Strategies to support families of children with end-stage renal failure

The burden of care for families looking after children with end-stage renal failure can be considerable, especially when it involves home peritoneal dialysis and supplementary feeding. Good communication with the family and between team members delivering the multidisciplinary care is essential. Stress may be partly reduced by meeting the information needs and supplementing the spoken word with booklets, videos, tape-recorded interviews and play preparation for children. Families greatly appreciate a continuum of care between the hospital and community which is enhanced by team members visiting the home, nursery/school and primary care physician to impart and update knowledge. Respite care is essential for home dialysis patients. It can be enhanced by an evening baby-sitting service and the involvement of a home care renal nurse, as well as a holiday support strategy. Participation of the families in a regular parents support group provides not only an opportunity to integrate families into the unit, but also allows direct feedback on issues affecting the quality of care.     

Prognosis of bedridden patients with end-stage renal failure after starting hemodialysis

Background The mean age of starting hemodialysis (HD) in patients with end-stage renal failure is gradually increasing in Japan. It is not uncommon for HD to be commenced in bedridden elderly patients who cannot give informed consent, because of brain damage. However, we have not been able to provide useful advice to their families because there was no relevant information available about the prognosis of bedridden patients on HD. Therefore, we examined the prognosis of bedridden HD patients. Methods Two hundred and nineteen patients who received HD were enrolled. These subjects were divided into five groups; (aged <50, 50–59, 60–69, 70–79, and ≥80 years at the commencement of HD), and we compared the overall prognosis between bedridden and nonbedridden patients, as well as that for each age group. Results There were 76 bedridden patients among the 219 HD patients, and the main cause of their bedridden state before starting HD was cerebrovascular disease. The 50% survival time after the start of HD was 120 months for the nonbedridden patients versus 56 months for bedridden patients. However, the mean (±SD) age of the bedridden patients was higher than that of nonbedridden patients (70 ± 13 versus 64 ± 14 years). In patients under age 50 years at the start of dialysis, the survival rate was lower in the bedridden than in the nonbedridden patients, but there were no differences between survival rates for bedridden and nonbedridden patients in the other four age groups. Conclusions The prognosis of HD patients is poor compared with the general life expectancy of the Japanese population, but whether these patients are bedridden or not has little influence on their survival.     

Stopping treatment for end-stage renal failure: the rights of children and adolescents

Dialysis and transplantation have been of obvious benefit to children with renal failure. Yet this is not always so. End-stage treatment can also create physical and mental suffering, for both patients and their families. For this reason, the availability of life-saving treatment creates difficult ethical and legal dilemmas concerning when it can morally and lawfully be rejected. These dilemmas are explored through a general account of the doctrine of informed consent and its application to the refusal of such therapy. Clinical and parental duties of care are outlined for young and adolescent children. For young children, the rights of parents are evaluated, recognising the significance of parental involvement and support for treatment for renal failure to be successful. It is further argued that adolescents who are deemed to be competent to give informed consent have the moral right to choose to die rather than to continue treatment. To minimise this prospect, the importance of effective counselling and peer group support is made clear, along with a partnership model of clinical negotiation.     

Therapeutic camping for children with end-stage renal disease

Therapeutic camping experiences for children with end-stage renal disease (ESRD) have proliferated in the United States and abroad. This report is based on the results of a survey designed to accumulate data on the development and implementation of 20 such camps. Children attending camp ranged in age from 1 year to 19 years. Single disease-specific camps were most common, while camps for children with a variety of chronic illnesses, including ESRD, and mainstream camps were also conducted. Facilities were available for hemodialysis and continuous ambulatory peritoneal dialysis, but not automated peritoneal dialysis, in the majority of surveryed camps. Dialysis nurses, pediatric nephrologists, dietitians and social workers were the medical personnel that most frequently participated in the camps. On average, 32 dialysis/transplant patient campers (range 6–100) attended camp for a 1-week session. Therapeutic camping experiences for children with ESRD are extremely successful and attempts to increase the availability of similar camps should be encouraged.Presented in part at the 13th Annual Conference on Peritoneal Dialysis, 7–9 March 1993, San Diego, California, USA.     

