The natural history of the multicystic dysplastic kidney--is limited follow-up warranted?

OBJECTIVE: Imaging of patients with multicystic dysplastic kidney (MCDK) has increased over the past three decades. This increased use of imaging has provided additional insights into the natural history of MCDK. The present study looked at this data for predictors of involution and associated anomalies. METHODS AND MATERIALS: Institutional review board approval was obtained for this retrospective study. The University of Michigan Departments of Urology and Radiology records were searched to identify unilateral MCDK patients during 1980-2012. Available clinical, radiological and surgical records were reviewed, and pertinent data were recorded. The log-rank test and a Cox proportional regression analysis were performed to identify predictors of MCDK involution. Probability of involution over time was assessed using Kaplan-Meier methodology. RESULTS: 301 unilateral MCDKs were identified; 195 (64.8%) were detected antenatally. Of the MCDKs found, 136 (45.2%) were in girls; 160 (53.2%) were right-sided. Mean size at baseline was 5.0 ± 0.2 cm (Mean ± SE). Associated abnormalities included: contralateral ureteropelvic junction obstruction (n = 10; 3.3%); contralateral ureterovesical junction obstruction/primary megaureter (n = 6; 2.0%); ipsilateral VUR (n = 21; 7.0%); contralateral VUR (n = 63; 20.1%); and renal fusion anomaly (n = 4; 1.3%). The cumulative probability of involution was: 9.8% at one year, 38.5% at five years, and 53.5% at ten years of age. Baseline MCDK size was the only significant predictor of involution at bivariate (p < 0.0001) and multivariate (p < 0.0001; HR 0.58 [95% CI: 0.49, 0.69]) analyses. No MCDK developed malignancy during the follow-up period. CONCLUSION: As many MCDKs eventually involute and the risk of associated malignancy appears to be very low, there is no absolute indication for nephrectomy. Based on the data and other recent studies, it is believed that pediatric MCDK patients with no other urologic abnormalities can safely tolerate more limited urological and radiological follow-up.

Two-Step Fowler-Stephens orchiopexy for intra-abdominal testes: a 28-year single institution experience.

PURPOSE: Two-Step Fowler-Stephens orchiopexy for high undescended testes allows for mobilization of the testicle to the scrotal position while preserving perfusion by collateral circulation after gonadal vessel ligation. Although used for decades, the long-term efficacy of this procedure has not been reported. We present our 28-year clinical experience with this technique. MATERIALS AND METHODS: We retrospectively studied a cohort of patients who underwent 2-step Fowler-Stephens orchiopexy at our institution between 1982 and 2009. Patients were excluded if either step was performed elsewhere or if followup was less than 6 months. Bivariate and multivariate analyses were performed to determine associations between clinical, surgical and anatomical factors and testicular viability at last followup. RESULTS: A total of 62 patients (79 testes) met inclusion criteria. Median followup was 3.1 years (range 0.6 to 20). Based on the most recent examination/ultrasound, 70.9% of testicles were considered to be normal, with the remainder exhibiting relative (15.2%) or complete (14.9%) atrophy. Of the 10 testes assessed at or after puberty 6 were normal and 4 showed relative atrophy. On bivariate and multivariate analyses only an open second stage approach was associated with normal testicular viability, with 69.9% of normal vs 18.9% of completely atrophic testes being managed by an open approach (p = 0.0084). CONCLUSIONS: Thought to be highly effective in short-term followup, our data suggest that 2-step Fowler-Stephens orchiopexy leads to complete testicular viability in 70.9% of cases. This viability is strongly associated with an open second step.

EUTUBS: a mnemonic for the complete endoscopic examination of the lower urinary tract.

Urethrocystoscopy is now routinely done in standard Urological practice. The availability of the flexible cystoscope for outpatient procedures has further increased the number of cystoscopists. However, there are currently no formal training schedules for urethrocystoscopy. This mnemonic has been developed to serve as a template for complete endoscopic examination of the lower urinary tract to which the cystoscopist may refer when undertaking this operation. It focuses on the common abnormalities that the endoscopist may encounter, and is not intended to be an exhaustive list of all abnormalities of the lower urinary tract. Furthermore, it is not meant to obviate the need for practical training of those wishing to carry out the procedure as part of their clinical practice.

