Cognitive and psychosocial outcome of infants dialysed in infancy.

OBJECTIVE: To contribute further to the understanding of cognitive and psychosocial outcome of children with end-stage renal disease undergoing long-term peritoneal dialysis. METHODS: In total, 16 surviving infants at a single centre beginning peritoneal dialysis in the first year of life were studied. The age range of the children at assessment was 1.6-12.1 years. Children were assessed using the Griffiths Mental Development Scales, the Wechsler Intelligence Scale for Children-Third Edition UK, and the Strengths and Difficulties Questionnaire. Information regarding the child's hospital stay and family background was also collated. A Pearson's Product Moment correlation was used to analyse the results. RESULTS: Although 67% of the children's scores fell within the average range, 87% were within at least two SDs of the norms (mean IQ = 86.6). Psychosocial adjustment measures revealed that 50% of scores fell within the borderline to abnormal category, suggesting that the frequency of psychological difficulties was above that of the normal population. CONCLUSIONS: These findings lend support to recent studies indicating that, developmentally, children undergoing long-term peritoneal dialysis are faring better than in the past. This may indeed be a reflection of improvements in renal treatment and diet. The behavioural results suggest the need to monitor psychological adjustment in this group of children.

Renal outcome in patients with cloaca.

PURPOSE: Cloaca is a complex malformation in which the rectum, vagina and urinary tract open into a single common channel. Functional results after reconstructive surgery have been documented but the renal outcome is less clearly understood. MATERIALS AND METHODS: The records of all patients with cloacal malformation treated at our institution from 1980 to 2000 were retrospectively reviewed to determine the renal outcome. All patients underwent serial ultrasound of the urinary tract, voiding cystography, nuclear renography, lumbosacral radiography, and measurement of serum creatinine and glomerular filtration rate when appropriate. RESULTS: We identified 64 patients 0.5 to 19 years old (mean age 11.2) at the time of the study. Of the 64 patients 53 (83%) were born with a structural abnormality of the urinary tract, including renal dysplasia in 17 (27%), ectopic kidney in 9 (14%), solitary kidney in 8 (13%), duplex kidneys in 6 (9%) and ureteropelvic junction obstruction in 3 (5%). Vesicoureteral reflux was noted in 34 cases (53%) and a sacral abnormality was diagnosed in 36 (57%). The glomerular filtration rate was measured in 38 patients (60%). Chronic renal failure developed in 32 patients (50%) with a glomerular filtration rate of less than 80 ml. per minute per 1.73 m.2, including 11 (17%) who progressed to end stage renal failure, 4 who (6%) required renal transplantation and 4 who died of chronic renal failure. Of the 32 children with a glomerular filtration rate of less than 80 ml. per minute per 1.73 m.2 44% had renal dysplasia, 25% had a solitary kidney, 44% had sacral anomalies, 72% had vesicoureteral reflux and 47% had secondary renal scarring. CONCLUSIONS: Renal impairment causes significant morbidity and mortality in patients with a cloacal malformation. These patients need a complete nephrourological assessment in the neonatal period due to the high incidence of urinary tract and sacral anomalies as well as careful post-reconstruction followup.

Outcome and growth of infants with severe chronic renal failure.

BACKGROUND: We aimed to assess the outcome and growth of infants with severe chronic renal failure (CRF). One hundred and one children presented between January 1, 1986, and December 12, 1998, with a glomerular filtration rate (GFR) of <20 mL/min/1.73 m2. The median (range) age at presentation was 0.3 (0 to 1.5) years, and follow-up was 7.6 (1.5 to 13) years. One- and five-year survival rates were 87 and 78%, respectively. The growth of the 81 children who survived over two years was evaluated. Eighty-one percent were enterally fed from age 0.7 (0 to 4.5) years for 1.9 (0.1 to 6.8) years. Forty-six percent had a gastrostomy, and 22% a Nissen fundoplication. Twenty-five were managed conservatively. Twenty were transplanted without dialysis at age 4 (1.7 to 8.5) years, and 36 were dialyzed at age 1.1 (0 to 9.8) before transplantation at age 2. 4 (1.3 to 10) years. RESULTS: The mean (SD) height standard deviation score increased from -2.16 (1.34) at 6 months (N = 63) to -1.97 (1.37) at 1 year (N = 75), -1.79 (1.29) at 2 years (N = 75), -1.33 (1.29) at 3 years (N = 68, P = 0.0006), -1.27 (1.04) at 5 years (N = 47, P = 0.0001), and -0.85 (0.82) at 10 years (N = 18, P = 0.001). The body mass index was in the normal range in the majority of patients. CONCLUSION: Mortality in infants with CRF occurs mainly in the first year of life. With early enteral feeding, the mean height standard deviation score is within the normal range from one year of age.

