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We report four cases of West Nile virus (WNV) transmission following a single multiorgan donation in north-eastern Italy. The transmissions were promptly detected by local transplant centres. The donor had been tested for WNV by nucleic acid amplification test (NAT) prior to transplantation and was negative. There were no detected errors in the nationally implemented WNV safety protocols.

GATA3 gene encodes a transcription factor expressed during thymus, liver, kidney, adrenal gland, central and peripheral nervous systems, placenta and T lymphocytes embryonic development. Mutations of GATA3 cause Hypoparathyroidism, sensorineural Deafness and Renal dysplasia syndrome (HDR). We report the case of a girl with a terminal deletion of the short arm of chromosome 10 (10p12.1-pter), including both HDR locus and the DiGeorge critical region 2 (DGCR2), with HDR phenotype but not DiGeorge syndrome 2 features. The girl developed chronic renal failure during the first year of life, associated with sensorineural hearing loss, facial dysmorphic features and psychomotor development. She had hypodysplastic kidneys and bilateral grade 3-vesicoureteric reflux. Her karyotype was 46,XX,del(10)(p12.1-pter). Quantitative analysis by Real Time PCR on blood DNA confirmed the lack of one copy of GATA3 gene. She underwent renal transplantation at the age of 11. Our patient is the first case with a large deletion of the short arm of chromosome 10 - that certainly involves DGCR2 - with the HDR phenotype but without the clinical features of DGS2. This peculiarity suggests the hypothesis that the mechanisms underlying this syndrome may be more complex. It is therefore possible that DGS2 may be determined by locus heterogeneity.

Background: Dialysis efficiency has a great influence on the outcome of patients. Few data are available on the removal of solutes with molecular weights higher than urea and creatinine. The aim of our study was to assess the transport and the removal of substances with molecular weights up to 15 kD and to evaluate the contribution of residual renal function in peritoneal dialysis (PD) children., Methods: Seventeen patients of 12 +/- 4 years undergoing automated PD were studied. Ten patients had 563 +/- 355 mL/day of urine output, and 7 were anuric. During a standardized nightly intermittent PD (NIPD) session, a single-injection inulin clearance was performed. Urea, creatinine, inulin (measured by HPLC), cystatin C and beta2-microglobulin (beta2m) were measured in blood, urine and dialysate. Clearances (L/week/1.73 m2) and weekly solute removal index (SRI) were calculated for all the solutes; weekly Kt/V was calculated for urea., Results: In non-anuric versus anuric patients the total clearances were: urea 82.6 +/- 18.3 versus 71.3 +/- 26.4; creatinine 82.7 +/- 28.6 versus 47.8 +/- 18.8; inulin 42.8 +/- 11.3 versus 32.8 +/- 20.4; beta2m 14.2 +/- 13.8 versus 9.2 +/- 8.3; cystatin C 20.2 +/- 9.4 versus 9.7 +/- 4.8. In the patients with residual diuresis, the urea was removed mainly by PD (69.2%), while inulin, beta2m and cystatin C were removed by renal clearance (64.0%, 79.5% and 62.8%, respectively). Total, peritoneal and renal weekly Kt/V values in the subjects with residual renal function, were 2.86 +/- 0.70, 1.99 +/- 0.40 and 0.87 +/- 0.43, respectively. Peritoneal weekly Kt/V in the anuric patients was 2.36 +/- 0.85; total weekly Kt/V in the total group was 2.65 +/- 0.78. Weekly SRIs in non-anuric versus anuric patients were: urea 2.56 +/- 0.58 versus 2.09 +/- 0.74; creatinine 2.66 +/- 0.73 versus 1.46 +/- 0.56; inulin 2.36 +/- 0.92 versus 1.64 +/- 1.60; beta2m 1.26 +/- 1.10 versus 1.20 +/- 1.90; cystatin C 1.72 +/- 0.83 versus 1.58 +/- 1.62., Conclusions: Solutes removed during PD tend to decrease following an increase in molecular weight of the substance. Since anuric patients are at higher risk of middle molecule and small protein accumulation, more attention should be paid to the removal of middle molecules. Further studies should be undertaken to evaluate whether removing them has a clinical impact and to determine their threshold levels.