Sales and science: changing patterns of pharmaceutical and medical device advertising in peer reviewed urology publications, 1975-2000.

PURPOSE: The effects of advertising on urological practice are controversial. We studied patterns of pharmaceutical and medical device marketing in peer reviewed urological journals in 1975 and 2000. MATERIALS AND METHODS: Pharmaceutical and medical device advertising in 1 European and 2 American peer reviewed urological journals were evaluated in 4 randomly selected issues of each journal published in 1975 and 2000, respectively. Advertising quantity and the qualitative characteristics of each advertisement were analyzed. RESULTS: We analyzed 574 advertisements in 24 issues. Advertising decreased between 1975 and 2000 based on the number of pages per issue (55.3 to 31.9, p = 0.04), number of advertisements per issue (30.4 to 17.4, p = 0.0098) and the ratio of advertising-to-scientific pages (0.399 to 0.151, p = 0.0016). Mean advertisement length was stable at 1.8 pages. The top 3 advertisers in 1975 were Eaton, Roche and Warner compared with Pfizer, AstraZeneca and Merck in 2000. Advertising for antibiotics comprised 70.3% of all pharmaceutical advertisements in 1975 but only 15.2% in 2000 (p = 0.0001), while advertising for benign prostatic hyperplasia, erectile dysfunction and hormonal therapy increased sharply. Nutritional supplement marketing increased from 0.5% of all advertisements in 1975 to 4.3% in 2000 (p = 0.0026). The incidence of advertisements citing peer reviewed literature increased from 16.7% to 33% (p = 0.0001) with a greater increase in the European than in the American journals. CONCLUSIONS: Advertising in peer reviewed urological journals has decreased since 1975 and fewer companies now market more products. Few advertisements cite the scientific literature. Better understanding of pharmaceutical marketing patterns may improve awareness of these efforts to influence physician practice.

External urethral sphincter dilation for the management of high risk myelomeningocele: 15-year experience.

PURPOSE: We investigate the long-term outcome using external urethral sphincter dilation for high risk myelomeningocele. MATERIALS AND METHODS: Since 1984 external urethral sphincter dilation was performed in 25 patients with myelomeningocele who demonstrated passive leak point pressure greater than 40 cm. H2O and/or poor bladder compliance. Mean followup from the first dilation was 8.4 years. Overall 2.4 dilations were performed per patient (range 1 to 8). Cystometrography, imaging study and continence status were evaluated retrospectively. RESULTS: Overall external urethral sphincter dilation produced durable improvements in mean leak point pressure (60.9 versus 34.4 cm. H2O), capacity (119.8 versus 233.3 ml.), initial compliance (11.5 versus 28.4 ml./cm. H2O) and terminal compliance (1.1 versus 7.7 ml./cm. H2O). Categorical analysis revealed 3 groups in terms of outcome. Group 1 consisted of 11 patients (44%) who demonstrated durable improvements in urodynamic parameters as well as preservation of the upper tracts. These patients demonstrated a 2-step compliance pattern on pre-dilation cystometrography, in which elevated leak point pressure was associated with excellent initial compliance. Group 2 consisted of 5 patients (20%) who failed to maintain safe leak point pressure and whose upper tracts deteriorated, including 4 who eventually underwent augmentation cystoplasty. This group demonstrated a 1-step hypertonicity in which elevated leak point pressure was associated with a steep pressure increase during early filling. Group 3 consisted of 9 patients (36%) who responded minimally in terms of leak point pressure reduction but whose upper tracts remained well preserved. They demonstrated a high pressure instability pattern associated with excellent baseline compliance. CONCLUSIONS: External urethral sphincter dilation provides an effective long-term solution for select high risk myelomeningocele cases. Those who demonstrate elevated leak point pressure and poor bladder compliance at the time of external urethral sphincter dilation are less likely to respond, suggesting that the bladder may have already undergone irreversible changes due to high outlet resistance. Patients who demonstrate instability patterns are less likely to respond to external urethral sphincter dilation in terms of leak point pressure reduction but the upper tracts appear to be well preserved.

A brief history of testosterone.