Evaluation of aortic stiffness in children with chronic renal failure

The measurement of aortic stiffness (As) [aortic strain (S), pressure strain elastic modulus (Ep) and pressure strain normalized by diastolic pressure (Ep*)] is suggested as an excellent marker of subclinical arterial sclerosis. We aimed to investigate the presence of As and to determine the relationship between As and some risk factors in children with chronic renal failure (CRF). Twenty-six pre-dialysis (PreD) [female/male (F/M) 7/19] patients and 23 chronic peritoneal dialysis (CPD) (F/M 13/10) patients were assessed. Twenty-nine healthy children were selected as a control group (F/M 14/15). We determined anemia, abnormal calcium/phosphate metabolism, hypertension, diastolic dysfunction, increased left ventricular mass (LVM), hypertriglyceridemia, increased stiffness (Ep, Ep*), and decreased strain (S) in the CRF (PreD and CPD) group compared with the controls (P < 0.05). Presence of renal disease, LVM and usage of angiotensin-converting enzyme inhibitor (ACE-I) in all groups; female gender, duration of disease and the usage of anti-hypertensive drug therapy in CRF patients; and LVM and LVM index in healthy children were found to be independent predictors for aortic stiffness and/or strain. In conclusion, CRF is associated with significant arterial functional abnormalities in uremic children and not controlled by dialysis treatment. These results suggest that, even in young children, uremia has a profound impact on arterial function.     

Familial hypomagnesaemia — hypercalciuria leading to end-stage renal failure

Several disorders of hypomagnesaemia of hetary renal origin are now recognised. The cases of two sisters from a consanguineous marriage with the syndrome of renal magnesium wasting, hypercalciuria and nephrocalcinosis are presented. Pathological examination of the heterozygous parental kidneys revealed mild focal interstitial fibrosis. This condition is a previously unreported cause of end-stage renal failure in chilhhood, and this report suggests that transplantation from heterozygous parental donors can be successfully undertaken without recurrence currence of the syndrome.     

Association of dominant dystrophic epidermolysis bullosa with end-stage renal failure and hearing loss

A 9-year-old boy with dominant dystrophic epidermolysis bullosa had hematuria. At the age of 14 he had proteinuria and at the age of 19 he had developed end-stage renal failure. Histopathological diagnosis of glomerular lesions was not made. During the course of his illness, high-frequency sensorineural hearing impairment developed and he became completely deaf. The hearing loss was thought to be caused by fradiomycin ototoxicity, as fradiomycin ointment had been used to treat secondary infections of the skin lesions since he was an infant. To our knowledge, an association of dominant dystrophic epidermolysis bullosa with end-stage renal failure and ototoxicity due to fradiomycin ointment have not been reported previously. Received: August 25, 1998 / Accepted: October 22, 1998     

Immunogenicity of hepatitis B vaccine (HEVAC B) in children with advanced renal failure

The immune response after hepatitis B (HB) vaccine HEVAC B was studied in 33 children (mean age 10±4 years) with advanced renal failure. Responders and protected patients were defined by antibody titres to HB surface antigen (anti-HBs) of greater than 10 and 50 mIU/ml, respectively. All received the initial recommended three injections at monthly intervals, and 23 received a booster injection (I_B) 11±1 months after the third injection (I₃). Loss of protection after I₃ led to additional injections in 8 patients (25%). Vaccine was well tolerated and no HB infection occurred during the follow-up period (19±10 months). The percentage of responders was 91% 2±1 months after I₃, and 100% 1 month, 13±1 months and 26±2 months after I_B. The percentages of protected patients at these dates were 91%, 95%, 100% and 100%. Anti-HBs titres 1–3 months after I₃ were useful for indicating those patients likely to have a rapid decline in anti-HBs titres, thus requiring serial anti-HBs determinations and additional injections to prevent a loss of protection. We conclude that, at the expense of a reinforced vaccination schedule in 25% of patients, HEVAC B vaccine can safely achieve a sustained protection in more than 90% of uracmic children.     

