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Biomedical subjects

C Chantler

Publications and source records attributed to C Chantler.

At least 163 records · Page 9Linked to original sources

The long-term prognosis of patients with focal segmental glomerulosclerosis.

Forty consecutive patients whose biopsies showed focal and segmental sclerosis were studied for 6 to 16 years to establish the long-term prognosis of this group of patients. By the end of a decade 21 were dead, on regular dialysis or transplanted; only one death was unrelated to renal failure. A further 8 patients had a GFR of less than 60 ml/min/1.73 m2. Only 11 patients had normal renal function, and of these eleven only four had no urinary abnormality. Actuarially calculated survival was 75% at 5 years, 50% at 10 years, and 38% at 15 years. There was no difference between the 28 adults and the 12 children in terms of evolution. Patients with a nephrotic syndrome at presentation had a poorer prognosis than those never nephrotic. Twenty nephrotic patients were treated with prednisone, and 14 of these with cyclophosphamide in addition. One patient responded with loss of proteinuria within two months to both drugs, and another lost proteinuria when treated with cyclophosphamide. Thirteen patients received allografted kidneys; a nephrotic syndrome recurred in three patients, with histological evidence of recurrent disease in two.

Adolescent↗

Oral essential amino acids in children on regular hemodialysis.

Plasma amino acids were measured by ion-exchange chromatography in 10 children on regular hemodialysis for renal failure before and after a 6 month period on an oral supplement of 8 essential amino acids. The supplement constituted 14.7 +/- 9.7 (1 SD) of the protein intake, the proportion varying inversely with protein intake, and mean energy and protein intakes which were normal for body size did not change significantly during the study. Before supplementation there were significant reductions compared to normal in the concentration of valine, leucine, isoleucine, lysine, histidine, tyrosine and serine, and elevations in glycine, proline and the methylhistidines. After supplementation methionine levels were abnormally high, isoleucine levels were in the lower range of normal and otherwise there were no significant changes. These modest changes suggest that supplemental essential amino acids in uremic children on adequate energy and protein intakes may not only be superfluour but may induce new imbalances which may themselves be harmful.

Administration, Oral↗

Focal segmental glomerulosclerosis with rapid decline in renal function ("malignant FSGS").

We describe 11 patients whose renal biopsies showed minimal changes with focal segmental glomerulosclerosis. These patients, in contrast to the majority of patients with similar renal histology, went into renal failure within 2 1/2 years of clinical onset. All were young, severely nephrotic, most hypertensive, with microscopic hematuria, non-selective proteinuria and extreme hypercholesterolemia. Treatment with corticosteroids and cytotoxic drugs was without effect in any patient. Despite rapid decline in renal function, profuse proteinuria and a nephrotic syndrome persisted into terminal uremia and continued even after dialysis had begun. Seven patients were given nine allografts; four grafts failed because of immediate vascular complications, and a persistant nephrotic syndrome was evident in two of the five surviving grafts. This did not, however, lead to graft failure. Two patients died on dialysis because of myocardial problems. These patients with rapid decline in renal function constitute a distinct clinical subgroup amongst those with focal and segmental glomerulosclerosis; it is possible that they have a different primarily vascular pathogenesis in contrast to other patients with similar renal biopsy appearances.

Adolescent↗

[Guy's Hospital home dialysis program (author's transl)].

The first home dialysis was carried out from Guy's Hospital in London in 1968. Since then, 141 patients have been treated in this manner. The age of the patients ranged from 4 to 64 years. 24 patients have died (cerebrovascular accidents, myocardial infarction, pulmonary edema, sepsis, peritonitis, hyperkalemia etc.). Some of the deaths were due to a slackening of discipline on the part of the patients and nursing staff during the years of constant dialysis. Of 60 adults 52 were able to start work again; full rehabilitation still seems possible in 6 cases. Nevertheless, many patients cannot lead a normal life because their social and sexual activity is greatly restricted. The present economic crisis led to financial limitations in the National Health Service. In future, home dialysis may have to be reduced and more transplantations performed.

Adolescent↗

Cryoglobulinaemia in Henoch-Schönlein purpura.

Sera from patients with Henoch-Schönlein purpura were examined for cryoglobulinaemia. Thirty patients had acute Henoch-Schönlein purpura, with or without renal diseas; 14 had chronic nephritis after a previous episode of purpura; and 17 were well, without urinary abnormalities, after recovering from Henoch-Schönlen purpura. Raised concentrations of cryoglobulins were present in 14 (47%) of those with acute purpura, nine (64%) of those with chronic nephritis, but none of those who had recovered completely from Henoch-Schönlein purpura. This suggests that acute Henoch-Schönlein purpura and the chronic nephritis that sometimes follows it have an immune-complex pathogenesis. IgA and properdin were found in several cryoglobulins, which suggested that complement had been activated via the alternative pathway, but isolated cryoglobulins capable of splitting C3 in vitro did so via the classical pathway.

Adolescent↗

Prognosis of Henoch-Schönlein nephritis in children.

All the survivors of a series of 88 patients with Henoch-Schönlein nephritis were examined after a follow-up of six and a half to 21 years (mean 9-9). Sixty-one patients had no demonstrable abnormality; six had minor urinary abnormalities; five had hypertension without urinary abnormally or renal dysfunction; four had heavy proteinuria; eight were in chronic renal failure, three of whom were on regular dialysis; and four patients had died within 25 months of onset. Neither corticosteroids nor immunosuppressive drugs alone or in combination appeared to influence the outcome. A clinical presentation with a combination of acute nephritis and a nephrotic syndrome and a high proportion of crescents in renal biopsy specimens was associated with a poor outcome. Neither the clinical presentation nor the renal morphology were, however, precise determinants of outcome. Outcome was not related to age, associated streptococcal infection, or recurrences of the rash. The clinical state two years after presentation was compared with the state six and a half years or more after presentation in 76 patients. The clinical state had changed in 32 patients, in 17 of whom it had deteriorated. It was not possible to identify with any certainty the patients who would deteriorate (or improve). Patients who have had Henoch-Schönlein nephritis should be followed up for at least five years.

