Kidney Intragraft Homing of De Novo Donor-Specific HLA Antibodies Is an Essential Step of Antibody-Mediated Damage but Not Per Se Predictive of Graft Loss.

Donor-specific HLA antibody (DSA)-mediated graft injury is the major cause of kidney loss. Among DSA characteristics, graft homing has been suggested as an indicator of severe tissue damage. We analyzed the role of de novo DSA (dnDSA) graft homing on kidney transplantation outcome. Graft biopsy specimens and parallel sera from 48 nonsensitized pediatric kidney recipients were analyzed. Serum samples and eluates from graft biopsy specimens were tested for the presence of dnDSAs with flow bead technology. Intragraft dnDSAs (gDSAs) were never detected in the absence of serum dnDSAs (sDSAs), whereas in the presence of sDSAs, gDSAs were demonstrated in 72% of biopsy specimens. A significantly higher homing capability was expressed by class II sDSAs endowed with high mean fluorescence intensity and C3d- and/or C1q-fixing properties. In patients with available sequential biopsy specimens, we detected gDSAs before the appearance of antibody-mediated rejection. In sDSA-positive patients, gDSA positivity did not allow stratification for antibody-mediated graft lesions and graft loss. However, a consistent detection of skewed unique DSA specificities was observed over time within the graft, likely responsible for the damage. Our results indicate that gDSAs could represent an instrumental tool to identify, among sDSAs, clinically relevant antibody specificities requiring monitoring and possibly guiding patient management.

Disparities in policies, practices and rates of pediatric kidney transplantation in Europe.

We aimed to provide an overview of kidney allocation policies related to children and pediatric kidney transplantation (KTx) practices and rates in Europe, and to study factors associated with KTx rates. A survey was distributed among renal registry representatives in 38 European countries. Additional data were obtained from the ESPN/ERA-EDTA and ERA-EDTA registries. Thirty-two countries (84%) responded. The median incidence rate of pediatric KTx was 5.7 (range 0-13.5) per million children (pmc). A median proportion of 17% (interquartile range 2-29) of KTx was performed preemptively, while the median proportion of living donor KTx was 43% (interquartile range 10-52). The median percentage of children on renal replacement therapy (RRT) with a functioning graft was 62%. The level of pediatric prioritization was associated with a decreased waiting time for deceased donor KTx, an increased pediatric KTx rate, and a lower proportion of living donor KTx. The rates of pediatric KTx, distribution of donor source and time on waiting list vary considerably between European countries. The lack of harmonization in kidney allocation to children raises medical and ethical issues. Harmonization of pediatric allocation policies should be prioritized.

Use of new peritoneal dialysis solutions in children.

Standard peritoneal dialysis (PD) solutions with low pH and containing high concentrations of lactate and glucose have been demonstrated to negatively affect the peritoneal membrane, mesothelial cell viability, residential peritoneal cells, and also to inhibit phagocytic functions. An increasing body of experimental evidence supports the idea that the peritoneal hypervascularization and fibrosis observed in long-term PD are causally related to the acute and chronic toxicity of conventional PD solutions. A Physioneal (lactate/bicarbonate mixed buffer pH 7-7.4), Physioneal, Extraneal (7.5% icodextrin), Nutrineal (1.1% amino-acid-containing solution) regimen, for example, offers a significant reduction in carbohydrate load (approximately 40-50%), lower exposure to and absorption of glucose degradation products, reduced oxidative stress, and improved volume control when compared with a first-generation DDDD (4 x Dianeal) regimen. The positive aspects of each solution that we have observed in our patients allow a recommendation on the potential benefit of using these solutions in children treated with PD. In fact, data from the literature as well as the results of the studies reported in this paper show that in children the application of neutral pH bicarbonate/lactate-buffered solution for the standard nighttime APD prescription, icodextrin solution for a long daytime dwell, and AA-based solution in malnourished patients is safe and effective. Extended clinical trials should be encouraged to better define the PD schedules for the combined use of these solutions that may be associated with the best clinical efficacy and the highest level of biocompatibility.

Proteolytic activity and free amino acid concentrations in polymorphonuclear leucocytes.

Polymorphonuclear granulocytes (PMN) are valuable tools for evaluating amino acid (AA) metabolism in nucleated cells, although variations of free amino acid concentrations due to the methods used for the separation of the cells and the procedures used for lysis have been reported. Furthermore, analytical variations in PMN AA concentration may be induced by protease activation during preparation, so that free AA detected in cells could originate from proteolysis other than from the physiological metabolic pathways and transport systems. To study this possibility we measured granulocyte protease activity and AA concentrations in cell suspensions processed with and without the addition of antiproteolytic agents. Granulocyte AA concentrations and protease activity in samples treated with antiproteolytics were 8-15 times lower than in samples processed without antiproteolytics. The use of protease inhibitors throughout the sample preparation is necessary for reliable estimation of free AA in granulocytes.

