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Abstract. Takayasu arteritis with renal artery involvement was the cause of severe persistent hypertension in eight children under 12 years of age. Features of severe hypertension dominated the clinical picture. Unequal or absent pulses were found in three patients. An irregular contour of the descending thoracic aorta on the chest roentgenogram was an early clue to the diagnosis. On aortography both renal arteries were affected in five patients, although a discrepancy in kidney size on excretory urography had suggested a unilateral lesion. Total aortography is mandatory to outline the full extent of the vascular abnormalities. All the patients had strongly positive tuberculin skin tests, and, although mycobacteria were not isolated, all patients received anti-tuberculous as well as antihypertensive therapy. Nephrectomy of the worse kidney is contraindicated if the renal arteries are asymmetrically involved, for fear of later extension of the arteritis. Takayasu arteritis is an important cause of severe persistent hypertension in nonwhite children. Pediatrics 62:209-217, 1978, renovascular hypertension, Tokayasu arteritis, renal artery stenosis. [ABSTRACT FROM AUTHOR]

SUMMARY Idiopathic arteritis of children is a widespread condition but it is most commonly reported in the'non-white races. It affects the large conducting arteries and, in childhood, the descending thoracic and abdominal aorta are mostly involved. The term Juvenile Main Vessel Arteritis is suggested rather than Takayasu's Arteritis as the cases described bear little relationship to the condition Takayasu originally reported. In non-caucasian children it may be an important cause of hypertension, ranking only second to renal parenchymal disease. [ABSTRACT FROM AUTHOR]

The incidence of persistent hepatitis B surface (HBs) antigenaemia was studied in 114 nephrotic children with glomerulonephritis. Twenty five (24 boys) of 28 cases of membranous glomerulonephritis were HBs antigen (HBsAg) carriers. Only 9 of the remaining 86 patients with nephropathies other than membranous glomerulonephritis were HBsAg positive. HBsAg immune complexes were seen in the sera by electron microscopy. On radioimmunoassay both HBsAg and antibody (anti-HBs), and HBeAg and antibody (anti-HBe) were often detected concurrently, HBsAg was not shown in the glomerular capillary wall. HBs antigenaemia persisted in 80% of patients after recovery from glomerulonephritis but remission of the proteinuria correlated well, although not fully, with seroconversion to anti-HBe. The natural history of hepatitis B virus (HBV) associated glomerulonephritis in childhood is one of slow recovery. A few patients are left with mild asymptomatic proteinuria but progressive renal failure is rare. The 14% incidence of membranous glomerulonephritis in nephrotic children in this area is much higher than that found by the international study of kidney disease in children in well developed countries and is probably related to a high HBV carrier rate. A search for HBV markers should be included in the investigation of persistent glomerulonephritis, particularly in countries with a high prevalence of HBV carriers. [ABSTRACT FROM AUTHOR]

Bilateral staghorn renal calculi in a 7-year-old girl with cystinuria were dissolved over a period of 6 months, using a high fluid intake, urinary alkalinisation, and D-penicillamine. Even in children with extensive cystine urolithiasis, medical management may avert the need for surgery. [ABSTRACT FROM AUTHOR]

Introduction: Renal transplantation is the therapy of choice for children with end-stage renal failure. There are many challenges associated with a paediatric programme in a developing country where organs are limited., Methods: A retrospective review was undertaken of 149 paediatric renal transplants performed between 1968 and 2006 with specific emphasis on transplants performed in the last 10 years. Survival of patients and grafts was analysed and specific problems related to drugs and infections were reviewed., Results: On review of the total programme, 60% of the transplants have been performed in the last 10 years, with satisfactory overall patient and graft survival for the first 8 years post transplant. At this point, transfer to adult units with non-compliance becomes a significant problem. Rejection is less of a problem than previously but infection is now a bigger issue--specifically tuberculosis (TB), cytomegalovirus (CMV) and Epstein-Barr virus (EBV) infections with related complications. A wide variety of drugs are available for tailoring immunosuppression to minimise side-effects., Conclusion: It is possible to have a successful paediatric transplant programme in a developing country. However, to improve long-term outcomes certain issues need to be addressed, including reduction of nephrotoxic drugs and cardiovascular risk factors and providing successful adolescent to adult unit transition.

