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Biomedical subjects

E Verrina

Publications and source records attributed to E Verrina.

At least 19 recordsLinked to original sources
PubMed2006

Nephronophthisis type 1 deletion syndrome with neurological symptoms: prevalence and significance of the association.

Type 1 nephronophthisis (NPHP) with homozygous deletions of nephrocystin [NPHP1, DEL] has been considered a pure renal disorder, but co-occurrence of extrarenal symptoms, mainly retinitis pigmentosa, is observed in a subset of patients. Recently, [NPHP1, DEL] has been detected in three patients with Joubert syndrome-related disorders (JSRDs), who associated neurological signs with a peculiar neuroradiological malformation known as the 'molar tooth sign' (MTS). To define the frequency of JSRD spectrum in NPHP1 patients, we re-examined 56 cases with [NPHP1, DEL] and found an overall incidence of 8.9% (five out 56 patients). All had small hyperechoic kidneys and had developed advanced renal failure within 15 years. Two patients presented the complete features of JSRD with cerebello-renal-retinal association and MTS. Two others showed, instead, severe intentional tremor and thick superior cerebellar peduncles on brain magnetic resonance imaging (MRI), and one of them had associated retinopathy. The fifth patient presented with hypotonia, developmental delay, central deafness, and ataxia associated with Leber congenital amaurosis and liver fibrosis but with normal brain MRI. Marked intrafamilial variability of associated extrarenal symptoms was observed in familial cases. Deletion extension did not differ in patients with isolated renal phenotype and in those with associated neurological symptoms. In conclusion, neurological defects varying from subtle involvement of cerebellum with thickened peduncle to both JSRD and diffuse central hypotonia are frequent in [NPHP1, DEL] patients. Prevalence of such association may justify systematic neurological and neuroradiological evaluation.

Adolescent↗
PubMed2006

Successful in vitro priming of EBV-specific CD8+ T cells endowed with strong cytotoxic function from T cells of EBV-seronegative children.

Epstein-Barr virus (EBV)-seronegative transplant recipients are at high risk of developing EBV-associated post-transplant lymphoproliferative disorder (PTLD), and would maximally benefit from an EBV-directed T-cell therapy for prevention or treatment of PTLD. So far, efforts to activate CD8+ EBV-specific cytotoxic T lymphocytes (CTL) endowed with high specific cytotoxicity from EBV-seronegative children have failed. We compared the CD8+ CTL priming efficiency of three different modified activation protocols, based on lymphoblastoid cell lines (LCL) stimulation potentially enhanced by either LCL presentation through dendritic cells, or selection of IFN-gamma+ cultured cells, or culture in the presence of rhIL-12 and rhIL-7, according to the standard protocol for reactivation of EBV-specific CTL. We found that only specific LCL stimulation in the presence of rhIL-12 and rhIL-7 was able to reproducibly expand EBV-specific CD8+ CTL endowed with strong cytotoxic activity from truly EBV-seronegative children. The lines thus activated, which included specificities toward EBV latent and lytic proteins, showed high percentage CD8+ T cells, with <10% naïve CD8+/CCR7+/CD45RA+ cells. Overall, the total number of CD8+ central memory cells, and of CCR7 T-cell effectors was comparable to that observed in healthy EBV-seropositive controls. In conclusion, it is feasible to activate EBV-specific CD8+ CTL with suitable characteristics for in vivo employment from EBV-seronegative children.

Adult↗
PubMed2001

Acute effects of simultaneous intraperitoneal infusion of glucose and amino acids.

BACKGROUND: The feasibility of simultaneously infusing glucose and amino acid (AA)-based peritoneal dialysis solutions was tested to determine whether peritoneal dialysis patients could achieve an adequate nonprotein calorie/nitrogen ratio while preventing a marked increase in blood urea nitrogen (BUN), which is usually seen if the AAs are administered without glucose. METHODS: An automatic peritoneal dialysis cycler was used to infuse glucose and AA solutions (3:1) simultaneously during the night. Eight infusions of 1000 mL m2 of body surface area (BSA), with a 60 minute dwell time, were performed in 10 children on peritoneal dialysis. The dialytic effluent was analyzed at every exchange and totaled at eight hours to evaluate volume, glucose, and AA concentration. Blood samples for plasma, glucose, insulin, and free AA determination were drawn at the beginning of automated peritoneal dialysis (APD) session and at each instillation of peritoneal dialysate. RESULTS: The mean glucose absorption was 33.7 +/- 10.0% and the AA absorption was 55.2 +/- 13.2% of the infused amount, and the ratio of nonprotein calorie (derived from glucose) to nitrogen (derived from AA) was 115.4:1. The insulin levels returned to normal only three hours after the beginning of APD. The free AA plasma levels were already increased two hours after dinner and remained high for the entire APD treatment because of the continuous absorption of AA from the peritoneum. The BUN levels did not increase despite the supply of AA. CONCLUSIONS: This APD procedure may improve utilization of AA for protein synthesis, as suggested by the lack of increase of the BUN levels with this regimen.

