Overview of a paediatric renal transplant programme.

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.

The clinical course of hepatitis B virus-associated nephropathy.

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 Lesch-Nyhan syndrome--an under-recognised condition in South Africa? A case report.

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.

Renal venous thrombosis in infancy.

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.

Dipstick screening for urinary tract infection.

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.

Bilateral renal malakoplakia in infancy.

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.

Membranous glomerulonephropathy in childhood.

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.

Calculation of glomerular filtration rate in infants and children. The use of one- and two-compartment models.

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.

Hepatitis B virus associated membranous glomerulonephritis.

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.

The bromide partition test and CSF adenosine deaminase activity in the diagnosis of tuberculosis meningitis in children.

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.

Nephrocalcinosis in children.

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.

Oxalosis of bone.

Skeletal survey in an 8-year-old girl with primary hyperoxaluria and in chronic renal failure revealed wide translucent metaphyseal bands and defects at the ends of the long bones. Similar disordered bone growth was seen as rims of rarefaction around the large epiphyses, the patella and the tarsal bones. Bone density was generally increased.

The radioactive bromide partition test in the diagnosis of tuberculous meningitis in children.

The radioactive 82Br partition test was performed on 83 children with suspected tuberculous meningitis. Three of 51 patients with a final diagnosis of TBM had a false negative serum to CSF bromide partition ratio of more than 1.6.

Congenital neurosyphilis and juvenile paresis: a forgotten entity?

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.

Spontaneous recanalization of renal artery stenosis in childhood Takayasu arteritis: A case report.

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.

Dissolution of bilateral staghorn cystine renal calculi.

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.

Chlorambucil therapy in childhood nephrotic syndrome.

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.

The nephrotic syndrome in families.

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.

Takayasu arteritis and renovascular hypertension in childhood.

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.

Familial vesico-ureteral reflux.

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.