Eighteen years experience in pediatric acute dialysis: analysis of predictors of outcome.

This study reviewed the 18-year experience of acute dialysis in the pediatric intensive care unit, in order to identify factors that could predict outcome, and to determine whether newer modalities of acute dialysis have influenced this outcome. Sixty-six children (ages 1 day to 19 years) received acute dialysis from May 1980 to April 1998. Factors predicting outcome were analyzed using univariate and Cox regression analysis. Modality of dialysis in the first 15 years was exclusively peritoneal dialysis. with a mortality of 63.9%. However, in the last 3 years, with increasing patient numbers, continuous hemodiafiltration (CHDF) was the modality of choice (56.7%), with a mortality of 73.3%. Univariate analysis showed that age <1 year, coma, acute tubular necrosis, disseminated intravascular coagulopathy, assisted ventilation, and hypotension were associated significantly with poor outcome (P<0.05). Cox regression analysis revealed that mortality was significantly higher in patients on mechanical ventilation (RR 5.96, 95% CI 1.82-19.50), or with age <1 year (RR 2.00, 95% CI 1.08-3.73). In conclusion, despite the increasing use of CHDF over the last 3 years, there was no significant improvement in mortality, probably related to the fact that more critically ill patients were dialyzed.

Membranous nephropathy with anti-tubular basement membrane antibody may be X-linked.

The association of membranous nephropathy with Fanconi syndrome and anti-tubular basement antibodies appears to be a distinct subset of familial membranous nephropathy. We studied two Chinese families with four affected boys to evaluate the mode of inheritance of disease. HLA haplotype analysis of the family members in these two pedigrees did not reveal any significant linkages. However, microsatellite analysis of both pedigrees using markers on the X chromosome suggested linkage to the long arm of the X chromosome between the microsatellite markers DXS1001 and DXS1227. Identification and analysis of additional pedigrees may allow more precise mapping of the disease gene for anti-tubular basement membrane antibody-associated membranous nephropathy.

Quantitation of peripheral blood cytomegalovirus DNA for monitoring recurrent cytomegalovirus retinitis in pediatric solid organ transplant recipients.

Cytomegalovirus (CMV) infection is a major concern following solid organ transplantation, especially in the pediatric population who remain at high risk of primary infection. CMV disease leads not only to increased patient and graft morbidity, but also to increased health care costs. This study describes the usefulness of a quantitative CMV polymerase chain reaction (PCR) technique for monitoring peripheral blood CMV DNA in pediatric recipients of kidney and liver allografts who had recurrent CMV retinitis. The incidence of CMV disease in 28 pediatric transplant recipients was 28.6%, one-half of whom developed retinitis. Two of these patients had recurrent retinitis on cessation of anti-viral treatment. A peripheral blood CMV DNA copy number of > or =500/microg of DNA was associated with recrudescence of the retinitis in these patients. We conclude that the measurement of peripheral blood CMV DNA by PCR is a useful tool for the surveillance of disease resolution and recurrence. This is particularly important in patients with CMV retinitis, who may remain asymptomatic for a period of time, despite recurrences.

Abnormal electrocardiographic patterns in renal failure. Ventricular tachycardia.

A 9-year-old girl was diagnosed to have renal failure. However, her family has not been compliant with medical treatment and opted for traditional therapy instead. She was admitted in an ill state with fluid overload. What is the ECG diagnosis?

Th1 and Th2 cytokine mRNA profiles in childhood nephrotic syndrome: evidence for increased IL-13 mRNA expression in relapse.

Idiopathic nephrotic syndrome of childhood is thought to be associated with T lymphocyte dysfunction often triggered by viral infections, with the production of circulating factor(s) resulting in proteinuria. In view of the conflicting evidence of T cell activation and Th1 or Th2 pattern of cytokine synthesis in this disease, this study examined the mRNA expression of interleukin-2 (IL-2), interferon-gamma, IL-4, and IL-13 from CD4+ and CD8+ T cells in steroid-responsive nephrotic patients in relapse and remission. Fifty-five children with steroid-responsive nephrotic syndrome were included in this study, together with 34 normal controls and 24 patient controls with viral infections. RNA was isolated from purified CD4+ or CD8+ cells from peripheral blood and subjected to reverse transcription-PCR. Cytokine mRNA expression was measured semiquantitatively, and a cytokine index was derived from densitometric readings, with cyclophilin as the housekeeping gene. Both cross-sectional and paired data showed an increased CD4+ and CD8+ IL-13 mRNA expression in patients with nephrotic relapse as compared to remission, normal, and patient controls (P < 0.008). This was also associated with increased cytoplasmic IL-13 expression in phorbol myristate acetate/ionomycin-activated CD3+ cells (6.66+/-3.39%) from patients with nephrotic relapse compared to remission (2.59+/-1.35%) (P < 0.0001). However, there was no significant difference in CD4+ or CD8+ IL-2, interferon-gamma and IL-4 mRNA expression. IL-13 is an important T cell cytokine with anti-inflammatory and immunomodulatory functions on B cells and monocytes. It is conceivable that IL-13 may act on monocytes to produce vascular permeability factor(s) involved in the pathogenesis of proteinuria in patients with relapse nephrotic syndrome.