PURPOSE: We explore the history of testosterone in the context of medical and scientific developments. MATERIALS AND METHODS: A review of the scientific and historical literature was conducted. RESULTS: The origins and effects of testosterone have been recognized throughout the history of humankind. Hunter performed testicular transplantation experiments in 1767 while studying tissue transplantation techniques, and almost a century later Berthold linked the physiological and behavioral changes of castration to a substance secreted by the testes. Brown-Séquard gave birth to the field of organotherapy in 1889 when he announced that his auto-injection of testicular extracts resulted in rejuvenated physical and mental abilities. Steinach and Niehans expanded upon Brown-Séquard's work with rejuvenation treatments involving vasoligation, tissue grafts and cellular injections. In 1935 David et al isolated the critical ingredient in organotherapeutic treatments, testosterone. CONCLUSIONS: The effects of the powerful hormone testosterone continue to inspire research and controversy 65 years later.

Toxinological and immunological studies of capillary electrophoresis fractionated Chrysaora quinquecirrha (Desor) fishing tentacle and Chironex fleckeri Southcott nematocyst venoms.

Repeated runs of capillary electrophoresis (CE) were used to study partially-purified jellyfish nematocyst venom protein in concentrations sufficient to perform toxinological assays. Nematocyst venoms from Chironex fleckeri (Cf) and Chysaora quinquecirrha were processed. The CE eluate was divided into quadrants by scanning protein content. The fourth fraction of both jellyfish venoms, contained proteins with the smallest molecular weight components, which were responsible for the highest hemolysins and the humoral and cell-mediated immunological activity. Cytotoxic Cf lethal factor activity against human liver cells was widely dispersed throughout both venoms but more prominent in fraction 4. A V(beta) receptor human T-cell repertoire was not species-specific for either crude or fractionated jellyfish nematocyst venom.

A comparison of the toxinological characteristics of two Cassiopea and Aurelia species.

A comparison of the toxinological properties of nematocyst venoms from Old and New World Cassiopea and Aurelia species was undertaken. The cnidom of venomous Cassiopea andromeda (Ca) and Aurelia (Aa(RS)) from the Red Sea was identical to that of nonvenomous Bahamian Cassiopea xamancha (Cx) and Chesapeake Bay Aurelia aurita (Aa(CB)), respectively. A clean nematocyst preparation of Ca and both Aurelias could be obtained but algal particles could not be separated completely from the Cx nematocysts. Further purification of all four nematocyst preparations showed significant differences in the action of their protein. Only the Cassiopea had coexisting dermonecrotic and vasopermeability producing properties and Ca's hemolytic activity was associated with mouse lethality. The protein, hemolysin and phospholipase gel filtration eluant curves of Ca venom were similar. Venomous Aa(RS) actively stung lips and contained more potent mouse lethal, demonecrotic, vasopermeability plus hemolytic factors than Aa(CB). Cross reactivity of convalescent human serum obtained from patients stung by Ca and venomous Cx collected in Central America occurred. This was also observed between sera of bathers stung by Aa(RS) and stinging Aurelia which appeared in Florida during the recent El Niño year. IgG was stimulated by several nematocyst proteins since many venom subfractions tested positive at high titers against convalescent sera. T-cell proliferation of mice primed with either Aurelia venom was positive against the homologous preparation with cross reactivity to the heterologous venom. Crude venoms of both Red Sea jellyfish metabolically stimulated cultured human hepatocytes more than their New World counterparts. This data shows that considerable similarities and differences exist in the venoms of these Old and New World Cassiopea and Aurelia medusae with the Eastern species being more potent.

Nephrolithiasis associated with the ketogenic diet.

PURPOSE: The ketogenic diet has been used for treating seizure disorders for more than 70 years. Nephrolithiasis is a known complication of this diet with a reported stone rate as high as 10% but there is sparse literature detailing the nature and treatment of these stones. We report on 4 children with nephrolithiasis on this diet. MATERIALS AND METHODS: We describe stone treatment and analysis as well as metabolic and urine abnormalities in 4 children with nephrolithiasis on the ketogenic diet who presented to our institution. RESULTS: All patients were treated with shock wave lithotripsy, fluid liberalization and oral citrate. One child was ultimately withdrawn from the diet due to persistent stone formation. Analysis revealed 3 calcium and 1 ammonium urate stones. Three patients had hypercalciuria, 2 elevated urinary uric acid and 1 hypocitruria. Serum studies revealed acidosis in 3 cases. CONCLUSIONS: The ketogenic diet induces several metabolic abnormalities that increase the propensity for stone formation. Urologists should be aware of this potential complication. Fluid liberalization and bicitrate are recommended as prophylaxis.

