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Aims: To evaluate the benefits and harms of treatments for vesicoureteric reflux in children. Methods: Meta-analyses of randomised controlled trials using a random effects model. Main outcome measures were incidence [...]

Objective: To test the medical belief that the micturating cystourethrogram (MCU) be deferred four to six weeks after acute symptomatic urinary tract infection (UTI) because of the risk of falsely detecting vesicoureteric reflux if performed earlier.Study Design: A cross sectional analytic study of preschool children with first time symptomatic UTI.Results: Of the 284 eligible children, 272 (95.8%) had MCU at a median time of 29 days after diagnosis (range 5 to 167 days). Vesicoureteric reflux was present in 77 children (28.3%). Beyond one week after diagnosis (270 children) the proportion and severity of vesicoureteric reflux detected was not associated with the timing of the MCU. Before one week, both children tested had vesicoureteric reflux.Conclusions: The presence and grade of vesicoureteric reflux is not influenced by the timing of the MCU one week after acute symptomatic UTI. There may be an association between the MCU and the presence of vesicoureteric reflux for children tested within one week after UTI. The MCU need not be deferred for four to six weeks after UTI. [ABSTRACT FROM AUTHOR]

Splendid Unmanned Aerial Vehicle (UAV) applications upshot its enormous use in densely inhabited areas, which is a matter of concern. In such areas, a proper tracking system is required to track an unauthorized/invader drone to ensure safety. With the flexibility of reaching inaccessible places, an Unmanned Aerial Vehicle Mounted Adaptable Radar Antenna Array (UAVMARAA) could be used. In this regard, a Hybrid Unscented Kalman-Continuous Ant Colony Filter (HUK-CACF) is proposed to estimate the position of the invader drone efficiently. Simulation results demonstrate the efficiency and robustness of the proposed filter for tracking system compared to the existing filters in terms of success rate. Further, for various Adaptable Radar Antenna Array (ARAA) patterns such as Uniform Linear Array (ULA), Uniform Rectangular Array (URA), and Uniform Circular Array (UCA), analysis is done for pertaining actual tracking effect for various parameters such as bearing, Doppler shift, ranging, and Radar Cross Section (RCS) by considering wobbling and mutual coupling (MC) effect. The result shows that the proposed filter outperforms in all the scenarios. Among the various ARAA, URA performs better than the other configurations., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 ISA. Published by Elsevier Ltd. All rights reserved.)

Background: Antibiotics are widely administered to children with the intention of preventing urinary tract infection, but adequately powered, placebo-controlled trials regarding efficacy are lacking. This study from four Australian centers examined whether low-dose, continuous oral antibiotic therapy prevents urinary tract infection in predisposed children., Methods: We randomly assigned children under the age of 18 years who had had one or more microbiologically proven urinary tract infections to receive either daily trimethoprim-sulfamethoxazole suspension (as 2 mg of trimethoprim plus 10 mg of sulfamethoxazole per kilogram of body weight) or placebo for 12 months. The primary outcome was microbiologically confirmed symptomatic urinary tract infection. Intention-to-treat analyses were performed with the use of time-to-event data., Results: From December 1998 to March 2007, a total of 576 children (of 780 planned) underwent randomization. The median age at entry was 14 months; 64% of the patients were girls, 42% had known vesicoureteral reflux (at least grade III in 53% of these patients), and 71% were enrolled after the first diagnosis of urinary tract infection. During the study, urinary tract infection developed in 36 of 288 patients (13%) in the group receiving trimethoprim-sulfamethoxazole (antibiotic group) and in 55 of 288 patients (19%) in the placebo group (hazard ratio in the antibiotic group, 0.61; 95% confidence interval, 0.40 to 0.93; P = 0.02 by the log-rank test). In the antibiotic group, the reduction in the absolute risk of urinary tract infection (6 percentage points) appeared to be consistent across all subgroups of patients (P > or = 0.20 for all interactions)., Conclusions: Long-term, low-dose trimethoprim-sulfamethoxazole was associated with a decreased number of urinary tract infections in predisposed children. The treatment effect appeared to be consistent but modest across subgroups. (Australian New Zealand Clinical Trials Registry number, ACTRN12608000470392.), (2009 Massachusetts Medical Society)