Adolescent↗
PubMed2001

A multicenter survey on automated peritoneal dialysis prescription in children.

Automated peritoneal dialysis (APD) is considered the first-choice chronic peritoneal dialysis modality for pediatric patients. Nighttime APD courses reduce the impact of PD treatment on a patient's and family's way of life, and the wide range of prescription options permit the dialysis schedule to be tailored to the needs of children of varying age and body size. We registered data concerning the dialytic regimens adopted in 12 pediatric dialysis centers for the treatment of 110 children on APD. Of the 110 children, 64 (aged 7.6 +/- 5.1 years) were on nightly intermittent peritoneal dialysis (NIPD); 29 (aged 9.2 +/- 4.3 years) were on tidal peritoneal dialysis (TPD); and 17 (aged 8.2 +/- 4.9 years) were on continuous cycling peritoneal dialysis (CCPD). The main prescription parameters for the various regimens (mean +/- standard deviation) were these: NIPD--exchanges: 13.0 +/- 5.8; duration: 10.0 +/- 1.1 hours; dwell volume: 36.5 +/- 6.2 mL/kg body weight (BW); glucose concentration: 1.69% +/- 0.41%. TPD--exchanges: 23.3 +/- 8.1; duration: 10.0 +/- 1.0 hours; dwell volume: 36.1 +/- 5.9 mL/kg BW; glucose concentration: 1.63% +/- 0.37%. CCPD--exchanges: 13.0 +/- 4.7; duration: 10.1 +/- 1.3 hours; dwell volume: 37.7 +/- 5.2 mL/kg BW; glucose concentration: 1.65% +/- 0.28%. Tidal volume was 52.2% +/- 9.0% of initial fill volume. Daytime dwell volume was 54.8% +/- 17.3% of night volume in CCPD patients, and 56.6% +/- 13.3% in 9 patients on continuous TPD. Because the patient population in this report varied in age, body size, and metabolic needs, the resulting range in prescription parameters was quite wide. Nevertheless, the duration of nightly PD sessions averaged 10 hours, fill volume averaged 36 mL per kilogram body weight, and daytime volume averaged 50% of nighttime fill volume.

Child↗
PubMed1998

Chronic renal replacement therapy in children: which index is best for adequacy?

BACKGROUND: The dialysis dose, Kt/V, and Solute Removal Index (SRI) have been proposed as tools to measure and compare adequacy of different renal replacement therapies in adults. The aim of our study was to elucidate whether the Kt/V and SRI could be appropriate parameters to compare different treatments and define adequacy targets in children. METHODS: Twenty-two pediatric chronic dialysis patients (2 to 17 years) were prospectively studied. Six patients were on continuous ambulatory peritoneal dialysis (CAPD), 7 patients were on automatic nightly peritoneal dialysis (ANPD), and 9 were on hemodialysis (HD). Patients had no peritonitis and were not hospitalized during the previous two months and, as proved by growth and subjective well being, were in steady state condition at the initiation of the protocol. As a consequence, the treatment delivered was assumed to be adequate and the prospective analysis was carried out within one month. Urea levels in dialysate, plasma and urine were measured to determine urea kinetics and measure adequacy parameters. RESULTS: Instantaneous urea clearance was much higher when hemodialysis was used (124.67 +/- 32.04 ml/min) compared to CAPD (2.79 +/- 0.29 ml/min) and ANPD (6.60 +/- 1.42 ml/min), as expected. The Urea dialytic clearance per week was greater in HD (67320 +/- 17299 ml) than in CAPD(28144 +/- 2895 ml) and ANPD (29910 +/- 4234 ml). Residual renal function contributed to the overall weekly clearance by 47% in CAPD, while it was only by 19% in HD and 26% in ANPD. The overall weekly clearance was therefore 79,842 ml/week in HD, 53,340 ml/week in CAPD and 41,012 ml/week in ANPD. Weekly dialytic Kt/V results were much higher in HD (3.75) than in CAPD (1.78) and ANPD (2.37). To these values, the renal Kt/V was added, reaching the values of overall (dialytic + renal) weekly Kt/V of 4.53 in HD, 3.41 in CAPD and 3.41 in ANPD. Although higher Kt/V values were observed in HD, when the SRI % was considered, HD appeared to be less efficient compared with the other two techniques. Since postdialytic rebound in HD patients averaged 22.5%, we may speculate that hemodialysis in children is less efficient than continuous or daily peritoneal dialysis because of a remarkable cardipulmonary recirculation and solute sequestration. CONCLUSION: In the global evaluation, dialysis SRI% appears to be more reliable as an index of adequacy compared to Kt/V in children. At least an integration between the two indices is strongly recommended.

Adolescent↗
PubMed1996

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.

Adolescent↗
PubMed1996

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.

