Search PubMed⌕ Search

Biomedical subjects

E C Kohaut

Publications and source records attributed to E C Kohaut.

At least 37 records · Page 2Linked to original sources

Treatment of IgA nephropathy in children: efficacy of alternate-day oral prednisone.

We have previously reported our experience with the use of alternate-day prednisone in the treatment of 6 patients with IgA nephropathy who have clinical or pathological risk factors for disease progression. We have now treated a total of 13 patients and followed them from 4 to 10 years. Patients received an alternate-morning dose of prednisone for 2-4 years. Dosage began at 60 mg/m2 for 3 month, was reduced to 30 mg/m2 by 1 year and 15 mg/m2 by 2 years. At last observation, urinary protein excretion was normal in 12 patients and no patient had hematuria. Twelve patients had normal estimated glomerular filtration rate (GFR) and one had renal insufficiency (GFR = 38 ml/min per 1.73 m2). A renal biopsy was performed in 11 patients after 2 years of treatment. Activity score decreased from 5.2 to 4.3 (P = 0.03) and chronicity score increased from 2.2 to 2.8 (P = 0.12). There were no complications of treatment. When compared with a historical group, the treated patients had a significant improvement in urinalysis (P < 0.00001) and preservation of normal GFR (P = 0.03). We conclude that alternate-day prednisone therapy may benefit patients with IgA nephropathy. A large prospective controlled trial is needed.

Administration, Oral↗

Treatment of growth failure in children after renal transplantation.

Growth failure continues to be a problem in the management of children with renal failure. Children have improved growth after successful renal transplantation, but seldom have "catch-up" growth, or normal adult height. In this study we report the findings of an open label pilot study to determine the safety and efficacy of the use of recombinant human growth hormone (rhGH) in children with growth failure after successful renal transplantation. Eleven children completing at least 1 year of treatment had a mean age of 11.5 years (+/- 3.3) and mean bone age of 8.4 years (+/- 2.4), and were significantly growth-retarded (mean standard deviation score of -3.18 [+1.1]). After receiving rhGH (0.05 mg/kg) subcutaneously each day the height velocity increased from 5.2 cm/year to 8.4 cm/year (P = 0.003), and the standard deviation score improved from -3.18 to -2.23 (P = 0.004). Treatment was associated with advancement in Tanner stage from 1 to 2.8 (P = 0.004), increased bone age from 8.4 years to 10.9 years (P = 0.0002), and although it was not at the point of statistical significance, moderate decrease in creatinine clearance from 75 ml/1.73m2/min to 60 ml/1.73m2/min (P = 0.1). The advancement in Tanner stage and bone age was not out of proportion to the advancement in height age. These data suggest that children with functioning renal allografts have improved growth with supraphysiologic doses of rhGH.

Adolescent↗

Pediatric renal transplantation at the University of Alabama at Birmingham, 1968 to 1993.

From May 5, 1968 through January 31, 1993, 272 renal allografts were performed in 233 children at the University of Alabama at Birmingham. Graft survival has improved since 1983 with the addition of cyclosporine. Acute and chronic rejections continue to be the major causes of graft failure. The use of living-related donor renal allografts optimizes long-term graft function, allows early transplantation, and minimizes potential long-term dialysis complications in children.

Academic Medical Centers↗

Growth hormone in the treatment of growth failure in children after renal transplantation.

Growth failure continues to be a problem in the management of children with renal failure. Children have improved growth after successful renal transplantation, but seldom have "catch-up" growth, or achieve normal adult height. Several investigators have reported the use of rhGH in children after renal transplantation and demonstrated improved height, growth velocity, and standard deviation score (SDS). We review the existing literature, present additional data from our center, and offer possible explanations.

Child Development↗

Methylprednisolone treatment of patients with steroid-resistant nephrotic syndrome.

Treatment with a combination of pulse methylprednisolone (MP) and an alkylating agent has been reported to induce long-term remission of proteinuria in patients with steroid-resistant nephrotic syndrome (SRNS). We have treated 13 patients with SRNS with a course of pulse MP. There were 8 black patients and 5 white; 10 had a biopsy diagnosis of focal segmental glomerulosclerosis (FSGS) and 3 nil lesion. Initially 5 patients responded and 2 partially responded. Of the responding patients, 5 relapsed while treated with alternate-week MP therapy. Of these relapsing patients, 3 received a second course of MP plus chlorambucil; 2 responded. The patients were observed for a mean of 47 months (range 4-64 months). When last seen only the 3 patients with a biopsy diagnosis of nil lesion were protein free. There were no complications of steroid therapy. Six patients currently have end-stage renal disease and 2 have renal insufficiency. All of the 6 patients with no response to treatment were black. These data suggest that a course of pulse MP therapy alone induces short-term remission of the nephrotic syndrome in some white patients with FSGS, but in almost no blacks. Patients who relapse may respond to retreatment, but addition of an alkylating agent does not appear to induce long-term remission in patients with FSGS.

