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Aims: Chronic renal failure in childhood jeopardizes both growth and development. In children with chronic renal failure from birth, growth in height and weight have been found to be generally poor. Few data on head circumference are available., Material: A cohort of 19 children with chronic renal failure from birth was studied from the early weeks of life to the age of 5 years. There were 18 boys; and 18 patients had congenital renal hypoplasia or hypodysplasia associated with obstructive uropathies. Eight patients received recombinant growth hormone (rhGH) after the age of 2 years. Only 2 patients needed renal replacement therapy before the age of 5 years. Data after transplantation were not included., Methods: The following variables were analyzed: body height, body mass index and head circumference. Data were expressed in median values of standard deviations scores (SDS)., Results: In the first 3 months of life there was a significant drop in height SDS, body mass index SDS and head circumference SDS. Thereafter, a stable growth velocity was observed for the rest of the study period, except for body mass index SDS. which improved after 36 months. There was a striking difference between patients who needed treatment or not with recombinant human growth hormone (rhGH). Patients without rhGH displayed a stable growth after the age of 3 months until 5 years of age. In the remaining 8 patients, rhGH treatment resulted in a significant increase not only in height SDS but also in head circumference SDS., Conclusions: In infants and young children with chronic renal failure from birth, growth in head circumference parallels growth in body height. This applies to all patients and to data before and during rhGH treatment.

In this study the outcome of kidney transplantation in 20 patients with end-stage renal disease (ESRD) due to the hemolytic uremic syndrome (HUS) was evaluated. The characteristics and severity of the primary disease in these patients were not different from those commonly reported in patients with HUS. Eleven children developed ESRD immediately following HUS, in nine others temporary partial recovery of kidney function for 0.5-13.2 years was observed. Twenty-four grafts were transplanted in these 20 patients. Graft survival was no different from overall cadaver graft survival. In nine patients failure of the first kidney graft occurred, while the four second grafts were still functioning at the time of writing. Recurrence of HUS was suspected but not proven to be the cause of first graft failure in two patients, and impaired graft function in a third one. A short interval between HUS and kidney transplantation seems to adversely affect graft survival. No other factors influencing outcome of transplantation could be detected.

A dynamic estimation of the complement system was obtained by immunochemical estimation of C3, C4, C5, C1q, C3b + C3c, C3d, Ba in children with hemolytic-uremic syndrome. The presence of increased breakdown products of C3 (C3b + C3c, C3d) and of factor B (Ba) suggests an activation of the complement system possibly by the alternative pathway. No definite explanation for these complement abnormalities can be given.

A 12 year-old boy was referred because of general weakness, enuresis and pallor which had been present for at least six months. Previously, the child had been hospitalized at the age of five, because of mental retardation and hepatosplenomegaly, for which no cause could be found. He had severe renal insufficiency, with all the hallmarks of nephronophthisis. In addition his vision was very poor and fundoscopy revealed tapetoretinal degeneration. The liver and spleen were grossly enlarged. Liver function was almost completely normal, but histology showed diffuse periportal febrosis with profiferation of the bile ducts. This observation seems to confirm the existence of a new syndrome, associating nephronophthisis and liver fibrosis as described by Boichis and coworkers (1973).

A 16-year-old girl with primary oxalosis type I presented with progressive claudication soon after being treated with chronic intermittent hemodialysis. Arterial insufficiency of the lower limbs was confirmed clinically (purple discoloration of the skin and absence of arterial pulses) and with Doppler sonography. The arteriogram showed diffuse and symmetric narrowing with smooth vessel walls. Treatment with sodium nitroprusside had a spectacular effect; nifedipine was less effective. Renal transplantation with the father's kidney resulted in a rapid, complete and sustained reversal of the ischemic features. Magnesium withdrawal is assumed to be a pathogenic factor of the vascular spasm in this patient.

In a child with the hemolytic uremic syndrome, plasma 6 keto-prostaglandin F1 alpha levels remained undetectable throughout the acute phase of the disease. The patient's plasma failed to stimulate prostacyclin production by "exhausted" rat aorta rings. In vitro study of the patient's vessels indicated that they retained the capacity to synthesize prostacyclin from exogenous arachidonic acid but that their endogenous arachidonic acid stores were either depleted or non-available. The response to repeated infusion of exogenous prostacyclin was equivocal, suggesting that abnormal prostacyclin metabolism in the hemolytic uremic syndrome may not be the only factor in its pathogenesis.

Forty-six patients who developed a Hemolytic Uremic Syndrome (HUS) during the period 1970-1976, were examined ten years later. Thirty-two individuals had no signs of renal disease whereas fourteen showed at least one abnormality. In the latter group a urinary osmolality below 800 mosmole per kg water was the most frequent defect found (eight cases). Three adolescents had both hypertension and proteinuria, which are considered as important late sequelae.

Three children with nephropathic cystinosis received cysteamine therapy, mostly in the form of phosphocysteamine, for more than six years. The patients were between two and three years of age at the start of the study. The daily dose of cysteamine was 60 mg/kg as cysteamine base. In all three, rapidly progressive renal failure occurred before their 10th birthday. When comparing their evolution with data on the natural history of childhood cystinosis, no improvement was observed in terms of growth and glomerular function. It is concluded that cysteamine therapy did not provide clear benefit to the three patients reported here.

A 7-year-old boy experienced recurrent attacks of acute renal failure over an 18 month period. Each attack was accompanied by a fluorescent yellow discoloration of his urine. Laboratory data and the kidney biopsy were consistent with acute tubulo-interstitial nephritis. Repeated contacts with the boy in a play setting and thorough toxicologic examination of his urine finally led to the conclusion that the mother systematically poisoned her child with glafenin. The clinical and experimental literature on glafenin-related disease is reviewed. This is the first report of glafenin intoxication in a child. This is also the first case of kidney disease as an expression of child abuse.

Ultrasonography has been used to assess renal bleeding after percutaneous renal biopsy in 57 infants and children. A perirenal hematoma was found in 10 patients i.e. an incidence of 16 per cent. An additional patient with heavy hematuria and temporary obstruction of the pelvis due to clot formation had an enlarged kidney and pelvicalyceal dilatation on ultrasound examination. We did not find a correlation between the clinical findings, the fall in hematocrit and the presence of a hematoma. Ultrasound can be regarded as the method of choice for monitoring renal bleeding in children who have undergone kidney biopsy.