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Biomedical subjects

L Rees

Publications and source records attributed to L Rees.

At least 37 records · Page 2Linked to original sources

Children with autism show local precedence in a divided attention task and global precedence in a selective attention task.

Children with a diagnosis of autism and typically developing children were given two variations of the Navon task (Navon, 1977), which required responding to a target that could appear at the global level, the local level, or both levels. In one variation, the divided attention task, no information was given to children regarding the level at which a target would appear on any one trial. In the other, the selective attention task, children were instructed to attend to either the local or the global level. Typically developing children made most errors when the target appeared at the local level whereas children with autism made more errors when the target appeared at the global level in the divided attention task. Both groups of children were quicker to respond to the global target than the local target in the selective attention task. The presence of normal global processing in the children with autism in one task but not in the other is discussed in terms of a deficit in mechanisms that inhibit local information in the absence of overt priming or voluntary selective attention to local information.

Adolescent↗

Normative data stratified by age and education for two measures of verbal fluency: FAS and animal naming.

Normative data stratified by three levels of age (16-59, 60-79, and 80-95 years) and three levels of education (0-8, 9-12, and 13-21 years) are presented for phonemic verbal fluency (FAS) and categorical verbal fluency (Animal Naming). The normative sample, aged 16 to 95 years, consisted of 1,300 cognitively intact individuals who resided in the community. Years of education ranged from 0 to 21. The total number of words in 1 minute for each of the letters F, A, and S was correlated r =.52 with the number of animal names generated in 1 minute. Regression analyses showed that FAS was more sensitive to the effects of education (18.6% of the variance) than age (11.0% of the variance). The opposite relationship occurred for Animal Naming, where age accounted for 23.4% of the variance and education accounted only for 13.6%. Gender accounted for less than 1% of variance for FAS and Animal Naming. The clinical utility of these norms is discussed.

Journal Article↗

Presymptomatic detection of familial juvenile hyperuricaemic nephropathy in children.

We studied 34 apparently healthy children and 2 propositi from kindreds with familial juvenile hyperuricaemic nephropathy (FJHN) - a disorder characterised by early onset, hyperuricaemia, gout, familial renal disease and a similarly low urate clearance relative to glomerular filtration rate (GFR) [fractional excretion of uric acid (FEur) 5.1+/-1.6%] in young men and women. In addition to the propositi, 17 asymptomatic children were hyperuricaemic -- mean plasma urate (368+/-30 micromol/l), twice that of controls (154+/-41 micromol/l). Eight of them had a normal GFR ( > 80 ml/min per 1.73 m2), and 11 renal dysfunction, which was severe in 5. The FEur in the 14 hyperuricaemic children with a GFR > 50 ml/min was 5.0+/-0.5% and in the 5 with a GFR < or =50 ml/min was still low (11.5+/-0.2%) compared with controls (18.4+/-5.1%). The 17 normouricaemic children (185+/-37 micromol/l) had a normal GFR (>80 ml/min) and FEur (14.0+/-5.3%). The results highlight the dominant inheritance, absence of the usual child/adult difference in FEur in FJHN and presence of hyperuricaemia without renal disease in 42% of affected children, but not vice versa. Since early allopurinol treatment may retard progression to end-stage renal failure, screening of all relatives in FJHN kindreds is essential.

Adolescent↗

Effect of enteral feeding on lipid subfractions in children with chronic renal failure.

The anorexia of chronic renal failure (CRF) is frequently managed with enteral feeds using combinations of commercial preparations, glucose polymers and fat emulsions. Such feeds might predispose to atherogenic blood lipid profiles. Our aim, therefore, was to compare the blood lipid profiles of enterally fed and non-enterally fed children. Plasma lipid subfractions were measured in 37 children with CRF managed conservatively and 10 managed with peritoneal dialysis (PD); 10 of the children were tube fed, 5 of whom were on PD. Results were compared between these groups. Overall, triglycerides (TGs, mean +/- SD) were high (2.3 +/- 1.4 mmol/l) and total cholesterol (TC) was at the upper limit of normal (5.2 +/- 1.5 mmol/l). Low-density lipoprotein (LDL), high-density lipoprotein (HDL), apoprotein A1 (apo A1), A2 (apo A2) and B (apo B), and lipoprotein (a) [Lp(a)] were within the normal range. There was an inverse correlation between TGs and glomerular filtration rate (P = 0.0001). There were no differences in the levels of TC, TG, LDL, HDL, apo A1, apo A2 or Lp(a) between tube-fed and non-tube-fed children. We conclude that enteral feeding does not enhance hyperlipidaemia.

