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We studied glucose metabolism using the hyperglycemic technique in a cross-section of 23 children (15 pubertal, 8 prepubertal) with stable chronic renal failure as a possible cause of their poor growth. Linear growth was expressed as growth velocity standard deviation score (GVSDS). GVSDS correlated with glucose disposal rate but not with insulin sensitivity index in the pubertal (r = 0.87, P0.001) and prepubertal (r = 0.86, P0.02) children with chronic renal failure. Thirteen children were followed longitudinally during medical suppression of hyperparathyroidism with dietary phosphate restriction and high-dose phosphate binders. Following significant suppression of serum parathyroid hormone (PTH) levels back to the normal range (932 +/- 240 ng/l to 199 +/- 50 ng/l), GVSDS, glucose disposal rate and insulin secretion all increased significantly (p0.01), with no change in insulin sensitivity index and renal function. The changes in GVSDS correlated with the changes in glucose disposal rate (r = 0.86, P0.02) and with the changes in insulin secretion (r = 0.80, P0.01). However, the changes in GVSDS did not correlate with the changes in PTH. The hypothesis that insulin may be more important than PTH in the pathogenesis of growth failure in chronic renal disease deserves further investigation.

Eight patients with the middle aortic syndrome are described. They were aged 2 months to 14 years at diagnosis; follow up was one to 11 years. Clinical presentations included asymptomatic hypertension (n = 5), severe headache, nose bleed, and chest pain (n = 1), and cardiac failure (n = 1). All had severe hypertension requiring multiple drug treatment. Diminished peripheral pulses were not helpful in the diagnosis, which is made on aortography. Associated clinical findings were Williams' syndrome (n = 3) and appreciable eosinophilia (n = 3). The differential diagnosis includes Takayasu's arteritis, fibromuscular dysplasia, and neurofibromatosis. Blood pressure was adequately controlled by medical treatment in six patients. Surgical angioplasty was performed in two. One patient remained normotensive without drug treatment 21 months after operation; the other died of sepsis and uncontrollable haemorrhage in the postoperative period. Medical treatment is satisfactory in most cases: surgery should be reserved for those in whom blood pressure cannot be controlled without unacceptable side effects of drug treatment. Although rare, the middle aortic syndrome should be considered in the differential diagnosis of hypertension when commoner causes have been excluded. Aortography is necessary for diagnosis.

Mid-aortic syndrome (MAS) is an uncommon condition characterized by segmental narrowing of the proximal abdominal aorta and ostial stenosis of its major branches. It is usually diagnosed in young adults, but may present in childhood as a challenging problem. Over the past 20 years 13 patients with MAS have presented to this institution. All had hypertension, four had associated neurofibromatosis, three persistent eosinophilia and three had Williams syndrome. In all cases arteriography showed a smooth segmental narrowing of the abdominal aorta with concomitant stenosis at the origins of the renal arteries. Six children were successfully treated with antihypertensive medication alone. Percutaneous transluminal angioplasty was attempted in two cases with poor result. Surgery was indicated in seven children with refractory hypertension and progressive renal impairment. Techniques used to revascularize the kidneys included thoracoabdominal to infrarenal aortic bypass with renal artery reimplantation, splenorenal bypass, gastroduodenal to renal bypass, aortorenal bypass and autotransplantation.

We analysed outcome retrospectively in relation to treatment and disease stage in patients with systemic lupus erythematosus and glomerulonephritis, and compared these with similar patients referred to us during 1969 - 78, and other published series from the same period. Eighty two patients with lupus nephritis were referred during 1979 - 89. Of these, 73 were followed up for a mean of 58.1 (SD 34.5) months. The histological pattern in renal biopsies was WHO Class IV (diffuse proliferative glomerulonephritis) in 59% of patients. In the acute phase 43 patients received intravenous nethyl-prednisolone IG daily, for three days, in 25 accompanied by daily 4 litre plasma exchanges on 5 to 10 days. Twelve patients received induction therapy using oral cyclophosphamide during the acute phase, for 8 - 12 weeks only. Maintenance therapy was with oral prednisolone in all patients, accompanied by azathioprine in 84% of cases. Long- term cyclophosphamide was never used, neither intravenously nor orally. At 10 years actuarially calculated patient survival was 87% and survival of renal function 86% compared with 57% and 65% respectively in 1970 - 78 ([P0.01]). Survival was no different in patients with renal biopsies classified into the various WHO classes. In patients with Class IV biopsies, survival in those treated with prednisolone and azathioprine only was the same as that in those given intravenous methylprednisolone and/or plasma exchange as well. However, patients treated with methylprednisolone or plasma exchange tended to have more severe disease. At last followup, 24/82 patients were in complete remission, 23 had normal plasma creatinine concentrations but abnormal urine, ie proteinuria, haematuria, or both, 16 had abnormal urine and elevated plasma creatinine concentrations, and 9 had started renal replacement therapy. Eight patients had died. The survival of patients with lupus nephritis has improved in the past decade in patients with comparable severity of disease, and renal failure is no longer the principle cause of death. Results of maintenance treatment using azathioprine as adjunct to oral prednisolone in patients with severe nephritis are as good as those in series published elsewhere describing regular intravenous cyclophosphamide. No clear advantage was evident from the additional use of intravenous methylprednisolone and/or plasma exchange in the acute phase, in patients with WHO Class IV severe diffuse proliferative glomerulonephritis.

