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Objective: We examined whether height less than the 1% for age (z score <-2.5) at dialysis initiation predicts adverse clinical outcomes for children with kidney failure., Design: National cohort study of children initiating dialysis, followed for a minimum of 1 month to a maximum of 8 years., Setting: Voluntary consortium of pediatric nephrology centers across the United States and Canada in the North American Pediatric Renal Transplant Cooperative Study., Patients: Two thousand three hundred six patients

Background: There are no large studies of the effect of pretransplant dialysis status on the outcome of renal transplantation (Tx) in children. This study evaluated the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS) registry data for the outcome of Tx in pediatric patients who either (1) received their transplants preemptively or (2) were maintained on dialysis before receiving their transplants., Methods: We compared graft survival and patient survival rates, incidence of acute tubular necrosis (ATN), acute rejection episodes, and causes of graft failure in peritoneal dialysis (PD) patients with those maintained on hemodialysis (HD) and those undergoing preemptive Tx (PTx)., Results: Primary Tx was performed in 2495 children (59% male; 61% Caucasian; 1090 PD, 780 HD, 625 PTx) between 1/1/1992 and 12/31/1996. The overall graft survival rates of the PD and HD groups were similar, but were less than that of the PTx group (3-year: 82% PD and HD, 89% PTx, overall P = 0.0003). Improved graft survival in the PTx group was present only in recipients of grafts from living donors. There was no difference in the overall patient survival rate at 3 years, or in time to first acute-rejection episodes in the three groups. The incidence of ATN in the first 7 days post-Tx was higher in PD and HD patients than in PTx patients (11% PD and 12% HD vs. 2% PTx, P<0.001; HD vs. PD, P = NS). The major single cause of graft failure in each group was: PD, vascular thrombosis (200%); HD, chronic rejection (27%); PTx, acute and chronic rejection (21% each)., Conclusion: NAPRTCS data show that graft survival is improved in patients receiving PTx, compared with those receiving PD and HD. Graft loss resulting from vascular thrombosis is more common in children who receive PD than in those receiving HD.

The use of recombinant human growth hormone (rhGH) in patients undergoing dialysis has not received substantial investigation. The available data would indicate that short-term growth velocity is improved with rhGH in children undergoing both peritoneal dialysis and hemodialysis. However, definitive assessment awaits long-term controlled studies. Although definitive studies are lacking, it does not appear that there is a differential effect of rhGH in the various dialytic modalities. Compared to patients with chronic renal insufficiency, the response to rhGH treatment in children undergoing dialysis appears inferior; however, definitive data are also lacking. rhGH is effective when administered intraperitoneally; however, long-term studies are required to substantiate efficacy. In summary, despite limited data, rhGH appears to be effective in growth-retarded children undergoing dialysis.

We examined the clinical significance of hepatitis Be antigenemia in 36 HBsAg positive pediatric dialysis and renal transplant patients. One hundred twenty-seven sera were tested for HBeAg and anti-HBe. Seventy-three sera (57%) from 29 patients (81%) contained HBeAg. The presence of HBeAg was associated with an increased titer of HBsAg (P < 0.005) and with the presence of the HBsAg carrier state (P < 0.001). HBeAg was found in 40% of specimens taken from dialysis patients, and in 70% of specimens from transplant patients (P < 0.001). No serum contained anti-HBe, although 28 of 29 sera (97%) tested had antibody to HBcAg. No association was found between the presence of HBeAg and serum aminoleucine transferase levels or the histologic evidence of chronic active hepatitis. Fifteen HBeAg negative sera from patients persistently positive for HBsAg were tested for HBV-specific DNA polymerase activity; 7 (47%) had significant activity. Since both HBeAg and DNA p are indicators of infectivity, many HBeAg negative sera from immunosuppressed HBsAg carriers may be infectious.

Orally administered calcium carbonate was evaluated as a phosphate binding agent in 15 children, ages 0.6 to 17.2 years, receiving maintenance dialysis. Changes in plasma aluminum concentration were assessed after discontinuation of treatment with aluminum-containing gels. The mean daily dose of calcium carbonate was 5.1 +/- 2.5 gm (384 +/- 315 mg/kg/day), and correlated inversely with body weight (r = 0.72, P less than 0.01) and age (r = 0.71, P less than 0.01). Mean serum calcium, phosphorus, and bicarbonate values were unchanged throughout the study. Plasma aluminum concentration fell from 90 +/- 51 to 34 +/- 22 micrograms/L (P less than 0.005). Dietary phosphorus intakes were 44 +/- 21 and 42 +/- 19 mg/kg/day during the control period and at the end of the study, respectively. Transitory hypercalcemia was the only side effect in 92% of the patients. In none of the patients did uncontrolled hyperphosphatemia, metabolic alkalosis, diarrhea, or symptoms or signs of hypercalcemia develop. Our data indicate that calcium carbonate is an effective phosphate binding agent in children receiving dialysis, and should be used in lieu of aluminum-containing gels in young children with renal failure.

