West Nile virus: the Italian national transplant network reaction to an alert in the north-eastern region, Italy 2011.

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.

Extracorporeal photochemotherapy after cardiac transplantation: a new therapeutic approach to allograft rejection.

UNLABELLED: Photopheresis (ECP) is a new immunomodulatory therapy in which recipient lymphocytes are treated extracorporeally with 8-methoxypsoralen and ultraviolet light. The treatment seems to induce an inhibition of both humoral and cellular rejection after transplantation. OBJECTIVE: Since recurrent rejection (RR) continues to be a severe complication after heart transplantation (HTx) and the immunosuppressive regimes used for the treatment are often associated with increased morbidity and mortality, we investigated whether ECP could have a beneficial effect on the number and severity of rejection episodes. METHODS: Eleven HTX recipients (5 M and 6 F, mean age 48.5 yrs) with RR were enrolled in the study. ECP was performed at weekly intervals during the 1st month, at 2 week intervals during the 2nd and 3rd month, and then monthly for another 3 months. RESULTS: The fraction of biopsies (EMB) with a grade 0/1A rejection increased during ECP from 46% to 72% while the EMB showing a 3A/3B rejection decreased from 42% to 18%. It is also noteworthy that out of the 78 EMB performed during ECP only one showed a 3B rejection in comparison with 13 out of 110 EMB in the pre-ECP period. Six rejection relapses were observed in a total follow-up of 60 months, two of them occurring during the tapering of oral steroid. Four relapses were reversed by ECP, one by i.v. steroids and the last by methotrexate after the failure of both i.v. steroids and ECP. The mean doses of immunosuppressive drugs resulted lower after 6 months of ECP: steroids were reduced from 13 to 8.25 mg/day, cyclosporine from 375 to 285 mg/day, azathioprine from 55 to 35 mg/day. CONCLUSIONS: ECP is a well tolerated treatment. Its administration allows better RR control and significant reduction in immunosuppressive therapy.

The Italian Registry of Pediatric Chronic Peritoneal Dialysis: a ten-year experience with chronic peritoneal dialysis catheters.

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.

Successful treatment of recurrent rejection in renal transplant patients with photopheresis.

Photopheresis (ECP) is a new form of photochemotherapy that induces a selective inhibition of the host response to foreign histocompatibility antigens and reverses allograft rejection after organ transplantation. This report describes four adolescent patients with recurrent rejection episodes after renal transplantation, all uncontrolled using standard protocols of immunosuppression (intravenous steroids and OKT3), yet successfully treated with a 6-mo course of ECP. The ECP treatment was performed at weekly intervals during the first month, at 2-wk intervals during the second and third months, and then monthly for another 3 mo. Creatinine clearance improved throughout the treatment in three patients and remained unchanged in one. All patients had a pre-ECP biopsy with a grade 2 or 3 rejection (Banff) with a diffuse infiltrate CD8, CD14, LFA-1 (166 cells positive/0.048 mm2), and VLA-4 (51 cells positive/0.048 mm2) positive, as well as a tubular expression of HLA-DR (6.2 sections of tubule positive/0.048 mm2), ICAM-1, and VCAM-1 (3.1 and 2.9 sections of tubule positive/0.048 mm2). A strong reduction of cell infiltrate and expression of LFA-1 (6.6 cells positive/0.048 mm2). VLA-4 (0.7 cells positive/0.048 mm2), HLA-DR (0.2 section of tubules positive/0.048 mm2), ICAM-1 (0.3 section of tubules positive/0.048 mm2), and a disappearance of VCAM-1 staining were observed in the biopsies performed after 3 mo of ECP. All patients remained rejection-free during ECP, without infections or other complications commonly observed with increasing doses of standard immunosuppression. The clinical improvement allowed a progressive reduction of oral steroids in three of the four patients treated.

Photopheresis in paediatric patients with drug-resistant chronic graft-versus-host disease.

