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Biomedical subjects

C Chantler

Publications and source records attributed to C Chantler.

At least 19 recordsLinked to original sources

Long-term follow-up of childhood Henoch-Schönlein nephritis.

A study of long-term outcome of 78 subjects who had had Henoch-Schönlein nephritis during childhood (at a mean of 23.4 years after onset) shows that severity of clinical presentation and initial findings on renal biopsy correlate well with outcome but have poor predictive value in individuals. 44% of patients who had nephritic, nephrotic, or nephritic/nephrotic syndromes at onset have hypertension or impaired renal function, whereas 82% of those who presented with haematuria (with or without proteinuria) are normal. 17 patients deteriorated clinically from an initial assessment in 1971; 7 of these had apparently completely recovered in 1976. 16 of 44 full-term pregnancies were complicated by proteinuria and/or hypertension, even in the absence of active renal disease. These findings indicate that childhood Henoch-Schönlein nephritis requires long-term follow-up, especially during pregnancy.

Biopsy

What makes red cells dysmorphic in glomerular haematuria?

Although red cell morphology has been used to localise the site of haematuria in the urinary tract, the cause of red cell deformity is still speculative. We have conducted experiments in vitro using venous red cells which indicate that hypochromia depends mainly upon sodium concentration and occurs when this falls below 75 mmol/l. We simulated the passage of red cells through the renal tubule by sequentially treating them with fluids of composition similar to those in different tubular segments, and produced anisocytosis and hypochromia but not the typical "bizarre deformity"--the hallmark of glomerular haematuria. We conclude that dual injury is required to produce the "typical" dysmorphic red cells in glomerular haematuria. First, mechanical damage caused by passage of red blood cells through the glomerular basement membrane followed by a second, osmotic, injury sustained by red cells during passage through the hypotonic tubular segment.

Basement Membrane

Transplantation--a new dimension for paediatrics.

Amazing advances have been achieved over the last 20 years in transplantation. As well as the problems of rejection and its control there are other difficulties which have to be confronted. There are shortages of donors, problems of selection for treatment and the problems of funding this expensive form of therapy. The need to make choices has to be considered and the criteria in determining choice have to be determined. Of particular concern in paediatrics are the problems that arise in relation to rehabilitation and the need to provide a multi-disciplinary approach to care. Finally, we have to remember that our enthusiasm for the possibilities of saving life and new treatments must not interfere with the basic responsibility to prevent suffering; at times the preservation of life at all costs is not justified, but in such circumstances doctors and other health care professionals have a responsibility for providing support both to the patient and to the family.

Child

The management of renal transplant artery stenosis in children by percutaneous transluminal angioplasty.

Between 1967 and 1989 in this unit 262 children (age at transplantation 9 months to 17 years, mean 9.6 years) had 345 renal transplants performed. Transplant artery stenosis (TAS) was found in 30 (8.7%) as demonstrated by arteriography, performed only when there was unexplained deterioration in transplant function, hypertension that was difficult to control, or in the presence of a vascular bruit. All patients with TAS except one had received a cadaveric allograft. From 1980 onward, percutaneous transluminal angioplasty (PTA) has been available for TAS, and this was attempted on 21 occasions in 16 patients. Nine patients demonstrated angiographic improvement following the procedure, and 7 showed immediate clinical improvement. On one occasion angioplasty precipitated graft loss. Five patients underwent planned corrective surgery, 4 after unsuccessful angioplasties. Our experience suggests that PTA should be the first method of intervention for TAS. Moderate success, both in angiographic and clinical terms, can be achieved, negating the need for surgery, while failure of PTA does not preclude surgical attempts at correction.

Adolescent

Duplicate publication.

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Duplicate Publications as Topic

Paediatric nephrology on the threshold of European integration.

The European Society for Paediatric Nephrology (ESPN) was founded 25 years ago. The progress of paediatric nephrology in Europe since then has been considerable, but we now face a number of problems. The care of the child with kidney disease is often expensive and more needs to be done to examine the cost effectiveness of the management of the child with kidney disease. International co-operation can also foster clinical research to determine the effectiveness of treatment through the institution of controlled trials and outcome studies. Particular problems are posed by the need to integrate the countries of Eastern Europe that have changed from command to market economies over the last year. In many instances there is no shortage of doctors, nurses and hospital beds but there is a need to change administrative and academic structures and to introduce appropriate technology. It is suggested that this may be assisted by twinning units. The integration of Europe requires that the role of the paediatric nephrologist in different countries needs to be examined, and appropriate training to fulfil these responsibilities needs to be agreed. Different countries obviously have different ways of organising and providing services for children with kidney disease. Kidney failure is rare in childhood and there are economic and academic advantages from close collaboration, both with adult nephrology services and with other paediatric specialities. Where it is intended to integrate children's hospitals into large multidisciplinary university hospitals for economic reasons, it is nonetheless necessary to make sure that the requirements of children are properly recognised and the requirements of the European Charter for children in hospital are met.(ABSTRACT TRUNCATED AT 250 WORDS)

Child

The influence of steroid therapy and recombinant human erythropoietin on the growth of children with renal disease.

Long-term steroid therapy has a depressant effect on hypothalamo-pituitary pulsatile secretion of growth hormone (GH), and this results in an attenuated pubertal growth spurt. Oxandrolone and recombinant human GH improve growth rates in children taking long-term steroid therapy for renal disease, but there are potential side effects. Treatment with recombinant human erythropoietin improved the growth of three prepubertal, but not three pubertal haemodialysis patients.

