How should we classify intersex disorders?

The term disorders of sex development (DSD) has achieved widespread acceptance as replacement for the term intersex, but how to classify these conditions remains problematic. The LWPES-ESPE (Lawson Wilkins Pediatric Endocrine Society and European Society of Paediatric Endocrinology) Consensus Group proposed using the karyotype as a basis for classification; however, this is but a crude reflection of the genetic makeup, is diagnostically non-specific, and is not in itself relevant to subsequent clinical developments. The historical classification of intersex disorders based on gonadal histology is currently out of favor, being tainted by association with the terms hermaphroditism and pseudohermaphroditism.

Continent cutaneous urinary diversion in children: experience with Charleston pouch I.

Purpose: Complete continent urinary diversion not incorporating the bladder is not commonly used in children. We evaluated the short and long-term outcome of a form of continent cutaneous urinary diversion (Charleston pouch I) in children.

Materials And Methods: A total of 17 children underwent Charleston pouch I continent cutaneous urinary diversion between 1988 and 2005.

Does deflux alter the paradigm for the management of children with vesicoureteral reflux?

The recent approval by the US Food and Drug Administration of Deflux (Q Med, Uppsala, Sweden), a particulate biodegradable polymer of dextran, has led to an understandable enthusiasm for treating children with vesicoureteral reflux by a routinely simple outpatient endoscopic procedure. However, Deflux is but one of a variety of particulate substances, both permanent and absorbable, that have been used to treat reflux in Europe for well over a decade with varying degrees of success. The purpose of this review is to place Deflux in this wider context to anticipate what can be expected realistically regarding the efficacy and potential hazards of this seductive technique and to make recommendations regarding its incorporation, in selected cases, in our therapeutic armamentarium.

Appendicovesical fistula in a girl with cystic fibrosis.

This report describes a teenage girl with cystic fibrosis in whom appendicitis developed complicated by appendicovesical fistula. This is a rather uncommon complication in the modern era.

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.

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.

Congenital renal arteriovenous malformations in infancy. The imaging features in two infants with hypertension.

Two infants with systemic hypertension were found to have congenital renal arteriovenous fistulas. The ultrasound, computed tomography, and angiographic features are presented. The hypertension subsided following nephrectomy in both patients.

Pathogenesis of adenocarcinoma complicating ureterosigmoidostomy. Experimental observations.

Columnar metaplasia, dysplasia, and adenocarcinoma occurred in patches of bladder epithelium exposed to the fecal stream in rats. This suggests that such tumors complicating ureterosigmoidostomy may arise from ureteric epithelium, and that urine is not essential for their development.

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.

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.

The AS 800 artificial urinary sphincter in children with myelodysplasia. Preliminary results.

Ten children with myelodysplasia and intractable urinary incontinence have been implanted with an AS 800 artificial sphincter. Eight remain dry and 2 are improved over a follow-up period of 12 - 14 months. However, most have shown a reduction in bladder compliance, which suggests that further surgery may be necessary to maintain continence.

Upper moiety pelviureteric obstruction in infant with Turner syndrome.

Upper moiety pelviureteric obstruction is reported in a duplex kidney of an infant with the Turner syndrome. An operative procedure for the correction of this anomaly is described.

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.

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.

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.

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.

Posterior urethral valve masquerading as the prune belly syndrome.

Four infants are presented who in addition to having a posterior urethral valve showed striking clinical and radiological features usually associated with the prune belly syndrome. Evidence is presented which suggests that these two conditions may coexist.