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R S Trompeter

Publications and source records attributed to R S Trompeter.

At least 19 recordsLinked to original sources
PubMed1995

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

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

Cystinosis↗
PubMed1994

Effect of cyclosporin A on glomerular filtration rate in children with minimal change nephrotic syndrome.

Cyclosporin A (CyA) is now commonly used in the management of children with steroid-dependent nephrotic syndrome. In order to assess nephrotoxicity related to CyA therapy, we measured glomerular filtration rate (GFR) on 123 occasions in 24 children with minimal change nephrotic syndrome receiving CyA. GFR was estimated from the plasma clearance of 51chromium-EDTA every 3 months during CyA therapy of up to 27 months duration. There was a significant reduction in GFR after 3 months of CyA therapy [118 +/- 33 (SD) to 93 +/- 24 ml/min per 1.73 m2] but no further fall thereafter, although the reduction in GFR was sustained for the duration of CyA therapy. This reduction in GFR appeared to be reversible upon cessation of CyA, but careful monitoring of renal function is necessary in such patients to prevent the development of longer term nephrotoxic sequelae.

Adolescent↗
PubMed1994

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

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

Adolescent↗
PubMed1994

Alternative treatment to corticosteroids in steroid sensitive idiopathic nephrotic syndrome.

A review was undertaken of the use of alternative immunosuppressive treatment in addition to corticosteroids in a cohort of 429 children with steroid sensitive nephrotic syndrome (SSNS) treated between 1980 and 1994. Two hundred and twenty two children (52%) received at least one course of alternative treatment, 98 (23%) two, and 43 (10%) three. Cyclophosphamide was administered to 196 children (46%); in 181 it was the first course of alternative treatment and in 104 (57%) of those it was also the last ('final course'). Levamisole was given to 56 children (13%) and cyclosporin to 53 (12%). Fifteen children in whom cyclosporin failed were treated with chlorambucil. A few patients received azathioprine or vincristine. Ten children developed secondary steroid resistance, of whom five progressed to chronic renal failure. Acute complications included reversible renal failure, septicaemia, peritonitis, convulsions, and cerebral thrombosis. There were three deaths. It is concluded that half of the referred children with SSNS were deemed to require at least one course of alternative immunosuppressive treatment, and that side effects of the treatment and complications of SSNS are infrequent but occasionally fatal.

Adrenal Cortex Hormones↗
PubMed1992

Cadaveric renal transplantation in children under 5 years of age.

From March 1987 to August 1990 23 cadaveric renal transplants were performed in 19 children under the age of 5 years at the time of transplantation. The mean age of the recipients was 3.3 years (range 1.3-4.7) and the mean weight 13.0 kg (range 9.3-19.2). The mean donor age was 7.8 years (range 1.5-25). All children received triple immunosuppression with prednisolone, cyclosporin A and azathioprine, and 4 who had 2 grafts during this period also received antithymocyte globulin at the time of the second transplant. Patient survival is 100%. Actuarial first cadaveric graft survival was 57% at 1 year and remains unchanged at 3 years. There were 10 graft losses, 4 were associated with renal venous thrombosis without apparent rejection. Two were lost due to acute vascular rejection with associated renal venous thrombosis, and the remaining 4 losses followed cellular or chronic vascular rejection. The mean glomerular filtration rate +/- SD was 51.4 +/- 23.6 ml/min per 1.73 m2 (n = 11) at 1 year and 43.5 +/- 25.3 at 2 years (n = 6). The mean height standard deviation score improved from -2.2 +/- 1.1 at the time of transplantation to -1.3 +/- 1.0 1 year post transplant (n = 11). The immunosuppression was well tolerated with a low incidence of side effects. Cadaveric renal transplantation remains a difficult but rewarding undertaking in children under 5 years of age.

Age Factors↗
PubMed1992

The evaluation of paediatric renal transplants using resistive index and renal blood flow.

Six children (aged 1.3-6.9 years) were examined with serial duplex Doppler sonography and diethylenetriaminepenta-acetic acid (DTPA) isotope renography in the post-renal transplant period. The resistive index (RI) was derived from sonographic studies and the renal blood flow (RBF) calculated from the isotope scans. The clinical status of the child and the corresponding plasma creatinine level were assessed together with these two parameters. The RIs ranged from 40% to 100% and the RBF from 0% to 16.8%. There were six rejection episodes in four patients. A significant fall in RBF mirrored a rise in plasma creatinine on each occasion, but there was no significant change in RI recorded. There were two graft losses, both associated with renal venous thrombosis. In both cases no significant RBF could be detected on DTPA renography. In one patient, the RBF remained low throughout a period of primary non-function associated with acute tubular necrosis, and increased as primary function was established and the plasma creatinine fell. Throughout this period there was no significant change in the RI. From our preliminary data RBF reflects graft dysfunction more accurately than does the RI.

