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Exstrophy

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https://www.readbyqxmd.com/read/29135412/surgical-repair-in-case-of-covered-exstrophy-of-bladder-with-complete-duplication-of-lower-genitourinary-tract-and-visceral-sequestration
#1
Sachin Sarode, Sunil Mhaske, Vinayak G Wagaskar, Bhushan Patil, Sujata K Patwardhan, Ganesh Gopalakrishnan
No abstract text is available yet for this article.
November 17, 2017: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/29126846/sexual-function-and-quality-of-life-in-adult-male-individuals-with-exstrophy-epispadias-complex-a-survey-of-the-german-cure-network
#2
J Traceviciute, N Zwink, E Jenetzky, H Reutter, K Hirsch, R Stein, W H Rösch, A K Ebert
OBJECTIVE: To investigate sexual function and quality of life (QoL) in adult male individuals with exstrophy-epispadias complex (EEC). Data from the German network for congenital uro-rectal malformations (CURE-Net) were used. PATIENTS AND METHODS: 51 males (≥18 years) recruited by CURE-Net between 2009 and 2012 were re-contacted per mail and asked to fill out four questionnaires including International Index of Erectile Function (IIEF-5), Cologne assessment of Erectile Dysfunction (KEED), the Short-Form 36 (SF-36) and one self-designed questionnaire about their medical history, current health status, sexual experience...
November 7, 2017: Urology
https://www.readbyqxmd.com/read/29126155/isl1-mediates-mesenchymal-expansion-in-the-developing-external-genitalia-via-regulation-of-bmp4-fgf10-and-wnt5a
#3
Saunders T Ching, Carlos R Infante, Wen Du, Amnon Sharir, Sungdae Park, Douglas B Menke, Ophir D Klein
Genital malformations are among the most common human birth defects, and both genetic and environmental factors can contribute to these malformations. Development of the external genitalia in mammals relies on complex signaling networks, and disruption of these signaling pathways can lead to genital defects. Islet-1 (ISL1), a member of the LIM/Homeobox family of transcription factors, has been identified as a major susceptibility gene for classic bladder exstrophy in humans, a common form of the bladder exstrophy-epispadias complex (BEEC), and is implicated in a role in urinary tract development...
November 8, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/29059756/re-use-of-human-acellular-dermal-matrix-during-classic-bladder-exstrophy-repair
#4
Douglas A Canning
No abstract text is available yet for this article.
November 2017: Journal of Urology
https://www.readbyqxmd.com/read/29059755/re-use-of-muscle-pedicle-flaps-for-failed-bladder-neck-closure-in-the-exstrophy-spectrum
#5
Douglas A Canning
No abstract text is available yet for this article.
November 2017: Journal of Urology
https://www.readbyqxmd.com/read/28986224/autophagy-apoptosis-and-cell-proliferation-in-exstrophy-epispadias-complex
#6
Mahsa Shabaninia, Ali Tourchi, Heather Di Carlo, John Gearhart
OBJECTIVE: To investigate the state of autophagy and its interactions with apoptosis and cell proliferation in patients who underwent successful early closure or delayed closure of exstrophy. They compared those outcomes with cell culture samples from patients with VUR as control. METHODS: Primary cultures of bladder smooth muscle cells were established from patients with successful neonatal bladder closure (group 1, N=5), delayed closure due to small bladder template (group 2, N=5) and vesicoureteral reflux as control (group3, N=5)...
October 3, 2017: Urology
https://www.readbyqxmd.com/read/28974870/preperitoneal-bladder-augmentation-feasibility-and-results
#7
Dhruva Nath Ghosh, Sampath Karl, Sudipta Sen
INTRODUCTION: Bladder augmentation is an important part of pediatric reconstructive urology. This study was conducted to assess the feasibility and results of our technique of preperitoneal bladder augmentation. MATERIALS AND METHODS: Thirty-three children underwent preperitoneal bladder augmentation for small inelastic bladders who had failed medical management or needed undiversion. The underlying diagnosis included neurogenic bladder, valve bladder, bladder exstrophy, non-neurogenic neurogenic, ectopic ureters, and urogenital sinus...
