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Biomedical subjects

I A Aaronson

Publications and source records attributed to I A Aaronson.

At least 19 recordsLinked to original sources

Defects of the testosterone biosynthetic pathway in boys with hypospadias.

PURPOSE: We determined the incidence of defects in 3 enzymes, namely 3beta-hydroxysteroid dehydrogenase, 17alpha-hydroxylase and 17,20-lyase, on the testosterone biosynthetic pathway in boys with hypospadias. MATERIALS AND METHODS: We evaluated 30 boys with a 46,XY karyotype, fully descended testes and penoscrotal or proximal shaft hypospadias. Serum concentrations of the metabolites mediated by these enzymes were measured, from which the precursor-to-product ratios were calculated. Seven patients underwent adrenocorticotropic hormone stimulation. Findings were compared to previously published data on age matched normal boys. RESULTS: A total of 11 boys had evidence of impaired function of 3beta-hydroxysteroid dehydrogenase alone or in combination with impaired 17,20-lyase or 17alpha-hydroxylase activity. An additional 4 boys had evidence of isolated 17,20-lyase deficiency. Thus, of the 30 boys studied 15 (50%) had evidence of a testosterone biosynthetic defect. The effect of adrenocorticotropic hormone stimulation varied with widening of the precursor-to-product ratios in some boys and narrowing in others. CONCLUSIONS: A high incidence of 3beta-hydroxysteroid dehydrogenase and 17,20-lyase deficiency was found in boys with proximal hypospadias. The response to adrenocorticotropic hormone stimulation suggests that enzymes in the adrenal glands and testes may be affected independently. Our findings support the hypothesis that hypospadias is the result of fetal endocrinopathy.

17-Hydroxysteroid Dehydrogenases↗

Endoscopic treatment of reflux: migration of Teflon to the lungs and brain.

We set out to determine experimentally whether particles of polytetrafluoroethylene migrate to the lungs and brain when relatively small volumes of Teflon paste are injected into the bladder in the manner used to correct reflux. Numerous particles of polytetrafluoroethylene were recovered from these organs within 2 weeks of injection. Those in the brain measured up to 15 microns in diameter, indicating that the pulmonary bed is an inefficient filter of particles gaining access to the venous circulation. Although clinically no adverse neurological effects have hitherto been reported, this study suggests that following the 'sting' procedure, some particles may lodge in the brain where they can block the cerebral microcirculation. We believe these findings represent a contraindication to the use of Teflon paste in children.

Animals↗

Urogenital tuberculosis in children.

The clinical and radiological features of 16 children with urogenital tuberculosis are reviewed. The possibility of urinary tract involvement should be considered in all children with evidence of past or active pulmonary tuberculosis, or who are primary contacts.

Child↗

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↗

Measurement of glomerular filtration rate in children using technetium-99m diethylenetriamine penta-acetic acid.

During the past 5 years, we have measured the glomerular filtration rate (GFR) by the slope-clearance method using technetium-99m diethylenetriamine penta-acetic acid (99mTc-DTPA) in 130 infants and children. The results in 22 children have been compared with inulin clearance, and a very good correlation between the two methods of measurement of GFR was demonstrated (r = 0,9616; P less than 0,0001). This study provides further evidence that 99mTc-DTPA is a satisfactory agent for the clinical measurement of GFR in children.

Child↗

True hermaphroditism. A review of 41 cases with observations on testicular histology and function.

Thirty-six children and five adults with true hermaphroditism have been reviewed. Twenty-five were raised as males and 16 as females. Those assigned as males had all ovarian tissue excised, leaving either a single testis or the testicular portion of one or both ovotestes. Post-operative testicular function was studied in 15 males. Basal testosterone levels were low in all four post-pubertal patients. Eleven children had plasma testosterone measured before and after stimulation with HCG. Response was poor in eight and normal in only three. Although social factors in South Africa usually result in true hermaphrodites being raised as males, the functional outcome of this decision is often disappointing.

Adolescent↗

Posterior urethral valve. A review of 120 cases.

One hundred and twenty infants and children with a posterior urethral valve treated at the Red Cross War Memorial Children's Hospital, Cape Town, during the period 1961 - 1982 are reviewed. Advances in diagnosis and management, particularly of the sick infant, have led to a dramatic reduction in the early mortality--from over 40% before 1977 to zero. However, the long-term follow-up of survivors suggests that many will require renal transplantation when they reach early adulthood.

Age Factors↗

Dysplasia of ureteric epithelium: a source of adenocarcinoma in ureterosigmoidostomy?

Dysplasia was found in the terminal portion of the ureters in two children with ureterosigmoidostomy followed by routine colonoscopy. These observations provide further evidence that adenocarcinomas complicating this type of diversion may be derived from ureteric epithelium and that urine is not essential for their development.

Adenocarcinoma↗