Objective: To describe the natural history and risk of early chronic kidney disease (CKD) in Indigenous Australian populations., Design, Setting and Participants: A prospective cohort of 2266 Aboriginal and non-Aboriginal children enrolled from primary schools throughout New South Wales from February 2002 to June 2004 and followed for 4 years., Main Outcome Measures: Urinalysis, height, weight, blood pressure, birthweight and sociodemographic status at baseline and 2- and 4-year follow-up; CKD risk factors: haematuria, albuminuria, obesity, and systolic and diastolic hypertension., Results: 2266 children (55% Aboriginal; 51% male; mean age, 8.9 years [SD, 2.0 years]) were enrolled at baseline. 1432 children (63%) were retested at 2-year follow-up, and 1506 children (67%) at 4-year follow-up. Prevalence of baseline CKD risk factors was frequent (2%-7%), but most abnormalities were transient. Besides persistent obesity (5.0%), persistence of CKD risk factors at final follow-up was low: haematuria (1.9%), albuminuria (2.4%), systolic hypertension (1.5%) and diastolic hypertension (0.2%). There was no difference in prevalence of persistent CKD risk factors between Aboriginal and non-Aboriginal children., Conclusions: Over 4 years of follow-up, Indigenous Australian children had no increased risk for early evidence of CKD. More than 70% of baseline risk factors were transient, and persistent risk factors were uncommon. Our findings suggest the increased risk for end-stage kidney disease seen in Indigenous adults is not yet manifest in these schoolchildren, and may be potentially preventable.

Albuminuria predicts cardiovascular and end-stage kidney disease in indigenous populations. Early detection in indigenous children may identify those who could benefit from early treatment. Community-based detection of albuminuria needs to be performed using a reliable, inexpensive, and widely available test, such as a proteinuria dipstick. Dipstick accuracy for detecting albuminuria in a community setting has not been evaluated. We assessed the accuracy of Multistix 10 SG dipsticks to detect baseline albuminuria and predict for persistent albuminuria at a 2-year follow-up in a population-based cohort of Australian Aboriginal and non-Aboriginal elementary-school-aged children. Variability in the accuracy of dipsticks in subgroups of higher risk children was analyzed using the relative diagnostic odds ratio (RDOR). Using Multistix 10 SG dipsticks, index-test-positive cases were defined as >/=0.30 g/L (1+) proteinuria and index-test-negative cases as <0.30 g/L (negative or trace) proteinuria. Referent-test-positive cases were defined as spot albumin:creatinine (ACR) >/=3.4 mg/mmol, and referent-test-negative cases as ACR <3.4 mg/mmol. There were 2,266 children (55.1% Aboriginal, 51.0% boys, mean age 8.9 years) enrolled. At the 2-year follow-up, 1,432 (63.0%) children were retested (54.0% Aboriginal, 50.5% boys, mean age 10.5 years). Prevalence of baseline albuminuria was 7.3%, and persistent albuminuria was 1.5%. Dipsticks had a sensitivity of 62% and specificity of 97% at baseline. In predicting persistent albuminuria, sensitivity was 75% and specificity 93%. Accuracy did not vary with ethnicity, gender, or body mass index. Accuracy was less in younger children (4.0-7.9 years), and in those with hematuria. The performance characteristics of Multistix dipsticks make them suitable for albuminuria detection in Aboriginal and other higher-risk groups of children. More than two thirds of children detected at a single test will have transient rather than persistent albuminuria. Multistix dipsticks are particularly useful for detecting children who will have persistent albuminuria.

Background: Aboriginal Australians have a 9-fold increased risk of end-stage renal disease. There is no information about the natural history and risk of chronic kidney disease (CKD) in Aboriginal and non-Aboriginal children., Study Design: Using a prospective study design, we aimed to determine the prevalence of persistent markers and risk factors for CKD in Australian Aboriginal and non-Aboriginal children and whether Aboriginal children are at increased risk of persistent markers of CKD after accounting for sociodemographic differences., Setting & Participants: Children were enrolled from elementary schools throughout New South Wales., Predictor: Aboriginal (Aboriginal and Torres Strait Islander Australians) versus non-Aboriginal ethnicity., Outcomes & Measurements: Urine analysis, height, weight, blood pressure, birth weight, and sociodemographic status were measured at baseline and 2-year follow-up. Albuminuria was defined as albumin-creatinine ratio of 3.4 mg/mmol or greater, hematuria as 25 or greater red blood cells/microL (>or=1+), obesity as body mass index of 2 SDs or greater, and systolic and diastolic hypertension as blood pressure greater than the 90th percentile., Results: 2,266 children (55.1% Aboriginal; 51.0% boys; mean age, 8.9 +/- 2.0 years [SD] years) were enrolled at baseline. Early markers and predictors of CKD at baseline were frequent: hematuria (5.5%), albuminuria (7.3%), obesity (7.1%), systolic hypertension (7.2%), and diastolic hypertension (5.8%). 1,432 children (63%) were available for retesting at 2-year follow-up (54.0% Aboriginal; 50.5% boys; mean age, 10.5 +/- 2.0 years). Persistent obesity (5.3%) was frequent, but persistent markers of CKD were infrequent (systolic hypertension, 1.1%; diastolic hypertension, 0.2%; hematuria, 1.1%; and albuminuria, 1.5%). Although there were more Aboriginal than non-Aboriginal children with baseline hematuria (7.1% versus 3.6%; P = 0.001), after adjustment for age, sex, birth weight, and sociodemographic status, there was no increased risk of persistent hematuria, albuminuria, obesity, or hypertension in Aboriginal children., Limitations: Persistent markers of CKD were much less frequent than anticipated, which may have affected study power. The group lost at follow-up was older children, which may have biased results., Conclusions: Overall, only 20% of children found to have markers of early CKD had persistent abnormalities (diastolic and systolic hypertension, albuminuria, and hematuria) 2 years later, equivalent to a population point prevalence of 1% to 2% in children with a mean age of 10 years. Aboriginal children had greater rates of baseline and transient hematuria, but no increased risk of persistent markers of CKD, suggesting that adolescence and young adulthood is a critical time for preventative strategies.

