A extrofia da bexiga (ou extrofia clássica da bexiga; ECB) é uma malformação geniturinária congênita que pertence ao grupo de condições do complexo de extrofia-epispádia (CEE) e se caracteriza por uma placa vesical exposta, epispádia e um defeito na parte da frente da pelve, do assoalho pélvico e da parede abdominal.
Introdução
O que você precisa saber de cara
A extrofia da bexiga (ou extrofia clássica da bexiga; ECB) é uma malformação geniturinária congênita que pertence ao grupo de condições do complexo de extrofia-epispádia (CEE) e se caracteriza por uma placa vesical exposta, epispádia e um defeito na parte da frente da pelve, do assoalho pélvico e da parede abdominal.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 6 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 13 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
2 genes identificados com associação a esta condição. Padrão de herança: Multigenic/multifactorial.
Acts as a sequence specific DNA binding transcriptional activator or repressor. The isoforms contain a varying set of transactivation and auto-regulating transactivation inhibiting domains thus showing an isoform specific activity. Isoform 2 activates RIPK4 transcription. May be required in conjunction with TP73/p73 for initiation of p53/TP53 dependent apoptosis in response to genotoxic insults and the presence of activated oncogenes. Involved in Notch signaling by probably inducing JAG1 and JAG
Nucleus
Acro-dermato-ungual-lacrimal-tooth syndrome
A form of ectodermal dysplasia. Ectodermal dysplasia defines a heterogeneous group of disorders due to abnormal development of two or more ectodermal structures. ADULT syndrome involves ectrodactyly, syndactyly, finger- and toenail dysplasia, hypoplastic breasts and nipples, intensive freckling, lacrimal duct atresia, frontal alopecia, primary hypodontia and loss of permanent teeth. ADULT syndrome differs significantly from EEC3 syndrome by the absence of facial clefting. Inheritance is autosomal dominant.
DNA-binding transcriptional activator. Recognizes and binds to the consensus octamer binding site 5'-ATAATTAA-3' in promoter of target genes. Plays a fundamental role in the gene regulatory network essential for retinal ganglion cell (RGC) differentiation. Cooperates with the transcription factor POU4F2 to achieve maximal levels of expression of RGC target genes and RGC fate specification in the developing retina. Involved in the specification of motor neurons in cooperation with LHX3 and LDB1 (
Nucleus
Variantes genéticas (ClinVar)
227 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
15 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Extrofia clássica da bexiga
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
Pesquisa e ensaios clínicos
2 ensaios clínicos encontrados.
Publicações mais relevantes
Comparison of internal and external rotation of corpora cavernosa in classic bladder exstrophy: A quantitative analysis of urethral coverage.
In classic bladder exstrophy (CBE), achieving optimal urethral coverage during epispadias repair (ER) remains surgically demanding. This study introduces a novel anatomical maneuver-external rotation (ExR) of the corpora cavernosa (CC)-and quantitatively compares it to internal rotation (InR) to determine which provides superior urethral coverage and penile length. We retrospectively analyzed 29 patients (aged 11-59 months) who underwent primary ER between 2020 and 2024 after prior bladder closure. In each case, the CC were fully mobilized, and neurovascular bundles were dissected to allow both InR and ExR. Each patient served as their own control. Urethral coverage and penile length were measured following both rotational techniques. ExR significantly improved urethral coverage compared to InR (mean difference: 9.79 mm; p < 0.0001). Urethral coverage gain correlated positively with patient age (rho = 0.6182, p = 0.0004), suggesting greater benefit in older children. All patients demonstrated an increase in coverage after ExR. A positive correlation was observed between age and the magnitude of coverage increase, although CC length was not directly measured. Two urethrocutaneous fistulas (6.9 %) occurred; one resolved after catheterization and one required surgical revision. ExR of the corpora cavernosa is a simple yet effective modification that offers greater urethral coverage in ER for CBE. These findings support its use-particularly in patients with an adequate urethral plate-as a preferable alternative to the traditional InR approach. Further studies with long-term outcomes are recommended.
