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https://www.readbyqxmd.com/read/28534165/-the-german-research-network-cure-net-a-benefit-for-patients-with-rare-diseases
#1
REVIEW
A-K Ebert
With the help of the media, there is growing public awareness for the problems associated with rare diseases and their impact on the lives of those affected and their families. Bladder exstrophy-epispadias complex (BEEC) is also a part of the group of rare diseases within the urological field. The German network CURE-Net was founded in 2009 to systematically collect data regarding the epidemiological and molecular causes, and clinical and psychosocial effects of congenital urorectal malformations. With the help of self-help groups a national registry could be established for systematic data retrieval...
May 22, 2017: Der Urologe. Ausg. A
https://www.readbyqxmd.com/read/28505946/re-clinical-pathway-for-early-discharge-after-complete-primary-repair-of-exstrophy-and-epispadias-by-using-a-spica-cast
#2
Douglas A Canning
No abstract text is available yet for this article.
June 2017: Journal of Urology
https://www.readbyqxmd.com/read/28481793/in-vivo-demonstration-of-psma-expression-in-adenocarcinoma-urinary-bladder-using-68ga-psma-11-pet-ct
#3
Shambo Guha Roy, Girish Kumar Parida, Sarthak Tripathy, Abhinav Singhal, Madhavi Tripathi, Chandrasekhar Bal
In vitro and in vivo studies have demonstrated prostate-specific membrane antigen (PSMA) expression in various malignant and benign tumors. Based on the recent immunohistochemical study showing PSMA expression in adenocarcinoma of urinary bladder, we hypothesized that PSMA expression in adenocarcinoma of urinary bladder can be demonstrated in vivo using Ga-PSMA 11 PET/CT. We present a man with exstrophy bladder, presenting with adenocarcinoma urinary bladder referred for staging PET/CT. Both F-FDG and Ga-PSMA-11 PET/CT were done, which showed PSMA expression in the primary tumor as well as metastatic lymph nodes...
May 6, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28467691/quality-of-life-amongst-women-in-bangladesh-following-ileal-conduit-urinary-diversion-operations-for-irreparable-vesicovaginal-fistula-and-bladder-exstrophy-observational-study
#4
Sarah H Walker, Beatrice Ambauen-Berger, Stacy L Saha, Sayeba Akhter
OBJECTIVE: To determine the quality of life (QOL) amongst fistula patients in Bangladesh, who have undergone Ileal Conduit (IC) urinary diversion operations, and to assess whether the risks and ethical challenges involved outweigh the reality of leaving a woman with urinary incontinence for the rest of her life. DESIGN: Observational study. SETTING: LAMB Hospital, Bangladesh. POPULATION: 17 women who had undergone IC between February 2012 and March 2016...
May 3, 2017: BJOG: An International Journal of Obstetrics and Gynaecology
https://www.readbyqxmd.com/read/28443240/repair-of-vesicocutaneous-and-urethrocutaneous-fistulae-with-rectus-muscle-flap-in-a-bladder-exstrophy-patient
#5
Daniel A Friedlander, Kathy M Lue, Jason E Michaud, John P Gearhart, Richard J Redett, Heather N Di Carlo
Urinary continence can be achieved in up to 90% of patients with bladder exstrophy. However, select patients remain incontinent despite modern reconstruction. Repeat operations for continence combined with the congenital pelvic abnormalities of exstrophy put patients at risk for urinary fistula formation. We report the use of a rectus muscle flap in the repair of two concomitant vesicocutaneous and urethrocutaneous fistulae in a patient with classic bladder exstrophy.
July 2017: Urology Case Reports
https://www.readbyqxmd.com/read/28436540/early-diagnosis-of-bladder-exstrophy-quantitative-assessment-of-a-low-inserted-umbilical-cord
#6
Michal Fishel-Bartal, Sharon Perlman, Baruch Messing, Ron Bardin, Zvi Kivilevitch, Reuven Achiron, Yinon Gilboa
OBJECTIVES: Bladder exstrophy is a rare severe congenital malformation. Early prenatal diagnosis is scarcely described in the literature. Low insertion of the umbilical cord is a constant anatomic feature of bladder exstrophy. The aim of our study was to assess whether early measurements of the umbilical cord insertion-to-genital tubercle length may serve as quantitative measurements for a low-inserted umbilical cord in cases of bladder exstrophy. METHODS: The umbilical cord insertion-to-genital tubercle length was prospectively measured in all cases referred for a nonvisualized urinary bladder before 18 weeks' gestation...
