Timing of orchiopexy in the United States: a quality-of-care indicator.

OBJECTIVE: To investigate whether orchiopexies are occurring later than recommended by American Academy of Pediatrics 1996 guidelines (around age 1). Adherence to guidelines is poorly studied. METHODS: The main cohort consisted of 4103 boys insured from birth (Innovus i3, insurance claims database). The complementary cohort consisted of 17 010 insured and noninsured boys (Pediatric Health Information System, PHIS). The inclusion criteria was age ≤ 5 years at time of International Classification of Disease, 9th revision-defined cryptorchidism diagnosis. The primary outcome was timely surgery (orchiopexy by age 18 months). RESULTS: In Innovus, 87% of boys who underwent an orchiopexy had a timely orchiopexy. Of those who did not undergo surgery (n = 2738), 90% had at least 1 subsequent well-care visit. Those who underwent timely surgery were referred to a surgeon at a younger age compared with those who underwent late surgery (4.1 vs 16.1 months, P < .00005). Predictors of timely surgery were number of well-care visits (odds ratio 1.5, 95% confidence interval 1.3-1.7), continuity of primary care (odds ratio 1.9, 95% confidence interval 1.3-2.7), and use of laparoscopy (odds ratio 4.5, 95% confidence interval 1.4-14.9). Family/internal medicine as referring provider was predictive of delayed surgery (odds ratio 0.5, 95% confidence interval 0.3-0.8). In the Pediatric Health Information System, 61% of those with private insurance had timely surgery compared with 54% of those without private insurance (P < .0001). CONCLUSION: We found an unexpectedly high adherence to guidelines in our continuously insured since birth Innovus population. Primary care continuity and well-care visits were associated with timely surgery. Further studies can confirm these findings in nonprivately insured patients with the ultimate goal of instituting quality improvement initiatives.

Virilization of a female infant by a maternal adrenocortical carcinoma.

OBJECTIVE: To describe a possible mechanism underlying the partial virilization of a 46, XX infant by a functional maternal adrenocortical carcinoma (ACC). METHODS: We performed immunocytochemical staining of tumor sections for luteinizing hormone (LH)/human chorionic gonadotropin (hCG) receptors. In addition, related reports in the literature are discussed. RESULTS: A previously healthy mother developed a large cortisol- and androgen-producing stage III adrenal tumor that did not interfere with conception or early morphogenesis. The tumor eluded detection until after delivery of a partially virilized 46, XX female infant with ambiguous genitalia. Immunohistochemical staining of tumor sections revealed overexpression of the LH/hCG receptor. Virilization of the genetically female fetus may have resulted from hCG-stimulated steroid secretion by the ACC. CONCLUSION: Because hypercortisolism and hyperandrogenism are associated with menstrual disturbances and spontaneous abortion, pregnancy in patients with functional adrenal tumors is uncommon. Rarely, maternal steroid excess from a functional adrenal tumor has caused 46, XX disordered sex differentiation. This unusual case demonstrates the influence of hCG on the functionality of an ACC and demonstrates the rare phenomenon of virilization of a female infant by a functional maternal adrenal tumor.

Selective use of voiding cystourethrography in children undergoing renal transplant evaluation.

PURPOSE: Voiding cystourethrography is a routine component in evaluating children awaiting renal transplantation. We examined whether this assessment is necessary in children with renal failure due to dysplasia/aplasia/hypoplasia syndrome and unknown etiology, which account for up to 25% of those with renal failure requiring renal replacement therapies. MATERIALS AND METHODS: We performed an institutional review board approved, retrospective review of 191 children undergoing transplantation between 2002 and 2007. We reviewed clinical factors associated with positive findings on voiding cystourethrogram. We also reviewed cystography results in children with chronic kidney disease due to renal dysplasia and unknown etiology. RESULTS: We identified 113 boys and 78 girls who underwent renal transplantation during the study period. Pre-transplant voiding cystourethrography was documented in 108 children (57%). Predictors of positive pre-transplant results included history of hydronephrosis, urinary tract infections and renal failure due to urological causes. No pre-transplant cystogram was positive in children with renal failure due to dysplasia or unknown etiology. CONCLUSIONS: We recommend selective use of voiding cystourethrography to evaluate children awaiting renal transplantation. We continue to support performing this test in children with renal failure due to urological causes and those with a history of urinary tract infection, hydronephrosis or voiding dysfunction. In the absence of these findings children with renal failure due to renal dysplasia/aplasia/hypoplasia syndrome or unknown etiology need not undergo pre-transplant voiding cystourethrography.

Rapid antimicrobial susceptibility determination of uropathogens in clinical urine specimens by use of ATP bioluminescence.

