MRI in the diagnosis of a peritoneal leak in continuous ambulatory peritoneal dialysis.

Mechanical problems in continuous ambulatory peritoneal dialysis (CAPD) can result in ultrafiltration failure and disruption of CAPD therapy. The recently described tool of CT peritoneography with water-soluble contrast medium has the disadvantage of radiation and instillation of nephrotoxic substances. We report a child with a peritoneal leak diagnosed by MRI after instillation of a gadodiamide-dialysate mixture. This method provided good anatomical detail without radiation or nephrotoxic agents.

Reduction of delayed renal allograft function using sequential immunosuppression.

Previous data suggested that outcome in small children with cadaveric renal transplantation might be improved with sequential therapy. This protocol combines augmented immunosuppression [by including antibody induction (ATG)] with avoidance of nephrotoxic medication in the immediate postoperative phase (by delayed start of cyclosporin therapy). In this report, we describe effects of this approach in 12 consecutively transplanted small children of less than 5 years of age (mean 3.2 years) who received a cadaveric renal graft at our institution between 1991 and 1998. Up to 1996 triple therapy (prednisolone, azathioprine, cyclosporin) and since 1997 sequential therapy (prednisolone, azathioprine, ATG until serum creatinine <2 mg/dl, then cyclosporin) was used for immunosuppression. Five children had delayed graft function (45.4%), all of whom were treated with triple therapy including cyclosporin from the very beginning, whereas children treated by the sequential protocol gained immediate graft function (P<0.05). There was no statistical difference between the two protocols concerning frequency or severity of rejections (67% vs. 60%, all steroid responsive), difference in the incidence of either bacterial or viral infections, or between the incidence of hypertension. Although not reaching statistical significance, 1-year graft survival rates also increased from 60% for triple therapy to 80% for sequential therapy. In conclusion, our findings confirm previous studies showing that outcome in small children undergoing renal transplantation may be improved by specially tailored treatment protocols such as sequential therapy.

Low serial serum neopterin does not predict low risk for chronic renal graft rejection.

Research has provided new and potent immunosuppressants which can potentially stop ongoing rejection. Subclinical rejection is a particular problem in the pediatric age group and early identification of children at risk is of the utmost importance. Neopterin has been previously shown to be a non-specific but sensitive marker for immunologic activity. In this study we hypothesized that low serum neopterin in the 1st year after transplantation predicts a low risk of chronic rejection. We retrospectively analyzed serial neopterin data obtained beyond the early postoperative period in 21 children and correlated the peak and average with glomerular filtration rate (GFR) loss during the subsequent years (P = 0.63, NS, r = 0.10). Our results show that serum neopterin did not differ between the majority of children who developed chronic transplant dysfunction and children with stable transplant function beyond the early post-transplant period. Thus serum neopterin failed to delineate a low-risk population who might be spared more invasive diagnostic procedures such as protocol biopsy.

Timing of peritoneal dialysis catheter removal after pediatric renal transplantation.

BACKGROUND: A peritoneal dialysis (PD) catheter is in place at the time of kidney transplantation in children receiving PD. Removal of the catheter eliminates the risk of catheter-related infections. However, the patient benefits from leaving the catheter in place if dialysis is necessary posttransplantation. There is currently no consensus on the proper timing of PD catheter removal after kidney transplantation in children. OBJECTIVE: To identify the risks and benefits of an in-dwelling PD catheter after renal transplantation in children. DESIGN: Retrospective single-center study of infectious complications and posttransplantation PD catheter use in 31 renal transplantations in 26 children. RESULTS: Peritoneal dialysis catheters were used postoperatively in 13 of the 31 transplantations. In 12 instances the catheter was needed during the first month after transplantation, and 2 of the patients involved did not have a catheter in place when needed. Six catheter-related infections occurred in 5 patients posttransplantation, with only 1 infection taking place within 1 month after transplantation. CONCLUSION: Our data suggest that the need for catheter use occurs predominantly during the first month, while infectious complications usually happen later. This strongly suggests that PD catheters should not be removed until approximately 1 month after kidney transplantation.

Reproducible erythroid aplasia caused by mycophenolate mofetil.

Anemia secondary to mycophenolate mofetil (MMF) was recently described in experimental animals. A clinical association between MMF and anemia has been observed, but there are no proven reports. We describe a girl with chronic graft failure who developed erythroid aplasia under immunosuppression with MMF. She showed prompt resolution when MMF was discontinued and a recurrence of this clinical course when MMF was restarted. As re-challenge with a medication is the most definitive approach for showing a direct relationship between the drug and the side effect, this case clearly demonstrates that MMF can cause erythroid aplasia.