Photopheresis (ECP) is a new type of photochemotherapy, used for the treatment of oncological and autoimmune diseases. Lymphocytes are drawn from the patients by leukapheresis, treated with 8-methoxypsoralen (8-MOP) and ultraviolet light A (UVA) in an extracorporeal system and then reinfused. Skin exposure to 8-MOP and UVA (PUVA) has been shown to relieve cutaneous symptoms of graft-versus-host disease (GVHD) in bone marrow transplant (BMT) recipients. ECP, which is similar in some ways to PUVA, has been used in this study to treat four paediatric patients who developed chronic GVHD following BMT and in whom GVHD had failed to respond to conventional immunosuppressive therapy. Following ECP, skin lesions cleared almost completely and pulmonary function tests improved in two of three patients with cutaneous and lung involvement. Serum bilirubin and transaminases gradually normalized, and gammaGT decreased considerably in the remaining patient who had a severe cholestatic hepatopathy. The Karnofsky performance score increased to 90% in the three patients with positive responses to ECP and remained unchanged (40%) in the patient who did not respond. Immunosuppressive therapy was reduced in three patients and eventually discontinued in two. No significant side-effects were observed during the treatment. Our results suggest that ECP is a non-aggressive treatment that may benefit patients with chronic GVHD who do not respond to standard immunosuppressive therapy.

[Chronic renal failure secondary to vesicoureteral reflux: is the prevention possible?]. / Insufficienza renale cronica secondaria a reflusso vescico-ureterale: è possibile la prevenzione?

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.

[Diagnostic and therapeutic protocol in malformative uropathies prenatally diagnosed]. / Protocollo diagnostico e terapeutico delle uropatie malformative con diagnosi prenatale.

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).

Complications linked to chronic peritoneal dialysis in children after kidney transplantation: experience of the Italian Registry of Pediatric Chronic Peritoneal Dialysis.

Our objective was to evaluate the infectious complications of the post-transplant period attributable to the persistence of catheter and other complications when chronic peritoneal dialysis (CPD) was performed post-transplantation. The design was a retrospective study, and the setting was an Italian registry of pediatric chronic peritoneal dialysis. There were 86 pediatric renal transplants (9/86 from living related donors, 2/86 simultaneous liver and kidney transplantation for oxalosis). Six of 86 transplants were lost at follow-up. Mean age of the children (n = 80) at transplantation was 9.3 years (range: 1.7-21 years). They had been on CPD for a mean period of 1.7 years (range: 0.2-4.6 years). During CPD, 67 peritonitis episodes (80% related to exit-site and/or tunnel infections) were observed, with an incidence of peritonitis of one episode per 16 months CPD. The mean safe interval of peritonitis and/or exit-site or tunnel infection was 208 days (range: 36-1897 days). The mean time of catheter removal was 80.3 days (range: 0-216 days) post-transplantation. During the first month post-transplantation, one episode of peritonitis secondary to a sepsis occurred in one child. No other episodes of peritonitis or exit-site and/or tunnel infections were observed. Two of 80 children returned to CPD (at four and at 12 months, respectively) because of persistent allograft failure. Furthermore, 12 patients were on CPD because of temporary graft failure. In all these patients the pretransplant peritoneal dialysis (PD) catheter was utilized, with no complications. These data show that the persistence of the PD catheter after kidney transplantation has produced no infections or other complications. What is more, the catheter was safely utilized during acute rejection or primary allograft nonfunction.

Comparison of patient hospitalization in chronic peritoneal dialysis and hemodialysis: a pediatric multicenter study.

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.

Extracorporeal photochemotherapy as adjuvant treatment of heart transplant recipients with recurrent rejection.

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.

Clinical experience in the treatment of infants with chronic peritoneal dialysis.

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.

Continuous peritoneal dialysis in newborns.

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.

Analysis of complications in a chronic peritoneal dialysis pediatric patient population. The Italian Registry of Pediatric Chronic Peritoneal Dialysis.

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.

Chronic peritoneal dialysis in paediatrics: experience of a national registry.

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%.

Peritonitis in children undergoing chronic peritoneal dialysis (CPD): data from the Italian Registry of Pediatric CPD.

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.

Benefits and risks of anemia correction with recombinant human erythropoietin in children maintained by hemodialysis.

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.

Familial occurrence of blind-ending bifid and duplicated ureters.

Blind-ending bifid ureter in a girl with urinary tract infection, and a blind-ending duplicated ureter in her younger sister are described. The embryology of this rare anomaly is briefly reviewed.