Hepatitis B virus (HBV) infection is recognised as an important cause of nephrotic syndrome in endemic areas. This paper retrospectively examines the natural history and treatment of 70 patients with membranous glomerulonephritis and 1 with mesangiocapillary glomerulonephritis associated with HBV infection. Thirty-seven patients were in complete remission by the end of the study. The average duration of proteinuria in these patients was 30 months. The cumulative probability of remission was 64% at 4 years and 84% at 10 years. Three patients were still nephrotic after more than 90 months of follow-up and 2 others had reached end-stage renal failure. Remission occurred within 6 months of clearing the antigen (HBeAg) in the majority of cases. Steroids alone were given to 10 patients and 2 received steroids and cyclophosphamide, with no beneficial effect. Three patients received interferon-alpha 2b. One cleared the HBeAg from the circulation and had a significant fall in proteinuria, but defaulted from follow-up a month after completing treatment. One had a reduction of proteinuria but remained HBeAg positive. There was no change in the condition of the third. Although the majority of children eventually enter remission, there is a significant morbidity associated with the disease. Steroids and other immunosuppressive therapy are of no benefit. Interferon therapy may be useful, but has not been adequately assessed.

The Lesch-Nyhan syndrome is a rare inborn error of purine metabolism caused by a deficiency of hypoxanthine-guanine phosphoribosyltransferase (HGPRT), which results in mental retardation with characteristic self-mutilation, spasticity, extrapyramidal signs and hyperuricaemia. The clinical and biochemical findings in an 18-month-old boy, who presented with renal calculi and was shown to have less than 1% of normal HGPRT activity, are reported. The obvious neurological abnormalities had previously been thought to be due to hypoxic-ischaemic encephalopathy. The expected incidence of this disease is much higher than the known number of cases diagnosed.

In screening for urinary tract infection the leucocyte esterase test will detect almost all samples with significant pyuria and bacteriuria, but is relatively nonspecific. The nitrite test is more specific but less sensitive and about one-third of the urinary tract infections in a large group of children were missed. The combination of screening tests results in greater overall accuracy both in the diagnosis and exclusion of urinary tract infection. Almost all cases of urinary tract infection were detected when either the leucocyte esterase or the nitrite screening test or both were positive. If both tests are negative, urinary tract infection is virtually excluded and unless the child is symptomatic, further urinalysis is unnecessary. Laboratory urinalysis is, however, necessary if any one screening test for leucocyte esterase or nitrite (or protein or haemoglobin) is positive. Combined biochemical screening for urinary tract infection with dipstick test strips is reliable and allows early diagnosis and management. By avoiding unnecessary urinalysis it is cost-effective for the patient and will significantly reduce the laboratory workload.

Membranous glomerulonephropathy (MGN) in South African black and mixed race children with the nephrotic syndrome is much commoner than in First-World countries. In this survey of 388 nephrotic children MGN was found in 51.9% of black and 20.9% of mixed race boys, and 25% of black and 5.6% of mixed race girls respectively, but was not present in 53 white and Asiatic nephrotic children. Aetiological or associated factors were documented in 84%: hepatitis B virus infection in 73%, congenital syphilis in 6% and systemic lupus erythematosus, D-penicillamine toxicity and Salmonella infective endocarditis in 1 case each. The prognosis depends on the cause and is much better than for adults with idiopathic MGN. After an average follow-up period of 4.5 years the overall remission rate was 78% and mean time to remission 30 months. One patient with syphilitic MGN died 15 years later; 3 patients are in mild renal failure. Corticosteroids and other immuno-suppressive therapy were ineffective and may do harm. The frequent occurrence of MGN is related to the high prevalence of predisposing infections in the affected population groups, and socio-economic rather than ethnic factors are important.

Bromide partition tests were performed on 58 children with suspected tuberculous meningitis (TBM). CSF adenosine deaminase activity (ADA) was measured at the same time. Four of the 33 patients with a final diagnosis of TBM had false-negative bromide partition ratios and 5 had false-negative CSF ADA levels. One of the 25 patients in whom TBM was excluded had a false-positive ratio and 4 had false-positive CSF ADA levels. The difference between the two tests was not significant. Both provide valuable evidence for or against a diagnosis of TBM.