Update and mid-term follow up of classic bladder exstrophy managed by the Toronto approach (Modified staged repair): Continence status, sexual function and upper tract functional outcomes.
The modified staged repair, or Toronto approach to reconstruct classic bladder exstrophy, involves bladder neck (BN) tailoring and bilateral ureteral reimplantation during primary closure, and later epispadias repair using external corpora rotation and a rotational penile skin flap. It aims to incorporate the advantages of complete primary repair while minimizing risks of upper tract deterioration and penile ischemia and improve cosmetic appearance of the genitalia. We present long-term outcomes for our initial patient series. All patients with initial operation between 2000 and 2014 were reviewed. Data on demographics, continence, erectile and ejaculatory function, cosmetic appearance of the genitalia, and upper tract status were collected. Twelve male and four female patients were identified, with median follow-up of 12.7 (IQR 10.9-15.4) and 12.5 years (IQR 10.6-15.6), respectively. Full continence (voiding with no leaks, dry periods ≥3 h) was achieved in two of 12 males and two of four females. Five of 12 males and all four females had dry periods longer than 1 h. Nine of 12 males and all females attained volitional voiding. Three of 12 males and one of four females underwent additional continence procedures. None have undergone augmentation cystoplasty or bladder neck closure. Of seven males with preliminary sexual function data, all experienced erections, straight in five, with recurrent dorsal curvature and ventral curvature in one patient each. Four of seven ejaculate and none have attempted penetrative intercourse. All seven males reported satisfactory cosmetic appearance despite a subjectively shorter penis. Although transitory dilations of the ureters were seen immediately post op, none had scarring, hydronephrosis, or febrile urinary tract infections at latest follow-up. One patient had an eGFR on the upper range of CKD 2, while the rest of the cohort had eGFR ≥ 90 mL/min/1.73m2. Mean bladder capacity on ultrasound was 145 mL for males and 97 mL for females. The present data suggests that the modified staged repair of exstrophy (Toronto approach) is associated with acceptable continence outcomes while minimizing escalation to augmentation cystoplasty and bladder neck closure. Most patients void volitionally and stay dry for 1-3 h, but few are fully continent or able to remain dry for 3 h or more. No patients in the cohort had CKD3 or worse, and none had hydronephrosis or history of febrile urinary tract infections. Most males experience ejaculation and straight erections. There was no glanular or corporal tissue loss.
Early findings on OnabotulinumtoxinA for postoperative pain control in bladder exstrophy.
Postoperative pain management in bladder reconstruction and exstrophy closure is challenging due to unique physiological differences in exstrophy patients, surgical complexity, and heightened risk of painful bladder spasms. While opioids and anticholinergics are used for pain relief, their prolonged use is associated with complications. We sought to evaluate postoperative benefits of OnabotulinumtoxinA (Botox) injections in patients with classic bladder exstrophy (CBE) and cloacal exstrophy (CE) undergoing bladder reconstruction or exstrophy closure. CBE and CE patients who underwent bladder reconstruction or exstrophy closure between 2018 and 2024 were identified from an institutional database. Bladder reconstruction was defined as any combination of the following procedures - bladder neck reconstruction, bladder neck transection, Mitrofanoff or Monti catheterizable channel creation, and bladder augmentation. Reconstructive patients were stratified by concurrent ureteral reimplants necessitating ureteral stent placement. Data on postoperative course, medications, and complications were collected. Among 48 patients undergoing bladder reconstruction, 14 received Botox and 34 did not. Of the 34 exstrophy closures, 12 received Botox and 22 did not. In patients undergoing bladder reconstruction without ureteral reimplants necessitating ureteral stents, Botox significantly reduced oxybutynin use (0.09 mg/kg/day vs. 0.15 mg/kg/day, p = 0.02) and oxycodone use (0.00 mg/kg/day vs. 0.11 mg/kg/day, p = 0.03). These patients also experienced fewer days with pain scores above 0 (4.00 days vs. 10.00 days, p = 0.04) and above 4 (2.00 days vs. 6.00 days, p = 0.04). In contrast, bladder reconstruction patients with ureteral reimplantation necessitating ureteral stents showed no significant differences in medication use or pain scores (all p > 0.05). Botox did not significantly impact postoperative course, medication requirements, in exstrophy closures (all p > 0.05). Botox injections significantly improved postoperative outcomes and reduced medication use in exstrophy patients undergoing bladder reconstruction without ureteral reimplantation that necessitate ureteral stent placement. However, there was no statistical significance noted in the intervention group compared to controls in cases involving ureteral reimplantation with stent placement. Furthermore, Botox showed no postoperative advantages in exstrophy closure, where surgical complexity may limit its therapeutic efficacy. Limitations include the retrospective design and small sample size. Intraoperative Botox reduced opioid and anticholinergic use, as well as postoperative pain, among exstrophy patients undergoing bladder reconstruction without ureteral reimplants requiring ureteral stents. This demonstrates its potential as an effective adjunct for postoperative pain in exstrophy patients undergoing select reconstruction.