Aim: Indigenous people have a two- to tenfold increased risk of premature death from cardiovascular disease. We aimed to determine whether some key risk factors for cardiovascular disease occur more commonly in Aboriginal than non-Aboriginal Australian children., Methods: Children were enrolled from primary schools throughout New South Wales, the state with the highest number of Aboriginal people. Associations between ethnicity, gender, birthweight, socio-demographic status and hypertension, obesity, baseline and persistent albuminuria were determined., Results: A total of 2266 children (55% Aboriginal) were enrolled. Mean age was 8.9 years (+/-3.8 years). Obesity (body mass index >or=2 standard deviations) was detected in 7.1%, systolic hypertension (blood pressure >90th percentile) in 7.2%, diastolic hypertension in 5.9%, baseline albuminuria (albumin : creatinine >or=3.4 mg/mmol) in 7.3% and persistent albuminuria in 1.5% with no differences between Aboriginal and non-Aboriginal children. Hypertension was less common with increasing social disadvantage (trend P < 0.02). Increasing body mass index standard deviation was strongly associated with systolic and diastolic hypertension (both P < 0.0001)., Conclusions: Many risk factors for cardiovascular disease are already common in young children but not more prevalent in Aboriginal than in non-Aboriginal children. In all children, overweight and obesity have the strongest association with hypertension, but social disadvantage appears protective for hypertension. Our findings suggest that risk for cardiovascular health disparities seen in indigenous adults manifests beyond childhood and that a window of opportunity exists to prevent some of these outcomes.

Indigenous people suffer substantially more end-stage kidney disease (ESKD), especially Australian Aboriginals. Previous work suggests causal pathways beginning early in life. No studies have shown the prevalence of early markers of chronic kidney disease (CKD) in both Indigenous and non-Indigenous children or the association with environmental health determinants--geographic remoteness and socioeconomic disadvantage. Height, weight, blood pressure, and urinary abnormalities were measured in age- and gender-matched Aboriginal and non-Aboriginal children from elementary schools across diverse areas of New South Wales, Australia. Hematuria was defined as>or=25 red blood cells/microl (>or=1+), proteinuria>or=0.30 g/l (>or=1+), and albuminuria (by albumin:creatinine)>or=3.4 mg/mmol. Remoteness and socioeconomic status were assigned using the Accessibility and Remoteness Index of Australia and Socio-Economic Indexes For Areas. From 2002 to 2004, 2266 children (55% Aboriginal, mean age 8.9 years) were enrolled from 37 elementary schools. Overall prevalence of hematuria was 5.5%, proteinuria 7.3%, and albuminuria 7.3%. Only baseline hematuria was more common in Aboriginal children (7.1 versus 3.6%; P=0.002). At 2-year follow-up, 1.2% of Aboriginal children had persistent hematuria that was no different from non-Aboriginal children (P=0.60). Socioeconomic disadvantage and geographical isolation were neither significant nor consistent risk factors for any marker of CKD. Aboriginal children have no increase in albuminuria, proteinuria, or persistent hematuria, which are more important markers for CKD. This suggests ESKD in Aboriginal people may be preventable during early adult life.