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).

Prevention of peritonitis in children on peritoneal dialysis.

We reviewed methods of preventing peritonitis in children. A considerable body of evidence indicates that peritonitis rates are lowest with the use of a double-cuffed catheter, with a downward directed tunnel, placed by an experienced surgeon. Evidence in adults, but lacking in children, suggests that exit-site mupirocin will lower Staphylococcus aureus exit-site infections and thus peritonitis rates. The risk of peritonitis due to contamination can be diminished by the avoidance of spiking and by the provision of a long training period. Catheter removal and replacement for catheter-related peritonitis may be done simultaneously in certain circumstances and is useful in decreasing the risk of recurrent peritonitis. Antibiotic prophylaxis at the time of catheter insertion, for contamination, during dialysate leaks, and for invasive procedures appears to be useful in diminishing peritonitis risk.

The use of the PD Adequest mathematical model in pediatric patients on chronic peritoneal dialysis.

OBJECTIVE: To test the accuracy of the PD ADEQUEST kinetic model in calculating peritoneal transport parameters and to quantify the differences between the results of software simulations and direct measurements in order to assess the reliability of this tool in chronic peritoneal dialysis (PD) pediatric patients. PATIENTS: Twenty-nine patients (mean age: 10 +/- 4 years; range: 4-17), 5 on continuous ambulatory PD, 4 on continuous cycling PD, 19 on nocturnal intermittent PD and 1 in nocturnal tidal PD, all free from peritonitis in the previous 2 months. Fourteen patients were anuric and 15 had a mean glomerular filtration rate of 1.79 +/- 1.23 mL/min, range 0.25-4.82. METHODS: In all patients, 24-hour dialysate and urine collections associated to standard peritoneal equilibration test (PET) were performed using their usual dialytic regimen and fill volume (1023 +/- 159 mL/m2 BSA, range 614-1361). PD ADEQUEST kinetic parameters were compared with pediatric and adult data from literature. The measured weekly normalized total creatinine clearance (CRCL), weekly total Kt/V, and daily net ultrafiltration (UF) were compared with corresponding mathematically modeled values. RESULTS: Kinetic parameters calculated by the PD ADEQUEST program were comparable to adult and pediatric values from previous studies after normalization for BSA. Measured and modeled CRCL and Kt/V showed a good agreement [concordance correlation (rc) 0.937 and 0.768, respectively] with limited median percentage absolute errors (11.6% and 10.2%, respectively). Ultrafiltration showed less favorable results (rc = 0.600 and median percentage absolute error 45%) probably owing to the wide variability of this parameter. When the analysis was restricted to the peritoneal component, the rc coefficients results were 0.745 for CRCL and 0.512 for Kt/V (median absolute error: 11.6% and 15.2%, respectively). CONCLUSIONS: The overall findings of our study show that the PD ADEQUEST kinetic model can be used in pediatric patients for the calculation of kinetic indexes and for mathematical simulation of the various regimens. We also feel that the results yielded by the PD ADEQUEST program are reliable enough for this computerized mathematical model to be used in the prescription management of pediatric patients. Only UF prediction needs to be used with a certain caution on account of the marked variability of this parameter.

Chronic peritoneal dialysis catheters in pediatric patients: experience of the Italian Registry of Pediatric Chronic Peritoneal Dialysis.

During the period 1986-1991, 140 pediatric patients [age < or = 15 years at the start of chronic peritoneal dialysis (CPD)], belonging to 15 dialysis centers, were enrolled in the Italian Registry of Pediatric Chronic Peritoneal Dialysis. Data on 188 peritoneal catheters were collected: 161 catheters were Tenckhoff (144 double-cuff, 17 single-cuff), and 27 were two-cuff Valli-type catheters. All catheters were surgically inserted; the entry site was in the midline in 84 cases and paramedian in 104. An omentectomy was performed in 78.8% of the cases. Apart from peritonitis, there were 161 catheter-related complications (103 exit-site infections, 17 leakages, 15 obstructions, 15 outer-cuff extrusions, 5 hemoperitoneum, 6 others) observed during 2687.5 dialysis-months, with an incidence of one complication every 16.7 dialysis-months. Fifty-five catheters (29.2%) were removed; infection (39 cases) was the main cause for removal, followed by obstruction (9 cases), dislocation, and outer-cuff extrusion (2 cases each). Actuarial survival of all catheters was 79.7% at 1 year, 66.6% at 2 years, 42.8% at 3 years, and 39.8% at 4 years. No difference in catheter survival was observed according to the entry site. When considering the age of the patients at catheter insertion, a difference close to statistical significance was found (p = 0.06).