October 2017: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/28960693/clinical-and-risk-factor-analysis-of-cloacal-defects-in-the-national-birth-defects-prevention-study
#8
Kim M Keppler-Noreuil, Kristin M Conway, Dereck Shen, Anthony J Rhoads, John C Carey, Paul A Romitti
Cloacal exstrophy (CE) and persistent cloaca (PC) (alternatively termed urorectal septum malformation sequence [URSMS]), represent two major cloacal defects (CDs). Clinical characteristics and risk factors often are studied for both defects combined, rather than exploring if these defects have different etiologies. We enumerated clinical features for 47 CE and 54 PC (inclusive of URSMS) cases from the National Birth Defects Prevention Study. Thirty-three CE cases were classified as isolated and 14 as multiple (presence of unassociated major defects); respective totals for PC cases were 26 and 28...
November 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28958802/cystectomy-in-the-pediatric-exstrophy-population-indications-and-outcomes
#9
Joan Ko, Kathy Lue, Daniel Friedlander, Timothy Baumgartner, Peter Stuhldreher, Heather N DiCarlo, John P Gearhart
OBJECTIVE: To explore the long-term outcomes and indications for cystectomy in bladder exstrophy patients. Although rare, cystectomy is the final surgical alternative to bladder repair among these patients with a poor quality bladder template. METHODS: A prospectively maintained database of 1298 patients with exstrophy-epispadias complex was reviewed for patients who underwent cystectomy between 1970 and 2015 at the author's institution. Demographic data, indication for cystectomy, surgical history, post-operative outcomes, and continence status were collected...
September 25, 2017: Urology
https://www.readbyqxmd.com/read/28924534/image-of-the-month-clinical-features-in-a-newborn-with-covered-cloacal-exstrophy
#10
Alejandra Vilanova-Sánchez, Christina B Ching, Alessandra C Gasior, Karen Diefenbach, Richard J Wood, Marc Levitt
Cloacal exstrophy is the most severe type of anorectal malformations that belongs to the bladder-exstrophy-epispadias complex of genitourinary malformations. Interestingly, its variant, the covered cloacal exstrophy, is often missed. The clinical findings of this variant may include an imperforate anus, low lying umbilicus, thick pubic bone, and pubic diastasis but with an intact abdominal wall. We present an interesting case of covered cloacal exstrophy with a side-by-side duplicated bladder and discuss important considerations for the time of colostomy creation in the newborn period...
January 2017: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/28920029/exstrophy-bladder-with-low-anorectal-malformation-a-rare-association
#11
Aditya Pratap Singh, Vinay Mathur, Ramesh Tanger, Arun Kumar Gupta
No abstract text is available yet for this article.
July 2017: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/28893435/gradual-bone-transfer-for-the-correction-of-the-pubic-diastasis-using-the-ilizarov-technique-in-closure-of-bladder-and-cloacal-exstrophy
#12
Hiroshi Kusakabe, Katsuhiko Ueoka, Shinichiro Takayama, Atsuhito Seki
BACKGROUND: In the patient of the cloacal exstrophy, cloaca with local abdominal wall is disrupted and exposed inner surface of the bladder needs early closure. Pelvic osteotomies are required for severe cases whose bladder cannot be closed by the suture of soft tissue only. We developed a technique involving the gradual positioning of bone fragments using a light, Ilizarov external fixator. The usefulness of the technique was assessed. METHODS: We enrolled 3 patients with cloacal exstrophy and 1 with bladder exstrophy as a gradual transfer group and 6 patients who were treated by other osteotomies as a control group...
September 8, 2017: Journal of Orthopaedic Science: Official Journal of the Japanese Orthopaedic Association
https://www.readbyqxmd.com/read/28892921/a-rare-case-of-genital-malformation-with-omphalocele-exstrophy-of-bladder-imperforate-anus-and-spinal-defect-complex-autopsy-findings
#13
K Mamatha, B R Yelikar, Varsha R Deshpande, B S Disha
Omphalocele, Exstrophy of cloaca, Imperforate anus and Spinal defects (OEIS) is a severe manifestation of exstrophy-epispadias sequence with a combination of defects including OEIS. It results from improper closure of anterior abdominal wall and defective development of cloaca and urogenital septum due to defect in blastogenesis during the 4(th) week of gestation. Identification of this complex is important through foetal autopsy as this condition can recur in siblings. Prenatal diagnosis also helps to prevent foetal death with appropriate management in the less severe cases...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28890277/-prenatal-diagnosis-of-abdominal-wall-defects
#14
H El Mhabrech, H Ben Hmida, H Charfi, A Zrig, C Hafsa
Anterior abdominal wall defects (AAWD) correspond to a wide spectrum of congenital defects affecting 6.3/10,000 pregnancies. They have in common a closure defect of the anterior abdominal wall and can be fatal or expose the fetus and the neonate (NN) to many complications. This study was based on a retrospective study of 22 cases of AAWD collected between May 2009 and December 2014. Its purpose was to specify the importance of prenatal ultrasonography in the diagnosis and prognosis assessment of these defects...