Background: Vesicoureteric reflux (VUR) results in urine passing, in a retrograde manner, up the ureter. Urinary tract infections (UTIs) have been considered to be the main cause of permanent renal parenchymal damage in children with reflux. Therefore management of these children has been directed at preventing infection by antibiotic prophylaxis and/or surgical correction of reflux. However controversy remains as to the optimum strategies for management of children with primary VUR., Objectives: To evaluate the benefits and harms of the different treatment options for primary VUR., Search Strategy: Published and unpublished randomised controlled trials (RCTs) were identified from the Cochrane Central Register of Controlled Trials, MEDLINE, EMBASE, reference lists of articles and abstracts from conference proceedings., Selection Criteria: RCTs were included if they compared any treatments of VUR including surgery (open and closed techniques), antibiotic prophylaxis of any duration, non-invasive techniques such as bladder training and any combination of therapies., Data Collection and Analysis: Two reviewers independently searched the literature, determined trial eligibility, assessed quality, extracted and entered data. For dichotomous outcomes, results were expressed as relative risk (RR) and 95% confidence intervals (CI). Data were pooled using the random effects model., Main Results: Ten trials involving 964 evaluable children comparing long-term antibiotics and surgical correction of VUR with antibiotics (seven trials), antibiotics with no treatment (one trial) and different materials for endoscopic correction of VUR (two trials) were identified. Risk of UTI by 1-2 and 5 years was not significantly different between surgical and medical groups (by 2 years RR 1.07, 95% CI 0.55 to 2.09; by 5 years RR 0.99; 95% CI 0.79 to 1.26). Combined treatment resulted in a 60% reduction in febrile UTI by 5 years (RR 0.43, 95% CI 0.27 to 0.70) but no concomitant significant reduction in risk of new or progressive renal damage at 5 years (RR 1.05, 95% CI 0.85 to 1.29). In one small study no significant differences in risk for UTI or renal damage were found between antibiotic prophylaxis and no treatment., Reviewers' Conclusions: It is uncertain whether the identification and treatment of children with VUR confers clinically important benefit. The additional benefit of surgery over antibiotics alone is small at best. Assuming a UTI rate of 20% for children with VUR on antibiotics for five years, nine reimplantations would be required to prevent one febrile UTI, with no reduction in the number of children developing any UTI or renal damage.

Purpose: We developed and tested the reliability of a new, structured, parent administered questionnaire to determine the prevalence of and risk factors for daytime urinary incontinence in children., Materials and Methods: A new questionnaire was developed and evaluated in a pilot study for ease of understanding and acceptability. It was then tested for reproducibility of responses in a randomly selected sample of new primary school entrants in Western Sydney 4 weeks after baseline data were collected. The questionnaire obtained data on demographics, prevalence of daytime incontinence, family history of incontinence, voiding symptoms and socioeconomic status. Categorical data agreement was assessed using the kappa statistic and continuous data agreement was analyzed using the Bland-Altman method., Results: A total of 166 subjects 3.5 to 7 years old (mean and median ages 5.6 and 5.7, respectively) completed the repeat questionnaire with a 78.5% response rate. Mean agreement of the responses to the first and second questionnaires was 91% (range 83% to 100%, mean kappa = 0.70, range 0.34 to 1.00). For continuous data the 95% confidence limits were narrow (0.3 for birth weight data)., Conclusions: We have developed a new daytime urinary incontinence questionnaire using parent reported data and demonstrated that it is reproducible. We consider it to be a useful instrument for ascertaining information on urinary incontinence and other voiding symptoms.

Objectives: To determine the prevalence and severity of, and risk factors for, daytime urinary incontinence in children starting primary school., Design and Setting: Population-based cross-sectional survey of new entrant primary school children in Sydney, Australia., Methods: A random cluster sample of 2020 primary school children was surveyed by using a daytime incontinence questionnaire with known substantial repeatability (mean kappa = 0.70)., Results: The questionnaire was returned for 1419 (70%) children with a mean age of 5.9 years; 16.5% of children had experienced one or more episodes of wetting in the last 6 months (mild), 2.0% had wet twice or more per week (moderate), and 0.7% were wet every day (severe) (overall prevalence of 19.2%). On multivariate analysis, recent emotional stress (odds ratio 5.7), a history of daytime wetting along the paternal line (odds ratio 9.3), and a history of wetting among male siblings (odds ratio 5.3) were independent risk factors for moderate to severe daytime wetting. Expressed as population attributable risk, 59% and 28% of moderate-severe and mild daytime wetting, respectively, can be attributed to these 3 factors. Only 16% of families with affected children had sought medical help., Conclusions: Daytime urinary incontinence in the first year of primary school is more common than previously reported, and only a small proportion of affected children seek medical help. Emotional stress and family history are likely to be major causal factors.