April 24, 2017: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
https://www.readbyqxmd.com/read/28401037/female-epispadias-presenting-as-urinary-incontinence
#7
Asmir Jonuzi, Nusret Popovic, Zlatan Zvizdic, Emir Milišic, Kenan Karavdic, Dewan Paddy
Isolated female epispadias without bladder exstrophy is a rare congenital anomaly affecting 1 in 484,000 females. The presenting features of female epispadias are urinary incontinence and abnormal anatomical features. A 6-year-old girl presented with primary urinary incontinence who on physical examination had a bifid clitoris and labia minora. The vagina and hymen were normal. Voiding cystourethrogram showed no reflux and a funnel shaped proximal urethra. With the diagnosis of isolated female epispadias, one-stage reconstruction of the urethra, bladder neck, labia minora and clitoris was performed...
March 2017: APSP Journal of Case Reports
https://www.readbyqxmd.com/read/28372999/commentary-to-short-term-outcomes-of-the-multi-institutional-bladder-exstrophy-consortium-successes-and-complications-in-the-first-two-years-of-collaboration
#8
John W Brock, Anne Dudley
No abstract text is available yet for this article.
March 16, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28371167/long-term-sexual-health-outcomes-in-men-with-classic-bladder-exstrophy
#9
Timothy S Baumgartner, Kathy M Lue, Pokket Sirisreetreerux, Sarita Metzger, Ross G Everett, Sunil S Reddy, Ezekiel Young, Uzoma A Anele, Cameron E Alexander, Nilay M Gandhi, Heather N Di Carlo, John P Gearhart
OBJECTIVES: To identify the long-term sexual health outcomes and relationships in men born with classic bladder exstrophy (CBE). MATERIALS AND METHODS: A prospectively-maintained institutional database comprised of 1248 patients with exstrophy-epispadias was utilized. Male patients 18 years or older with CBE were included. A 42-question survey was designed utilizing a combination of demographic information and previously validated questionnaires. RESULTS: A total of 215 men inclusion criteria, of which 113 (53%) completed the questionnaire...
March 31, 2017: BJU International
https://www.readbyqxmd.com/read/28314702/short-term-outcomes-of-the-multi-institutional-bladder-exstrophy-consortium-successes-and-complications-in-the-first-two-years-of-collaboration
#10
J G Borer, E Vasquez, D A Canning, J V Kryger, A Bellows, D Weiss, T Groth, A Shukla, M P Kurtz, M E Mitchell
INTRODUCTION/BACKGROUND: Bladder exstrophy is a rare diagnosis that presents major reconstructive challenges. To increase experience and proficiency in the care of bladder exstrophy (BE), the Multi-Institutional BE Consortium (MIBEC) was formed, with a focus on refining technical aspects of complete primary repair of bladder exstrophy (CPRE) and subsequent care. OBJECTIVE: Outcome measures included successful CPRE (absence of dehiscence), complications, and integrated points of technique and care over the short-term...
February 20, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28277470/patient-reported-impact-of-pelvic-organ-prolapse-on-continence-and-sexual-function-in-women-with-exstrophy-epispadias-complex
#11
Ross G Everett, Kathy M Lue, Sunil S Reddy, Daniel A Friedlander, Cameron E Alexander, Ezekiel E Young, Mahmoud Abdelwahab, Nilay M Gandhi, Edward J Wright, John P Gearhart
OBJECTIVE: This study aimed to characterize long-term urogynecologic issues of women with a history of bladder exstrophy and pelvic organ prolapse (POP) and to assess the impact of POP repair on continence and sexual function. DESIGN: Patient demographics and surgical history related to exstrophy and POP were collected through chart review. Patient perceptions regarding sexual function, urinary continence, and quality of life were assessed through Web-based administration of validated questionnaires: International Consultation on Incontinence Questionnaire-Urinary Incontinence Short Form and POP-Urinary Incontinence Sexual Questionnaire...
March 9, 2017: Female Pelvic Medicine & Reconstructive Surgery
https://www.readbyqxmd.com/read/28277359/vaginal-delivery-after-d%C3%A3-hrssen-incisions-in-a-patient-with-bladder-exstrophy-and-uterine-prolapse
#12
Ruben Lachica, Yen Chan, Kristen R Uquillas, Richard H Lee
BACKGROUND: Bladder exstrophy is a rare congenital anomaly affecting the lower abdominal wall, pelvis, and genitourinary structures. Pregnant women with bladder exstrophy present a unique challenge to the obstetrician. CASE: The patient is a 35-year old pregnant woman with bladder exstrophy, an extensive surgical history, and uterine prolapse with an abnormal, rubbery consistency to her cervix. Prenatally, she was counseled on the potential use of Dührssen incisions to facilitate vaginal delivery...