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.

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.

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.

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.

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.

A multicenter study on the hospitalization of pediatric patients on chronic peritoneal dialysis.

The Italian Registry of Pediatric Chronic Peritoneal Dialysis (CPD) carried out a special study on patient hospitalization during the years 1989-1992. Ninety-two children (mean age 8.4 +/- 4.7 years) entered the study, for a total of 1406 CPD-months. The contribution of the different causes of hospitalization for a total of 4683 hospital days was: CPD training 31%; routine controls 14%; CPD-related complications 35%; clinical complications 14%; other causes 6%. The rate of patient hospitalization that resulted was 3.33 days/CPD-month; it was higher in the first year (4.32 days/CPD-month) than in the second year (1.64 days/CPD-month) or in the third year (2.25 days/CPD-month). This difference was mainly due to the need for the training at the start of the CPD treatment. The evaluation of the hospitalization rate in different age groups showed a statistically significant difference (p < 0.05) between the group 0-2 years (5.47 days/CPD-month) and the group 3-15 years (2.78 days/CPD-month). Complications were the cause of 150 admissions to the hospital (1:9.6 CPD-months). Ninety-eight admissions were due to CPD-related complications: peritonitis (33%), problems with the catheter (19%), abdominal hernias (4%), and others (9%). Among clinical complications (52 admissions), the main cause of hospitalization was hypertension (15%), followed by infections (4%), and malnutrition (3%).

Five years' experience of the Italian Registry of Pediatric Chronic Peritoneal Dialysis.

The results of the first 5 years' experience of the Italian Registry of Pediatric Chronic Peritoneal Dialysis (CPD) (1986-1990) are presented. Patients of less than 15 years of age at start of dialysis were enrolled and clinical data collected until the age of 19. The number of the dialysis centres participating in the Registry increased from 7 in 1986 to 15 in 1990. The total number of patients on CPD was 119, the number of new patients per year ranged from 15 to 28 and the percentage of all dialysed children treated with CPD increased from 40% in 1986 to 49% in 1990. The age of patients at start of CPD was 8.5 +/- 4.9 years and 16% of them were under 2 years. Only CAPD was utilized in 1986, while CCPD/NPD accounted for 53% and 65% of the treated patients in 1989 and 1990, respectively. At 4 years, patient survival was 91.3% and technique survival 79.3%. A comparison between data of 48 patients on CPD and 34 on hemodialysis, who started dialysis in the period 1989-1990, showed that CPD was the most frequent form of initial therapy (56%) and was the treatment of choice for children younger than 4 years.

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.

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.

The branchio-oto-renal syndrome (report of two family groups).

The major features of the Branchio-Oto-Renal syndrome (BOR syndrome), an autosomal dominant disorder, are branchial remnants, ear anomalies, deafness and renal dysplasia. We report two family groups affected by the BOR syndrome: in two-thirds of the affected children renal abnormalities led to severe renal insufficiency in early life. The necessity for a meticulous search for renal anomalies in individuals with aural and/or branchial abnormalities is emphasized. In affected families, genetic counselling is suggested.

[Clinical aspects of the nephropathy in Schoenlein-Henoch syndrome]. / Aspetti clinici della nefropatia della sindrome di Schoenlein-Henoch.

Fourty-four children with Henoch-Schoenlein nephritis were studied at the onset of the nephropathy and during a follow-up from 6 to 110 months. The extra-renal manifestations were purpura (100%), abdominal pain (63.5)% or melena (27%), arthlagias (61.5%), neurological symptoms with convulsions (4.5%) and retinal involvement (4.5%). The clinical presentation of the nephropathy consisted in haematuria and proteinuria (41%), isolated haematuria (30%), acute renal failure (ARF) (23%), nephrotic syndrome (4%) or isolated proteinuria (2%). Hypertension was present in 17 patients. Renal biopsy was performed in 18 patients and the glomerular changes were graded according to the classification of ISKDC; the renal histopathology ranged from minimal lesions to severe crescentic glomerulonephritis and was found to correlate with clinical state. Twenty-four patients, who showed severe clinical presentation and/or diffuse mesangial proliferation with high proportion of crescents, received a corticosteroid therapy. Most of our patients followed a relatively benign course: all but one of patients with ARF have normal renal function at the end of follow-up and no patients with less severe renal presentation got a bad outcome. Only 2 patients showed relapse of nephropathy and purpura at the 6th and 8th year of follow-up, respectively. After 24 months of follow-up the clinical outcome of a group of 19 patients receiving corticosteroid therapy was not very different from that of 11 untreated patients.