Adolescent↗

Catch-up growth occurs after renal transplantation in children of pubertal age.

OBJECTIVE: Assessment of growth after renal transplantation in children of pubertal age by analyzing the annual increment in height standard deviation score (Ht SDS) in all girls > or =10 years and boys > or =11 years of age at the time of transplantation until latest follow-up (minimum 2 years). PATIENTS: A total of 59 grafts were placed in 54 recipients (30 boys) between December 1984 and January 1995. Mean (range) age at transplantation was 13.6 years (10.1 to 17.7 years). Fifty-one percent had congenital renal disease, 36% acquired renal disease, and 13% had hereditary nephropathies. Eighty-seven percent were first grafts; of these, 29% were performed pre-emptively, and 23% were from living related donors. RESULTS: Mean (SD) Ht SDS at transplantation was -1.8 (0.2) and increased significantly thereafter, such that it was -1.6 (0.2) at 1 year, n = 52; -1.5 (0.2) at 2 years, n = 47; -1.0 (0.2) at 3 years, n = 27; -0.7 (0.3) at 4 years, n = 19; and -0.6 (0.3), n = 13, at 5 years after transplantation (analysis of variance, P < .001). The greatest improvement in Ht SDS in the first year was seen in children with the highest glomerular filtration rate (r = 0.429, P = .002) and in those who were shortest at the time of transplantation (r = -0.356, P = .009). CONCLUSION: Catch-up growth occurs in children receiving renal transplants during the expected time of puberty.

Adolescent↗

Combined liver-kidney transplantation in methylmalonic acidemia.

A 13-year-old boy with non-B12-responsive methylmalonic acidemia (MMA) had chronic renal failure. Hemodialysis led to symptomatic and biochemical improvement. He subsequently received a combined liver-kidney transplant. After 16 months of follow-up he has a normal lifestyle and a marked reduction in plasma and urine methylmalonate.

Adolescent↗

High incidence of bacteriuria following renal transplantation in children.

BACKGROUND: Bacteriuria is common post-transplant. However, most studies are in adults with a short follow-up. We have assessed the incidence of bacteriuria, predisposing causes and its effect on short and long-term graft function in children. METHODS: The notes of 142 children (67% male) who received 168 kidney transplants (138 cadaveric) between 1987 and 1994 were studied. The mean age at transplantation was 9.0 +/- 4.5 years, and 32 children were transplanted pre-emptively. Diagnoses reflected those found in any children's renal failure programme. RESULTS: Two hundred and thirty one episodes of bacteriuria were detected in 66 patients patients (46%): a rate of one episode per 23 patient months of follow-up. Fifty two percent were during the first year, and 29% of these during the first 4 weeks post-transplant. Forty two children (28%) had recurrences. The incidence was not affected by sex, vesico-ureteric reflux into native kidneys, donor source, circumcision in boys, dialysis pre-transplant or acute rejection. Bacteriuria was significantly more common in patients with a history of bacteriuria before transplant (P < 0.005) and with bladder pathology (P < 0.001). Organisms were predominantly coliforms (41%); 70% were Gram-negative. Sixty percent were resistant to the prescribed antibiotic prophylaxis. There was an associated transient rise in plasma creatinine concentration: mean pre-episode 111 +/- 86 mumol/l vs mean post-episode 134 +/- 108 mumol/l (P < 0.0001). Seventy two percent of episodes were asymptomatic, but even in this group 81% had an associated rise in plasma creatinine (P < 0.001). Despite this, there was no significant decrease in glomerular filtration rate in patients with bacteriuria compared with patients without at the end of follow-up: 50 vs 56 ml/min/1.73 m2 respectively. CONCLUSION: Bacteriuria is common post-transplant, occurring most often in those with bladder pathology or with a history of bacteriuria pre-transplant.

Adolescent↗

Randomised controlled trial of recombinant human growth hormone in prepubertal and pubertal renal transplant recipients. British Association for Pediatric Nephrology.