April 2017: Obstetrics and Gynecology
https://www.readbyqxmd.com/read/28262538/bladder-perforation-after-augmentation-cystoplasty-determining-the-best-management-option
#13
Ted Lee, David J Kozminski, David A Bloom, Julian Wan, John M Park
INTRODUCTION: Delayed bladder perforation is a well-described complication after augmentation cystoplasty. Although the frequency, risk factors, and diagnostic challenges are well documented, discussions regarding management strategies are sparse. OBJECTIVE: We evaluated our experience of managing augmented bladder perforation to interrogate the hypothesis that non-operative management can be used effectively. STUDY DESIGN: We retrospectively evaluated the management of 10 patients with augmented bladder perforations over a 16-year period (Jan 2000-Jan 2016)...
February 10, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28262536/challenges-in-salvaging-urinary-continence-following-failed-bladder-exstrophy-repair-in-a-developing-country
#14
Moneer K Hanna, Ibrahim Bassiouny
INTRODUCTION: The management of urinary incontinence following failed bladder exstrophy repairs is not well defined, some surgeons recommend urinary diversion, others would pursue reconstructive surgery. OBJECTIVE: Herein we review our experience with various strategies for management of urinary incontinence and their outcome in 61 patients born with bladder exstrophy who failed their initial repairs. We also examine the impact of diverse factors in making the surgical decision for patients with limited resources in a developing country...
January 29, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28254239/psychosexual-development-management-of-bladder-exstrophy-epispadias-in-complex-patients
#15
M Di Grazia, S Pellizzoni, L G Tonegatti, W Rigamonti
INTRODUCTION: Bladder-exstrophy-epispadias complex (BEEC) represents a spectrum of urogenital step-wise malformations: epispadias, complete exstrophy, and cloacal exstrophy. Psychosexual development in adolescent patients with BEEC may become especially problematic. At present, there are few contributions in the literature investigating the validity of psychosexual treatment in order to tackle this particularly emotional and personal development phase. OBJECTIVE: The study aimed at verifying the efficacy of an intervention methodology for psychosexual support of a group of adolescents with BEEC...
June 9, 2016: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28247629/-percutaneous-nephrolithotripsy-in-patients-after-different-methods-of-urinary-diversion
#16
B G Guliev, E O Stetsik
The study presents the results of percutaneous nephrolithotripsy (PNL) in patients who underwent different methods of urinary diversion. MATERIALS AND METHODS: The study included the results of PNL in 16 patients (study group) with kidney stones who were subjected to different methods of urinary diversion. Earlier, 2 (12.5%) patients underwent bilateral ureterosigmoidostomy for bladder exstrophy, 12 (75%) experienced radical cystectomy with orthotopic ileocystoplasty and 2 (12.5%) - heterotopic cystoplasty...
August 2016: Urologii︠a︡
https://www.readbyqxmd.com/read/28209547/pregnancy-after-urinary-diversion-at-young-ages-risks-and-outcome
#17
Nina Huck, S Schweizerhof, P Honeck, A Neisius, J W Thüroff, R Stein
OBJECTIVE: To assess the urologic and obstetric outcomes during and after pregnancy following urinary diversion (UD) performed during childhood or adolescence. MATERIALS AND METHODS: From our UD database, we identified 25 women who became pregnant between 1981 and 2013. Reasons for UD were neurogenic bladder, exstrophy, trauma, sinus urogenitalis, and interstitial cystitis. Seventeen had continent cutaneous diversion, 4 had continent anal diversion, and 4 had colonic conduit...
February 14, 2017: Urology
https://www.readbyqxmd.com/read/28208931/pseudo-exstrophy-of-bladder-with-unilateral-renal-agenesis-a-rare-combination-of-two-anomalies
#18
Abdolhamid Amouei, Mohammad Baghi Yazdi, Fatemeh Ehsani, Mojtaba Babaei Zarch
Pseudo-exstrophy of bladder is an uncommon condition characterized by the major musculoskeletal defects without urinary system defects. A two-day-old female neonate was presented with pseudo-exstrophy of the bladder and unilateral renal agenesis- A rare combination of two anomalies. She was born at 37 weeks gestational age with caesarean section delivery. The X-ray of hip revealed pubic diastasis. Ultrasonography clearly showed absence of the left kidney with a normal right kidney. The patient was treated successfully with proper surgical management...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28208572/re-impact-of-concomitant-hernia-repair-at-the-time-of-complete-primary-repair-of-bladder-exstrophy
#19
Douglas A Canning
No abstract text is available yet for this article.
March 2017: Journal of Urology
https://www.readbyqxmd.com/read/28208571/re-peri-operative-transfusion-risk-in-classic-bladder-exstrophy-closure-results-from-a-national-database-review
#20
Douglas A Canning
No abstract text is available yet for this article.
March 2017: Journal of Urology
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