Human parvovirus B19 is considered an etiologic agent of aplastic anemia in immunosuppressed patients. Microscopic vasculitis, with or without renal involvement, has recently been attributed to this viral infection in immunocompetent patients. This study describes four cases of thrombotic renal graft microangiopathy presumably secondary to B19 infection. Twelve to 50 days after transplantation, four patients presented a renal graft dysfunction with creatinine rising to 360 to 1088 micromol/L and requiring hemodialysis in three cases. Renal involvement appeared after a systemic illness characterized by fever, fatigue and arthralgia, aplastic anemia (hemoglobin ranged from 5.3 to 7.8 g/dl), and thrombocytopenia. A thrombotic microangiopathy was observed in the renal biopsies, and the parvovirus B19 genome was isolated by PCR from the specimens. All four patients also became IgM-positive for parvovirus. Three of the four renal biopsies taken at the time of transplantation (T0) from the same patients were found positive for the B19 genome. Graft function recovered, with resolution of the aplastic anemia, within 22 to 110 d. Twenty biopsies performed as routine controls or for suspected acute rejection and nine T0 biopsies of patients with no signs of B19 infection were used. The B19 genome was found in two of 20 posttransplant biopsies and in one of nine T0 biopsies. The temporal association between aplastic anemia and the onset of thrombotic graft microangiopathy, isolation of the viral genome in renal specimens, seroconversion, and endothelial tropism of the virus suggests that B19 could be the etiologic agent of thrombotic microangiopathy in these cases. The development of the disease after infection could depend on other detrimental cofactors, which make the patient more susceptible to microthrombi formation in the renal microvasculature. The renal graft could represent the route of B19 transmission.

Objective: To analyze the data from 347 peritoneal catheters implanted in 249 pediatric patients aged < or = 15 years at start of chronic peritoneal dialysis (CPD)., Design: Restrospective study of the data collected between 1986 and 1995, in 20 dialysis centers, from the Italian Registry of Pediatric Chronic Peritoneal Dialysis. Data collection for each pediatric catheter included: catheter type, site and technique of insertion, complications, duration, and reason for removal or replacement., Results: Fifty catheters were inserted in patients under 2 years of age, 50 in patients aged 2 - 5 years and 247 in patients over 5 years of age. Catheter types included 307 (88.5%) Tenckhoff (286 double cuff, 21 single cuff) and 40 (11.5%), double-cuff, Valli-type catheters. All catheters were surgically implanted and omentectomy was performed in 83.5% of cases; the entry-site was in the midline in 136 cases (39.2%) and paramedian in 211 (60.8%). During 6076 CPD months we observed 274 catheter-related complications: 182 catheter infections (exit-site and/or tunnel infection), 23 leakages, 19 obstructions, 19 cuff-extrusions, 14 dislocations, 6 hemoperitoneum, 10 other (incidence of one complication every 21.8 dialysis-months). A significant reduction of catheter-related complications occurred in the last five years, compared with the first 5 years. One hundred and six catheters were removed due to catheter-related causes: infection (83 cases), obstruction (11), dislocation (4), outer-cuff extrusion (3), leakage (2), bowel incarceration (2), and bowel infarction (1). Catheter survival was 72.2% at 12 months, 52.3% at 24 months, 32.8% at 36 months, and 25.7% at 48 months. Significantly lower catheter survival was found in younger children (0 - 2 years) compared with two other age groups (2 - 5 years, and > 5 years). No significant correlation was found between catheter survival and catheter entry-site (midline vs paramedian)., Conclusions: Catheter-related infections were confirmed to be the most common complication and most frequent cause of peritoneal catheter removal. In addition, catheter survival rate was worse in younger children, indicating that more effort should be made to improve peritoneal catheter survival particularly in this age group.