Skeletal status in adolescents with end-stage renal failure: a longitudinal study

In a longitudinal study, bone status was assessed in adolescents and young adults aged 15.3±3.4 years at the onset of the study with end-stage renal failure (ESRF). The group consisted of 18 subjects (11 females and seven males), of whom nine patients were on hemodialysis and nine patients on peritoneal dialysis. Six patients were previous or current glucocorticoid (GCS) users. Renal failure was recognized before 6.1±4.1 years, and dialysis was performed for 3.0±2.0 years. Follow-up took placed 8.6±0.8 and 21.7±2.5 months later, and the following data were collected: bone mineral density (BMD) at the spine (s-BMD) and total body (TB-BMD) using DPX-L (Lunar, USA); quantitative ultrasound by DBM 1200 (IGEA, Italy) at the hand phalanges (Amplitude-dependent Speed of Sound, Ad-SoS), serum concentration of i-PTH, total calcium, ionized calcium and phosphate. Tanner stages were also evaluated. The mean values of BMD measurements and Ad-SoS were stable during a period of observation, and a mean Z-score for TB-BMD was significantly lower at the third versus baseline value (–1.87±1.75 versus –1.49±1.53, P<0.05). Z-scores for s-BMD and Ad-SoS decreased non-significantly. Changes in s-BMD and TB-BMD Z-scores were influenced by changes in body size and changes in biochemical parameters, and a change in Ad-SoS Z-score was not dependent on these factors. The values of second (P<0.05) and third (P<0.01) s-BMD Z-score were significantly lower in GCS treated subjects, and longitudinal change in spine Z-score was greater in GCS treated patients versus others (P<0.05). Duration of ESRF, duration and type of dialysis and gender did not influence skeletal variables. Skeletal measurements correlated significantly with Tanner stages (besides the correlation with Ad-SoS in the first measurement, r ranged from 0.5 to 0.72, P<0.05), and changes in Tanner stages observed over a period of observation did not correlate with changes in skeletal variables. Among laboratory variables, the following non-significant tendencies to change were observed: serum concentration of i-PTH and phosphate increased, and total and ionized calcium decreased. In conclusion, adolescent subjects with ESRF treated with dialysis showed stable mean values of skeletal measurements, and these were expressed as Z-scores, a tendency to drop was observed. The lack of an increase observed in normal healthy subjects of the same age, and low values in Z-scores, indicates that skeletal status is seriously affected in subjects with ESRF.     

Growth failure,risk of hospitalization and death for children with end-stage renal disease

Growth failure remains a significant problem for children with chronic renal insufficiency and end-stage renal disease (ESRD). We examined whether growth failure is associated with more-frequent hospitalizations or higher mortality in children with kidney disease. We studied data on prevalent United States pediatric patients with ESRD in 1990 who were followed through 1995. Patients were categorized according to the standard deviation score (SDS) of their incremental growth during 1990: severe (<–3 SDS), moderate growth failure (>–3 and <–2 SDS), and normal growth (>–2 SDS). Among 1,112 prevalent pediatric dialysis and transplant patients (<17 years, Tanner I–IV), those with severe and moderate growth failure had higher hospitalization rates {relative risk (RR) 1.14 [95% confidence interval (CI) 1.1, 1.2] and 1.24 [95% CI 1.2, 1.3]} respectively than those with normal growth after adjustment for age, gender, race, cause and duration of ESRD, and treatment modality (dialysis or transplant) in 1990. Kaplan-Meier survival analysis showed 5-year survival of 85% and 90% for patients with severe and moderate growth failure, respectively, compared with 96% for patients with normal growth (P<0.001, log-rank). Cox proportional hazards analysis revealed that those with severe (RR 2.9, 95% CI 1.6, 5.3) and moderate growth failure (RR 2.01, 95% CI 1.1, 3.6) had an increased risk of death compared with youths with normal growth, after adjustment. A higher proportion of deaths in the severe and moderate growth failure groups were attributed to infectious causes (22% and 18.7%, respectively) than in the normal growth group (15.6%). We conclude that growth failure is associated with a more-complicated clinical course and increased risk of death for children with kidney failure. Received: 15 August 2001 / Revised: 14 January 2002 / Accepted: 15 January 2002     

School adjustment of children with end-stage renal disease

We examined adjustment to school of children with end-stage renal disease (ESRD) and the psychological condition of their mothers; 30 children on continuous ambulatory peritoneal dialysis (CAPD), 35 children with a transplant and 33 healthy children were studied. A factorial ANOVA statistic found that the mean scores for non-academic problems (psychological factors) differed significantly across the three groups, being highest in CAPD children and lowest in healthy children. No significant differences between CAPD and transplant children were found for school maladjustment with academic problems, although the prevalence differed significantly across the groups, being highest in transplant children and lowest in healthy children. The mothers of children with ESRD were prone to feel various anxieties and dependency.     

