Results of a vesicoureteral reflux practice pattern survey among American Academy of Pediatrics, Section on Pediatric Urology members.

PURPOSE: We evaluated practice patterns among urologists managing vesicoureteral reflux, and identified areas of consensus and controversy requiring further research. MATERIALS AND METHODS: A 1-page questionnaire addressing various aspects of reflux management, including screening, diagnosis, treatment and followup, was mailed to a cohort of 225 practicing urologists identified by the membership roster of the American Academy of Pediatrics, Section on Pediatric Urology. Coded returned mailers were blindly analyzed and tabulated by 2 reviewers. Questionnaires were subdivided using a coding system by decade (1970s or earlier, 1980s and so forth) of participant membership in the American Urological Association. Select questions were analyzed to determine whether time of training affected the response. RESULTS: Of 225 surveys 155 were returned and usable (overall 60.8% response rate). General agreement exists regarding several important points in the management of vesicoureteral reflux, including evaluation of patients at the initial urinary tract infection (78.1% of respondents), use of voiding cystourethrography and ultrasound at the initial evaluation (99.4 and 96.8%, respectively), and evaluation of sibling reflux (83.2%). Certain absolute and relative indications for surgical intervention require further clarification. Various reimplantation techniques are used today, stents are not placed by the majority of practitioners and significant variations exist in the duration of postoperative hospital stay. CONCLUSIONS: Today there is substantial consensus among urologists on many issues regarding vesicoureteral reflux management. However, the areas of controversy will only be clarified by further scientific and clinical outcomes based research.

Postoperative pain following outpatient pediatric urologic surgery: a comparison of anesthetic techniques.

OBJECTIVES: To evaluate and compare the efficacy of caudal and local nerve blocks in preventing pain following outpatient pediatric urologic surgery. METHODS: Seventy-seven boys undergoing outpatient urologic surgery were randomized into one of three groups: general anesthesia alone, general anesthesia and caudal nerve block, or general anesthesia and local nerve block. Pain scores, frequency of medication, and total dosage of medication were recorded in the immediate postoperative period. Long-term pain and discomfort were measured using a 24-hour and 1-week questionnaire. RESULTS: The caudal and local groups had lower pain scores and significantly lower medication requirements than the control group in the short-term postoperative period. The caudal group had lower pain scores than the local group in all categories; however, the only significant difference was at 24 hours postoperatively. At 1 week postoperatively, the only difference among the three groups was a lower pain score in the caudal group compared with the control group. CONCLUSIONS: This study demonstrates that for outpatient pediatric urologic procedures, both caudal and local nerve blocks significantly reduce pain and medication given in the immediate postoperative period and indicates that caudal block may decrease pain for up to 1 week following surgery.

Erosion of an inflatable penile prosthesis reservoir into the bladder, presenting as bladder calculi.

We report a case of erosion of an inflatable penile prosthesis reservoir int the bladder 4 years after implantation for organic impotence. In contrast to previously reported cases this complication was not associated with incontinence, urinary tract infection or a revision procedure. Microscopic hematuria was present but there was no pyuria. Diagnosis evaded radiographic and cystoscopic confirmation. Bladder erosion of the reservoir was discovered incidentally during treatment of bladder calculi.

Vitamin D resistance and alopecia: a kindred with normal 1,25-dihydroxyvitamin D binding, but decreased receptor affinity for deoxyribonucleic acid.

A new kindred exhibiting vitamin D resistance and alopecia is described. Clinically, three of seven sisters demonstrated rickets, hypocalcemia, elevated serum 1,25-dihydroxyvitamin D3 [1,25-(OH)2D3] levels, and alopecia. Biochemical analysis of cultured fibroblasts from skin biopsy explants in two affected and one normal sister revealed normal [3H]1,25-(OH)2D3 binding to receptors (Kd = 0.05 nM; Nmax = 30-50 fmol/mg protein). Despite normal steroid binding, cells from the two affected sisters failed to respond to 1,25-(OH)2D3 in vitro, as measured by induction of the enzyme 25-hydroxyvitamin D-24-hydroxylase. The cells from the normal sister showed a response within the range of five normal cell lines. Sucrose gradient analysis yielded a typical 3.2S protein under high salt conditions in extracts from the three siblings, but with reduced capacity to aggregate to a 6S moiety in low salt gradients in the two affected cells. Whole cell [3H]1,25-(OH)2D3 binding studies revealed nearly normal localization of bound receptor to the nuclear compartment. Elution of bound receptors by KCl gradients from both DNA-cellulose or fibroblast nuclei demonstrated that the receptors from the affected sisters exhibited decreased affinity for DNA compared to those from normal subjects. We conclude that 1,25-(OH)2D3 receptors from these resistant fibroblasts have a normal steroid-binding domain, but a defective nuclear binding domain. We believe that this abnormality may be responsible for the vitamin D resistance observed both in vivo and in vitro.

Precocious puberty and hypothalamic hamartoma.

A 3-year-old boy with true precocious puberty and hamartoma of the hypothalamus is described. Preliminary diagnosis was established by the presence of unusually advanced puberty and skeletal age without other evidence of central nervous system dysfunction, elevated blood gonadotropin and testosterone concentrations, and positive computed tomography scans. Pneumoencephalography further delineated the tumor. Hypothalamic-pituitary-gonadal and adrenal functions were studied. Release of gonadotropins after the injection of synthetic luteinizing hormone-releasing hormone and the suppression of luteinizing hormone and of testosterone by oral fluoxymesterone were comparable to maximal responses of normal men. The diagnosis was confirmed by histologic and electron microscopic study of an excised portion of the tumor. Immunofluorescence studies indicated the presence of luteinizing hormone-releasing hormone in the hamartoma and radioimmunoassays detected thyroid-stimulating hormone-releasing hormone and somatostatin. The hamartoma resembles an autonomously functioning accessory hypothalamus. The ectopic neuroendocrine pathways, however, are responsive to hormonal stimulation and feedback. The clinical, endocrinologic, and pathologic features of published cases of precocious puberty and hypothalamic hamartoma are reviewed. Advanced or rapidly progressive true precocious puberty in the very young with elevated concentrations of blood gonadotropins and gonadal steroids and positive pneumoencephalography appear to be characteristic.

Chloride losing diarrhoea and metabolic alkalosis in an infant with cystic fibrosis.

A case of hypochloraemic metabolic alkalosis in an infant with chloride losing ileostomy drainage and cystic fibrosis is described. It is speculated that intestinal loss of chloride played a major role in the development of metabolic alkalosis.