Adolescent↗
PubMed1996

Protein and calorie intake, nitrogen losses, and nitrogen balance in children undergoing chronic peritoneal dialysis.

We performed 22 nitrogen balance (NB) studies of three days' duration in 19 children (8.7 +/- 3.8 years) on chronic peritoneal dialysis (CPD) for 19.4 +/- 16.4 months. The dietary intakes were assessed by the double weighing method. Total nitrogen, protein, urea, and creatinine were analyzed in the dialysate and urine collected daily. Total nitrogen was also determined in the feces collected over the whole NB study period, using vegetable charcoal as a marker. The protein intake was 1.64 +/- 0.50 g/kg/day, corresponding to 126 +/- 40% of the recommended daily allowance (RDA) for normal children of the same age, and the calorie intake (diet+glucose from dialysate) reached 75 +/- 26% of RDA. Nitrogen losses were: 0.177 +/- 0.052 g/kg/day with peritoneal fluid and urine, and 0.028 +/- 0.018 g/kg/day with feces. The NB, positive in 17 out of 22 studies, ranged from -116 to +167 mg/kg/day (mean 44.0 +/- 66.2 mg/kg/day). A direct and significant correlation between NB and nitrogen intake (g/kg/day) (r = 0.562, p < 0.05) and total calorie intake (cal/kg/day) (r = 0.483, p < 0.05) has been observed. These data confirm the need to ensure in children on CPD an adequate nutritional intake, and further support the efforts to improve calorie intake.

Blood Proteins↗
PubMed1996

Children on continuous ambulatorial peritoneal dialysis: muscle and plasma proteins, amino acids and nutritional status.

The aim of the study was to investigate plasma and muscle amino acid (AA) levels in children on continuous ambulatory peritoneal dialysis (CAPD) and their relationship to various indices of nutritional status. Ten children with a mean age of 6.4 +/- 5.6 yrs were evaluated. Muscle biopsies and venous blood samples were taken after an overnight fast. Muscle samples were obtained from rectus abdominis. Data were compared with those of a control group of 22 children who were undergoing elective surgery. Informed consent was obtained from the parents. The plasma concentration of most of the essential AA (valine, leucine, isoleucine, lysine, methionine and tyrosine) were significantly reduced and the levels of some non essential AA (aspartic acid, glycine, citrulline, 1-3 methihystidine, taurine + alanine) were significantly higher than in the controls. Muscle intracellular free essential AA concentrations, except the low levels of valine and leucine did not differ significantly from values in the controls. Among non essential AA, aspartic acid, glutamic acid and ornitine showed significantly increased intracellular concentrations. No significant correlations were found between plasma and muscle AA concentration and ASP (alkali-soluble protein)/DNA ratio, serum albumin, transferrin, bicarbonate levels and duration of CAPD. Instead, a significant correlation was noted between the muscle ASP/DNA ratio, an indicator of the amount of cell proteins per cell unit, and age (r = 0.714, p < 0.05). Muscle Branched chain AA levels were significantly correlated to body mass index (BMI) (r = 0.648, p < 0.05).

Adolescent↗
PubMed1996

Is the response to rhGH in peritoneal dialysis patients less effective than in patients with chronic renal insufficiency.

Data on the effects of rhGH treatment in children on peritoneal dialysis are limited. In general rhGH therapy seems to be less effective compared with results on patients with chronic renal failure on conservative treatment. Our experience suggests that rhGH can stimulate growth in children on CPD, and that the efficacy of such therapy is reduced after the first year of treatment, although the rhGH is still active.

Child↗
PubMed1995

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.

Catheters, Indwelling↗
PubMed1994

Muscle and plasma amino acids and nutritional status in kidney-transplanted children.

Nutritional status, assessed by anthropometric and biochemical methods, protein and amino-acid (AA) composition and muscle water were evaluated in 11 kidney transplanted children and in a control group of 10 children with normal renal function who were undergoing elective surgery. Samples of the rectus abdominis muscle were taken when surgery was performed in the control children and when the peritoneal catheter was removed in the transplanted children. The mean time from the transplantation to the study time was 97 +/- 14 days (range 72-114 days). Height was reduced in the transplanted children compared to the controls but skinfold thickness, arm muscle circumference and serum proteins (total protein, albumin, transferrin, pseudocholinesterase) were normal. The body mass index was over the 50 degree percentile in nine of the eleven children. The muscle contents of total, extracellular, and intracellular water, alkali-soluble protein (ASP), DNA and the ASP/DNA ratio were not significantly different in transplanted children from those in the controls. Plasma leucine and taurine levels were significantly decreased, whereas plasma citrulline and alanine levels and the glycine/serine ratio were increased. Muscle threonine, alanine+taurine, glycine and aspartic acid levels as well as the glycine/serine ratio were increased in the transplanted children. Transplanted children show an almost normal muscle AA profile and a plasma AA pattern that seems to be related to the prednisone therapy.

Adolescent↗