Intracapsular hip fractures in end-stage renal failure

Patients with end-stage renal failure (ESRF) have metabolic bone disease. This increases the risk of femoral neck fracture and increases the risk of complications associated with fracture fixation such as non-union and avascular necrosis (AVN). We report the results of treatment in a consecutive series of 15 intracapsular fractures of the hip occurring in 13 patients with ESRF over a 5-year period. Six intracapsular hip fractures (of which five were undisplaced) were treated by internal fixation. Five out of these six (mean=83.3%) required conversion to total hip arthroplasty because of non-union or AVN. In all six of these patients, internal fixation was considered adequate post operatively. Of the remaining nine intracapsular hip fractures treated by hemiarthroplasty, only one required conversion to total hip arthroplasty because of stem subsidence (mean=11%). The difference in the revision rate for the two groups i.e. primary fixation versus primary hemiarthroplasty was statistically significant (p-value=0.01). The six patients with undisplaced intracapsular fractures treated by internal fixation required a total of 14 major operations, at an average rate (including initial fracture fixation and revision surgery) of 2.3 per patient. The 9 displaced fractures treated by hemiarthroplasty required just 10 operations in total, at an average rate of 1.1 per patient. (The difference was significant; p-value=0.006.) The 1-year mortality in the whole group (13 patients with 15 fractures) was 44.4%. We suggest that patients with ESRF with an intracapsular fracture of the neck of femur should be treated by replacement arthroplasty irrespective of femoral head displacement because of the high risk of revision surgery associated with internal fixation.     

Acacia gum supplementation of a low-protein diet in children with end-stage renal disease

Patients with end-stage renal disease (ESRD) die in the absence of renal replacement therapy (RRT). In developing countries RRT is not uniformly available and treatment often relies on conservative management and intermittent peritoneal dialysis (IPD). This study investigates the possibility of using acacia gum supplementation to improve the quality of life and provide children with ESRD with a dialysis-free period. Three patients referred to our hospital with ESRD during a 3-month period were enrolled in a therapeutic trial to investigate the efficacy of acacia gum (1 g/kg per day in divided doses) as a complementary conservative measure aimed at improving the quality of life. Inclusion criteria included a pre-dialysis creatinine clearance of <5 ml/min, current dietary restrictions and supplementation, at least one dialysis session to control uremic symptoms, absence of life-threatening complications, and sufficient motivation to ensure compliance with the study protocol. One patient complied with the protocol for only 10 days and died after 6 months, despite IPD. Two patients completed the study. Both reported improved well-being. Neither became acidotic or uremic, and neither required dialysis during the study period. Both patients maintained urinary creatinine and urea levels not previously achieved without dialysis. In conclusion, dietary supplementation with acacia gum may be an alternative to renal replacement therapy to improve the quality of life and reduce or eliminate the need for dialysis in children with ESRD in some developing countries.     

Bariatric surgery in patients with obesity and end-stage renal disease

BackgroundBariatric surgery has been suggested as a treatment for obesity and end-stage renal disease (ESRD). Although the number of bariatric surgeries in patients with ESRD is increasing, its safety and effectiveness in these patients are still controversial and the surgical method of choice in these patients is under debate.ObjectivesTo compare the outcomes of bariatric surgery between patients with and without ESRD and to assess different methods of bariatric surgery in patients with ESRD.SettingMeta-analysis.MethodsA comprehensive search was conducted in Web of Science and Medline (via Pubmed) until May 2022. Tow meta-analyses were performed: A) to compare bariatric surgery outcomes among patients with and without ESRD, and B) to compare outcomes of Roux-en-Y gastric bypass (RYGB) and sleeve gastrectomy (SG) in patients with ESRD. Using a random-effect model, odds ratios (ORs) and mean differences (MDs) with 95% confidence intervals (CIs) were computed for surgical and weight loss outcomes.ResultsOf 5895 articles, 6 studies were included in meta-analysis A and 8 studies in meta-analysis B. The risk of bias was moderate to serious among studies. Major postoperative complications (OR = 2.82; 95% CI = 1.66–4.77; P = .0001), reoperation (OR = 2.66; 95% CI = 1.99–3.56; P < .00001), readmission (OR = 2.37; 95% CI = 1.55–3.64; P < .0001), and in-hospital/90-d mortality (OR = 4.03; 95% CI = 1.80–9.03; P = .0007) were higher in patients with ESRD. Patients with ESRD also had a longer hospital stay (MD = 1.23; 95% CI = .32–2.14; P = .008). Bleeding, leakage, and total weight loss were comparable among groups. SG showed a 10% lower rate of overall complications and significantly shorter hospital stay than RYGB did. The quality of evidence was very low for the outcomesConclusionsBariatric surgery in patients with ESRD seems to have higher rates of major complications and perioperative mortality than in patients without ESRD, but a comparable rate of overall complications. SG has fewer postoperative complications and could be the method of choice in these patients. These findings should be interpreted cautiously in light of the moderate to high risk of bias in most included studies.     