Adolescent

Effects of oral phosphocysteamine and rectal cysteamine in cystinosis.

Diurnal variation in leucocyte cystine and the effects of equimolar single doses of oral phosphocysteamine and rectal cysteamine were studied in eight patients with cystinosis, aged 1.8-16.5 years. No significant diurnal variation in leucocyte cystine was found. Absorption of cysteamine was reduced after rectal administration compared with the oral dose: mean (SD) peak concentration 17.2 (6.3) mumol/l v 36.4 (5.5) mumol/l at 40 min and mean (SD) area under the curve 22.3 (14.3) v 59.4 (33.1) mumol/h/l. Oral phosphocysteamine significantly reduced the mean (SD) leucocyte cystine from 8.09 (0.47) to 3.26 (1.48) nmol 1/2 cystine/mg protein at three hours. At 12 hours the mean leucocyte cystine was significantly lower than the pretreatment concentration. Rectal cysteamine did not significantly reduce the mean leucocyte cystine concentration. In conclusion, phosphocysteamine suspension may be administered every 12 hours. Rectal cysteamine administration is feasible but higher doses are required before efficacy can be judged.

Administration, Oral

Evaluation of light microscopy to localise the site of haematuria.

Red cell morphology was examined by light microscopy in 122 urine specimens from 99 patients with haematuria in an attempt to define the site of origin. Altogether 84% of glomerular bleeding and 91% of non-glomerular bleeding was correctly assigned according to diagnoses determined by renal biopsy in 51 patients and clinically in 48. The test should be interpreted with caution as both false positive and false negative results were observed, some in the same individual with unchanged pathology at different times. No advantage of phase contrast over bright field microscopy was apparent even when examining uncentrifuged urines.

Erythrocytes, Abnormal

Familial juvenile gouty nephropathy with renal urate hypoexcretion preceding renal disease.

This paper reports investigations in a young woman with renal disease and six other seemingly healthy young members of a new kindred (four male:two female) with familial juvenile gouty nephropathy (McKusick 16200). The family had previously been known to have a "familial" renal disease, but came to attention through an isolated episode of gout in the propositus when renal function was already impaired. A reduced GFR was found in three of the other six subjects. Hyperuricemia associated with a grossly reduced fractional uric acid clearance (Cur/Ccr x 100) was present in the propositus and five kindred members, three of whom were children. The finding of this abnormality in two subjects with normal GFR suggests that this apparent hallmark of the disease precedes the onset of renal damage. The results confirm the dominant nature of the disorder, and highlight the need to investigate all kindred members of patients with juvenile gout and renal failure. Early recognition is important, since allopurinol therapy in doses adjusted to the reduced renal function may ameliorate the progression of the renal lesion.

Adolescent

Measurement of beta 2-microglobulin, retinol-binding protein, alpha 1-microglobulin and urine protein 1 in healthy children using enzyme-linked immunosorbent assay.

Enzyme-linked immunosorbent assays (ELISA) have been developed for the measurement of beta 2-microglobulin (B2M), retinol-binding protein (RBP), alpha 1-microglobulin (A1M) and urine protein 1 (UP1) in children. Results from random urine samples in 43 children (31 for B2M) are, when corrected for urine creatinine (geometric mean (range)): B2M 9.8 (6.0-40.7) micrograms/mmol, RBP 8.1 (less than 1-24.5) micrograms/mmol, A1M 0.4 (0.1-2.2) mg/mmol and UP1 17.8 (less than 2-309.4) micrograms/mmol. Fractional excretions (FE) in 23 children (14 for B2M) are (geometric mean (range)): FEB2M 0.04% (0.02-0.10%) and FEUP1 0.10% (0.01-1.21%). Results in overnight urine collections are also presented. Our results extend existing data for normal ranges in adults to include children and provide data on UP1 concentrations.

Adolescent

Recurrence of focal segmental glomerulosclerosis in transplanted kidneys: analysis of incidence and risk factors in 59 allografts.

Fifty-nine allografts were placed in 43 patients with renal failure from focal segmental glomerulosclerosis (FSGS): 27 allografts were put into 16 children aged less than 15 years, and 32 allografts into 27 adolescents and adults. Recurrence of FSGS was noted histologically in 13 allografts, 10 in 8 children and 3 in adults. None of the 9 children and 24 adults who never developed an allograft nephrotic syndrome showed FSGS in their allograft biopsies. The age of onset was a strong risk factor for recurrence: recurrent FSGS developed in 8 of 16 children (50%) but only in 11% of adolescents and adults (3 of 27 patients). Although the time from apparent onset to renal replacement treatment was shorter in those with recurrence than those without in the children, there was no difference in the time spent on dialysis prior to transplantation. Mesangial prominence was observed in the original biopsy in 12 of 13 patients with recurrence, and recurrence rate was similar in living and cadaver donor allografts; class I MHC matching was similar in those with and without recurrence. Three allografts treated with cyclosporin A as well as 9 with azathioprine showed recurrence. Of 9 second or subsequent allografts placed in those with recurrence in the first allograft, only 3 showed further recurrence. In 3 re-grafted after 13, 11 and 5 years, normal function was seen.

Adolescent