Administration, Oral↗

Renal transplantation in children with posterior urethral valves.

We compared retrospectively the outcome of renal transplantation in 18 children with a diagnosis of posterior urethral valves to 18 matched children with nonobstructed renal failure to identify the disadvantages of renal transplantation into the valve bladder. There was no statistical difference in patient survival. The 5-year graft survival rate was 62.3% in children with posterior urethral valves and 48% in the control group. The difference was not statistically significant (p = 0.32). A statistically significant increase in serum creatinine from 1.2 to 2.2 mg./dl. was noted in the patients with posterior urethral valves, which was not seen in the control group (p = 0.0009). The clinical significance of this finding is unknown. Renal transplantation can be performed successfully in patients with posterior urethral valves. A prospective study is required to determine the benefits of preoperative lower urinary tract urodynamic assessment and the true effect of the valve bladder.

Adolescent↗

Zinc and copper balance in children on continuous ambulatory peritoneal dialysis.

We monitored serum zinc and copper levels for 4 months in six patients treated with continuous ambulatory peritoneal dialysis (CAPD). Zinc and copper fluxes were studied during a single dialysis exchange and over a 3-day period. Routine oral trace element supplements were then discontinued for 2 months. Serum zinc levels declined but serum copper levels remained unchanged. One month after oral supplements had been restarted, serum zinc levels returned to normal and serum copper levels rose above initial values. Zinc and copper concentrations in dialysis exchange indicated that the patients absorbed zinc and lost copper in significant amounts. The patients had poor dietary intakes of both minerals. These data suggest that patients treated with CAPD benefit from oral zinc supplementation.

Adolescent↗

Renal angiomyolipoma associated with lymph node involvement and renal cell carcinoma in patients with tuberous sclerosis.

Renal angiomyolipomas are found in more than half of the patients with tuberous sclerosis. We report on 3 patients with tuberous sclerosis and pathologically aggressive renal angiomyolipoma with retroperitoneal lymph node involvement and/or renal cell carcinoma. All patients have had a benign course. The literature is reviewed and supports the benign nature of this seemingly aggressive disorder. An approach to the evaluation and treatment of a child with tuberous sclerosis and renal angiomyolipoma is presented.

Adolescent↗

Alternate-day prednisone therapy in children with IgA-associated nephritis.

IgA nephropathy (IgAN) leads to renal failure in up to 30% of affected children and adults. There is currently no consensus on therapy in IgAN. Six patients with risk factors for disease progression were identified based on clinical or histologic findings. These patients were treated with alternate-day prednisone for 12 to 60 (mean, 36) months and followed for 28 to 60 (mean, 54) months. Follow-up biopsies were available in four patients. At last examination all treated patients had normal urinalysis and serum creatinine level. Follow-up biopsies showed stable or improved glomerular histology in three of four patients. One patient had a slight worsening of the interstitial disease. No steroid toxicity was observed. The outcome of these treated patients was compared with that of 15 comparable patients from another center who received no treatment and with patients from two published clinical pathology series. A normal urinalysis was found at follow-up in all treated patients, compared with one of 15 untreated patients (P = 0.003). None of the patients in the published series with comparable disease had normal urinalysis at follow-up. End-stage renal disease or renal insufficiency occurred in seven of 15 untreated and no treated patients (P = 0.19). The data strongly support the need for a prospective control trial of prednisone therapy in IgAN.

Adolescent↗

Aggressive therapy of infants with renal failure.

Nine infants, who presented with renal failure within the first 3 months of life, were treated with continuous ambulatory peritoneal dialysis (CAPD). Seven infants survived to an age of 12-15 months, when they received transplants. Two patients died while on CAPD. Six infants are alive with a functioning renal allograft, at an average age of 35.5 months and an average of 22 months post-transplant. Neurological development is normal in four of the six infants tested. The mean current height of the six transplant recipients is just below 2 SD from the mean.

Humans↗

Therapy of focal segmental glomerulosclerosis with cyclosporine A.

Six patients with biopsy-documented focal segmental glomerulosclerosis were treated with cyclosporine A (CyA) a new and potent T-cell suppressant. Dosage was adjusted to maintain trough levels between 100 and 300 ng/ml (whole blood radioimmunoassay). Patients were treated for 6 weeks or until unacceptable drug toxicity occurred. In one patient proteinuria was significantly reduced. This same individual was the only patient treated within 6 months of diagnosis. Therapy was discontinued in two patients at week 4, one because of worsening renal failure and one because of hypertension. These complications occurred despite trough CyA blood levels of less than 300 ng/ml. The results suggest that further and controlled studies of CyA are warranted in the therapy of nephrotic syndrome associated with focal segmental glomerulosclerosis, especially when used early in the course of disease. The data also suggest enhanced toxicity of CyA in patients with active nephrotic syndrome.

Adolescent↗