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OBJECTIVE: To determine the patient and donor characteristics important for short-term and long-term renal transplant survival at Cincinnati Children's Hospital Medical Center. METHODS: Cumulative transplant survival was calculated and univariate analysis of graft survival performed on 206 transplants done since 1970 in 148 pediatric patients. Grafts to black recipients were analyzed separately. Short-term graft survival is defined as 1-year allograft survival and long-term graft survival as graft half-life (t1/2) survival for allografts functioning after the first posttransplant year. RESULTS: One-year graft survival of living-related donor (LRD) and cadaver donor (CAD) transplants was 77% and 62%, respectively. Graft t1/2 was 11.2 years for LRD and 9.8 years for CAD grafts. The CAD 1-year graft survival when the recipient or donor was younger than 7 years was 36% and 41%, respectively. The LRD 1-year graft survival to children younger than 7 years was 88% versus 75% in older children. Graft survival at 1 year was similar for CAD primary and retransplants (60% vs 65%), but graft t1/2 better for CAD primary grafts (17.8 years vs 5.0 years, P < 0.001). Preemptive LRD grafts performed similarity at 1 year and better over the long term compared with patients who had long-term dialysis (85% vs 74%, P = NS; and 16.9 years vs 8.0 years, p < 0.001). Preemptive CAD grafts did poorly, with 1-year graft survival of 38%. Administration of Cyclosporine A (CsA) improved CAD 1-year graft survival (76% vs 54%, p < 0.001) but not long-term survival. Thirty grafts to 24 black children had a 1-year survival of 48%, with no graft surviving more than 5 years. CONCLUSIONS: Living-related donor transplantation should be aggressively pursued for young children. If a LRD is unavailable and the young child's medical condition is stable, delay in CAD transplantation should be considered, with dialysis before transplant. Use of CsA improves 1-year pediatric graft survival, but does not improve graft survival after 1 year at the Children's Hospital Medical Center. New strategies to improve graft survival in black children should be pursued.

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Because controlled trials in adults have shown accelerated deterioration of renal function in a small number of patients receiving calcitriol for renal osteodystrophy, we initiated a prospective, randomized, double-blind study of the use of calcitriol versus dihydrotachysterol in children with chronic renal insufficiency. We studied children aged 1 1/2 through 10 years, with a calculated glomerular filtration rate between 20 and 75 ml/min per 1.73 m2, and with elevated serum parathyroid hormone concentrations. Ninety-four patients completed a mean of 8.0 months of control observations and were randomly assigned to a treatment period; 82 completed the treatment period of at least 6 months while receiving a calcitriol dosage (mean +/- SD) of 17.1 +/- 5.9 ng/kg per day or a dihydrotachysterol dosage of 13.8 +/- 3.3 micrograms/kg per day. With treatment the height z scores for both calcitriol- and dihydrotachysterol-treated groups showed no differences between the two groups. In relation to cumulative dose, there was a significant decrease in glomerular filtration rate for both calcitriol and dihydrotachysterol; for calcitriol the rate of decline was significantly steeper (p = 0.0026). The treatment groups did not differ significantly with respect to the incidence of hypercalcemia (serum calcium concentration > 2.7 mmol/L (> 11 mg/dl)). We conclude that careful follow-up of renal function is mandatory during the use of either calcitriol or dihydrotachysterol because both agents were associated with significant declines in renal function. There was no significant difference between calcitriol and dihydrotachysterol in promoting linear growth or causing hypercalcemia in children with chronic renal insufficiency. Dihydrotachysterol, the less costly agent, can be used with equal efficacy.

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Seventy-six children with idiopathic MPGN have been followed at this medical center since 1957 (mean 10.6 years of disease). Seventy-one of the children have been treated with a regimen of prednisone (mean 7.7 years of treatment). Monotherapy with alternate-day prednisone was used in 50 of the children. Other immunosuppressive and cytotoxic drugs have not been used since 1981. In the 71 treated patients, the cumulative renal survival (creatinine less than 3.0 mg/dl (265.5 mumol/L)) was 82% in the tenth year and 56% in the twentieth year after disease onset. Cumulative renal survival, calculated from the date of initiation of the prednisone regimen, was 75% in the tenth year and 59% in the twentieth year. As our experience with this disease has increased, long-term cumulative renal survival has improved.

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During the control period of the Growth Failure in Children With Renal Diseases Study, investigators at 23 centers were able to observe and characterize growth and to make anthropometric and nutritional measurements in 82 children with mild to moderate renal insufficiency. As a multicenter, controlled clinical trial designed to study the relative efficacy of 1,25-dihydroxyvitamin D3 and dihydrotachysterol in the treatment of renal osteodystrophy, no prior vitamin D exposure and a creatinine clearance of 25 to 75 ml/min/1.73 m2 were criteria for entrance into the clinical trial. Ages ranged from 18 months to 11 years (mean 5.6 +/- 3.1 years), and distribution by age category was as follows: 38%, 1 to 3 years; 28%, 4 to 6 years; and 34%, 7 to 10 years. There was a 3:1 male/female ratio; 72% of the patients had congenital disease by the International Classification of Diseases (ninth revision). Mean creatinine clearance was 49.5 +/- 20 ml/min/1.73 m2. The C-terminal parathyroid hormone values (1121 +/- 1562 pg/ml) were well above 2 SD of the mean of a normal growing population of similar age. Parathyroid hormone values correlated with degree of renal insufficiency (r = -0.57) and with height by bone age but not with chronologic height or growth velocity. The bone age/height age ratio, a predictor of growth potential in normal children, was low for the entire series of patients (0.88 +/- 0.35) but failed to correlate with growth velocity and was negatively correlated with rising parathyroid hormone levels. Average values for height, weight, triceps skin fold, mid-arm muscle circumference, and body mass index were within 2 SD of the mean of the normal population, although measurements for the 1- to 3-year age group were significantly less than those of the older patients. Total energy intake averaged less than 86% of the recommended dietary allowances; total protein intake was more than 161% of the allowance. Nitrogen balance in 23 patients was positive and correlated most significantly with increasing energy intake (r = 0.6). Growth velocity, calculated from the interval gain during the month control period, averaged +0.3 SD, with the highest growth velocity z scores recorded for those with acquired disease. A growth velocity index, expressed as the slope of the regression between change in height SD and growth velocity z score, was used to describe the growth accomplished in the control period by age category.(ABSTRACT TRUNCATED AT 400 WORDS)