AIMS: To evaluate the efficacy (height velocity (HV), change in height standard deviation score (delta HSDS)), and safety (glomerular filtration rate (GFR), incidence of rejection, and calcium and glucose metabolism) of recombinant human growth hormone (rhGH) treatment after renal transplantation. DESIGN: A two year randomised controlled trial. SUBJECTS: Fifteen prepubertal and seven pubertal children: mean (SD) age, 13.0 (2.6) and 15.2 (2.4) years, respectively; mean (SD) GFR, 51 (30) and 48 (17) ml/min/1.73 m2, respectively. Six prepubertal and three pubertal children were controls during the first year; all received rhGH in the second year. RESULTS: In the first year, mean (SE) HV and delta HSDS in the prepubertal treated group increased compared with controls: 8.1 (0.9) v 3.7 (0.6) cm/year and 0.6 (0.1) v -0.3 (0.2), respectively. In the pubertal treated group, mean (SE) HV and delta HSDS were also greater: 10.1 (0.6) v 3.9 (1.3) cm/year and 0.6 (0.1) v -0.1 (0.2), respectively. Comparing all treated and control children, there was no significant change in GFR: treated group, mean (SE) 9.9 (5.4) ml/min/1.73 m2 v control group, -1.6 (7.6) ml/min/1.73 m2. There were also no differences in the incidence of rejection in the first year: eight episodes in 13 patients v five episodes in nine patients, respectively. Phosphate, alkaline phosphatase (ALP), parathyroid hormone (PTH), and fasting insulin concentrations rose during the first year of treatment, but not thereafter. In the second year of treatment, HV remained above baseline. CONCLUSION: Treatment with rhGH improves growth in prepubertal and pubertal children with renal transplants, with no significant change in GFR or the incidence of rejection. Phosphate, ALP, PTH, and insulin increased during the first year of treatment.

Adolescent↗

The frequency and distribution of spontaneous attention shifts between social and nonsocial stimuli in autistic, typically developing, and nonautistic developmentally delayed infants.

Spontaneous shifts of attention were observed in autistic, typically developing, and nonautistic developmentally delayed infants. Three types of attention shifting behaviour were observed; (1) between an object and another object, (2) between an object and a person, and (3) between a person and another person. The two control groups shifted attention more frequently between an object and a person than between an object and another object or between a person and another person. The infants with autism showed a different pattern, shifting attention between an object and another object more than any other type of shift. Furthermore, infants with autism showed fewer shifts of attention between an object and a person, and between person and person, than did the two control groups. They also spent less time overall looking at people and looked more briefly at people and for longer durations at objects, compared to the two control groups. These results indicate an abnormality in social orientation in autism even at the early age of 20 months.

Attention↗

Retransplantation after post-transplant lymphoproliferative disease.

We present a boy who developed post-transplant lymphoproliferative disease (PTLD) 3.5 months after a first kidney transplant. The diagnosis was made after histopathological examination of the renal graft which was removed because of Pseudomonas aeruginosa septicaemia. After 2 years on dialysis, the patient received a second renal transplant. This graft continues to function after 5 years and there has been no evidence of recurrence of PTLD. This suggests that retransplantation can be undertaken in patients who have recovered from PTLD in a previous graft.

Adolescent↗

Compliance with growth hormone therapy in chronic renal failure and post transplant.

An anonymous questionnaire to investigate non-compliance with recombinant human growth hormone (rhGH), its causes and correlation with growth response, was sent to 60 children with renal disease; 48% returned the questionnaires; 69% reported that they were concerned about their size and 83% reported being treated as younger than they were. Understanding of the purpose and administration of rhGH was good; 62% missed some rhGH injections, but there was no correlation with understanding or difficulty with the injections, concern regarding size or growth response.

Adolescent↗

Physiology and biochemistry of endothelial function in children with chronic renal failure.

Premature atherosclerosis is a major cause of morbidity and mortality in chronic renal failure (CRF). Endothelial dysfunction is a key early event in atherogenesis. The aim of this study was to assess the effect of CRF on endothelial function using physiological and biochemical measures. To focus on the effect of CRF itself, 23 children (matched with 23 controls for age and vessel diameter) were selected because they were normotensive, had normal total cholesterol (TC) levels, and were not on vasoactive drugs. Their mean (range) age was 12.0 (7.8 to 17.0) years; GFR 17.5 (8.8 to 34.5) ml/min/1.73 m2. The physiology of endothelial function in the brachial artery was assessed using high resolution ultrasound by measuring its diameter at rest, during reactive hyperemia (endothelium dependent dilation) and after sublingual glyceryl trinitrate (GTN; endothelium independent dilation). Nitric oxide (NO) metabolites and endogenous NO synthetase (eNOS) inhibitors were measured as an assessment of endothelial metabolism. Brachial artery dilation to flow [FMD, mean (SEM)%] was reduced in CRF to 4.9 (0.6) and controls 8.6 (0.6), P < 0.0001. In contrast, the response to GTN was similar in both groups: CRF 25.1 (1.6), controls 23.3 (1.2), P = 0.31. There was no difference in TC, low density lipoprotein (LDL) or high density lipoprotein (HDL) between the patients and the controls. Triglycerides (TG) were higher in the patients but within the normal range. Antibodies against oxidized LDL (ox-LDL) were high in CRF. Endogenous NOS inhibitors were high in CRF, and intermediate NO metabolites were low. There was no correlation between FMD of the brachial artery and lipid subfractions, or with NO metabolites or eNOS inhibitors. Endothelium dependent dilation of the brachial artery is impaired in children with CRF who do not have co-existing risk factors for atherosclerosis. This may represent early evidence of atherogenic vascular disease.