Letter to the Editor re: "Long-term sexual outcomes in classic bladder exstrophy: Insights from standardized scores".
Response to Letter to the Editor re: "Long-term sexual outcomes in classic bladder exstrophy: Insights from standardized scores".
Publicações recentes
Comparison of internal and external rotation of corpora cavernosa in classic bladder exstrophy: A quantitative analysis of urethral coverage.
Long-term sexual outcomes in classic bladder exstrophy: Insights from standardized scores.
📚 EuropePMC124 artigos no totalmostrando 154
Comparison of internal and external rotation of corpora cavernosa in classic bladder exstrophy: A quantitative analysis of urethral coverage.
Journal of pediatric urologyLong-term sexual outcomes in classic bladder exstrophy: Insights from standardized scores.
Journal of pediatric urologyCommentary to "Update and mid-term follow up of classic bladder exstrophy managed by the Toronto approach (modified staged repair): Continence status, sexual function and upper tract functional outcomes".
Journal of pediatric urologyUpdate and mid-term follow up of classic bladder exstrophy managed by the Toronto approach (Modified staged repair): Continence status, sexual function and upper tract functional outcomes.
Journal of pediatric urologyManagement of pregnancy in a patient with previous surgical repair of classic bladder exstrophy.
International journal of surgery case reportsManagement of incontinence after classic bladder exstrophy closure.
Actas urologicas espanolasOne-hundred-fifteen consecutive bladder exstrophies successfully closed in a single nationally commissioned centre.
Journal of pediatric urologyEarly findings on OnabotulinumtoxinA for postoperative pain control in bladder exstrophy.
Journal of pediatric urologyAugmentation cystoplasty and continent catheterizable channels in the bladder exstrophy-epispadias complex: A 20-year experience.
Journal of pediatric urologyLong-term follow-up reveals complexity of urinary and faecal continence outcomes in patients with classic bladder exstrophy.
BJU internationalMultidisciplinary Management of Pregnancy in Bladder Exstrophy: A Case Report.
The American journal of case reportsFailure of Prenatal Ultrasonography to Identify Classic Bladder Exstrophy in a Heavily Screened Population.
UrologyLong-term urinary outcomes in classic bladder exstrophy: results of an extensive follow-up.
BJU internationalWill my Child Walk Funny? The Rotational Profile of Infants and Children With Classic Bladder Exstrophy.
Journal of pediatric orthopedicsDuplicate bladder exstrophy in a female infant: A case report.
Urology case reportsBladder exstrophy-epispadias-cloacal exstrophy complex: characteristics, aetiologies, and epidemiologic findings.
African urologyClassic Bladder Exstrophy - Timing of initial closure and technical highlights.
African urologySubperiosteal Tunneled Allograft Reconstruction of the Symphyseal Ligaments (STARS) in Bladder Exstrophy Epispadias Complex.