Long-term outcome of peritoneal dialysis in infants.

Debate continues concerning the treatment of infants with end-stage renal disease. We evaluated progress and outcome of 20 infants with a mean age of 0.34 year (range, 0.02-1 year) in a long-term peritoneal dialysis program at a single center. Mean weight at the start of dialysis was 4.8 kg (range, 1.7-11.4 kg), and the duration of dialysis was 17.3 months (range, 1-59 months). Eleven infants received renal transplants, 4 were switched to hemodialysis and then received transplants, 4 died, and 1 continues to receive peritoneal dialysis. There was significant co-morbidity in 6 infants who died or required hemodialysis. Catheter interventions were frequent, with 12 infants requiring at least one replacement. There were 1.1 episodes of peritonitis per patient-year; 70% of infants had 0 to 1 episode. Mean weight standard deviation score (SDS) was -1.6 at the start, -0.3 at 1 year (P =.0008), and 0.3 at 2 years (P =.0008). Height SDSs were -1.8 at the start, -1.1 at 1 year (P =.046), and -0. 8 at 2 years (P =.06). Head circumference SDSs were -1.9 at the start, -1.3 at 6 months (P =.003), and -0.9 at 1 year (P =.015). Fourteen of 16 survivors are achieving normal developmental milestones or attend mainstream school. Peritoneal dialysis in infancy is a demanding treatment, but outcome for growth, development, and transplantation justifies this intensive approach. When parents are counseled, the importance of non-renal co-morbidity must be emphasized.

Long-term enteral nutrition in infants and young children with chronic renal failure.

An inadequate nutritional intake is common in infants and young children with chronic and end-stage renal failure (CRF/ESRF), causing poor weight gain and growth retardation. In a programme of enteral feeding (EF), growth, nutritional intake and outcome for oral feeding were evaluated in 35 children with CRF/ESRF, mean (range) age 1.6 (0-4.9) years at start of EF for 30 (12-60) months. Twenty-nine had a glomerular filtration rate of 12.1 (6-26) ml/min per 1.73 m(2) and 6 were on peritoneal dialysis. Mean (SD) weight standard deviation scores (SDSs) in the 0 to 2-year age group (n=26) were -3.3 (1.0) 6 months before EF, -3.1 (1.3) at the start, -1.7 (1. 4) at 1 year, (P=0.0003) and -1.4 (1.8) at 2 years, (P=0.0008). Height SDSs were -2.9 (0.7), -2.9 (1.2), -2.2 (1.2) (P=0.008) and -2. 1 (1.3) (P=0.004). Weight SDSs in the 2 to 5-year age group (n=9) were -2.3 (1.2), -2.0 (1.1), -1.1 (1.3) (P=0.002) and -0.9 (1.0) (P=0.04). Height SDSs were -2.8 (0.6), -2.3 (0.7), -2.0 (0.7) and -2. 0 (0.8). There was no change in energy intake as a percentage of the estimated average requirement, nor was this exceeded. Percentage energy from the EF in the 0 to 2 year age group remained unchanged despite an absolute increase in energy intake with age. Twenty-one have had renal transplants, of whom 86% eat and drink normally. Long-term EF prevents or reverses weight loss and growth retardation in children with CRF/ESRF, with the achievement of significant catch-up growth if started before age 2 years.

Early-onset chronic renal failure as a presentation of infantile nephropathic cystinosis.