Can dialysis adequacy be achieved by tailoring the dialysis prescription in an Asian pediatric population on nightly intermittent peritoneal dialysis?

This study was undertaken to determine whether tailoring the dialysis prescription in Asian children on nightly intermittent peritoneal dialysis (NIPD), without adding high-dose therapy for cost-savings purposes, was able to achieve dialysis adequacy and improvement in nutrition. Eight children (age range: 5.5-20 years) on NIPD for a mean of 2.1 +/- 0.6 years, were studied at baseline and at 3 months and 9 months after their dialysis dose was tailored. Dialysis adequacy was measured by weekly Kt/Vurea and creatinine clearance (CCr). Fat-free mass (FFM) and percent body fat (%FAT) as measured by bioelectrical impedance, together with anthropometric measurements, serum total protein, and albumin, were used as indicators of nutrition. After the dialysis prescription was tailored, the mean weekly Kt/Vurea increased from 1.89 +/- 0.35 to 2.12 +/- 0.54 at 9 months, and total CCr increased from 36.4 +/- 11.51 L/1.73 m2 to 48.30 +/- 14.30 L/1.73 m2. The increase occurred despite a decline in residual renal function and was attributable to significant improvements in the peritoneal clearances of urea and creatinine (p < 0.05). The mid arm muscle circumference (MAMC) increased significantly (p = 0.006), while FFM increased from 25.68 +/- 7.92 kg to 26.95 +/- 9.83 kg, and %FAT decreased from 21.56% +/- 8.41% to 18.66% +/- 8.16%. The increase in FFM correlated significantly with a decrease in serum creatinine (r = -0.94, p = 0.005). In conclusion, tailoring the dialysis prescription in NIPD, without adding high-dose therapy, resulted in a trend of improvement in dialysis adequacy and some nutritional parameters.

Efficacy and safety of oral desmopressin in the treatment of primary nocturnal enuresis in Asian children.

OBJECTIVE: To determine the efficacy and safety of oral desmopressin (DDAVP) treatment in Asian children with nocturnal enuresis. METHODOLOGY: This was a multicentre randomized placebo-controlled double-blind cross-over study. Patients were randomized to either active treatment with oral 400 mg DDAVP or placebo, with a 2-week medication-free period between the cross-over. Children with primary monosymptomatic nocturnal enuresis, aged between 7 and 18 years, with a minimum frequency of wetting of 6 nights or more during a 2-week observation period were recruited. Efficacy was measured by reduction in the average number of wet nights per week. RESULTS: Of the 37 children initially recruited, the outcomes for 34 children were included in the final cross-over analysis, as they had complete data for both the treatment periods. Statistical analysis by ANOVA showed that there was no significant difference between the medication-free period and the pretreatment period. However, the average number of wet nights per week for the DDAVP treatment period (2.5+/-2.7) was significantly lower than that of the placebo treatment period (4.5+/-2.1) (P < 0.0001). In terms of the safety profile, there was no significant change in bodyweight, blood pressure, serum sodium, serum osmolality, and urine osmolality following DDAVP treatment. CONCLUSION: Oral DDAVP is a safe and efficacious drug for the short-term treatment of children with primary nocturnal enuresis.

Subclinical activation of lupus nephritis by recombinant human growth hormone.

The effect of growth hormone (GH) on subclinical disease activity in a 15-year-old boy with previously quiescent lupus nephritis and chronic renal failure is described. Institution of supraphysiological doses of GH resulted in a rise in erythrocyte sedimentation rate, decrease in serum complement, rise in anti-DNA antibody titers, and increase in T-cell activation markers, all of which improved following cessation of GH treatment.

