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Biomedical subjects

R S Trompeter

Publications and source records attributed to R S Trompeter.

At least 19 recordsLinked to original sources
PubMed2006

Perinatal renal venous thrombosis: presenting renal length predicts outcome.

BACKGROUND: Renal venous thrombosis (RVT) is the most common form of venous thrombosis in neonates, causing both acute and long term kidney dysfunction. Historical predisposing factors include dehydration, maternal diabetes, and umbilical catheters, but recent reports highlight associations with prothrombotic abnormalities. STUDY: Twenty three patients with neonatal RVT were analysed over 15 years. Predisposing factors, presentation, and procoagulant status were compared with renal outcome using multilevel modelling. RESULTS: Median presentation was on day 1: 19/23 (83%) had pre/perinatal problems, including fetal distress (14), intrauterine growth retardation (five), and pre-identified renal abnormalities (two); 8/18 (44%) had procoagulant abnormalities, particularly factor V Leiden mutations (4/18). Long term abnormalities were detected in 28/34 (82%) affected kidneys; mean glomerular filtration rate was 93.6 versus 70.2 ml/min/1.73 m2 in unilateral versus bilateral cases (difference 23.4; 95% confidence interval 6.4 to 40.4; p = 0.01). No correlation was observed between procoagulant tendencies and outcome, but presenting renal length had a significant negative correlation: mean fall in estimated single kidney glomerular filtration rate was 3 ml/min/1.73 m2 (95% confidence interval 3.7 to -2.2; p = 0.001) per 1 mm increase, and kidneys larger than 6 cm at presentation never had a normal outcome. CONCLUSIONS: This subgroup of neonatal RVT would be better termed perinatal RVT to reflect antenatal and birth related antecedents. Prothrombotic defects should be considered in all patients with perinatal RVT. Kidney length at presentation correlated negatively with renal outcome. The latter, novel observation raises the question of whether larger organs should be treated more aggressively in future.

Blood Coagulation Disorders, Inherited↗
PubMed2004

Experience with cyclosporine in pediatric renal transplantation.

Three decades ago renal transplantation had become the accepted therapy for end-stage renal disease in children. Cyclosporine (CsA) was introduced into the majority of clinical immunosuppressive protocols in the 1980s and attained a vital place in the armamentarium of antirejection drugs for children. However, CsA therapy is not without adverse effects, notably posttransplant hypertension, hyperlipidemia, and nephrotoxicity. The cosmetic side effects of CsA, principally hirsutism and gum hyperplasia, are significant and very important to children particularly when drug compliance issues are vital to achieve success. The central role CsA has played to date in the development of successful treatment protocols for children undergoing renal transplantation is explored, including the importance of therapeutic drug level monitoring to optimize clinical outcomes.

Age Factors↗
PubMed2004

Oral health and caries related microflora in children during the first three months following renal transplantation.

UNLABELLED: There is little information on the oral health of children undergoing renal transplantation during the early transplant period. METHODS: Twenty-four children undergoing renal transplantation aged 4-13.2 years and their matched controls were recruited. The dmfs, dmft, DMFS and DMFT, plaque, gingivitis and gingival enlargement scores were recorded. The oral microflora was sampled and cultured for S. mutans, Lactobacllus species and Candida species. RESULTS: There was a significantly lower mean dmfs (0.3 +/- 0.9; P = 0.03), dmft (0.3 +/- 0.9; P = 0.03), DMFS (2.3 +/- 5.3; P = 0.01) and DMFT (1.5 +/- 2.6; P = 0.02), respectively, in the transplant group. There was a significantly greater mean plaque score (14.7 +/- 11) for the permanent dentition, at baseline only, compared with 90 days post-transplantation (9.4 +/- 10.4; P = 0.02). There was a significantly greater gingival enlargement score (1.8 +/- 1.4; P = 0.04) 90 days post-transplantation compared with baseline. The S. mutans and Lactobacillus counts were significantly lower both at baseline (P = 0.0001 and P = 0.004) and 90 days post-transplantation (P = 0.02; and P = 0.05), respectively, compared with the controls. CONCLUSIONS: The transplant children had less active dental disease than the controls although gingival enlargement needs careful monitoring.

Adolescent↗
PubMed2003

Risk of relapse after meningococcal C conjugate vaccine in nephrotic syndrome.