October 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28888708/ureteric-urethral-engraftment-as-a-new-surgical-technique-for-management-of-incontinence-in-bladder-exstrophy-complex-a-retrospective-cohort
#15
Abdol-Mohammad Kajbafzadeh, Shabnam Sabetkish, Nastaran Sabetkish
OBJECTIVES: To report the results of a novel surgical technique for achieving urinary continence in patients with bladder exstrophy complex (BEC) by ureteric-urethral engraftment (UUE) technique. PATIENTS AND METHODS: Sixteen female patients with BEC and a mean ± SD age of 3.48 ± 1.75 years were referred for primary exstrophy repair from 2009 to 2012. From these, 9 patients were operated by single-stage bladder closure (group I); while 7 patients underwent the novel technique of UUE to compare the continence achievement (group II)...
October 2017: International Journal of Surgery
https://www.readbyqxmd.com/read/28868232/penile-reconstruction-with-skin-grafts-and-dermal-matrices-indications-and-management
#16
Paloma Triana Junco, Mariela Dore, Vanesa Nuñez Cerezo, Javier Jimenez Gomez, Miriam Miguel Ferrero, Mercedes Díaz González, Pedro Lopez-Pereira, Juan Carlos Lopez-Gutierrez
Introduction  The penis eventually needs specific cutaneous coverage in the context of reconstructive procedures following trauma or congenital anomalies. Local flaps are the first choice but are not always available after multiple previous procedures. In these cases, skin graft and dermal matrices should be considered. Materials and Methods  This study was a retrospective review of the past 4 years of four patients with severe loss of penile shaft skin who underwent skin reconstruction. Dermal matrices and skin grafts were utilized...
January 2017: European Journal of Pediatric Surgery Reports
https://www.readbyqxmd.com/read/28861290/mystery-and-realities-of-phalloplasty-a-systematic-review
#17
REVIEW
Selçuk Sarıkaya, David John Ralph
The neophallus creation is still a mystery and it remains challenging even today. In this article, we performed a comprehensive review of the literature regarding phalloplasty and penile reconstructive surgery between January 2008 and May 2016. In this review, we have included 15 research articles and the results of 276 patients were examined. Studies revealed several indications and when indications were reviewed, 191 patients were female-to-male transgender, 9 patients had disorder of sex development/micropenis, 16 had penile amputation/trauma, 9 had ambiguus genitalia, 40 had exstrophy and/or epispadias, 11 had other problems...
September 2017: Turkish Journal of Urology
https://www.readbyqxmd.com/read/28833084/pregnancy-outcomes-among-patients-with-prior-bladder-exstrophy
#18
Matthieu Dap, Olivier Larmure, Olivier Morel, Jean-Louis Lemelle
No abstract text is available yet for this article.
August 21, 2017: International Journal of Gynaecology and Obstetrics
https://www.readbyqxmd.com/read/28815980/sexuality-in-men-with-exstrophy
#19
EDITORIAL
Christopher R J Woodhouse
No abstract text is available yet for this article.
September 2017: BJU International
https://www.readbyqxmd.com/read/28810634/misdiagnosis-of-a-cloacal-exstrophy-variant-as-urorectal-septum-malformation-in-a-fetus-by-ultrasound-a-case-report
#20
Yang-Qing Xu, Xiao-Hong Yang, Xin-Lin Chen, Xiu-Qiin Ji, Sheng Zhao
Cloacal exstrophy variants are comprised of a wide range of characteristics, of which there are four primary features, including omphalocele, bladder exstrophy, an imperforate anus and spina bifida. The existing literature regarding the differential diagnosis from alternative urinary diseases prenatally are limited. If the bladder is present, defects in the ventral wall may not be visualized with prenatal ultrasound in certain conditions, including oligohydramnios, and differential diagnosis from urorectal septum malformation sequence is a challenge...
August 2017: Experimental and Therapeutic Medicine
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