Lymphocyte subsets in renal transplant recipients during growth hormone therapy.

OBJECTIVE: To evaluate the effect of growth hormone treatment on growth, levels of insulin-like growth factor I (IGF-I) and lymphocyte subsets in immunosuppressed renal allograft recipients. METHODS: 18 children (aged 8.0-16.6 years) received growth hormone 1 IU/Kg/week daily for two years. Height, IGF-I levels and in 11/18 patients, lymphocyte subsets were evaluated serially. RESULTS: Standardized growth velocity increased from -1.0+1.5 to +1.2+2.2 and standardized IGF-I levels from +0.8+1.5 to +3.1+1.1 (1 year) and to +1.4+1.7 (2 years). The total lymphocyte count and the number of T lymphocytes (CD3+) decreased. The decrease was more marked in CD8+ (from 1.5+0.3 x10(9)/L to 0.9+0.3 x10(9)/L, 1 year and to 0.8+0.1 x10(9)/L, 2 years) compared to CD4+ (from 1.5+0.3 x10(9)/L to 1.0+0.2 x10(9)/L, 1 year and to 1.3+0.2 x10(9)/L, 2 years), resulting in an increment of the CD4+/CD8+ index. CONCLUSIONS: The differential effect of growth hormone treatment on CD4+ and CD8+ lymphocytes might be explained by different expression of the IGF-I receptor in these distinct subsets.

Treatment of peripubertal children after renal transplantation (RTX) with recombinant human growth hormone: auxological data and effects on insulin-like growth factor-I (IGF-I) and IGF-binding protein-3 (IGFBP-3) during 24 months.

OBJECTIVE: To evaluate growth and endocrine parameters in RTX children with GH treatment during 24 months. SUBJECTS: 18 children (13 boys), age 13.1 yr (8.0-16.6), bone age 10.1 yr (5.4-15.3). Patients were 2.8 yr (0.5-7.5) after RTX and had immunosuppressive therapy, prednisone 0.16 mg/kg/d (0.08-0.68). METHODS: GH (4 IU/m2/day s.c.) was given and patients were seen every 3 months for evaluation of height, height velocity, bone age, and hormone parameters. Serum IGF-I was determined by RIA, IGFBP-3 by RIA and Western ligand blotting (WLB). Renal function and adverse effects (GFR, glucose tolerance, rejection episodes) were monitored. RESULTS: Height (+1 SDS) and height velocity (+2.2 SDS) increased significantly during 24 months GH treatment, but delta BA/delta CA was 1.7 and 1.5 during the first and second treatment year, respectively, and all patients entered puberty during the treatment period. GFR decreased slightly during 2 yr (p = 0.048), two patients had chronic rejection and GH therapy was terminated in one patient because of glucose intolerance. The ratio IGF-I/IGFBP-3 rose during the first year (p = 0.002) indicating more bioavailable IGF-I. IGFBP-3 determined by WLB was decreased, but IGFBP-1, -2 and -4 were elevated as compared to a standard. CONCLUSIONS: GH treatment increased height and growth rate in children after RTX. This may be due to significant changes in IGF-I and IGFBP-3 relationship. However, bone maturation was also accelerated thus diminishing height potential. From month 12 to 24 a continuous decrease of IGF-I was observed. There was a slight but significant deterioration of graft function. Adverse events that led to termination of GH therapy were observed in 3 of 18 patients.

[Chronic peritoneal dialysis as home therapy in childhood--risks and complications]. / Chronische Peritonealdialyse als Heimtherapie im Kindesalter--Risiken und Komplikationen.

BACKGROUND: The aim of our study was to ascertain the complications of chronic peritoneal home dialysis in childhood. PATIENTS: 17 children were treated by ambulatory peritoneal home dialysis between 1984 and 1994 at the paediatric dialysis unit of the University Children's Hospital in Vienna, Austria. Their average age was 6.5 years (1 week to 12 years); 7 (41.2%) children were below school age (< 6 years). RESULTS: In our observation period of 369 dialysis months (DM), the average duration of dialysis was 21.7 months (4.0-74.3). In relation to total DM the incidence of peritonitis was 1:23.1 of exit site infection 1:14.8 and of catheter related complications 1:41.0. 5 children developed hernias. 5 children were switched to haemodialysis and 8 children received kidney transplants. 2 children died from non-dialysis-associated causes. CONCLUSION: Peritoneal dialysis, in contrast to haemodialysis, is a home treatment modality applicable even to infants. The most common complication is infection. Our data and the European and North American literature show that by close ambulatory monitoring and special hygenic procedures peritonitis frequency can be markedly reduced.

[Function and structure of families with chronically ill children]. / Funktion und Struktur von Familien mit chronisch kranken Kindern.