In November, 1999, all children under age 18 years in the UK were offered immunisation with the newly introduced meningococcal C conjugate vaccine (MCCV). In a cohort of 106 patients with nephrotic syndrome, there were 63 relapses during the 12 months before vaccination, and 96 during the equivalent period postvaccination (p=0.009). The relapse rate of nephrotic syndrome increased significantly after administration of MCCV. Whether to vaccinate such children needs to be carefully considered.

Ambulatory Care↗
PubMed2003

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.

Adolescent↗
PubMed2003

The significance of a defect on DMSA scan in children with renal transplants.

Since December 1995, pediatric renal transplant recipients in our unit have received a DMSA scan as soon as possible post-transplant in order to provide a baseline for comparison in the event of subsequent complications. We retrospectively reviewed the case notes and DMSA scans of the 45 patients who underwent a scan within 9 wk of their transplant to see if pre or peri-transplant factors or post-transplant complications were associated with defects on scanning. Forty percentage of scans had defects. The presence of defects was not associated with potential predisposing factors such as patient or donor age, cadaveric or live donation, cold ischemia time, multiple donor vessels, the use of non-heart beating donors, the mean time to scan, the serum creatinine, or the presence of structural renal tract anomalies predisposing to UTI. However, 87% of patients had complications before the scan, including UTI, rejection, acute tubular necrosis, transplant biopsy and drug toxicity. Children with no clinical complications had a significantly reduced risk of a defect (p = 0.035), while biopsy was associated with the presence of defects (p = 0.0034). Twenty patients had one or more follow up DMSA scans: one patient developed a new focal defect. In conclusion, renal transplant defects are frequently found on DMSA scanning even early after transplantation and are non-specifically associated with many different complications.

Adolescent↗
PubMed2002

Immunosuppressive therapy of childhood idiopathic nephrotic syndrome.

Childhood nephrotic syndrome (NS) is a distressing chronic renal disorder with potentially life threatening complications. Over 80% of cases in children are due to minimal change disease and the majority will respond to corticosteroid therapy. Steroid-sensitive NS is considered a relatively benign condition, since progression to end stage renal failure (ESRF) is extremely rare and > 80% will enter spontaneous long-term remission in later childhood. However, the disease is characterised by a relapsing course, placing the child at risk of acute complications, such as infection, hypovolaemia and thrombosis. Frequent relapses can result in a not inconsequential corticosteroid burden or prescription of cytotoxic immunosuppressive therapy to control the disease. In contrast, steroid-resistant and -refractory NS has an unfavourable outcome with a propensity to progress to ESRF. While these clinical entities have an unpredictable response to cytotoxic immunosuppressive therapy, the favourable long-term renal survival associated with children who enter sustained remission has revived the enthusiasm to treat steroid-resistant NS with more aggressive immunosuppressive regimens.

Adrenal Cortex Hormones↗
PubMed2001

Familial steroid-responsive nephrotic syndrome and HLA antigens in Bengali children.

We investigated the major histocompatibility complex class I and II loci in three Bengali families with nine children affected with steroid-sensitive nephrotic syndrome (SSNS). A sequence-specific primer (SSP) of DNA typing method was used to detect human leukocyte antigens (HLA). The unaffected siblings and their parents were also studied. Similar to previous reports, there was a high frequency of HLA-DR7.1 (DRB1*0701), DR53 (DR B4*01011-0104) and DQ2 (DQB2*0201-3) antigens in the affected children. However, there was a similar finding in the unaffected children and their parents. HLA-DR7.1 probably was not a causative factor, since it had no predictive value for the occurrence or the severity of SSNS in the affected families. Siblings with identical HLA typing behaved differently (they either had or did not have SSNS). In these families there was no correlation between predisposition to the nephrotic syndrome and the genetic determinant responsible for HLA.

Adolescent↗
PubMed2000

Cardiac malformations associated with the congenital nephrotic syndrome.

The association of cardiac malformation with the congenital nephrotic syndrome (CNS) has been previously reported in only one family. We report four patients with CNS: three with pulmonary valve stenosis (one requiring valvuloplasty) and one with discrete subaortic stenosis requiring surgical resection. We conclude that the cardiac status of all patients with CNS should be reviewed regularly by a paediatrician, with a low threshold for referral to a cardiologist, as flow murmurs due to chronic anaemia may obscure cardiac pathology. It is important to diagnose any associated cardiac lesions as these may require intervention, and may also predispose to the development of bacterial endocarditis if surgical or dental procedures are undertaken without appropriate antibiotic prophylaxis.