Adolescent↗

Quantity of cytomegalovirus viruria is a major risk factor for cytomegalovirus disease after renal transplantation.

Studies have shown that risk factors for human cytomegalovirus (HCMV) disease after renal transplant include primary infection (virus of donor origin infecting a non-immune individual), re-infection (virus of donor origin infecting a immune individual), and the detection of viraemia (as a marker of virus dissemination). We now report that viral load in the urine is also a significant factor in HCMV disease and is one of the main mechanisms underlying the risk associated with viraemia and donor serostatus. Longitudinal analysis of a group of 196 renal recipient identified 35 recipients who were PCR positive for HCMV in urine. Elevated viral loads were present in symptomatic patients, viraemic patients, and patients experiencing primary HCMV infection. Disease was associated with the peak quantity of virus present in the urine during the post-transplant period (P = 0.0001), with viraemia (P = 0.0003), and with transplantation of a seropositive donor (P = 0.03). Univariate logistic regression analysis showed that increases of 0.25 log10 in viral load were associated with a 179% increased risk of disease (odds ratio = 2.79; 95% C.I. 1.22-6.39; P = 0.02). This effect persisted in a multivariate logistic analysis when viraemia was incorporated (odds ratio = 2.77; 95% C.I. 1.07-7.18; P = 0.04). In contrast, the significant association between viraemia and disease observed in univariate analysis (odds ratio = 23.75; 95% C.I. 3.69-152.90; P = 0.0009) became marginally non-significant in multivariate analysis once viral load had been controlled for (odds ratio = 34.54; 95% C.I. 0.75-1599.00; P = 0.07). The computed probability of disease showed that a rapid transition occurred at viral loads between 10(5.7) and 10(6.5) genomes/ml urine in non-viraemic patients compared to viral loads between 10(5.0) and 10(5.7) genomes/ml urine in patients with concurrent viraemia. The implications of these findings for understanding HCMV pathogenesis, improving patient management, and optimising trials of antiviral treatment are discussed.

Adolescent↗

Factors influencing the response to growth hormone in children with renal disease.

The effects of age, height velocity over the preceding year, glomerular filtration rate (GFR) and prednisolone dose on growth response have been assessed by single and multiple linear regression analysis in 23 prepubertal children [age, mean (SD), 8.2 (2.5) years] with chronic renal failure (CRF) and 16 prepubertal children [12.1 (2.3) years] with renal transplants treated for 1 year with recombinant human growth hormone (rhGH), 30 U/m2 per week. Height velocity [mean (SD), cm/year] increased from 4.7 (1.3) to 9.7 (2.1) (P < 0.0001) in the CRF group and 3.1 (1.6) to 7.3 (2.8) (P < 0.0001) in the transplant group. In the CRF group, there was a correlation between age and height velocity, both in the pretreatment year (r = -0.755, P < 0.0001) and during treatment (r = -0.421, P = 0.045). There was no correlation between pretreatment height velocity or GFR and response to rhGH. In the transplanted children height velocity during the treatment year correlated with age (r = -0.647, P = 0.007), prednisolone dose (r = -0.689, P = 0.003), GFR (r = 0.542, P = 0.030) and pretreatment height velocity (r = 0.655, P = 0.006). Multiple regression analysis showed prednisolone dose and age to be the most important predictors of response.

Anti-Inflammatory Agents↗

Psychological and clinical effects of renal biopsy performed using sedation.

A sedation regimen using sequential oral trinepazine, intravenous Pethco (pethidine, chlorpromazine and promethazine) and diazemuls was evaluated in children having native kidney (n = 17) and transplant kidney (n = 17) biopsies. Biopsy was successful in all cases, with no serious side effects. A self-reported scale of memory recall and pain perception showed the optimal time for biopsy to be between 30 and 90 min after the intravenous Pethco. The child's level of distress was measured by a self-reported scale, a parent-reported scale and an observational scale for doctors and nurses; 45% of children rated themselves highly distressed prior to the procedure, their parents being the best assessors of this distress. Younger children and those undergoing native kidney biopsy had less understanding of the procedure. Children's worries could be clearly categorised into procedural and outcome issues: those undergoing transplant biopsy were more worried about outcome, whereas those undergoing native kidney biopsy were more worried about the procedure.

Adolescent↗