Vesicoureteral reflux (VUR) is the commonest cause of end-stage renal failure reported by the ITALKID (Italian Register of Chronic Renal Failure) (CRF) in children (0-15 years). Herein we report 131 children (53 M and 78 F) with a diagnosis of VUR made in our Centre between 1987 and 1994. Diagnosis was made in 69% of cases (85/131) during the 1st year of life and in 31% of cases (47/131) afterwards. VUR was demonstrated following a prenatal ultrasonography suspicion in 24/131 and after symptoms such as urinary tract infection (94/131) and poor growth (6/131). 75/131 of patients were treated conservatively (continuous chemoprophylaxis) while 56/131 underwent an antireflux surgery. CRF was found in 14% (19/131) of cases even though the diagnosis of VUR was performed in most of the cases (79%) during the 1st year of life. In conclusion prenatal ultrasonography has a low sensibility in the diagnosis of VUR. In addition, an early diagnosis made during the 1st year of life do not prevent CRF. The study was carried out in a Centre where patients with urological problems are referred from a large part of Northern East of Italy and this may explain the high prevalence of CRF and of children receiving a surgical treatment.

The increased frequency of prenatal detection of urinary tract abnormalities has brought to the attention of the pediatrician a high number of neonates and infants with asymptomatic hydronephrosis, which represents the most common form of uropathy. As it becomes clear that hydronephrosis does not necessarily mean obstruction, the purpose of the evaluation is to identify those infants in whom hydronephrosis will cause progressive renal deterioration, because of an ongoing restriction to urinary outflow. In the present paper, the discussion of the post-natal diagnosis and management focuses on the embryologic background, the pathophysiology of the obstruction and the rationale to utilise the different diagnostic techniques (ultrasonography, associated with the use of the Doppler; intravenous urography; avoiding cystourethrography; renal scintigraphy).

Patient hospitalization was compared in 207 pediatric patients (age < or = 15 years at the start of dialysis) on chronic peritoneal dialysis (CPD) (127 patients) or center hemodialysis (HD) (80 patients), treated in 17 dialysis centers during the period 1989 to 1994, and followed up for at least three months. The hospitalization rate was expressed as hospital days per patient-month, and was calculated on the overall period of treatment and separately for the first and second year. Since the age at start of dialysis markedly differed between CPD (8.2 +/- 4.7 years) and HD (11.2 +/- 2.9 years) patients (with no HD patient younger than five years), results are separately presented in three patient groups: CPD patients aged < 5 years (A); CPD patients aged five to 15 years (B); HD patients (C). The duration of hospitalization was subdivided according to the following different causes: routine (monitoring of dialysis adequacy), complications of the modality, patient primary renal disease, and other causes. The results are presented in Table 1. A statistically significant difference in total days hospitalized was found between each of the two groups of CPD patients and the HD patients; the results for hospitalization for dialysis-related complications were higher in the group of younger children on CPD, while the difference between the two age-matched groups of patients on CPD and HD was not significant.

Recurrent rejection is an uncommon, severe complication after heart transplantation that is associated with a poor long-term prognosis. Photopheresis (ECP), a new form of extracorporeal photo-chemotherapy used for the treatment of cutaneous T cell lymphoma and several autoimmune diseases, has also been used for prevention and treatment of acute rejection in heart transplant recipients. It seems to induce specific suppression of both cellular and humoral rejection. In this study, we evaluated whether ECP added to standard therapies allowed better control of rejection and reduction of conventional immunosuppressive drugs in patients with repeated rejection episodes. Eight heart transplant recipients (6 men and 2 women, mean age 48 yr), with recurrent rejection were treated with ECP for 6 months. Endomyocardial biopsies (EMB) were performed monthly. As a result of treatment, 7 patients on ECP experienced a reduction of the number and severity of rejection episodes. The fraction of EMB negative for rejection increased from 13 to 41%, whereas the fraction of specimens with multifocal and/or diffuse moderate lymphocytes infiltration (grades 3A and 3B) decreased from 41 to 21%. ECP allowed reductions of daily immunosuppressive therapy: prednisone by 44% (16.9 vs. 9.4 mg), cyclosporine by 21% (366 vs. 291 mg), and azathioprine by 29% (137 vs. 97 mg). No major side effects were observed. We conclude that, although the number of patients is small, the use of ECP was safe and associated with improved control of recurrent rejection. This allowed tapering of immunosuppressive drugs, which was particularly useful in two patients with insulin-dependent diabetes and one with sternal wound osteomyelitis.