Cinacalcet for secondary hyperparathyroidism in children with end-stage renal disease

The efficacy and acceptability of cinacalcet for treatment of secondary hyperparathyroidism (SHPT) was assessed in seven pediatric patients suffering from end-stage renal disease (ESRD) presenting with inadequately controlled SHPT despite conventional management. Patients received daily treatment with cinacalcet (dosage 0.25 mg/kg body weight) for a total of 4 weeks. Within 4 h after application of the first dose, median levels of serum parathyroid hormone (PTH) had decreased from 932 pg/ml (range 511-1,938 pg/ml) to 584 pg/ml (88-937 pg/ml), and final pre-dose values after 4 weeks were 199 pg/ml (121-940 pg/ml; each P < 0.05 versus baseline). Median concentrations of serum calcium (Ca) decreased within 4 h of the first administration, from 2.56 mmol/l to 2.38 mmol/l, returning to 2.58 mmol/l at 24 h, and they remained slightly decreased compared to baseline values thereafter (each P < 0.05 versus baseline). Both the median levels of serum phosphorus (P) and the Ca x P ion product decreased significantly during the 4-week period. Cinacalcet was well tolerated and without drug-related adverse effects. Thus, even with approximately half of the dose usually given to adult dialysis patients, PTH and the Ca x P ion product were markedly reduced in pediatric ESRD patients presenting with inadequately controlled SHPT. Therefore, our results support the initiation of a randomized, controlled, long-term trial in children.     

Renal replacement modalities for childhood end-stage renal failure

Summary: An overview is presented of the current management of children with end-stage renal disease (ESRD), focusing on specific therapies such as haemodialysis, peritoneal dialysis and renal transplantation. Also discussed is the overall management of such children, with attention directed at growth, nutrition and development.     

Cardiovascular complications in pediatric end-stage renal disease

Mortality from end-stage renal disease (ESRD) is often due to cardiac causes. Although cardiovascular complications of ESRD have long been recognized, only recently has the presence of traditional cardiovascular risk factors been associated with late cardiovascular complications. This review presents a history of cardiac involvement in ESRD, the pathophysiology of accelerated atherosclerosis and left ventricular hypertrophy, and a summary of the literature on cardiovascular risk assessment in children. Techniques for non-invasive assessment of cardiac end-organ injury are also discussed. Recommendations for monitoring of risk factors and treatment in the pediatric ESRD population are presented.     

Towards guidelines for dialysis in children with end-stage renal disease

There are few data describing the current practices of treatment selection for children with end-stage renal disease (ESRD). In an effort to establish a consensus among Spanish pediatric nephrologists for inclusion and exclusion criteria for renal replacement therapy in children with ESRD, in 1995 we surveyed members of the Spanish Pediatric Nephrology Association. Although only 43% of members responded, pediatric nephrologists and bioethicists studied the results and compiled a list of ten guidelines for treatment of children with ESRD. The proposed guidelines are meant to be a starting point for further discussion. An emphasis on flexibility, individual case assessment, and consideration of the best interests of the patient must remain central to any treatment plan. Decision making should ideally be shared by parents, professionals, the child, when appropriate, and ethics committees, as necessary. Received: 9 February 1999 / Revised: 21 June 1999 / Accepted: 28 June 1999