Search PubMed⌕ Search

Biomedical subjects

J Wiggelinkhuizen

Publications and source records attributed to J Wiggelinkhuizen.

At least 19 recordsLinked to original sources
PubMed1992

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. The expected incidence of this disease is much higher than the known number of cases diagnosed.

Athetosis↗
PubMed1989

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. 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.

Humans↗
PubMed1988

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. 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.

Adolescent↗
PubMed1987

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 20.9% 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.

Black People↗
PubMed1986

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.

Adolescent↗
PubMed1983

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. 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.

Antigen-Antibody Complex↗
PubMed1982

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. 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.

Adenosine Deaminase↗
PubMed1982

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 translucent metaphyseal bands were separated from the diaphyses by an irregular line of increased bone density. Biopsy of a bone defect showed massive deposition of oxalate crystals indicative of advanced oxalosis of bone.

Bone Diseases↗
PubMed1982

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.

Acidosis, Renal Tubular↗
PubMed1980

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. Four of the 32 patients in whom TBM was excluded had a false positive ratio of less than 1.6; three of these had sever purulent bacterial meningitis. The test distinguishes TBM from viral meningitis. Antituberculous therapy does not affect the diagnostic value of the bromide partition test in the first few weeks of treatment of TBM.

Bromides↗
PubMed1980

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.

Child↗
PubMed1979

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. 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.

Adolescent↗
PubMed1979

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.

Child↗
PubMed1979

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.

Child↗