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Bladder 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/28958802/cystectomy-in-the-pediatric-exstrophy-population-indications-and-outcomes
#8
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
#9
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
#10
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
#11
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
#12
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/28888708/ureteric-urethral-engraftment-as-a-new-surgical-technique-for-management-of-incontinence-in-bladder-exstrophy-complex-a-retrospective-cohort
#13
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/28833084/pregnancy-outcomes-among-patients-with-prior-bladder-exstrophy
#14
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/28810634/misdiagnosis-of-a-cloacal-exstrophy-variant-as-urorectal-septum-malformation-in-a-fetus-by-ultrasound-a-case-report
#15
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
https://www.readbyqxmd.com/read/28794909/role-of-the-lf-sine-derived-distal-isl1-enhancer-in-patients-with-classic-bladder-exstrophy
#16
Rong Zhang, Michael Knapp, Franziska Kause, Heiko Reutter, Michael Ludwig
A genome-wide association study and meta-analysis identified ISL1 as the first genome-wide significant susceptibility gene for classic bladder exstrophy (CBE). A short interspersed repetitive element (SINE), first detected in lobe-finned fishes (LF-SINE), was shown to drive Isl1 expression in embryonic mouse genital eminence. Hence, we assumed this enhancer a conclusive target for mutations associated with CBE formation and analyzed a cohort of 200 CBE patients. Although we identified two enhancer variants in five CBE patients, their clinical significance seems unlikely, implying that sequence variants in the ISL1 LF-SINE enhancer are not frequently associated with CBE...
September 2017: Journal of Pediatric Genetics
https://www.readbyqxmd.com/read/28771836/editorial-comment-to-lessons-learned-from-the-management-of-adults-who-have-undergone-augmentation-for-spina-bifida-and-bladder-exstrophy-incidence-and-management-of-the-non-lethal-complications-of-bladder-augmentation
#17
Kazuyoshi Johnin
No abstract text is available yet for this article.
August 3, 2017: International Journal of Urology: Official Journal of the Japanese Urological Association
https://www.readbyqxmd.com/read/28771821/lessons-learned-from-the-management-of-adults-who-have-undergone-augmentation-for-spina-bifida-and-bladder-exstrophy-incidence-and-management-of-the-non-lethal-complications-of-bladder-augmentation
#18
REVIEW
Douglas A Husmann
The present review provides clinical insights and makes recommendations regarding patient management garnered by the long-term follow up of patients undergoing enteric bladder augmentation for the management of congenital anomalies. A prospectively maintained database on 385 patients that have experienced an enteric bladder augmentation, using either the ileum or colon, was reviewed. Evaluations included methods used to prevent bladder calculi formation and recurrence, the incidence and etiology of renal calculi development, the incidence and treatment of vitamin B12 deficiency, and the complications and need for surgical revisions for continent catheterizable stomas...
August 3, 2017: International Journal of Urology: Official Journal of the Japanese Urological Association
https://www.readbyqxmd.com/read/28768624/pseudoexstrophy-a-forme-fruste-of-bladder-exstrophy
#19
Edmond Wright, Edwin Harmon, Michael Steiner, Michael Nowicki
No abstract text is available yet for this article.
August 2, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28721463/exstrophy-bladder-reconstruction-or-diversion-for-the-underprivileged
#20
REVIEW
Yogesh Kumar Sarin, Virender Sekhon
The surgical techniques for management of bladder exstrophy epispadias complex have evolved from staged reconstruction, complete primary repair to radical mobilization. Post-operative complications add to the multiplicity of surgical procedures at each step. The end results are variable with many achieving continence rates of 85-89% only after bladder augmentation and clean intermittent catheterization. The situation is further complicated in resource-poor settings, where illiteracy and poverty are the driving factors for choosing a single operative procedure for creation of low pressure reservoir aiming at upper tract preservation and good primary continence...
September 2017: Indian Journal of Pediatrics
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