Journal of pediatric orthopedicsEnd-stage and chronic kidney disease in classic bladder exstrophy: A retrospective muti-institutional cohort study.
Journal of pediatric urologyEZH2 specifically regulates ISL1 during embryonic urinary tract formation.
Scientific reportsModified penile reconstruction in classic bladder exstrophy: Can complete corporal covering of the urethral closure be achieved using incomplete disassembly technique?
International braz j urol : official journal of the Brazilian Society of UrologyStomal Stenosis After Continent Urinary Diversion in Bladder Exstrophy: Risk Factors and Management.
UrologyRedo surgery to improve urinary function, sexual function and cosmesis in male patients with exstrophy-epispadias complex complications. Technical principles and pearls based on case scenarios.
Journal of pediatric urologyBladder exstrophy-epispadias complex: The effect of urotherapy on incontinence.
Journal of pediatric urologyLong-term psychosexual adjustment of adults born with classic bladder exstrophy.
Journal of pediatric urologyAn Analysis of the Efficacy of Multilayered Repair and Reconstruction Using Combined Tissue Pedicle Flaps for Abdominal Wall Defects in Adult Bladder Exstrophy Patients.
Annals of plastic surgeryInnervation pattern of the unclosed detrusor muscle in classic bladder exstrophy: a study of patients with urothelial overexpression of nerve growth factor.
Pediatric surgery internationalOptimizing prenatal diagnosis and referral of classic bladder exstrophy: Lessons from a single-institution experience.
Journal of pediatric urologyInterposing Rectus and Gracilis Muscle Flaps for Pelvic Reconstruction in Bladder Exstrophy after Bladder Neck Closure.
Plastic and reconstructive surgerySupravesical Fissure Variant of Bladder Exstrophy in a Female Patient: Case Report and Review of Literature.
UrologyPerioperative management of primary classic bladder exstrophy: A single institutional pathway to success.
Journal of pediatric urologyThe true pelvic volume change with various corrective osteotomy techniques for exstrophy-epispadias complex spectrum: the value of computer-assisted virtual surgery.
Journal of pediatric orthopedics. Part BSuperior Vesical Fissure as an Incomplete Form of Bladder Exstrophy: A Case Report with Clinico-Pathological Correlation and a Comprehensive Literature Review.
International journal of surgical pathologyClassic Bladder Exstrophy Closure Without Osteotomy or Immobilization: An Exercise in Futility?
UrologyPresentations of bladder exstrophy in a resource-limited setting and the role of Mainz II continent diversion for late referrals or failed primary closures: a multicentric report.
La Pediatria medica e chirurgica : Medical and surgical pediatricsExome Survey and Candidate Gene Re-Sequencing Identifies Novel Exstrophy Candidate Genes and Implicates LZTR1 in Disease Formation.
BiomoleculesThe outcome and complications of modern staged repair surgery in newborns with classic bladder exstrophy in different genders: A retrospective study.
Birth defects researchClassical bladder exstrophy in an adolescent: A case report on management, challenges and outcome.
African journal of paediatric surgery : AJPSLong term outcomes in classic bladder exstrophy - The adult picture.
Journal of pediatric urologyUrethral Plate Grafting for Deficient Urethral Plate in Exstrophy: An Alternative Method to Augment Urethral Plate.
Journal of pediatric surgeryMucosal Violations and Their Effect on Successful Bladder Neck Closure in Cloacal Exstrophy.
Journal of pediatric surgeryAchieving goal capacity for continence surgery: A cumulative event analysis of bladder exstrophy patients.
Journal of pediatric urologyBladder capacity and growth in classic bladder exstrophy: A novel predictive tool.
Journal of pediatric urologyRare case of superior vesical fissure with hypospadias: variants of classic bladder exstrophy.
World journal of pediatric surgeryThe impact of repeated bladder surgery on successful bladder neck closure in classic bladder exstrophy: The role of mucosal violations.