Chronic peritoneal dialysis (CPD) is the first treatment modality for most infants with end-stage renal failure; this group of patients shows peculiar clinical and technical problems. We present the data from a National Registry on 22 children starting CPD under one year of age, representing 11.6% of the total population of the Registry (189 patients). Mean weight at start of CPD was 6.1 +/- 1.8 kg and duration of dialysis was 22.1 +/- 15.5 months. During the follow-up period, 9 patients were transplanted, 1 was shifted to hemodialysis, and 4 died. Patient survival was 89.1% and 82.2% at 1 and 2 years (97.9% and 96.5% in the group of 167 older children); technique survival results were 89.1% at 1 year and 77.1% at 2 years (vs 92.5% and 85.7%, respectively). The incidence of peritonitis was 1 episode every 15.6 CPD-months (1:16.1 in the older children). Catheter-related complications occurred more frequently in infants (1:11.8 vs 1:17 episode:CPD-months), even if this difference was not statistically significant. Statural growth was on average -0.29 +/- 0.66 SD/year with a significant improvement between the first (-0.50 +/- 0.79) and the second (+0.23 +/- 0.77) year of CPD. Our data confirm that infants represent a higher risk group and that they can be treated satisfactorily with CPD while awaiting renal transplantation.

Objectives: To report the complications and outcome of 10 newborns affected by acute renal failure (ARF), treated by continuous peritoneal dialysis (CPD)., Design: All newborns admitted for tertiary treatment to the Neonatal Intensive Care Unit of the University of Padova, who underwent CPD between February 1986 and December 1990, were analyzed retrospectively., Patients: Ten newborns (mean weight 2077 g, range 540-4930 g) received CPD, 6 of whom were preterm. All the survivors completed the study., Interventions: A number 9, 5 French Tenckhoff catheter was used, and a closed circuit was created by means of a modified continuous ambulatory peritoneal dialysis (CAPD) technique. The mean duration of dialytic therapy was 7 days., Results: At the end of the dialytic period, 7 of the 10 patients had normal serum potassium and sodium values. CPD produced two different types of complications: leakage of the dialytic fluid in very low weight newborns and one episode of peritonitis during a chronic dialysis treatment. Six died of severe respiratory failure (in no case, however, was this attributable to ARF or CPD procedure). All but one of the survivors regained normal renal function. The only exception necessitated a kidney transplant., Conclusion: We believe that this technique, although invasive, improves the outcome of both preterm and low birth weight newborns affected by ARF.

During the period 1986-1991, the Italian Registry of Pediatric Chronic Peritoneal Dialysis collected data from 140 patients younger than 15 years at the start of chronic peritoneal dialysis (CPD). In this study we review the Registry's complications and patient hospitalization data. A total of 395 complications directly related to CPD were registered during 2722 dialysis-months. There were 176 episodes of peritonitis (44.5%), 161 catheter-related complications (40.7%) (103 exit-site infections, 17 leakages, 15 obstructions, 15 cuff extrusions, 5 hemoperitoneum, and 6 other complications), and 58 technique-related complications (14.8%) (39 abdominal hernias, 10 hydroceles, 5 with abdominal pain, 4 hydrothorax complications). The patient hospitalization rate during the period 1989-1991 was evaluated; the analysis referred to 106 patients who underwent treatment for a total of 1520.5 dialysis-months. Patients starting CPD in the year and patients already on CAPD spent 5.8 and 2.1 days per patient-month in the hospital, respectively; the difference was not statistically significant. The evaluation of complications (both technical and systemic) causing patient hospitalization showed that peritonitis was responsible for 43.2% of patient admissions and 36.3% of days hospitalized, catheter-related complications for 22% and 19.8%, technique-related complications for 8.3% and 5.1%, and other clinical complications for 26.5% and 38.8%, respectively.

