[Psychosocial adaptation in adolescents with chronic kidney disease]. / L'adattamento psico-sociale nell'adolescente con nefropatia cronica.

BACKGROUND: Chronic disease represents a very critical experience for personal identity. This is far more true in adolescence when corporeal, emotional, relational and cognitive changes are taking place. The aim of the present study is to draw attention to the social and emotional balance and the psychosocial adaptation in a group of nephropathic adolescents and to formulate a suitable assistance program. METHODS: We studied two adolescent groups, one group of nephropathics and one group of healthy individuals, by administering the MMPI (Minnesota Multiphasic Personality Inventory) personality test. RESULTS: We found significant differences between the mean score of the two groups in the clinical scale for depression, hypochondria and hysteria (p < 0.5); in addition, we observed a statistically significant difference in the emotional balance indicators. CONCLUSIONS: Our experience suggests that nephropathic adolescents require global awareness of their psychological problems through the use of innovative assistance methods. A new strategy involving a more modern approach (i.e., educational camps) and specific psychosocial rehabilitation programs should be introduced to improve the quality of life.

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.

Renal-retinal syndromes: association of retinal anomalies and recessive nephronophthisis in patients with homozygous deletion of the NPH1 locus.

Tapeto-retinal degeneration is frequent in patients with nephronophthisis. Association of the most severe forms of tapeto-retinal dystrophy with NPH identifies a syndrome described first by Senior et al and Loken et al. This syndrome is distinct on molecular grounds from pure renal nephronophthisis (NPH1), which has its gene locus mapped on chromosome 2q13. We describe three families with large homozygous deletion of the NPH1 locus in which mild to moderate ocular lesions due to tapeto-retinal degeneration coexisted and were correlated to renal defects. This new association of NPH1 with retinal dystrophy is characterized by focal lesions of retina and is pauci-symptomatic in clinical presentation. For this reason it may remain unrecognized in most NPH1 patients.

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.

Chronic cholestatic liver disease with associated tubulointerstitial nephropathy in early childhood.

We report the clinical and morphological features of an unusual hepatorenal disorder in 2 patients. The main clinical features were early onset of cholestatic liver disease and progressive tubulointerstitial nephritis, leading to renal death in early childhood. Renal histology showed interstitial fibrosis, tubular atrophy and dilatation, glomerular cysts in the cortex and periglomerular fibrosis; liver histology was characterized by portal fibrosis and bile duct abnormalities. Evaluating the 12 patients published in the literature, the long-term prognosis of the liver function appears bad, suggesting the possibility of a combined liver and kidney transplantation.

[Hyperechogenicity of the renal medulla in children: clinico-echographic correlations]. / Iperecogenicità della midollare renale nel bambino: correlazioni clinico-ecografiche.

Increased renal medullary echogenicity by renal ultrasound associated with hypercalciuria and nephrocalcinosis is often present in childhood. 17 children, 9 boys and 8 girls, aged from 8 months to 10 years were classified into three groups based on ultrasound findings according to Patriquin and Robitaille: type A faint hyperechogenic rim around the sides and tip of the medullary pyramid; type B more intense echogenic rim of the pyramids; type C intense echoes throughout the pyramid. Clinic-echographic correlations showed a pattern C in 4 children with distal renal tubular acidosis and in an infant treated with furosemide; pattern B in 3 patients having different types of tubulopathy associated with hypercalciuria; pattern A in 6 children with congenital tubulopathy and in 3 children treated with vitamin D. Abdominal X-rays detected medullary calcinosis in 2 (11.7%) of total 17 patients. Ultrasonography appears to be an important tool in early diagnosis of renal medullary nephrocalcinosis.

[Echographic diagnosis of nephro-urological neoplasia in a pediatric nephrology center]. / Diagnosi ecografica di neoplasie nefro-urologiche in un centro di nefrologia pediatrica.

The use of ultrasound for the diagnosis of urologic neoplasms in simple and accurate. Nowadays nephrologists and urologists advocate the use of ultrasound and the procedure now plays a complementary role of the clinical examination. We present a retrospective study regarding our experience with ultrasonography in the diagnosis of urologic problems. In the period february 95-february 96 we performed 672 ultrasound examination on children ranging in age from 30 days to 16 years. All examinations were performed using a 3.5-5 MHz real time convex scanner. The patients were examined in the supine position for imaging of the bladder and in the prone position for imaging of the kidneys. We report 3 cases of neoplasm of the genito-urinary tract screened with the use of ultrasounds in a selected paediatric population.

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.

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%).

Hepatitis C virus antibodies in dialysis pediatric patients.

A new 4-antigen recombinant immunoblot assay (4-RIBA) for confirmation of hepatitis C virus (HCV) C-100 enzyme-linked immunosorbent assay (ELISA) reactivity was tested in serum samples of 11 pediatric patients on dialysis. Of 6 HCV C-100 ELISA-positive samples, all were 4-RIBA positive. The 2nd generation ELISA picked up 1 additional case confirmed by 4-RIBA. The 2nd generation tests increased the prevalence of HCV-positive cases.