Infections in paediatric renal transplant recipients.

A retrospective study of all children who had undergone renal transplantation at the National University Hospital was undertaken in order to investigate the types and frequency of infections in paediatric renal transplant recipients. A total of 13 children ranging in age from 3 to 17 years were transplanted over a 7-year period from 1989 to 1996. The mean follow-up period was 40.5 months. Ten patients (77%) had at least one episode of infection post-transplant, 70% of which occurred within the first 6 months post-transplant. These infections included urinary infections (8), disseminated cytomegalovirus (CMV) disease (4), Pneumocystis carinii pneumonia (1) and mycoplasma pneumonia (1). Urinary tract infections were significantly associated with the presence of ureteral stents. CMV disease was significantly associated with the use of mycophenolic acid in the early post-transplant period. Infection therefore continues to be a major cause of morbidity in paediatric renal transplant recipients.

Primary monosymptomatic nocturnal enuresis in Singapore--parental perspectives in an Asian community.

Primary monosymptomatic nocturnal enuresis (PMNE) is often not openly discussed in Asian societies. We report the parental view of PMNE in Singapore, its impact on patients and their families and the traditional beliefs and its influence on subsequent management. A screening questionnaire was used in evaluating 30 children enrolled in a clinical trial on the use of oral Desmopressin for the treatment of PMNE. Primary monosymptomatic nocturnal enuresis was familial in 56.7% of patients. Fifty per cent of them were previously unevaluated. Earlier remedial attempts included bedtime fluid restriction and voiding (100%), incentive measures (43.3%), traditional practices (26.7%), punishment (20%), drugs (16.7%), psychotherapy (100%) and bladder training (3.3%). Perceived causes of PMNE were maturational delay (50%), deep sleep (50%), familial (43.3%), behavioural problems (43.3%) and excessive fluid intake (26.7%). Reasons for seeking treatment included restricted outdoor activities (90%), parental fatigue (86.7%), disrupted sleep for the household (46.7%) and fear of underlying pathology (26.7%). Perceived adverse effects on patients included social stigma (83.3%), disrupted sleep (33.3%) and impaired school performance (13.3%). Primary monosymptomatic nocturnal enuresis can thus be a chronic distressing problem in Asian communities.

Familial renal adysplasia.

Renal dysplasia and agenesis as isolated findings are usually considered sporadic, noninherited abnormalities. We report three kindreds with familial renal adysplasia. Two or more children were affected in each of the families and at least one member--whether proband, sibling, or parent--had a clinically silent anomaly. Normal kidneys in the parents did not preclude the occurrence of renal adysplasia in more than one child. The empiric risks for offspring and first-degree relatives were 50% and 25%, respectively, suggesting a strong genetic factor such as a major dominant gene with variable expression. Because the disease appears to be genetic in some cases of renal adysplasia, careful screening of the proband's family, subsequent children, and pregnancies is important for the purpose of accurate genetic counseling.

Comparison of progression of renal failure in children with hypoplastic-dysplastic kidneys and chronic glomerulonephritis.

A comparative study was made on two groups of children comprising 20 patients with renal hypoplasia/dysplasia in one group and 12 patients with chronic glomerulonephritis (GN) in the other, presenting with chronic renal failure (CRF) in the Department of Paediatrics, Singapore General Hospital and National University Hospital between 1975 and 1989. The age of onset of CRF, the progression of renal failure and the presence of various clinical complications were analysed and compared. The mean age of onset of CRF was earlier in patients with renal hypoplasia/dysplasia (p less than 0.001) but the progression of renal failure in these patients were slower (p less than 0.005). Hypertension occurred more frequently in the chronic GN group (p less than 0.001) while urinary tract infection (UTI) occurred more frequently in the renal hypoplasia/dysplasia group (p less than 0.004). With the early onset of renal failure and slow deterioration of renal function in patients with renal hypoplasia/dysplasia, the provision of good conservative treatment for renal failure is most important in the management of these patients. In the chronic GN patients however, with the rapidity of deterioration of renal function, early preparation for replacement therapy becomes more imminent. However, renal replacement therapy in end-stage renal failure (ESRF) is costly and not readily available, it is more prudent to delay the onset of ESRF by providing effective conservative treatment of renal failure which includes the early recognition and treatment of hypertension in chronic GN and UTI in renal hypoplasia/dysplasia.