Aortic Valve Stenosis↗
PubMed2000

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.

Body Weight↗
PubMed1999

Renal transplantation in children under 5 years of age.

Between March 1987 and December 1997, 59 renal transplants [49 cadaveric, 10 live related (LRD)], were performed in 54 children aged 5 years and younger. Six children required a second transplant. The median (range) age of the recipients was 2.9 (1.4-5.0) years; mean weight was 12.6 kg (7.4-23) and donor age 11 (2-50) years. Immunosuppression was cyclosporin or FK506, prednisolone, and azathioprine. Antithymocyte globulin was given as induction therapy for second transplants. Patient survival was 98.3%; 1 patient died from upper gastrointestinal haemorrhage. Graft survival was 67.7% at 1 year, 57.4% at 5 years, and 45.2% at 10 years. No LRD graft was lost during 7 years of follow-up. Thrombosis was the main cause of graft loss (10 cases) followed by vascular rejection (2 cases). There was no significant difference in graft survival between recipients aged less than 2, 2-3, and 3-5 years. The height standard deviation score (+/-SD) improved from -2.1+/-1.3 at transplantation to -1.0+/-1.3 at 1 year, -1.1+/-1.5 at 5 years, and to -0.14+/-1.1 at 10 years.

Adolescent↗
PubMed1999

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.

Age Factors↗
PubMed1998

Psycho-social outcome of parents and young children after renal transplantation.

This cross-sectional study aims to assess whether renal transplantation in children under the age of 6 years has an effect on the child's later behaviour and eating and whether this outcome is related to differences in the levels of stress and coping skills shown by the parents. In this small sample of 14 children aged under 8 years, renal transplantation in the pre-school age range did not have any marked adverse effect on the children's emotional or behavioural state. The children's severe eating problems dramatically improved after transplant. Long-term, early tube-feeding does not impair the development of normal feeding patterns in these children. Parental stress levels were not elevated in the post-transplant period and parents tended to use passive coping strategies to manage the chronic illness in their child.

Adaptation, Psychological↗
PubMed1998

Renal transplantation in the management of bilateral Wilms' tumour (BWT) and of Denys-Drash syndrome (DDS).

BACKGROUND: Wilms' tumour (WT) occurs bilaterally in approximately 5-7% of affected children. In some patients, complete surgical removal of the malignant tissue cannot be achieved without bilateral total nephrectomy. In Denys-Drash syndrome (DDS), bilateral nephrectomy is indicated both because of the associated nephropathy usually progressing rapidly to end-stage renal failure and because of the high risk of WT development in any residual renal tissue. METHODS: Case records of patients with a diagnosis of either bilateral WT (BWT) or DDS, who underwent bilateral nephrectomy and subsequent renal transplantation between 1980 and 1996 at the Hospital for Sick Children, London, were reviewed. RESULTS: Allogeneic renal transplantation was performed in two children with BWT and four with DDS, three of whom had developed unilateral WT by the time their kidneys were removed. Renal transplantation was performed 15-49 months after bilateral nephrectomy at a mean age of 45 (26-76) months, with a minimum of 1 year tumour-free survival after completion of chemotherapy in those with WT. One patient died after renal transplantation. Five children had a favourable outcome, with a mean follow-up of 80 (29-121) months post-renal transplantation. CONCLUSION: Advances in dialysis and transplantation programmes for young children offer the potential for a marked improvement in the prognosis for patients with BWT and for those with DDS.

Child, Preschool↗
PubMed1998

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↗
PubMed1997

Autosomal recessive polycystic kidney disease: long-term outcome of neonatal survivors.

Autosomal recessive polycystic kidney disease causes renal and hepatic dysfunction in childhood. We describe the clinical outcome of 52 children with this diagnosis born between 1950 and 1993. Currently 23 are alive, 24 dead and 5 have been lost to follow-up; 1 has been dialysed and 7 transplanted. Life-table analysis of the patients surviving the 1st month of life revealed an actuarial renal survival of 86% at 1 year and 67% at 15 years. The probability of requiring anti-hypertensive treatment was 39% at 1 year and 60% at 15 years of age. Bleeding from gastro-oesophageal varices occurred in 8 patients at a mean age of 12.5 years, and was preceded by haematological evidence of hypersplenism in 6 of them. The study indicates a relatively good prognosis for patients with this condition who survive the neonatal period and emphasises the importance of early detection and appropriate management of systemic and portal hypertension.

Adolescent↗