We report an 18-month-old girl who presented in chronic renal failure after an illness characterised by protracted diarrhoea, poor weight gain and anaemia. There were no symptoms and signs suggestive of a renal Fanconi syndrome, but a diagnosis of nephropathic cystinosis was suggested by renal biopsy and confirmed by an elevated leucocyte cystine concentration. We suggest that the diagnosis of cystinosis should be considered in any child with chronic renal failure of unknown aetiology.

Serum intact parathyroid hormone and ionised calcium concentration in children with renal insufficiency.

We report our experience of the use of an immunoradiometric assay for intact parathyroid hormone (i-PTH) and the measurement of plasma ionised calcium concentration (PCa2+) in 73 children with chronic renal insufficiency (CRI); plasma creatinine concentration (PCr) 52-856 mumol/l. There was a poor correlation between i-PTH and PCr (r = 0.10, n = 552) compared with that for C-terminal PTH and PCr (r = 0.60, n = 248), suggesting that the i-PTH assay is independent of renal function in this group of treated children. A clear response of i-PTH to a low total plasma Ca (tPCa) and PCa2+ was observed. There was a significant positive correlation between both tPCa and PCa2+ (r = 0.50, n = 389) and the fraction of Ca2+ (the fraction of tCa which was ionised) and PCa2+ (r = 0.50, n = 389). The finding of a low or normal PCa2+ with a low calculated fraction of Ca2+ was frequently observed, i.e. the measured tPCa was unexpectedly high, suggesting complexing of Ca2+ by accumulated anions in CRI. There was a poor relationship between the plasma albumin concentration and both bound plus complexed Ca (tPCa minus PCa2+) and the fraction of Ca2+ (r = 0.15 and -0.17, respectively). The positive predictive value for a raised i-PTH of a tubular reabsorption of phosphate of less than 80% was 0.87, and of an alkaline phosphatase greater than 800 U/l was 0.37.(ABSTRACT TRUNCATED AT 250 WORDS)

Foregut motor function in chronic renal failure.

In children with chronic renal failure (CRF) anorexia, nausea, and vomiting are common yet poorly understood symptoms. We studied oesophageal and gastric motor function in 12 children (age 7 months-6.8 years) with severe CRF not undergoing dialysis who had persistent anorexia and vomiting. Eight of 12 patients had significant gastro-oesophageal reflux (reflux index 5.2% to 21.9%, mean 11.3%; controls < 5%), 7/10 had altered gastric half emptying times (T1/2) for 5% glucose or milk (glucose meal--controls: 8-14 min, two CRF patients: 18-25 min; milk meal--controls: 48-72 min, five CRF patients 27, 28, 82, 83, and 110 min). Gastric antral electrical control activity was abnormal in 6/11 patients, with different types of gastric dysrhythmias whereas the remainder and controls showed a regular dominant frequency of 0.05 Hz. In 7/9 patients fasting serum gastrin concentration was raised (53 to > 400, mean 168 pmol/l, controls < 40 pmol/l). All CRF patients with anorexia and vomiting had one or more disorder of foregut motility. The nature and variety of the motor disorders and the raised concentrations of circulating gastrin suggest that the normal environment generated by CRF affects the function of the smooth muscle of the foregut.

Losses of calcitriol to peritoneal dialysis bags and tubing.

Peritoneal dialysis provides a convenient means of administering calcitriol. We investigated in vitro the efficiency with which this approach would deliver the drug to patients. We used an injectable preparation of calcitriol, Calcijex (Abbott Laboratories, Montreal, Quebec, Canada), which we radiolabelled by adding radioactive 1,25-dihydroxyvitamin D3 to it. The preparation was injected into dialysis bags and drained through appropriate tubing to monitor delivery of the radiolabelled calcitriol. By 2 h after injecting calcitriol into dialysis bags, 50% of the dose was left in the fluid, by 20 h only 26% was left. The delivered drug was pure 1,25-dihydroxyvitamin D3 based on chromatographic analysis of the recovered radiochemical. To study what would happen if the drug was in the bag for a minimal length of time the calcitriol was injected immediately prior to draining the fluid. We recovered 62.9% +/- 5.2% SD of the dose from the tubing which would theoretically have entered a patient. There was no significant change in calcitriol dose delivered if the fluid was warmed to 37 degrees C prior to injecting the drug and immediate drainage of the bag.