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https://www.readbyqxmd.com/read/28810634/misdiagnosis-of-a-cloacal-exstrophy-variant-as-urorectal-septum-malformation-in-a-fetus-by-ultrasound-a-case-report
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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...
July 18, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28694586/duplicate-bladder-exstrophy-with-hypospadias-a-rare-exstrophy-variant-with-unusual-association
#7
Bibekanand Jindal, K Lalatendu Kumar, Sadasivan Jagdish
No abstract text is available yet for this article.
July 2017: Journal of Indian Association of Pediatric Surgeons
https://www.readbyqxmd.com/read/28687949/controversies-of-sex-re-assignment-in-genetic-males-with-congenital-inadequacy-of-the-penis
#8
Venkatachalam Raveenthiran
Sex assignment in 46XY genetic male children with congenital inadequacy of the penis (CIP) is controversial. Traditionally, children with penile length less than 2 cm at birth are considered unsuitable to be raised as males. They are typically re-assigned to female-sex and feminizing genitoplasty is usually done in infancy. However, the concept of cerebral androgen imprinting has caused paradigm shift in the philosophy of sex re-assignment. Masculinization of the brain, rather than length of the penis, is the modern criterion of sex re-assignment in CIP...
July 8, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28666504/pregnancy-in-a-treated-case-of-ectopia-vesicae
#9
Fazeela Shahzad, Shazia Khalid Khan, Madiha Siraj
The patient was born with bladder exstrophy and underwent multiple surgeries for its correction. Finally, she had ureterosigmoidostomy at the age of 9 years, which ultimately led her to live till reproductive age. The children born with this defect are capable of surviving till adult life. She was received by us with preterm labour, referred from Hafizabad. She also had hyperchloremic metabolic acidosis and mild hydronephrosis. She was managed with multidisciplinary, modified care but the pregnancy ended up in an emergency cesarean section due to non-reactive cardiotocograph and persistently reduced fetal movements...
November 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28652161/the-surgical-management-of-bladder-polyps-in-the-setting-of-exstrophy-epispadias-complex
#10
John Jayman, Ali Tourchi, Mahsa Shabaninia, Mahir Maruf, Heather DiCarlo, John P Gearhart
OBJECTIVE: To investigate the surgical course for treating bladder polyps in patients with exstrophy epispadias complex (EEC). EEC bladder polyps are unique pathologic entities, with a distinct difference between polyps discovered at birth and polyps developed after failed exstrophy closure. METHODS: A prospectively maintained database of 1300 patients with EEC was reviewed for bladder exstrophy patients with bladder polyps. The following data were obtained: patient demographics, polyp type, past medical and surgical history, and continence outcomes...
June 23, 2017: Urology
https://www.readbyqxmd.com/read/28627397/long-term-efficacy-of-young-dees-bladder-neck-reconstruction-role-of-the-associated-bladder-neck-injection-for-the-treatment-of-children-with-urinary-incontinence
#11
A Faure, G Hery, E Mille, P Orillac, B Da Silva, T Merrot, M Haddad, J M Guys
OBJECTIVE: To compare the long-term efficacy of Young-Dees bladder neck reconstruction (YDBNR) alone versus YDBNR plus bladder neck injection (BNI) in patients with urinary incontinence caused by urethral sphincter insufficiency. PATIENTS AND METHODS: Between 1987 and 2006, we assessed the continence rates obtained with YDBNR and BNI as a supplementary treatment for persistent outlet insufficiency in patients with neurogenic bladder (group 1, n = 35) and bladder exstrophy (group 2, n = 20)...
June 13, 2017: Urology
https://www.readbyqxmd.com/read/28617177/discordant-anomalies-with-combined-features-of-pentalogy-of-cantrell-and-oeis-complex-a-case-report-in-monochorionic-twins
#12
Nique Kunapinun, Jitsupa Treetipsatit
INTRODUCTION: Ventral body wall defects have various manifestations. Among others, pentalogy of Cantrell (PC) and omphalocele exstrophy imperforate anus spinal abnormalities (OEIS) complex are defects that involve upper and lower anterior midline of body wall, respectively. Although both entities are in a spectrum of ventral body wall defects, the combination of PC and OEIS complex has not been described. CASE REPORT: In this report, we describe an unusual case of congenital ventral body wall defect with combined features of PC and OEIS complex, which discordantly occurred in monochorionic monoamniotic twins...
June 15, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28592391/long-term-fate-of-the-upper-tracts-following-complete-primary-repair-of-bladder-exstrophy
#13
Jonathan S Ellison, Jennifer Ahn, Margarett Shnorhavorian, Richard Grady, Paul A Merguerian
INTRODUCTION: Complete primary repair of bladder exstrophy (CPRE) is widely used for classic bladder exstrophy (CBE) closure. Long-term renal function with or without dilation in these patients is poorly characterized and may be impacted by bladder storage parameters or recurrent urinary tract infections (UTIs). OBJECTIVE: We sought to assess our long-term experience with CPRE and investigate factors that may influence upper-tract deterioration. STUDY DESIGN: A retrospective review of patients at our institution with CBE undergoing CPRE from 1990 to 2015 was performed...
May 17, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28554748/nephrogenic-adenoma-of-the-urinary-tract-a-6-year-single-center-experience
#14
Didem Turcan, Mustafa Fuat Acikalin, Evrim Yilmaz, Funda Canaz, Deniz Arik
BACKGROUND: Nephrogenic adenoma is an uncommon benign lesion that occurs at several sites in urinary tract, from the renal pelvis to urethra, with the highest frequency in urinary bladder. Nephrogenic adenoma displays a broad spectrum of architectural and cytological features. Hence, recognition of its characteristic histopathological features is needed to distinguish this lesion from its mimickers. MATERIALS AND METHODS: A retrospective series of 21 cases of nephrogenic adenoma in 18 patients, which were diagnosed in our department between 2010 and 2016, were analyzed...
March 25, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28552751/-ultrasound-of-the-fetal-urinary-system-during-the-first-trimester-of-pregnancy
#15
N Hamdaoui, A Dabadie, E Lesieur, E Quarello, M Kheiri, G Hery, B Guidicelli, F Bretelle, G Gorincour
The detection of abnormalities of the fetal urinary system in the first trimester of pregnancy is constantly improving, namely owing to the improved resolution of the image, the use of the endovaginal approach and thanks to sonographers' constant training. The pathological aspects, usually detected in the second trimester of pregnancy, can be suspected early in the first trimester and range from kidneys' cavity dilation to bilateral renal agenesis, polycystic kidney disease, multi-cystic dysplasia and bladder megavessia or bladder exstrophy...
May 25, 2017: Gynecologie, Obstetrique, Fertilite & Senologie
https://www.readbyqxmd.com/read/28551609/exstrophy-variant-in-a-female-child
#16
Eliana Costantino, George S Ganesan
No abstract text is available yet for this article.
May 27, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28545812/liver-exstrophy-in-a-neonate-with-poland-syndrome
#17
Xenophon Sinopidis, Antonios Panagidis, Vasileios Alexopoulos, Sotirios Tzifas, Gabriel Dimitriou, George Georgiou
No abstract text is available yet for this article.
May 10, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28534165/-the-german-research-network-cure-net-a-benefit-for-patients-with-rare-diseases
#18
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...
July 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
#19
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
#20
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...
July 2017: Clinical Nuclear Medicine
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