In modern medicine especially the sick child often points out the limits of the psychosocial potentialities. This project investigates the function, structure, coping mechanisms, power and weakness of families with chronically ill children. We investigated 28 children from the nephrological ward and 55 patients from the cardiological department and also their families with the FAM III and compared the obtained T-scores with the results of the control-group (76 families, t-test, analysis of variance). Families with patients after renal transplantation (NTX) pointed out significant worse T-scores than the group with patients on CAPD or with preterminal renal insufficiency and CG (p < 0.05). Within the cardiological groups the differences were not statistically significant, on the other hand the group with patients before heart-operation and the group with patients after palliative heart-operation had better T-scores than the CT (p < 0.05). These results demonstrate that families with children suffering from a chronic renal or heart disease mobilize substantial resources to cope with these problems. By contrast the results of the families with patients after NTX or successful heart surgery are significant worse than the control-group and the other investigated patients groups. Our results come to the conclusion that especially after successful NTX or heart-surgery a psychosocial care of these families is necessary.

Use of prostaglandin I2 in three small children at high risk of early renal graft thrombosis.

We report the use of prostaglandin I.2. (PGI2) in three small children weighing less than 15 kg at high risk of graft thrombosis after cadaveric renal transplantation complicated by acute tubular necrosis. PGI2 was started at a dose of 5 ng/kg per min within the first 6 h after transplantation, and was continued for 12-15 days. Before and during PGI2 infusion, color-coded and pulsed Doppler sonography was performed. We found immediate restoration of diastolic flow, consistent with a decrease in vascular resistance. During the subsequent days, the sonographically assessed flow pattern and clinical graft function improved gradually. None of the three consecutively treated children developed graft thrombosis or lost his graft; no clinically relevant bleeding or adverse hemodynamic or pulmonary effects were seen.

Estimation of total creatinine clearance is unreliable in children on peritoneal dialysis.

OBJECTIVE: To test the reliability of creatinine clearance in children on peritoneal dialysis (PD). DESIGN: Longitudinal, case-controlled. SETTING: Routine clinic visits at the pediatric dialysis unit of the Universitätskinderklinik of Vienna. PATIENTS: Eleven children (2-13 years, 10-55 kg) with end-stage renal disease on PD. INTERVENTIONS: Creatinine clearance (CCr) was determined by measuring creatinine excretion (ECr) over 24 hours in both dialysate and urine. Each child had three to five separate measurements of their CCr. At the same time we also calculated the Schwartz formula clearance from the patient's height and serum creatinine, using a modified correlate. MAIN OUTCOME MEASURES: Reliability of CCr was assessed by two approaches. First, we compared each serial measurement with the mean value for each patient and thereby assessed the "intramethodical" variability. Second, we compared each CCr with the simultaneous formula clearance and assessed the "intermethodical" disagreement. RESULTS: Twenty-seven percent of the measurements of CCr were classified as unreliable based on a comparison with the mean value for each patient. Reliability was closely correlated with residual renal function (p < 0.01); only 12% of the measurements in the anuric patients were classified as unreliable (vs 31% in the patients with residual renal function). The simultaneous formula clearance was less variable than the CCr. The formula clearance had a sensitivity of 93% and a specificity of 60% for detecting unreliable values of CCr. CONCLUSION: Estimation of total CCr is unreliable in pediatric patients on PD. A simultaneous formula clearance can be used to detect which values are unreliable.

[Spontaneous growth of children with chronic renal failure]. / Spontanes Längenwachstum bei Kindern mit chronischer Niereninsuffizienz.

Several pathogenetic factors may contribute to the growth failure in patients with CRF. We have analysed retrospectively spontaneous growth in 30 patients with CRF and investigated the influence of various therapies (conservative therapy, hemodialysis and transplantation with different immunosuppressive therapy) on spontaneous growth. At diagnosis (age 8.5 +/- 0.8 years; mean +/- SEM) height was reduced in the whole group (-1.46 +/- 0.2 SDS; x +/- SEM). Height SDS decreased further during the observation period of two and three years in the hemodialysis group (-2.19 +/- 0.69) and in the group with conservative treatment (-2.58 +/- 0.93), respectively. After Tx (n = 26) patients received different types of immunosuppressive therapy: 18 patients received cyclosporin A and prednisone (4-6 mg/m2 BS) daily; 8 transplanted patients received azathioprine (2 mg/kg BW) additionally. After Tx height improved not significantly (-2.02 +/- 0.30 vs. -1.49 +/- 0.36 SDS and -2.52 +/- 0.64 vs. -2.05 +/- 0.24 SDS after three and two years, respectively) irrespective of the immunosuppressive therapeutic regime. In the whole patient group neither hemodialysis nor conservative treatment nor Tx caused a significant change in height SDS; the possible factors, that might be involved in growth failure, are discussed.