Child↗
PubMed1992

Interleukin-8 and polymorphoneutrophil leucocyte activation in hemolytic uremic syndrome of childhood.

Polymorphoneutrophil leucocytes (PMNLs) are implicated in the pathogenesis of diarrhea-associated hemolytic uremic syndrome (D+ HUS). We investigated mechanisms of PMNL involvement by measuring tumor necrosis factor alpha (TNF alpha) and the novel cytokine, interleukin-8 (IL-8), a potent activator of neutrophils, together with alpha 1- antitrypsin-complexed elastase (alpha 1-AT-E) as a marker of neutrophil degranulation, and anti-neutrophil cytoplasmic antibodies (ANCA). IL-8 was not detected in the 17 normal children, but was significantly elevated in 20 of 25 D+ HUS children (P less than 0.005), and in three of nine children with non-diarrhea-associated (D-) HUS. Sequential data showed that IL-8 peaked transiently in the circulation, reaching a maximum just before a more protracted burst of alpha 1-AT-E. The IL-8 levels correlated significantly with circulating alpha 1-AT-E concentrations (r = 0.50, P less than 0.05). In D+ HUS IL-8 levels also correlated with the PMNL count (r = 0.63, P less than 0.005), and the highest values were seen in those children who died in the acute phase of the disease. TNF alpha was raised in only 1 of 16 D+ HUS children and in no patients were ANCA detected. The data suggest that PMNLs in HUS are recruited by IL-8, that this cytokine plays a key role in the PMNL activation which occurs, and that agents which suppress this recruitment and activation might play a therapeutic role in this disorder.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1992

Foregut motor function in chronic renal failure.

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

Child↗
PubMed1991

Long term renal outcome of childhood haemolytic uraemic syndrome.

OBJECTIVE: To evaluate the long term outcome of renal function in infants and children after diarrhoea associated haemolytic uraemic syndrome. SETTING: The Hospital for Sick Children, Great Ormond Street, and the Royal Free Hospital, London. SUBJECTS: 103 children with the syndrome who presented between 1966 and 1985; 88 attended for follow up investigations (40 male, 48 female) with a mean age 11.6 (range 5.2-22.6) years and a mean duration of follow up of 8.5 (range 5.1-21.3) years. MAIN OUTCOME MEASURES: Blood pressure, ratio of early morning urine albumin to creatinine concentration, glomerular filtration rate, and plasma renin activity. RESULTS: The mean (SD) systolic blood pressure standard deviation score was 0.38 (0.67) and diastolic blood pressure SD score was 0.10 (0.76). The geometric mean ratio of overnight urine albumin to creatinine concentration was 1.27 (range 0.03-48.2), significantly higher than the value observed in 77 normal children (0.32 (0.05-1.95), p less than 0.0001). Glomerular filtration rate estimated from the plasma clearance of chromium-51 EDTA was 95.1 (22.7) ml/min/1.73 m2 surface area, and 16 children had a rate of less than or equal to 80 ml/min/1.73 m2. Significant negative correlations were found between glomerular filtration rate and urinary albumin to creatinine ratio (r = -0.41, p less than 0.0001) and glomerular filtration rate and systolic blood pressure SD score (r = -0.48, p less than 0.0001). A significant positive correlation was found between urinary albumin to creatinine ratio and systolic blood pressure SD score (r = 0.25, p = 0.02). CONCLUSIONS: After an acute episode of diarrhoea associated haemolytic uraemic syndrome 31% (27/88) of children had an increased albumin excretion, 18% (16/88) had a reduced glomerular filtration rate and 10% (9/88) had both, in association with a higher systolic blood pressure, indicating considerable residual nephropathy in this group.

Adolescent↗
PubMed1990

Sclerema neonatorum and subcutaneous fat necrosis of the newborn in the same infant.

Sclerema neonatorum (SN) is a rare disease of the newborn characterised by diffuse hardening of the subcutaneous adipose tissue. The condition is distinct from the localised violacious nodules observed in subcutaneous fat necrosis of the newborn (SCFN). Both conditions were diagnosed within the first 2 weeks of life in the same infant. SN and SCFN are unusual conditions in the newborn, the former usually associated with a severe clinical illness, following delivery or major surgery. The skin lesions of these two conditions are distinct and do not usually occur simultaneously. SN is associated with a poor prognosis.