Fungal peritonitis in children treated with peritoneal dialysis and gastrostomy feeding.

Feeding gastrostomies were placed in three children treated with chronic peritoneal dialysis at our center because of persistent, severe malnutrition and inadequate growth. Two had frequent fungal infections of the gastrostomy site and all three developed Candida peritonitis which occurred at 1 month, 2 months and 2 years after insertion of gastrostomy. Complications included multiple intra-abdominal adhesions, abscess formation and loss of peritoneal function necessitating transfer to hemodialysis. The presence of a gastrostomy may predispose to the development of fungal peritonitis with its high morbidity and should be avoided in children on chronic peritoneal dialysis.

Cell-mediated immunity in patients on hemodialysis: relationship with hepatitis B carrier status.

We compared cell-mediated immune responses in two groups of patients on hemodialysis. One group of patients were chronic carriers of hepatitis B virus (HBV), and the patients of the other group were HBV antigen negative. Our results show that despite the presence of normal numbers of T cells and an increased CD4/CD8 ratio in both groups of patients compared to healthy controls (p less than 0.0001), only the group of patients who were chronic HBV carriers showed depressed lymphoproliferative responses to phytohemagglutinin (p less than 0.001) and concanavalin A (p less than 0.0001). In contrast, a control group of healthy adult HBV carriers showed normal T cell subsets and lymphoproliferative responses to mitogens, indicating that HBV infection per se did not result in depressed lymphoproliferative responses. These results further substantiate the notion that depressed cell-mediated immunity in chronic renal failure is an important factor in predisposing patients to HBV infection with subsequent development of the chronic carrier state.

Acute glomerulonephritis--changing patterns in Singapore children.

This study compared the pattern of acute glomerulonephritis (AGN), a disease known to be influenced by socioeconomic and environmental factors, in children 12 years and under, for the years 1971 and 1985. All children admitted to the four major paediatric departments with haematuria and at least two of the following (oedema, hypertension or oliguria) had an initial diagnosis of AGN. A sample population from one unit from 1980 to 1984 showed that over 70% of these children had evidence of a post-streptococcal aetiology. In 1971, 411 children were admitted with AGN, as compared with only 58 in 1985. The age-sex-race standardized rates for 1971 and 1985 were 0.632 and 0.023/1,000 children 12 years and under, respectively (P less than 0.001). The mean age of presentation was lower in 1971. Over this period, Singapore saw a threefold rise in the gross national product, accompanied by rapid urbanization. On analysis of the housing pattern, only 31% of the children lived in high-rise apartments in 1971, in contrast with 86% in 1985 (P less than 0.001). The majority of non-apartment dwellers had homes in rural districts. From an epidemiological perspective, factors which could have led to the highly significant decline in prevalence of AGN in Singapore children included improvement in the socioeconomic status and health care system, and urbanization of the country.

Sonographic screening for renal tract anomalies associated with congenital heart disease.

The incidence of structural renal tract abnormalities in 109 children with documented congenital heart disease (CHD) was studied using real-time sonography. In these children, 11.9% had associated urologic anomalies, which included hydronephrosis, duplication, ectopia, agenesis, and dysplasia of kidneys. Children with associated extracardiac anomalies had a significantly higher incidence of renal tract anomalies (39.1%) compared to those with isolated CHD (4.7%). Therefore, sonographic screening of the renal tract should be performed routinely in patients with CHD, especially in those with multiple congenital defects.

Transferrin subtypes and spontaneous abortion in a Chinese population.

A series of Chinese newborns of consecutive normal vaginal deliveries were investigated for the distribution of serum transferrin subtypes by polyacrylamide gel iso-electric focusing at pH 3.5-9.5. Newborns whose mothers had a history of previous spontaneous abortion (n = 189) had a significantly higher frequency of the C2 variant and the C2 gene compared to those (n = 864) without a history of spontaneous abortion. There was no significant difference in the frequency of transferrin alleles between newborns with normal and low birth weight (n = 147).

Adriamycin-induced cardiomyopathy aggravated by cis-platinum nephrotoxicity requiring dialysis.

Complications of chemotherapy for a synovial sarcoma in an eight-year old boy included cisplatinum nephrotoxicity and adriamycin cardiotoxicity. The resultant renal failure and fluid overload necessitated peritoneal dialysis. Intractable hypomagnesemia was also a prominent feature.