The results of the first 3 years' collaboration of the Italian Registry of Paediatric Chronic Peritoneal Dialysis (CPD) (1986-1988) are presented. This Registry acquired data on the majority of the paediatric patients treated with CPD in Italy, thus providing a national picture in a field where few nationwide surveys are available. Patients of less than 15 years of age at the start of dialysis were enrolled and clinical data collected until the age of 19 years. The number of nephrological paediatric centres participating in the Registry increased from 7 in 1986 to 11 in 1988. The total number of patients on CPD was 70 and the percentage of dialysed children treated with CPD ranged from 40.2% to 43.6%. Data on 89 peritoneal catheters were collected: during 1417 dialysis-months 70 catheter-related complications were observed (1:20.8 dialysis-months); actuarial catheter survival was 92.7% at 6 months, 84.8% at 1 year and 68.8% at 2 years. the incidence of peritonitis changed from 1 episode every 10.9 patient-months in 1986 to 1 every 19.8 in 1988. Abdominal hernias were the other main clinical complication observed. The survival of patients was 92.5% at 3 years, while the technique survival at the same time was 84%.

During the period 1986-1990, 119 patients were enrolled in the Italian Registry of Pediatric CPD. CAPD was largely predominant in the first 3 years, while CCPD accounted for 48% of dialysis months in the period 1989-1990. The connect-disconnect system was a Y set for all patients during the whole observation period. The incidence of peritonitis decreased from 1 episode: 10.9 patient-months in 1986 to 1:19.8 in 1988, and then passed to 1:16.2 in 1990. A comparison of the incidence of peritonitis between CAPD and CCPD, referring to the 1989-1990 period, showed no significant difference. The percentage of positive peritoneal fluid cultures changed from 48% in 1986 to 73% in 1990. Gram-positive bacteria, primarily Staphylococcus aureus and Staphylococcus epidermidis, accounted for most of the isolated organisms. Candida albicans was cultured in 3 cases both in 1986 and 1987. Exit site infection was the predominant (82%) complication, followed by leakage and catheter cuff extrusion. The hospitalization rate for peritonitis resulted persistently high (61% of episodes) and the mean duration was 12.7 days. Of the 8 patients who were switched to hemodialysis, 4 had recurrent peritonitis and 1 Candida albicans peritonitis.

Ten children with renal failure (age range 2 years 6 months to 18 years 9 months; median 11 years 10 months), maintained by long-term hemodialysis, had successful correction of their anemia after intravenous administration of recombinant human erythropoietin in a dosage escalating every 2 weeks (75 to 150 to 300 to 450 IU/kg/wk). Mean hemoglobin concentration increased from 6.4 +/- 0.9 to 11.5 +/- 1.0 gm/dl. Blood cell counts used to evaluate the correction of anemia were done after dialysis; this was especially important for children less compliant with water restriction. The higher hemoglobin concentration resulted in improvement of the quality of life, a greater tolerance for physical effort (exercise tolerance doubled and the ventilatory anaerobic threshold increased significantly), correction of some subclinical central nervous system abnormalities detected by evoked potentials testing, and reduction of bleeding time. Few side effects were noted; severe hypertension developed in one patient when postdialysis hematocrit was only 28%, and there were two episodes of hypertransaminasemia with no other evidence of liver dysfunction. We conclude that in children with renal failure the use of recombinant human erythropoietin to correct anemia is safe and strongly advisable, because of the resolution of many of the symptoms correlated with anemia.