Journal of pediatric urologySpinal dysraphism in exstrophy: a single-center study of a 39-year prospective database.
Journal of neurosurgery. PediatricsInflammatory Expression Profiles in Bladder Exstrophy Smooth Muscle: Normalization Over Time.
UrologyA Single Center's Changing Trends in the Management and Outcomes of Primary Closure of Classic Bladder Exstrophy: An Evolving Landscape.
UrologyA Triplicated Bladder with Diphallia; Rare Variant of Duplicate Exstrophy.
Research and reports in urologyComplications of delayed and newborn primary closures of classic bladder exstrophy: Is there a difference?
Journal of pediatric urologyFixation with lower limb immobilization in primary and secondary exstrophy closure: A saving grace.
Journal of pediatric urologyDelayed Surgical Management of an Unusual Classic Bladder Exstrophy Variant.
UrologyThe use of a modified tanagho flap in the repair of posterior urethral stricture after primary exstrophy closure.
Journal of pediatric urologyA genome-wide association study with tissue transcriptomics identifies genetic drivers for classic bladder exstrophy.
Communications biologyApplication of Tunica Vaginalis Flap for Epispadias Repair in the Epispadias-Exstrophy Complex.
UrologyOrthopedic complications after osteotomy in patients with classic bladder exstrophy and cloacal exstrophy: a comparative study.
Journal of pediatric urologyMini-open Anterior Pelvic Osteotomy and Spica Casting for Primary Closure of the Pelvis in Infants With Classic Bladder Exstrophy.
Journal of pediatric orthopedicsExstrophy-Epispadias Complex With Isolated Ectopic Bowel Segment: A Case Series and Literature Review.
UrologyBladder exstrophy: Modern staged repair experience in our institution.
African journal of paediatric surgery : AJPSInsights and outcomes of single-staged repair of female bladder exstrophy-epispadias complex without osteotomy: 15 Years experience of a single institution.
Journal of pediatric urologyAn evaluation of perioperative surgical procedures and complications in classic bladder exstrophy patients Using the National Surgical Quality Improvement Program-Pediatric (NSQIP-P).
Journal of pediatric urologySexual health outcomes after penile reconstruction in the exstrophy-epispadias complex.
Journal of pediatric urologyPelvic Floor Anatomical Variations in Children With Exstrophy-Epispadias Complex Using Magnetic Resonance Imaging.
UrologyDelayed primary closure of bladder exstrophy without osteotomy: 12 year experience in a safe and gentle alternative to neonatal surgery.
Journal of pediatric surgeryBilateral ureteral reimplantation at complete primary repair of exstrophy: Post-operative outcomes.
Journal of pediatric urologyLong-term urological and gynecological outcomes following complete primary repair in females with bladder exstrophy.
Journal of pediatric urologyPersistent urothelial differentiation changes in the reconstructed exstrophic bladder: Congenital or acquired dysfunction of the epithelial barrier?
Journal of pediatric urologyCommentary to 'Osteotomy in the newborn classic bladder exstrophy patient: A comparative study'.
Journal of pediatric urologyThe Richard Grady Monsplasty: A vertical Z-plasty technique.
Journal of pediatric urologyOsteotomy in the newborn classic bladder exstrophy patient: A comparative study.
Journal of pediatric urologyExpression of Low Affinity Nerve Growth Factor Receptor p75 in Classic Bladder Exstrophy.
Frontiers in pediatricsCongenital Heart Defects in Patients with Classic Bladder Exstrophy: A Hitherto Neglected Association?
European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift fur KinderchirurgieUrothelial Differences in the Exstrophy-Epispadias Complex: Potential Implications for Management.
The Journal of urologyRole of the Pubic Symphysis in Osseous Pelvic Development: A Novel Model of Bladder Exstrophy in Rabbits.
Journal of pediatric orthopedicsPedicled Anterolateral Thigh and Radial Forearm Free Flap Phalloplasty for Penile Reconstruction in Patients With Bladder Exstrophy.