Unlabelled: Technetium-99m-dimercaptosuccinic acid (DMSA) scintigraphy is a frequently used diagnostic test in pediatric practice to assess the presence and severity of renal damage. Most commonly it is performed after urinary tract infection. The aim of this study was to investigate the variability in the interpretation of DMSA scans by pediatric nuclear medicine physicians in this clinical setting., Methods: We selected all 441 scans from children with first-time urinary tract infection who presented between 1993 and 1995 to a pediatric casualty department and who are participants in a prospective cohort study. Two hundred and ninety-four scans were performed at a median time of 7 days after diagnosis, and 147 scans were from children who were free from further infection over a 1-yr follow-up period. Two experienced nuclear medicine physicians independently interpreted the 441 scans according to whether renal damage was present or absent and using the modified 4-level grading system for DMSA abnormality of Goldraich. Apart from being informed that urinary tract infection was the indication for DMSA scintigraphy, no other clinical information was given to the nuclear medicine physicians. The indices of variability used were the percentage of agreement and the kappa statistic. For the grading scale used, both measures were weighted with integers representing the number of categories from perfect agreement. Disagreement was analyzed for children, kidneys and kidney zones., Results: There was agreement in 86% (kappa = 69%) for the normal-abnormal DMSA scan dichotomy, and the weighted agreement was 94% (weighted kappa = 82%) for the grading of abnormality. Disagreement of DMSA scan interpretation of > or =2 grades was present in three cases (0.7%). The same high level of agreement was present for patient, kidney and kidney zone comparisons. Agreement was not influenced by age or timing of scintigraphy after urinary tract infection., Conclusion: Two experienced nuclear medicine physicians showed good agreement in the interpretation of DMSA scintigraphy in children after urinary tract infection and using the grading system of Goldraich.

Objective: To assess whether improving energy intake by tube feeding could prevent growth failure and improve growth rates in children with congenital renal failure., Design: Prospective descriptive study., Setting: Renal Units, Royal Alexandra Hospital for Children, and Westmead Hospitals., Patients: All children with advanced chronic renal disease (glomerular filtration rate < 30 mL/min/1.73 m2) between 1992 and 1994., Intervention: Tube feeding was commenced if height or weight standard deviation score (SDS) was below the normal range (> -2 SDS) or when height SDS was decreasing and oral intake was not meeting energy requirements. Energy requirements were calculated for median weight for chronological age and sex to provide for catch-up growth., Main Outcome Measures: Growth rate was measured by comparing height and weight SDS at the beginning and end of the study period. Normal growth rate is defined as no change in SDS over time, whereas catch-up growth is defined as an increase in SDS over time., Results: Seven children, mean age 0.6 +/- 0.7 years, with advanced renal failure (mean glomerular filtration rate = 17 mL/min/1.73 m2) caused by congenital renal hypoplasia/dysplasia were studied. All subjects were eventually tube fed for a mean time of 18. 6 +/- 4.5 months. There was no significant change in height SDS (-0. 9 to -1.1) or weight SDS (-0.4 to -0.2)., Conclusion: Optimizing nutritional intake by tube feeding children with advanced chronic renal failure from an early age resulted in no decline in growth rate; however, catch-up growth was not achieved.

Variability in the interpretation of micturating cystourethrography by paediatric radiologists for the diagnosis of vesicoureteric reflux in children was evaluated. All 265 micturating cystourethrograms (MCUs) that were available from 304 consecutive children aged 0.5-61 months-who were investigated after their first urine infection between 1993 and 1995 as part of a prospective cohort study-were selected for interpretation. Three experienced paediatric radiologists from the same department independently interpreted the MCUs according to the grading system of the International Reflux Study in Children, from grades 0 to V, with the presence of intrarenal reflux also noted. Apart from being informed that urine infection was the indication for the MCU, no other clinical information was given to the radiologists. The indices of variability used were the percentage of agreement and the kappa statistic, expressed as a percentage. Both measures were weighted with integers representing the number of categories from perfect agreement. Disagreement was analysed for children and kidneys. For the diagnosis of vesicoureteric reflux in individual patients, including grade, the percentage of agreement was 96%-97% (kappa 90%-91%) and the weighted percentage of agreement was 96%-98% (weighted kappa 93%-94%). The same high level of agreement was present for individual kidneys, with a percentage of agreement of 97%-98% (kappa 89%-92%) and a weighted percentage of agreement of 98%-99% (kappa 94%-95%). There was near perfect agreement in the interpretation of radiological micturating cystourethrography among three experienced paediatric radiologists for the diagnosis and grade of vesicoureteric reflux. Any variations in the medical care of children suspected of having vesicoureteric reflux are not explained by differences in the reporting of this diagnostic test.