Chlorambucil (Leukeran), a cytotoxic agent, was administered to 13 children with the nephrotic syndrome who had responded to steroid therapy, but frequently relapsed. In all patients corticosteroid therapy had become unsatisfactory. Eleven patients have so far remained in remission for an average follow-up period of 31 months since chlorambucil therapy. No side-effects of therapy were observed in this study, but several grave complications of high-dosage therapy have been reported in the recent literature. A dose of 0.2 mg/kg/d for 8 weeks should not be exceeded.

Takayasu arteritis with renovascular hypertension in an 8-year-old girl is described. At the onset both renal arteries were affected, but 2 years later one renal artery had been recanalized and the uncontrolled hypertension was cured by contralateral nephrectomy.

Nephrocalcinosis is an uncommon condition is childhood. The commonest cause is renal tubular acidosis, although this may not manifest itself radiographically until adolescence. Recognizing the calcification as either cortical, medullary or mixed is not always possible, but may sometimes be an aid to differentiate metabolic from vascular causes.

Four families of which 2 or more members were affected with primary vesico-ureteral reflux are reported. A multifactorial inheritance pattern subject to environmental factors is likely. Early examination and detection of the disorder in relatives at risk provide an opportunity to avoid the serious sequelae of vesico-ureteral reflux.

Renal venous thrombosis (RVT) in infancy occurs in situations associated with reduced renal blood flow and hypercoagulability. The clinical diagnosis is based on finding enlarged kidney(s), haematuria and thrombocytopenia in a setting where the infant is at risk of RVT. Ultrasonography is the imaging modality of choice and should replace the more invasive excretory urography and venography for confirmation of the diagnosis. Impairment of renal function is best documented by radionuclide studies. Treatment is supportive with heparinisation for severe bilateral RVT and inferior vena cava thrombosis. The role of thrombectomy and fibrinolytic therapy is limited in infancy. Survival rates have much improved in recent years. Severe venous infarction leads to atrophy of the affected kidney, which may later be mistaken for congenital renal hypoplasia. RVT may be complicated by hyperreninaemic hypertension, which is curable by nephrectomy.

Juvenile neurosyphilis is rare and may be arrested or modified in the early stage by antibiotic treatment for intercurrent infections. Inadequate treatment may weaken the signs and symptoms of neurosyphilis so much that a high index of clinical suspicion is required for early and correct diagnosis. It should be remembered, however, that benzathine penicillin does not constitute adequate treatment for neurosyphilis.

Two male infants aged 6 months presented with Escherichia coli septicaemia and urinary tract infection. Despite seemingly appropriate antibiotic therapy, a swinging fever, painful enlargement of both kidneys, sterile leukocyturia and renal failure persisted. Excretory urography, ultrasound scan, computerized tomography and magnetic resonance imaging showed diffuse infiltrative disease. 99mTc dimercaptosuccinic acid uptake was minimal, but a 67Ga-citrate scan showed striking diffuse uptake by both kidneys suggestive of inflammatory cell infiltration. Bilateral renal parenchymal malakoplakia was diagnosed on tissue examination. Bilateral parenchymal involvement of the kidneys by this chronic granulomatous disease has not previously been reported in infancy and is fatal if untreated. The lesion is believed infection-induced, due to defective bacterial activity of the macrophages, possibly related to an abnormally low cGMP/cAMP ratio. Treatment with intracellularly active trimethoprim-sulphamethoxazole, rifampicin and ascorbic acid resulted in complete recovery of the one infant so treated over a period of months.

Six families, each with 2 siblings suffering from the nephrotic syndrome, are reported. The incidence, causes, clinical picture and management of the nephrotic syndrome occurring in families are discussed, as well as possible ways of preventing this syndrome.

Radiopharmaceuticals such as chromium-51-ethylenediamine tetra-acetic acid and technetium-99m-diethylenetriamine penta-acetic acid are often used to measure glomerular filtration rate. Some assumptions are inherent in the method while others are made to simplify the procedure. The assumption of a one-compartment model falls into the latter group and is an important cause of misleading results.