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This report describes the serum osteocalcin values in children with mild to moderate, but relatively stable, renal dysfunction followed in the Growth Failure in Children With Renal Diseases Study. This report is derived from data obtained during the control period (6 months) before the initiation of vitamin D therapy. Up to three measurements per patient were obtained. Serum osteocalcin concentration was compared with creatinine clearance (glomerular filtration rate) calculated by the Schwartz formula; with serum concentrations of alkaline phosphatase, parathyroid hormone, and bicarbonate; and with the percentages of the recommended dietary allowances of calories and protein ingested. By standard correlation techniques, there appeared to be an inverse correlation between calculated creatinine clearance and serum osteocalcin concentration, and a direct correlation between serum osteocalcin and parathyroid hormone values. However, when we employed a statistical technique that takes into account repeated measures in the same patient, no correlation was found between calculated glomerular filtration rate and serum osteocalcin concentration, and no direct correlation was found between serum osteocalcin and parathyroid hormone values. The lack of a correlation between calculated glomerular filtration rate and serum osteocalcin values may be due to large fluctuations in the serum osteocalcin concentration, even though renal function is relatively stable.

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We reviewed the records of 154 children who received 207 renal transplants for end-stage renal disease from 1965 to 1987, and discovered that 48 (31%) had had convulsive seizures, some before transplant surgery, others only after transplant, and some during both before and after transplantation. The majority of children had minimal long-term problems, and 60% of the children had only a single convulsion. In six of the patients, convulsions were a manifestation of more serious underlying conditions that produced significant morbidity. Seizures of differing clinical type occurred, with hypertension being the most significant etiologic factor. In children with renal failure, there are minimal symptoms heralding the hypertensive encephalopathy. Rapid resolution without recurrence of seizures after control of hypertension is a major sign that hypertension was the cause and that the long-term prognosis is good.

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A 7-year-old boy with mild renal failure and signs and symptoms of acute poststreptococcal glomerulonephritis including severe hypocomplementemia had, by renal biopsy, numerous crescents but no deposits in the glomerular capillary loops. Instead, deposits identical in location and composition to those described for children with idiopathic rapidly progressive glomerulonephritis were present. The severe hypocomplementemia was found to be due to high levels of C3 nephritic factor; niether nephritic factor nor hypocomplementemia has been reported in rapidly progressive glomerulonephritis of the idiopathic type. Following prompt therapy with methylprednisolone intravenously, serologic abnormalities disappeared and renal function greatly improved, but a later biopsy showed 50% of the glomeruli obliterated by scarring. The case is of importance not only in indicating that severe hypocomplementemia does not rule out idiopathic rapidly progressive glomerulonephritis but also in adding to the list of diseases in which nephritic factor can be found.

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Of 14 potential anencephalic donors, the kidneys of six were transplanted to recipients ranging in age from 4 to 19 years and in weight from 8 to 49 kg. In three recipients, serum creatinine levels are less than 1.0 mg/dl 1.5 to 9.5 years after transplant. The transplants failed in two for technical reasons; in one, the kidneys were lost by rejection. The other anencephalic infants were not suitable as donors because of rapid deterioration of vital signs (4), ABO incompatibility (3), and inability to perfuse the kidney (1). Successfully transplanted kidneys from this source rapidly increase in size and function to accomodate even large recipients.

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In all of eight patients with mesangiocapillary glomerulonephritis who received prednisone in an alternate-day schedule for 3 to 9 hr, the glomerule showed a marked reduction in mesangial cellularity and complete regression of mesangial interposition. Subendothelial deposits disappeared but intramembranous dense deposits, when present previously, persisted. Repair was incomplete in that a few mesangial deposits were still present, immunoflourescence was unchanged, and several of the patients developed focal mesangial scarring and increased numbers of global bodies. Functional glomerule and glomerular tuft segments, however, showed greatly improved morphology. There was no evidence of deterioration of renal function over a follow-up period of 5 to 11.5 yr.

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Of 182 patients with acute glomerulonephritis, 20 had normal C3 levels at onset. Normocomplementemic and hypocomplementemic patients were similar with respect to incidence and site of preceding streptococcal infection, elevation of ASO titer, distribution by age, sex, race, season, and year,\and glomerular morphology by light and electron microscopy. They differed in that the normocomplementemic patients tended to have normal serum C5 levels and, for reasons not clear, reduced serum albumin and elevated cholesterol levels. The consistent absence by immunofluorescence of IgG in the glomeruli of five hypocomplementemic patients and its presence in five normocomplementemic patients was considered a chance observation. The data suggest that in each group the nephritis was poststreptococcal and that the mechanism producing poststreptococcal glomerulonephritis is capable of acting independently of that activating circulating C3.

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