Neonatal Bartter syndrome differs from the classical Bartter syndrome in the occurrence of antenatal presentation with polyhydramnios. Nephrocalcinosis and severe growth retardation are common sequelae. Indomethacin has been reported to improve linear growth, but its use in the early newborn period has been infrequently described. In this paper we report normal growth and development and the absence of nephrocalcinosis in an infant now aged 19 months with neonatal Bartter syndrome treated from day 3 of life with indomethacin. With early diagnosis and treatment with indomethacin plus adequate water, calories, and sodium, normal growth can be achieved and nephrocalcinosis may be prevented in children with neonatal Bartter syndrome.

Objective: To determine whether circumcision decreases the risk of symptomatic urinary tract infection (UTI) in boys less than 5 years of age., Study Design: A case-control study (1993 to 1995) in the setting of a large ambulatory pediatric service. Case subjects and control subjects were drawn from the same population. One hundred forty-four boys less than 5 years of age (median age, 5.8 months) who had a microbiologically proven symptomatic UTI (case subjects), were compared with 742 boys (median age, 21.0 months) who did not have a UTI (control subjects). The proportion of case and control subjects who were circumcised in each group was compared with the use of the chi-square test, with the strength of association between circumcision and UTI expressed in terms of an odds ratio. To determine whether age was a confounder or an effect-modifier, we stratified the groups by age (< 1 year; > or = 1 year) and analyzed by the method of Mantel-Haenszel., Results: Of the 144 preschool boys with UTI, 2 (1.4%) were circumcised, compared with 47 (6.3%) of the 742 control subjects (chi-square value = 5.6; p = 0.02; odds ratio, 0.21; 95% confidence intervals, 0.06 to 0.76). There was no evidence that age was a confounder or modified the protective effect of circumcision on the development of UTI (Mantel-Haenszel chi-square value = 6.0; p = 0.01; combined odds ratio, 0.18; 95% confidence intervals, 0.05 to 0.71; Breslow-Day test of homogeneity chi-square value = 0.6; p = 0.4)., Conclusions: Circumcision decreases the risk of symptomatic UTI in preschool boys. The protective effect is independent of age.

We have studied renal biopsies from three groups of patients to determine if alpha beta T cells or gamma delta T cells are present, and whether their presence is correlated with disease progression in IgA nephropathy (IgAN). Group one comprised thin basement membrane disease biopsies (non-immunological control, N = 7); group two were patients with IgAN and stable renal function one year following biopsy (stable, N = 7); and group three were IgAN patients with rapidly declining renal function after one year (progressive, N = 7). Immunohistochemical staining using monoclonal antibodies (CD3, TcR beta, TcR delta) and molecular studies utilizing polymerase chain reaction amplification of cDNA transcribed from biopsy RNA, with primers specific for either the alpha beta TcR or gamma delta TcR, were undertaken. On immunohistochemistry a significant increase in CD3 + cells in progressive biopsies was seen (vs. control P = 0.002, vs. stable P = 0.002). The progressive biopsies infiltrate consisted of both alpha beta TcR (vs. control P = 0.001, vs. stable P = 0.003) and gamma delta TcR cells (vs. control P = 0.01). The RNA study demonstrated an increase in TcR C alpha transcription in the progressive (vs. control P = 0.003) biopsies. Increased TcR C delta transcription was seen in the progressive group (vs. control P = 0.01, vs. stable P = 0.02). We confirm that the presence of lymphocytes in IgAN biopsies predicts progressive disease. While alpha beta T cells are found in both stable and progressive disease, the presence of gamma delta T cells is only associated with progressive IgAN.

The accumulation of toxic metabolites in children with inborn errors of metabolism may cause acute metabolic crises and result in long-term neurological dysfunction or death. Peritoneal dialysis often provides insufficient clearance to protect against these complications, while intermittent haemodialysis cannot prevent reaccumulation of metabolites between dialysis sessions. We describe the use of continuous venovenous haemofiltration (CVVH) or haemodiafiltration (CVVHD) in three infants with maple syrup urine disease (MSUD) and one child with carbamyl phosphate synthetase (CPS) deficiency. All children with MSUD had a satisfactory reduction in branched-chain amino acids within 24 h of onset of haemofiltration, and are now neurologically normal. The child with CPS deficiency had an ammonia level of < 100 mumol/l within 24 h of onset of therapy, but died 3 days later from unrelated cardiovascular complications. Complications of the therapy included the clotting of one haemofilter and the replacement of two vascular access catheters per patient on average per therapy. Two patients required blood transfusion. We report the successful use of CVVH and CVVHD in the acute management of metabolic crises associated with inborn errors of metabolism, and believe that these may be the optimal techniques for the acute clearance of toxic metabolites.