Adipose Tissue↗
PubMed1990

Renal transplantation in children.

Transplantation with a renal allograft is the treatment of choice for the child with end-stage renal failure. Over the past 20 years rapid advances have made it possible to transplant a kidney of adult proportions into a child aged 1 year or less. Actuarial graft survival at 5 years for first cadaveric kidneys is more than 60% in children aged less than 5 years. Long-term studies have demonstrated an improvement in linear growth and preservation of glomerular filtration rate.

Child↗
PubMed1989

Purine enzyme defects as a cause of acute renal failure in childhood.

Acute renal failure (ARF) is not listed as a usual form of presentation in hypoxanthine-guanine phosphoribosyltransferase deficiency, despite the gross uric acid overproduction in the defect. We found that a third of such patients may present in ARF when the urinary uric acid/creatinine ratio may be normal, not raised, and the defect may be suspected from the disproportionate increase in plasma uric acid. This is important in view of the potential confusion of uric acid with 2,8-dihydroxyadenine, the even more insoluble purine excreted in the other salvage enzyme disorder, adenine phosphoribosyltransferase deficiency. In that disorder, presentation in ARF is well recognized, the uric acid/creatinine ratio is also normal, but plasma urate is not raised. Our combined experience in these two disorders underlines the importance of early recognition and treatment with carefully adjusted doses of allopurinol, which may reverse or postpone renal failure.

Acute Kidney Injury↗
PubMed1989

Immunosuppressive therapy in the nephrotic syndrome in children.

The high incidence of remission and prevention of relapse of minimal change nephrotic syndrome (MCNS) in children, produced by corticosteroids is reviewed. With the introduction of corticosteroids over 30 years ago and the increased expertise in their use, the mortality rate has been reduced to less than 5%. There is no justification for a clinical trial to test the effect of corticosteroids in inducing remission, but the need remains to evaluate methods of administration in order to achieve therapeutic benefit with minimum toxicity. Children with frequently relapsing, steroid-dependent MCNS will usually enter remission following treatment with an alkylating agent such as cyclophosphamide. In about 50% no further relapse in experienced. The results of recent experience using cyclosporin A immunosuppression suggest a beneficial effect associated with steroid responsiveness. Approximately 30% of children with focal segmental glomerulosclerosis enter remission following treatment with corticosteroids. Some 30% require dialysis and transplantation within 5 years of diagnosis and immunosuppressive therapy to prevent deterioration of renal function is probably justified.

Adrenal Cortex Hormones↗
PubMed1989

Antenatal screening for hepatitis B is medically and economically effective in the prevention of vertical transmission: three years experience in a London hospital.

We describe our experience over a three-year period in the detection of antenatal patients with hepatitis B virus (HBV) carriage, and the successful prevention of vertical transmission in the infants of HBsAg-positive women. Nine of the 26 infants born to HBsAg-positive mothers were considered to be at high risk of infection. Eight of these nine remained HBsAg-negative after passive or passive/active immunization. The majority of infants tested had anti-HBs antibody titres above 10 i.u./l from the first month. In comparison with the cost of caring for patients with chronic hepatitis B infection and decompensated cirrhosis, non-selective testing of pregnant women for HBsAg is found to be cost effective.

Carrier State↗
PubMed1989

[Triple immunosuppression in renal transplantation in children: 3 years' experience].

Cyclosporin A in combination with low-dose steroid therapy and azathioprine was given to 50 children who received 53 renal transplants. In spite of a limited period of observation, the use of cyclosporin A, either on a post operative intravenous regime or on oral regime when primary graft function is achieved, has no significant influence on graft survival rate, which is 82% after 1 and 2 years, utilising cadaver donors. Vascular complications were responsible for the loss of 4 grafts and were associated with the use of kidneys from young donors, rather than the type of immunosuppressive agents used.

Adolescent↗
PubMed1987

A review of drug prescribing in children with end-stage renal failure.

This review provides a guideline for the prescribing of drugs in children with end-stage renal failure. The altered physiology of renal failure affects the metabolism of most pharmacological agents and modification of the drug dosage or extension of the prescribed drug interval become necessary with declining glomerular filtration rate (GFR). Most dose adjustments are dependent on a knowledge of renal function since GFR is inversely proportioned to the elimination rate constant of a drug. The details of drugs listed by generic name are those commonly used in paediatric practice, and the recommendations for dose adjustments are related to a reduction in GFR and not to body mass. Familiarization with the normal dose prescription of any drug used in paediatric practice is therefore necessary when referring to the table.

Child↗