Adolescent↗

Presentation, management, complications, and outcome of acute renal failure in childhood: five years' experience.

During 1971-5, 72 episodes of acute renal failure were treated in 70 children aged up to 16 years. The commonest causes were renal hypoperfusion (31 cases), haemolytic-uraemic syndrome (12), glomerulonephritis (9), septicaemia (5), and congenital abnormalities (6). Though referral from other hospitals was generally prompt, 10 out of 51 patients had been observed for up to seven days before transfer. Dailysis was used in 44 cases, the most common complications of which were peritonitis in those treated with peritoneal dialysis and acute changes in fluid balance in those treated with haemodialysis. Altogether 37 patients fully recovered, 10 were discharged with chronically impaired renal function, 17 died, and six entered the dialysis and transplantation programme. The mortality fell from 33% in 1972 to 20% in later years, which was due solely to maintenance dialysis being available. Though all patients with irrevocable kidney failure who were suitable entered the dialysis and transplantation programme, with current financial restrictions we doubt whether we shall be able to find places for all such patients in the future.

Acute Kidney Injury↗

Home haemodialysis in children. Report of the London Children's Home Dialysis Group.

In January, 1973, a study was established to evaluate, prospectively and independently, the growth and psychosocial adaptation of children in end-stage renal failure treated by home haemodialysis at Guy's Hospital and the Royal Free Hospital. By January, 1975, 26 children had entered the study, but 2 died before they were established on home haemodialysis. In this paper a specified 1-yr period starting 6 mo after the onset of haemodialysis was selected for analysis. School attendance, diet, plasma-biochemistry, bone disease, growth, and emotional symptoms were investigated in each child. Rehabilitation was satisfactory, and average school attendance was 65%. Growth in all the prepubertal children was poor, whereas in most pubertal children it was adequate. At the end of the year, only 5 children were emotionally disturbed, but half the families had stress symptoms. The children at Guy's Hospital were dialysed more intensively than those at the Royal Free Hospital, and they had significantly lower mean plasma urea and creatinine concentrations and a greater energy and protein intake. On the other hand, the children at the Royal Free Hospital had a better school attendance. We conclude that home haemodialysis is an acceptable treatment for children in end-stage renal failure.

Adaptation, Psychological↗

Hyperlipidemia.

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Adolescent↗

Effect of uremia on nutritionally-induced variations in protein metabolism.

The effect of uremia on nutritionally-induced variations in protein metabolism was studied in growing rats with chronic, moderate uremia. Plasma and muscle protein synthetic activities and related values were measured from incorporation rates of 14C-leucine infused over a six-hour period. Synthetic activities were compared in the postabsorptive (2 to 14 hr after feeding) and in the fasting (18 to 24 hr after feeding) states. Differences between uremic and control rats were seen in fasting: plasma and intracellular leucine declined in control rats as fasting developed; they rose in uremic rats. In fasting uremic rats, a smaller percent of leucine efflux from extracellular fluid was used for protein synthesis. In a second study, muscle protein synthesis (Sm) and net urea nitrogen production (UNPr) were compared between control and uremic rats which were a) food-deprived but given carbohydrate for 36 hr, and b) fasted for 36 hr. Fasting, as contrasted with carbohydrate feeding, was associated with depressed muscle protein synthesis and increased UNPr in both control and uremic rats; the changes were greater in uremic rats. We conclude that food deprivation for more than 14 hr incurred greater catabolic responses of body protein in moderately uremic rats. The effect was mediated in part by greater suppression of muscle protein synthesis. In contrast, moderate uremia had little discernible effect upon protein metabolism in the postabsorptive state or food deprived state when gluconeogenesis was suppressed with glucose administration.

Alanine↗

Hyperlipidaemia in children on regular haemodialysis.

Fasting plasma concentrations of triglycerides (TG), cholesterol, immunoreactive insulin (IRI), and blood glucose were raised in 16 children with chronic renal failure on regular haemodialysis compared with 18 healthy children. In the patients plasma IRI correlated positively with plasma TG, while blood glucose did not correlate with IRI or lipid concentrations. Dietary intake, expressed as percentage of recommended intake for height-age, did not correlate with plasma lipids, but there was a positive correlation between plasma TG and the proportion of calories derived from carbohydrate. The children were not malnourished as evidenced by normal plasma albumin and transferrin concentrations. The mechanism of the hyperlipidaemia is unclear but it may be related to the glucose intolerance with hyperinsulinaemia which is found in uraemia. In view of the risk of premature atherosclerosis, plasma lipid concentrations should be monitored in children with chronic renal failure and attempts made to ameliorate hyperlipidaemia with appropriate dietary manipulations.

Adolescent↗

Congenital renal abnormalities in the Laurence-Moon-Biedl syndrome.

Two children with Laurence-Moon-Biedl syndrome had radiographic evidence of cysts in the renal medulla, and one had impaired renal function and infection. The frequency of cystic disease in this syndrome implies that intravenous urography should be carried out on all patients with this syndrome. Children with renal abnormalities should be followed to avoid further damage from urinary infection, or from dehydration in those with a concentrating defect. Regular renal function tests should be carried out on other children. and full investigation should follow if abnormalities are found in order that the natural history of the often fatal renal lesion can be clarified.

Child↗