Objective: To directly assess the body protein content of children with chronic renal failure (CRF) and short stature., Setting: A tertiary referral paediatric hospital., Subjects: There were: (i) 17 patients (10 male, nine pre-pubertal; mean age 12.90 +/- 3.20 years) with CRF and height standard deviation (SD) score < -2.00, and (ii) 43 normal children (18 male, 27 pre-pubertal; mean age 10.34 +/- 3.34 years)., Interventions: CRF patients had the following measurements: anthropometry, total body nitrogen (TBN) by neutron capture analysis, 4 day weighed food record and serum albumin levels. Control subjects had TBN and anthropometric measurements only., Results: Although older than the controls, the CRF patients had significantly lower TBN values (645 +/- 265 vs 930 +/- 365 g, P < 0.01). Mean values for TBN and TBN/height (percentages of expected) in the CRF patients were significantly reduced to 54% and 63% respectively, when predicted from age. However, their TBN predicted from height was 100% of expected. %TBN (predicted from age) correlated significantly with height SD score (r = 0.79), weight SD score (r = 0.87), upper arm muscle area percentile (r = 0.62) and serum albumin (r = 0.62). Mean oral energy and protein intakes were 65% and 172% of recommended dietary intake respectively., Conclusions: Children with CRF and short stature are significantly protein-depleted for age although not for height. Chronic energy deficiency may contribute to impaired protein deposition which, in turn, may be important in the pathogenesis of growth failure in CRF.

Insulin-like growth factor-binding protein-3 (IGFBP-3) and insulin-like growth factor-1 (IGF-1) levels were measured by specific radioimmunoassays in children with all degrees of chronic renal failure (CRF). Study group 1 comprised 29 children (10 on dialysis) who had been studied one to four times over 2 years to determine whether IGF-1 and IGFBP-3 levels differed from those in age-matched healthy children and to examine the relationship between these levels and heights. IGF-1 and IGFBP-3 levels did not differ from those in normal children. IGF-1 and IGFBP-3 were significantly correlated, increased with pubertal stage in all children and with age in non-dialysis patients. IGF-1, but not IGFBP-3, correlated with age in dialysis patients. There was no correlation between IGF-1 or IGFBP-3 levels (corrected for age) and height standard deviation score (SDS) in either non-dialysis or dialysis patients. Study group 2 comprised 19 children (7 on dialysis) who were studied prospectively for 1-2 years to examine the relationship between IGF-1 and IGFBP-3 levels, growth rates and nutritional parameters. Mean values of IGF-1 and IGFBP-3 (corrected for age) did not change over 1-year periods, while height SDS fell by -0.38 +/- 0.21 SD/year in dialysis patients and by -0.11 +/- 0.29 SD/year in non-dialysis patients. No significant correlations were found between IGF-1 or IGFBP-3 levels and growth rates or nutritional parameters. Thus growth retardation in children with CRF is not related to circulating levels of IGF-1 or IGFBP-3.

A 10-year-old boy with chyluria due to a congenital fistulous communication between the lymphatic system and the bladder is described. Chyluria can be parasitic or non-parasitic. Many causes of non-parasitic chyluria have been reported. Lymphography is the preoperative imaging procedure of choice since it demonstrates the site, the calibre and the number of the fistulous communications. Lymphoscintigraphy shows very well the site of the fistula but is not as precise as lymphography. However, it has the advantage to be less invasive and is an excellent alternative in the non-surgical cases. The prognosis of non-parasitic chyluria is usually very good and the treatment is mostly conservative.

Patients with apparent mineralocorticoid excess (AME) have low or absent activity of the enzyme 11 beta OH steroid dehydrogenase (11SD), and inappropriately high intrarenal levels of cortisol resulting in Na+ retention and hypertension. Pseudohypoaldosteronism (PHA), in contrast, is characterized by salt wasting despite hyperaldosteronemia, reflecting low or absent mineralocorticoid receptors (MR). Although AME is presumed to reflect inappropriate cortisol occupancy of MR, several features also suggest inappropriate occupancy of glucocorticoid receptors (GR). To test this possibility, we administered carbenoxolone, which is known to block 11SD, to four patients with PHA, and observed marked mineralocorticoid effects, e.g., antinatriuresis and elevated plasma bicarbonate. To further test the possibility that occupancy of renal GR may induce a classical mineralocorticoid response, we administered the highly specific glucocorticoid RU 28362 to adrenalectomized rats and showed that it has profound antinatriuretic effects. Finally, by selectively blocking MR with RU 28318 or GR with RU 38486, we have shown that corticosterone, the physiologic glucocorticoid in rats, has an antinatriuretic effect in adrenalectomized rats via either MR or GR occupancy. Previous studies have clearly shown that MR are inherently nonselective and have equivalent intrinsic affinity for aldosterone, corticosterone, and cortisol. The present studies suggest that this nonselectivity includes the nuclear response element to which either MR or GR may bind to elicit a mineralocorticoid effect, and further underscore the importance of the enzyme 11SD in the specific mineralocorticoid action of aldosterone.