Effect of growth hormone treatment on glucose tolerance in a patient with cystinosis after kidney transplantation.

A 16 year-old boy with nephropathic cystinosis and kidney transplantation was successfully treated with rhGH because of growth retardation. After 15 months of rhGH therapy he developed impaired glucose tolerance. Various causes like cystinosis itself, the immunosuppressive therapy with cyclosporine A and cortisone, but rhGH too might have been the responsible factors for that. Treatment with rhGH was initiated again after 4 months of interruption of therapy because no relation between impaired glucose tolerance and GH could be established.

Tubulointerstitial nephritis and uveitis: an immunological disorder?

A 14-year-old boy with tubulointerstitial nephritis and uveitis (TINU syndrome) is described. Nephropathy improved without systemic cortisone treatment, whereas uveitis relapsed and was treated with topical steroids. Blood cell immunological analysis and serum analysis revealed signs of cytotoxic T-cell, macrophage and granulocyte activation, which declined as the clinical symptoms improved. This may be interpreted as an indication of their significance as markers in the pathogenesis of this syndrome or as part of a microbial-triggered immune response.

[Formula creatinine clearance as a substitute for 24-hour creatine clearance in children with kidney transplantation]. / Formula-Kreatininclearance als Ersatz der 24-Stunden-Kreatininclearance bei nierentransplantierten Kindern.

Despite many theoretical advantages, formula-creatininclearance (Schwartz et al, Journal of Pediatrics 1976) has not found broad clinical acceptance in everyday pediatric patient care. In this study we report our results of long term observations (11.7 +/- 6.8 (1.7-24.8) months) of measured and computed creatininclearance in 27 children after renal transplantation (15 boys, 12 girls, mean age 14.5 +/- 4.2 (5.5-20) years) at the Kinderdialyse of the Universitäts-Kinderklinik of Vienna. We found a wide scattered correlation between the measured and computed creatininclearance values with a 90% confidence interval between -30% to +60% of the 24 hour creatininclearance. Formula creatininclearance (SD 17.8%) was markedly better reproducable than the 24 hour creatininclearancethe (SD 37.8%), the intraindividuell collecting error (36.1%) was almost twice the interindividuell "coefficient" error (20.27%). We therefore conclude that the 24 hour creatininclearance is by far not as accurate as the complexity of the procedure pretends and support broad clinical acceptance for the formula creatininclearance.

Subcutaneous recombinant human erythropoietin in children with renal anemia on continuous ambulatory peritoneal dialysis.

Subcutaneous recombinant human erythropoietin (rHuEpo) treatment of renal anemia was performed in four boys and eight girls on CAPD, aged 0.8-12.5 (mean 7.4) years. In contrast to previous studies, our therapeutic goal was not set with a hematocrit of 30% but with full correction of anemia. Following a maximum weekly rHuEpo dosage of median 120 (range 100-240) IU/kg body weight, hematocrit increased in 10 children from 24 (14-29)% within 12 (4-17) weeks to 40.1 (33.5-48.4)%. The weekly increase in hematocrit was 1.27 (0.5-3.1)%. The corrected reticulocyte count increased from 1.3 (0.7-1.8)% to 2.3 (1.4-3.9)% within 4 (2-6) weeks. Eight children fulfilled the protocol; six with an uncomplicated course were able to maintain a hematocrit of 37.1 (35.1-42.7)% with only one sc medication per week of approximately two-thirds of their highest weekly rHuEpo dosage. No serious adverse effect of rHuEpo therapy was observed.

Renal transplant hemodynamics in children: prospective analysis of colour coded versus pulsed Doppler sonography.

In 30 children with renal allografts the diagnostic validity of pulsed Doppler (PD) versus colour coded Doppler (CD) sonography was assessed prospectively. 46 PD examinations were performed calculating the resistive index (RI) in the segmental arteries in comparison to 46 CD scans, where renal blood flow throughout the grey-scale image was displayed. In addition, point-spectral analysis with calculation of the RI was also performed on the CD scans. The time for examination ranged from five to ten minutes for the PD and from three to five minutes for the CD study. Concordant findings for the PD and CD technique were generally obtained (normal blood flow pattern on PD-excellent visualization of renal blood flow on CD, reduced or reversed diastolic flow on PD-poor visualization of renal blood flow on CD). There was close correlation of the RI values obtained by the PD and CD scans. CD sonography facilitated point-spectral analysis in shortening the time for examination. The ability to visualize focal hemodynamic alterations provided a higher diagnostic accuracy in comparison to PD sonography.