Five cases of children with nontuberculosis pericardial constriction with effusion are reported. The disease is not as rare as has been thought, and the aetiology in these cases was probably a previous virus infection. A previous diagnosis of hepatitis, nephrotic syndrome, or protein-losing enteropathy had been made and the correct diagnosis was delayed for months or even years. Pericardiectomy produced immediate relief from symptoms in 2 patients but in 2 others there was evidence of poor myocardial function postoperatively.

Summary— Forty-three children with overt neurological disease and neuropathic vesicourethral dysfunction were entered into a trial comparing clean intermittent catheterisation (CIC) with manual expression combined with drug treatment (non-CIC). The 22 children in the CIC group showed a significantly greater improvement in continence than the 21 children in the non-CIC group (P

Three children with nephropathic cystinosis were treated with indomethacin 3 mg/kg a day for periods ranging from 9 to 18 months. The drug produced worthwhile clinical improvement in all, with marked beneficial effects on polyuria, polydipsia, and general wellbeing. Clearance studies performed under conditions of maximal water diuresis showed that proximal tubular sodium reabsorption was increased in all children, with consequent reduction in sodium delivery to the distal nephron leading to reduced free water clearance and distal tubular cation exchange. Plasma sodium and potassium concentrations became normal in all patients, with improvement in phosphate and bicarbonate concentrations in one. Renal function continued to deteriorate, but without obvious acceleration of the process by the drug. We were unable to demonstrate a beneficial effect on growth; nevertheless, indomethacin is a useful adjunct to the symptomatic treatment of children with severe nephropathic cystinosis.

Carbohydrate metabolism was studied by the hyperglycemic clamp technique in 14 children with moderate to severe chronic renal failure (CRF) (8 pubertal and 6 prepubertal) and was compared with 5 healthy prepubertal children and 7 healthy young adults. Constant hyperglycemia was maintained for 120 min. Under these conditions, the average glucose infusion rate (M) is an index of glucose metabolic rate, the average insulin response (I) is an index of beta cell responsiveness to hyperglycemia, and the M/I ratio is an index of insulin sensitivity. Children with CRF were glucose intolerant (lower M), hyperinsulinemic (higher I), and insulin resistant (lower M/I) compared with the control children. An age-related difference in glucose tolerance and insulin sensitivity was noted. Pubertal children with CRF had lower M and lower M/I compared with prepubertal children with CRF. The 7 adult controls also had lower M and lower M/I compared with the 5 children controls. M and M/I correlated with glomerular filtration rate (GFR) in the 8 pubertal children, while M/I also correlated with GFR in the 6 prepubertal children with CRF. The hyperglycemic clamp provides a useful tool in studying the pathogenesis of carbohydrate disturbances in children with CRF.

Glucose metabolism was studied using the glucose clamp technique in 8 children with CRF (4 pubertal and 4 prepubertal) before and after correction of secondary hyperparathyroidism (HPD) by a regime of phosphate binders and dietary phosphate restriction. Glucose metabolic rate (M) increased by 34%, insulin response (I) increased by 32%, and insulin sensitivity (M/I) did not change. Compared to corresponding normal values, these patients were glucose-intolerant (low M) before treatment and became glucose-tolerant after treatment (normal M). They were insulin-resistant (low M/I) before treatment and remained so after treatment. The 5 uremic children without secondary HPD were treated with the same regime to prevent the secondary HPD. Their M, I, and M/I did not change after treatment. The change in I correlated closely with the change in M in all 13 patients. One pubertal patient with uremia was studied on three occasions. Initially, without secondary HPD, he had a normal M and a low M/I. When he developed secondary HPD, M decreased by 39%, I decreased by 37%, and M/I did not change. Treatment of his secondary HPD restored M and I values back to initial levels, and M/I again did not change. Treatment of secondary HPD by correction of phosphate retention led to an improvement of glucose intolerance with an increase in insulin secretion.

A commercially available closed dialysis system and a new peritoneal cannula with potential advantages for infants have been developed. The dialysis set includes three dialysate bags that may be connected to the filling burette; the warming coil of the set is placed in a thermostatically controlled water bath. The peritoneal catheter comprises a flexible tube with side holes and a sharp short bevelled needle with obturator. Advantages of the new equipment over previously available equipment are that the cannula is easier to insert; there is less risk of contaminating the dialysate since the tubing set is unbroken from supply to cannula; the mix of the dialysate may be changed easily without interrupting dialysis; and the equipment may be assembled easily by unskilled staff.