The occurrence of hyperglycaemia and insulin deficiency in a young child receiving peritoneal dialysis during the course of haemolytic uraemic syndrome (HUS) is described. This unusual complication may have been due to microvascular disease involving the pancreas. Plasma glucose should be monitored during HUS, particularly if dialysis with fluids containing high dextrose concentrations is required.

Sixty-five children over one year and under 15 years of age began treatment for end-stage renal failure between 1973 and 1988. Sixty-one renal transplants were performed in 53 children, 39 of these were from living donors (38 were first-degree relatives and one was an emotionally related volunteer). Thirteen children, of whom seven had received transplants and six had not, died, including three children with functioning transplants; nine deaths occurred in the first eight years of the programme. Cumulative five-year and 10-year patient survival rates were 78% and 75%, respectively. Eighteen transplants failed, 12 as a result of rejection, five as a result of disease recurrence and one due to primary non-function. Cumulative five-year and 10-year transplant survival rates for first grafts were 66% and 53%, respectively. For living donor transplants these rates were 85% and 68%, respectively. Growth rates fell by 0.4 +/- 0.05 standard deviation score (SDS) per year in children undergoing dialysis, were normal in children with renal transplants receiving prednisone (change in SDS per year, -0.02 +/- 0.08) and increased by 0.36 +/- 0.07 SDS per year in children with transplants receiving cyclosporin A alone. Currently, 32 (82%) of 39 transplant recipients and 7 (58%) of 12 patients undergoing dialysis attend school or work full time. Although both dialysis and transplantation are acceptable therapies for children with end-stage renal failure, successful transplantation provides the best opportunity for satisfactory growth and development.

Kidney biopsies from ten children with recurrent macroscopic haematuria, showed mesangial deposition of IgG, IgA and complement. Eight of these ten biopsies also showed disposition of properdin, a basic euglobulin intimately involved in the alternate pathway of complement activation. Serum haemolytic complement activity was normal in the eight patients tested. Incubation of the serum at 4 degrees C for 24 dours did not result in any change in complement activity. Theses data suggest that the mesangial inflammatory process in these patients may be medicated in part by the alternate pathway of complement activation and that the mechanism is activated locally.

Height, expressed as standard deviation scores for chronological age and for bone age, was studied in relation to glomerular filtration rate, bone age delay, and bone histology in 47 children with chronic renal disease and GFR less than 80 ml/min/1.73 m2. In multiple regression in all 47 patients, only GFR and bone age delay significantly affected height; 40% of children were short (height standard deviation score less than -2) for chronological age, and 9% were short for bone age. Renal osteodystrophy, which only occurred at GFR less than 30 ml/min/1.73 m2, significantly affected height only in children with congenital renal disease and GFR less than 20 ml/min/1.73 m2. Although radiological and biochemical changes of renal osteodystrophy were seen more often in short children, histological bone disease occurred just as frequently in tall children as in short children. Thus much of the observed height retardation in chronic renal failure is associated with delayed skeletal maturation. In addition, although severe renal osteodystrophy may contribute to growth retardation in advanced renal failure, our data suggest that milder degrees of bone disease evident only on histological study cannot be implicated in the etiology of growth failure in chronic renal impairment.

The complement system was investigated in 34 patients with infectious mononucleosis. Three had specific complications: one haemolytic anaemia, one severe arthralgia/myalgia and one proliferative glomerulonephritis. Complement changes consistent with classical pathway consumption were seen in ten of the uncomplicated group and the patients with haemolytic anaemia and arthralgia/myalgia. The patient with glomerulonephritis showed evidence of alternative pathway utilisation including C3 splitting activity and the deposition of properdin on renal biopsy. The complement findings suggest that circulating immune complexes are common in such patients and are likely to play a role in the pathogenesis of the complications. It is proposed that both complement pathways may be required for the effective clearance of viral material from the circulation.

The complement system was examined in fifty patients with acute, apparently uncomplicated measles; forty-six were children less than 10 years old. Twenty showed evidence of pathological complement activation. In thirteen of these the pattern was consistent with activation of the classical pathway while in the other seven data suggested utilization of an alternative pathway. An additional eleven patients had isolated reduction in Clq without alteration in concentration of other components; these were excluded from the classical pathway group. No patient had detectable immune complexes or C3 splitting activity in serum; however, it is suggested that the abnormal complement patterns observed are likely to indicate the presence of circulating immune complexes in a high percentage of patients with this infection.