The leech and the physician: biology, etymology, and medical practice with Hirudinea medicinalis.

The history of the word "leech" and the practice of leeching reveal interconnected social histories. We give the linguistic and medical histories of the word, and explore its biology and clinical history. Our historical account extends from the earliest known record of leeching to current research. Despite historical variation in its reputation as a therapeutic technique, leeching remains useful today in a number of applications. Further investigation may well disclose even more uses for the leech, particularly for its enzymes with anesthetic, anticoagulant, and antimetastatic properties.

Tissue engineering from Adam to the zygote: historical reflections.

Tissue engineering, long a matter of myth and dream throughout the history of medicine, is now a practical reality. A wide spectrum of biological materials are used in the field of urology to treat disease and to overcome human disabilities, including tissue grafts and organ transplantation. Laboratory-engineered bioproducts for the off-the-shelf replacement and reconstruction of tissue is now almost at hand. This article presents a glimpse into the past by highlighting a number of early pioneering works in the field of tissue transplantation and cell culture technologies.

Barometers and bladders: a primer on pressures.

PURPOSE: We develop a "consilient" (unified) view of pressure as a physical phenomenon and "clinimetric" tool, making a connection between barometers and bladders. MATERIALS AND METHODS: The philosophy, physics and clinical applications of pressure during the last 2 millennia were examined from Lucretius to the modern medical subspecialties. RESULTS: A variety of units and systems of pressure quantification developed as the physics of pressure became understood. Applications of pressure in clinical medicine with distinct physiological relevance have been created for organ systems across the subspecialties. Some measurements have become useful for management of urinary tract and other diseases. CONCLUSIONS: Despite a broad range of units, systems and applications, a consilient view of pressure in medicine can be approached. This perspective is fundamental to understanding the significance of pressures in the expanding clinimetric arena and should mitigate against misplaced concreteness that is tempting in modern medical practice, whereby laboratory tests become virtual realities and are mistaken for patients.

Caroli's disease diagnosed in a child by MRCP.

We describe a case of Caroli's disease associated with a choledochal cyst and autosomal recessive polycystic kidney disease in a child whose diagnosis was confirmed with magnetic resonance cholangiopancreatography (MRCP), after initial abnormalities were seen by ultrasonography. Invasive procedures such as liver biopsy or endoscopic retrograde cholangiopancreatography (ERCP) were, therefore, not necessary. Recent radiological advances in the diagnosis of Caroli's disease with particular emphasis on MRCP are discussed.

Delayed presentation of posterior urethral valves: a not so benign condition.

PURPOSE: Posterior urethral valves are usually detected during infancy by prenatal sonography. Rarely they may be diagnosed during later childhood, adolescence or even adulthood. Less is known about presentation and outcome in these older patients. We reviewed our experience at 4 institutions with the late presentation of posterior urethral valves. MATERIALS AND METHODS: A 13-year retrospective review revealed the late presentation of posterior urethral valves in 47 patients 5 to 35 years old (mean age 8). Data collected included presenting symptomatology, radiographic findings and renal function. Statistical analysis determined the relationships among presenting symptoms, patient age at diagnosis and renal function. RESULTS: The most common presenting symptoms were diurnal enuresis in 60% of the cases, urinary tract infection in 40% and voiding pain in 13%. Other presenting symptoms in less than 10% of the cases included poor stream, gross hematuria and proteinuria. At diagnosis hydronephrosis and vesicoureteral reflux were present in 40 and 33% of the patients, respectively, while serum creatinine was elevated in 35% and end stage renal disease had developed in 10%. The severity of presenting signs and symptoms was significantly associated with renal impairment, while patient age at diagnosis was not. CONCLUSIONS: Posterior urethral valves is not merely a disease of infancy. Voiding cystourethrography should be considered in boys older than 5 years who have voiding complaints, especially in association with diurnal enuresis or urinary tract infection. Patients who present late with posterior urethral valves are at risk for progression to end stage renal disease.