Two girls are described, aged 7 and 10 years respectively, who presented with arterial hypertension associated with unilateral kidney disease. Both were cured by nephrectomy, according to criteria for cure which are described in the paper. Only 45 similar cases could be found in the literature; these results are summarized and compared with those obtained in adult patients. The cure rate in children was 76%; similar large series including patients of all ages, but consisting predominantly of adults, show cure rates of only about 25%. It is inferred that the age of the patient is an important factor in determining prognosis, and should be considered in the selection of patients for nephrectomy. Methods of investigation of these patients, and possible mechanisms concerned in the pathogenesis of the hypertension, are briefly discussed.

Creatinine concentration was measured by a kinetic method in 475 plasma samples obtained from 260 term and preterm infants during the first 2 months post-natal age. In term infants plasma creatinine fell from 88 +/- 4.4 mumol/L (mean + SEM) at birth, to 60 +/- 1.9 mumol/L by the fourth day remaining stable thereafter; in preterm infants creatinine fell to 86 +/- 7 mumol/L by the fourth day. Analysis of pooled data from all infants of more than 4 days post natal age revealed a progressive fall in creatinine with increasing post conceptual age. The normal range of values varies with post conceptual age and was not influenced by gestational age at the time of birth.

Summary— Ten children with vesicoureteric reflux who had moderate impairment of renal function before corrective surgery have been analysed retrospectively. A significant improvement in renal function occurred after successful anti-reflux surgery. The possible reasons for this improvement are discussed with emphasis on the functional rather than the morphological effects of reflux.

The safety and clinical efficacy of calcium carbonate therapy in children with chronic renal failure were assessed in 68 patients (average age 8.38 years) during a mean follow-up period of 19.9 months (range 1.2-49.4). Forty-seven episodes of hypercalcaemia occurred in 29 children (3.5 episodes per 100 patient-months). There were no significant differences in mean GFR or biochemical parameters between these patients at the start of calcium carbonate therapy and the group of children who never experienced hypercalcaemia. Good control of secondary hyperparathyroidism and a significant reduction in serum aluminum were achieved. Two of 23 hypercalcaemic patients showed nephrocalcinosis on ultrasonography. 99Tc pyrophosphate scanning failed to detect any other ectopic calcification. The incidence of hypercalcaemia increased significantly when the GFR was less than 15 ml/min per 1.73 m2 and was most frequent in children receiving dialysis (48 episodes per 100 patient-months). The decrease in GFR during therapy was significantly more in the hypercalcaemic group compared to the normocalcaemic group (P less than 0.01), despite no irreversible acute effects of hypercalcaemia being observed on the rate of decline of GFR. We believe that the reduced renal homeostatic reserve is a major factor predisposing to hypercalcaemia. Consequently calcium carbonate is safe to use in children with severe chronic renal failure with close biochemical monitoring; the benefits over aluminium phosphate binders far outweigh the risks of hypercalcaemia and ectopic calcification.

Of 104 patients with idiopathic mesangiocapillary glomerulonephritis studied for at least two years, 69 patients had type I disease and 35 had type II. Forty-five patients were children, and 59 were adults. Type II mesangiocapillary glomerulonephritis was more common in children than in adults, but no other clinical feature distinguished the two types at onset. Complement studies revealed that patients with type II had lower serum C3 concentrations and more frequently showed C3-splitting activity (C3 nephritic factor) in the serum. Children had hypertension or a lowered glomerular filtration rate less frequently at onset than did adults, but children had a higher incidence of a hematuric onset; C3 nephritic factor was also more frequent in the children. During a follow-up period of two to 21 years (mean eight years), only seven patients (five with type I and two with type II) showed clinical remission, whereas 38 percent of patients with type I and 49 percent of patients with type II died or required dialysis; a further 23 percent of patients with type I and 16 percent of patients with type II had continuing disease and reduced glomerular filtration rate. Only the presence and persistence of a nephrotic syndrome in type I predicted renal failure. In both types, the presence of sclerosis or crescents in the initial renal biopsy specimen was associated with a poorer prognosis, but no other feature was of major prognostic value.

To assess the effectiveness of the surgical correction of vesico-ureteric reflux, current renal function was determined in 56 children with scarred kidneys who had undergone ureteric reimplantation between 1978 and 1983. The children were aged between 2 and 15 years, had no coexisting urological disease and a glomerular filtration rate (GFR) of less than 90 ml/min/1.73 m2. In the 32 patients who had bilateral reimplantation of ureters draining bilaterally scarred kidneys the improvement in GFR following surgery was highly significant (P less than 0.001), with improvement occurring in 75%. The greatest improvement was in patients where the GFR was less than 50 ml/min/1.73 m2 (29%). Individual kidney GFR was estimated in 42 kidneys and 81% improved after surgery. This improvement was highly significant (P less than 0.001).

We investigated 47 children in whom vesicoureteric reflux was clinically suspected by both conventional radiological and indirect radionuclide cystography. Comparison of the two methods showed no significant difference in their accuracy in detecting vesicoureteric reflux. The advantages and disadvantages of, and indications for, indirect radionuclide cystography are discussed.

A 9-year-old boy with a clinical illness similar to juvenile rheumatoid arthritis was found to have rubella virus in the synovial fluid. There was complete remission of symptoms after 3 months. The role of rubella virus as a possible possible aetiological agent in juvenile rheumatoid arthritis is discussed.