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75 children aged under 15 years have entered the regular haemodialysis/renal transplant programme at Guy's Hospital in the 10 years since its inception; 13 children have subsequently died. A combination of hospital and home haemodialysis and renal transplantation was used. 64 children received 80 renal allografts; 37 1st grafts were from live, related donors and 27 were from cadaver donors. The 5-year acturial patient survival for the whole group and for those who had transplants was 76%. Live donor graft survival was 65% at 3 years, and 55% at 5 years; 1st cadaver graft survival was 42% at 3 years. Results obtained during the last 6 years of the 10-year period showed an improvement with a live related donor graft survival rate of 71%, and a 1st cadaver graft survival rate of 47% at 3 years. Particular experience was gained with home haemodialysis and live, related donor transplantation. Despite growth, and psychosocial and rehabilitation problems, the overall results were encouraging, particularly for the 46 children who had successful transplants. These children grew better, had fewer psychosocial difficulties, and were rehabilitated more successfully into normal life than those on long-term dialysis. We conclude that dialysis and transplantation should be offered to all suitable children with terminal renal failure. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

The relation between the true plasma creatinine concentration (Pc) and the glomerular filtration rate corrected for body surface area (GFR/SA) was investigated in 108 individuals, and the following formula was derived: GFR/SA (ml/min per 1-73m2SA) = 0-43 Ht (cm)/Pc (mg/100 ml). This formula was tested in a second group of 83 children, and its accuracy and precision was compared to the 24-hour creatinine clearance. It was found to be superior to the creatinine clearance overall, and was as good, even if all results involving suspect 24-hour-urine collections were eliminated from analysis. The formula in SI usage is: GFR/SA (ml/min per 1-73 m2SA) = 38 Ht (cm)1Pc (mumol/l). [ABSTRACT FROM AUTHOR]

Skin tests and exercise tests were performed on children with cystic fibrosis (CF) and first-degree relatives of CF children. Positive skin tests were found in 56% of patients and 19% of relatives, but the prevalence of atopy in CF patients bore no relationship to its prevalence in their relatives. Increased 0ronchial lability, mainly due to bronchoconstriction, was present in 54% of patients and 27% of relatives. The results showed that increased prevalence of bronchial lability, but not atopy, is found in first-degree relatives of CF children, and that the abnormal lability and atopy found in CF children does not indicate a familial allergic background. [ABSTRACT FROM AUTHOR]

Presents several correspondence concerning the articles published in the 'British Medical Journal.' Prevalence of childhood malaria; Suggestion in the management of children with nephrotic syndrome; Prevention of vitamin D deficiency in the elderly.

The initial plain chest radiograph of 60 infants with transposition of the great arteries and all less than 29 days old are reviewed. The previously described appearances of an oval heart, a narrow pedicle and increased pulmonary markings were only seen in 6 infants (10%). In 10 infants the appearances were normal (16%), and corresponded to the appearances seen in normal infants whose radiographs were used as controls. The relationship of the aortic arch as seen in the angiograms is related to the width of the pedicle as seen in the plain radiographs. The pattern of origin of the aortic arch vessels was observed, and a common brachiocephalic trunk or innominate artery displaced to the left was seen in 10 of the 41 infants with suitable angiograms. The radiographic appearances are also related to the presence of heart failure and to the mixing site or sites, associated pulmonary stenosis or coarctation of the aorta. [ABSTRACT FROM AUTHOR]

The prognosis of 28 infants presenting consecutively to a general paediatric unit with convulsions between 1 and 6 months of age was far better than had been reported previously. Between 1 and 4 years after presentation the condition of 19 patients was normal, 3 were mildly handicapped, 4 severely handicapped, and 2 had died. Normal physical examination and electroencephalogram at time of presentation, and easy control of convulsions were associated with a satisfactory outcome. [ABSTRACT FROM AUTHOR]

A patient with progressive osteolysis of the carpal and tarsal bones with glomerulonephritis of unusual severity is described. There was a notable absence of osteodystrophy in this and other reported cases who had chronic renal failure. [ABSTRACT FROM AUTHOR]

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.

Three hundred and fifty-eight renal imaging studies with quantitation of renal perfusion were performed following injection of 99Tcm-DTPA in 22 children aged 6--15 years with renal transplants. In 58 of 62 scans performed at the onset of a rejection episode, renal perfusion was decreased. This was the only feature of rejection in 6 episodes, in 5 of which the patient was oliguric due to post-operative acute tubular necrosis. In 4 episodes perfusion fell, but antirejection treatment was not given as a repeat scan was normal. On 18 occasions the plasma creatinine concentration (Pc) rose, but the scan was unchanged; no treatment was given and repeat Pc was normal. Three patients had a ureteric obstruction and 2 patients a lymphocoele detected from the images. There was no morbidity. Sequential renal scintigraphy was valuable in the early diagnosis of rejection and in the avoidance of unnecessary therapy.

Body composition was estimated in 12 pubertal and 7 pre-pubertal children (age range 7-17 years) on regular hemodialysis for chronic renal failure (CRF). The results were expressed as a ratio of observed/predicted. Total body water (TBW) was normal and extracellular water (ECW) was significantly increased in both groups. Intracellular water (ICW), which reflects body cell mass, was significantly decreased in the pre-pubertal children but not in the pubertal children. The deviation in ICW from predicted normal was significantly more marked in the prepubertal children compared with pubertal children. Similarly, fat-free solids (FFS) were significantly decreased in the pre-pubertal, but normal in the pubertal children. Total body fat, estimated from body water, correlated significantly with skinfold thickness measurements and was normal in both groups, with the pre-pubertal children having significantly higher body fat ratio than pubertal children. Body fat was increased in some of the patients whose body cell mass was decreased. It is suggested that these changes are the result of a failure of normal energy metabolism with consequent body protein depletion, and that energy supplements as carbohydrate, particularly in the pre-pubertal patients, will increase body fat without affecting cell mass.

Henoch-Schönlein nephritis now appears to be an immunologically mediated disease, common in children but rare in adults, and initiated by unknown antigen or antigens. We have reviewed this form of nephritis with particular attention to immunological changes, occurrence in adults and prognosis. Assessment and treatment are made difficult by varying diagnostic criteria, especially in adults, and lack of controlled data.

Fasting plasma amino acid concentrations were measured in 16 children on regular hemodialysis for renal failure. Reductions compared to normal were found in valine, leucine, isoleucine, lysine, histidine, tyrosine, and serine; and increases were found in glycine, citruline, proline, and 1- and 3-methylhistidine. Acute reductions in amino acid concentrations occurred in response to i.v. glucose, similar to those reported in normal adults, but plasma alanine, which was raised only in those with poor glucose tolerance, fell to normal and did not vary in those with normal glucose tolerance. No correlations were found with growth, but the plasma glycine concentration was highest in those patients with poorest energy intakes. Plasma alanine concentrations correlated with raised triglyceride concentrations. It is suggested that many of the abnormalities are due to the excessive utilization of protein for energy because of impaired availability of conventional energy sources in uremia.