We describe the first direct testing of the antimicrobial susceptibilities of bacterial pathogens in human clinical fluid samples by the use of ATP bioluminescence. We developed an ATP bioluminescence assay that eliminates somatic sources of ATP to selectively quantify the bacterial load in clinical urine specimens with a sensitivity of <1,000 CFU per milliliter. There was a log-log relationship between light emission and the numbers of CFU in clinical urine specimens. A clinical study was performed to evaluate the accuracy of the ATP bioluminescence assay for determination of the antimicrobial susceptibilities of uropathogens in clinical urine specimens tested in a blinded manner. ATP bioluminescent bacterial density quantitation was used to determine the inoculation volume in growth medium with and without antibiotics. After incubation at 37 degrees C for 120 min, the ATP bioluminescence assay was repeated to evaluate the uropathogen response to antibiotics. The ability of the ATP bioluminescence assay to discriminate between antimicrobial susceptibility and resistance was determined by comparison of the results obtained by the ATP bioluminescence assay with the results obtained by standard clinical microbiology methods. Receiver operator characteristic curves were used to determine the optimal threshold for discriminating between susceptibility and resistance. Susceptibility and resistance were correctly predicted in 87% and 95% of cases, respectively, for an overall unweighted accuracy of 91%, when the results were stratified by antibiotic. For samples in which the pathogen was susceptible, the accuracy improved to 95% when the results for samples with less than a 25-fold increase in the amount of bacterial ATP in the medium without antibiotics were excluded. These data indicate that a rapid bioluminescent antimicrobial susceptibility assay may be useful for the management of urinary tract infections.

Enhanced objective quantitative cystometric analysis of compliance and contractility.

OBJECTIVE: To show, for pressure-time data from cystometrography (CMG), the potential practical clinical application of automatically identified, displayed, analysed and quantified compliance and contractility, as undesirable high-pressure detrusor storage may be caused by inefficient compliance or uninhibited contractions (UNC). MATERIAL AND METHODS: Bladder contractility was measured by UNC and compliance by relaxed-state detrusor pressure (RSDP), i.e. the detrusor (bladder-abdominal) pressure with all UNC removed. Forty-one CMG examinations were used retrospectively to: (i) validate the separation and identification, by comparing the resulting separate graphs (data) of UNC and RSDP with an expanded time scale for raw vesical and rectal data; (ii) show that the separation is correct by examples; and (iii) show the potential practical utility by results for typical cases. RESULTS: Separation into RSDP and UNC was correctly identified and plotted. The examples showed the utility and four types of UNC ('high', contractions of >25 cmH(2)O of long duration; 'medium', >25 cmH(2)O of short duration; 'low', 4-25 cmH(2)O of short duration; and 'frequent', of 2-6 cmH(2)O). CONCLUSIONS: UNCs as small as 2 cmH(2)O can be detected and measured. The explicit enhanced estimate of compliance and contractility will be useful in the follow-up when comparing different patients and studies, and assist in more appropriate diagnosis and treatment. Because the treatment for bladders with poor contractility differs greatly from those with detrusor instability, the ability to reliably and accurately differentiate between these causes is important.

Detrusor pressure rise in a normal bladder.

OBJECTIVE: To determine the detrusor pressure rise of a normal bladder (P(cap,NL)) when filled to capacity. PATIENTS AND METHODS: Twenty-four patients with an apparently normal bladder, i.e. with symptoms but whose cystometrogram (CMG) showed no pathology of any kind and a near-normal pressure vs volume plot, were selected from 218 clinical CMGs. A straight line was fitted to the PV plot of these CMGs and then the P(cap,NL) calculated, e.g. by a straight line extending to the point of the normal expected cystometric bladder capacity (V(cap.NL)). Published data relative to P(cap,NL) were evaluated and compared with the values obtained. RESULTS: The mean (range) P(cap,NL) was 6 (4.5-7) cmH2O for patients with apparently normal bladders infused at the maximum physiological diuresis (MPD). This value was substantiated by three other studies of published values. The P(cap,NL) was 6 cmH2O at infusion rates of 10% of glomerular filtration rate (GFR), 10 cmH2O at 20% of GFR and 7-15 cmH2O for 3-10 times the infusion rate of 10% of GFR. CONCLUSION: P(cap,NL) is independent of age, size and sex and, at constant infusion rates of approximately MPD, will be 6. For infusion rates up to 35 mL/min, it is in the range of 6-15 cmH2O.

Left-colon antegrade continence enema (LACE) procedure for fecal incontinence.

PURPOSE: Antegrade continence enemas (ACE) are an efficacious therapeutic option for patients with fecal incontinence. The authors review their institution's experience with a variation of the Monti-Malone ACE procedure using the left colon as a source of an intestinal conduit and enema reservoir. METHODS: From 2000 to 2002, 18 patients with fecal incontinence or intractable constipation underwent left-colon ACE (LACE) procedure. Concomitant Mitrofanoff appendicovesicostomy was performed in 15 patients and bladder augmentation in 9. The majority of patients had neural tube defects. A segment of left colon was tubularized, tunneled into the muscular wall of the distal colon, and exteriorized through the left upper quadrant or midabdomen. Stomal catherization and enema installation were started one month postoperatively. RESULTS: Fifteen patients (83%) achieved fecal continence, 2 remain incontinent of stool, and 1 experienced stomal closure (mean follow-up was 24 +/- 9 months). Two patients had stomal stenosis that required revision. The mean enema volume in patient's achieving continence was 360 +/- 216 mL, and the mean transit time was 18 +/- 12 minutes. CONCLUSIONS: LACE is an efficacious procedure for fecal incontinence that can be performed safely at the time of major urologic reconstruction. Administration of enemas into the left colon has several physiologic advantages that result in predictable bowel evacuation.