systemic lupus erythematosus, vasculitis, Wegeners granulomatosis, anaphylactoid purpura and Goodpastures syndrome. Immune mechanisms, such as immune complexes and/or autoantibodies, play a role in the pathogenesis of pulmonary and glomerular injury. Acute pulmonary hemorrhage can lead to respiratory failure and has a high mortality. Therapy with immunosuppressive agents such as pulse methylprednisolone and cyclophosphamide will control the hemorrhage and improve pulmonary function in most cases.