Fasting serum cholesterol and serum triglyceride levels were determined in 15 maintenance hemodialysis (MH) and 35 renal transplant (RT) patients. Fourteen of 15 MH patients (93%) had elevated triglyceride levels (greater than 140 mg/100 ml) compared to 11 of 35 RT recipients (31%) (P less than .001). Two of 15 MH patients (13%) had elevated cholesterol levels (greater than 230 mg/100 ml), compared to 18 of 35 RT recipients (51%) (P = .03). In MH patients, a positive correlation was noted between serum triglyceride levels and carbohydrate intake (P = .03). Autopsy material from 12 children who underwent MH or RT was compared to material from 16 age-matched controls; an increased collagenous content of intima, a possible early indicator of coronary artery disease, was noted more frequently (P less than .006) in index patients compared to controls. Our data demonstrate that hyperlipidemia is a frequent finding in pediatric patients treated with MH and RT, and may be associated with premature coronary artery disease.

Acquired cystic kidney disease (ACKD) occurs in adult patients undergoing long-term dialysis. Early detection is important because clinically significant hematuria and malignancies are associated with ACKD. We evaluated by magnetic resonance imaging (MRI) and ultrasonography (US) the incidence of ACKD in 15 patients aged 7.3-21.6 years (mean 15.9 years) with non-cystic primary renal disease. Nine patients had been treated with peritoneal dialysis only, and 6 with both hemodialysis and peritoneal dialysis for 24-73 months (mean 37 months). Three patients (20%) had no cysts. In 5 patients (33%) with bilateral multiple cysts, the diagnosis of ACKD was made by MRI and US. In another 5 patients, solitary cysts were localized to one kidney by MRI, and in 2 patients solitary cysts were seen in both kidneys. This study documents that ACKD is not limited to older patients with end-stage renal disease. Early detection of these cysts can be accomplished by MRI and is warranted since 1 patient developed neoplastic tubular changes which can precede tumor formation.

Bioassayable serum somatomedin activity could be estimated in uremic subjects only after appropriate correction for increased circulating inorganic sulfate. Somatomedin activity increased after hemodialysis in six of ten patients, possibly due to removal of somatomedin inhibitors.

Reduced somatomedin activity was uniformly present pre-dialysis in 7 children with end-stage renal disease. Somatomedin activity improved in 6 of the 7 patients following a single 5 hr hemodialysis. Percent normal growth correlated with post-dialysis somatomedin activity in 6 of the 7 children. No correlation was present between percent normal growth and calorie or protein intake. Reduced somatomedin activity in children with end-stage renal disease appears to be due to a circulating uremic inhibitor(s). Somatomedin activity may be a useful in vitro test for adequacy of dialysis in children. Further efforts are necessary to identify the inhibitor(s) of somatomedin activity in uremia.

Serial HB Ag determinations were obtained on 62 children undergoing hemodialysis during a 31/2-year period. Thirty six (58%) of the patients had atleast one positive HB Ag determination (titer larger than 1:8) either during the period of dialysis or within 2 months after transplantation. Of the children who became HB Ag positive during the period of dialysis, 89% demonstrated hepatitis B antigenemia during the initial 6 months of dialysis. Becuase of the temporal relationship between the development of HB Ag positivity and the duration of dialysis, contamination of equipment was proposed as a contributing source for transmission of HB Ag. Although 67% of the patients who were HB Ag positive during dialysis developed biochemical evidence of hepatic dysfunction, only two patients had clinical manifestations. The incidence of hepatic involvement appeared to be related to age; only one of nine children less than 12 years of age had evidence of hepatic disease. Intermittent persistence of HB Ag for periods larger than 3 years was observed. Both the incidence and persistence of HB Ag indicate the need to prevent its acquisition by pediatric patients undergoing hemodialysis.

Peroneal motor nerve conduction velocities (MNCVs) were performed on 58 children aged 20 months to 12 years undergoing chronic hemodialysis. No patient had any clinical manifestations of uremic polyneuropathy. The mean MNCV in 21 children at the onset of dialysis was 42.0 m/sec; significantly slower than the control group of 51.4 +/- 5.3 m/sec (p less than 0.001). 16 studied performed between the 1st and 6th month had a mean MNCV of 43.2 +/- 5.7 m/sec, also slower than the normal controls (p less than 0.001). We conclude that peroneal MNCVs are reduced in most children at the initiation of chronic hemodialysis and do not change significantly during the next 6--12 months and that the routine practice of obtaining such studied is of no value in the clinical management of children undergoing chronic hemodialysis.

Factors involved in choosing a treatment modality for the infant, child, and adolescent with ESRD differ from those used when counseling an adult patient. Age at the time ESRD develops, mental status, psychosocial status, and the primary renal disease must be taken into consideration when contemplating the optimal therapeutic modality for the pediatric patient with ESRD. The ideal approach to optimize growth in the pediatric patient with ESRD remains to be delineated.

Hemodialysis was initiated in a mild-moderate hemophiliac at 15 years of age. Hematuria had been a frequent and persisting feature from the age of five years without documented cause. Anemia and proteinuria was first detected at 13 years. A cadaver donor renal transplant was carried out after three months of hemodialysis. Massive intravesical bleeding complicated the immediate post-transplantation period. The allograft rejected after three months and the patient was maintained for eight years on home hemodialysis. A second cadaver donor allograft was carried out at 23 years of age. Again, massive intravesical hemorrhage was a problem post-transplant. The allograft is currently functioning 27 months post-transplant. Factor VIIIc activities have fluctuated between 5% and 40% in the absence of factor infusions.