The Journal of urologyAssessment of the anterior osteotomy role in the restoration of normal pelvic floor anatomy for bladder exstrophy patients using pre and postoperative pelvic floor MRI.
Journal of pediatric urologyDelaying primary closure of classic bladder exstrophy: When is it too late?
Journal of pediatric urologyIleal Neobladder With A Continent Cutaneous Catheterizable Channel Using the Extramural Serous Lined (Mansoura) Technique in A Bladder Exstrophy Patient.
UrologyVolumetric and acetabular changes in the bony pelvis associated with primary closure of classic bladder exstrophy.
Journal of pediatric urologySingle-staged male bladder exstrophy-epispadias complex reconstruction with pubic bone adaptation without osteotomy: 15-year single-center experience.
International urology and nephrologySLC20A1 Is Involved in Urinary Tract and Urorectal Development.
Frontiers in cell and developmental biologyDevelopment of Late Continence in Bladder Exstrophy and Epispadias Patients.
UrologyEvaluation of outcomes following complete primary repair of bladder exstrophy at three individual sites prior to the establishment of a multi-institutional collaborative model.
Journal of pediatric urologyClassic bladder exstrophy and complete rectal prolapse in a 10 year-old child with no previous surgical intervention: what to achieve?
Journal of surgical case reportsPractice patterns in classic bladder exstrophy: A global perspective.
Journal of pediatric urologyPenile Disassembly in Complete Primary Repair of Bladder Exstrophy: Time for Re-evaluation?
UrologyProbability of Bladder Augmentation, Diversion and Clean Intermittent Catheterization in Classic Bladder Exstrophy: A 36-Year, Multi-Institutional, Retrospective Cohort Study.
The Journal of urologyUrinary Continence Outcomes in Classic Bladder Exstrophy: A Long-Term Perspective.
The Journal of urology[Appllication of human acellular dermal matrix in surgical treatment of genitourinary disease].
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciencesThe role of anatomic pelvic dissection in the successful closure of bladder exstrophy: an aid to success.
Journal of pediatric urologyCommentary to 'Impact of pelvic immobilization techniques on the outcomes of primary and secondary closures of classic bladder exstrophy'.
Journal of pediatric urologyClassic bladder exstrophy and adenocarcinoma of the bladder: Methylome analysis provide no evidence for underlying disease-mechanisms of this association.
Cancer geneticsAnatomy of Classic Bladder Exstrophy: MRI Findings and Surgical Correlation.
Current urology reportsIsolated bladder exstrophy in prenatal diagnosis.
Archives of gynecology and obstetricsImpact of pelvic immobilization techniques on the outcomes of primary and secondary closures of classic bladder exstrophy.
Journal of pediatric urology3-Dimensional Magnetic Resonance Imaging Guided Pelvic Floor Dissection for Bladder Exstrophy: A Single Arm Trial.
The Journal of urologyContinuous caudal epidural analgesia and early feeding in delayed bladder exstrophy repair: a nine-year experience.
Journal of pediatric urologyManagement of Recurrent Pelvic Fluid Collections in Adult Male Bladder Exstrophy Patients With Maintenance of Erectile Function.
UrologyTowards a Central Role of ISL1 in the Bladder Exstrophy⁻Epispadias Complex (BEEC): Computational Characterization of Genetic Variants and Structural Modelling.
GenesModern Management of the Failed Bladder Exstrophy Closure: A 50-yr Experience.
European urology focusOne-stage combined delayed bladder closure with Kelly radical soft-tissue mobilization in bladder exstrophy: preliminary results.
Journal of pediatric urologySingle-stage Abdominoplasty Using Groin Flaps Without Osteotomies: Management of Exstrophy-epispadias Complex.
UrologyA Borderline Ovarian Tumour in a Patient with Classic Bladder Exstrophy; a Case Report.
Irish medical journalPredictors and outcomes of perioperative blood transfusions in classic bladder exstrophy repair: A single institution study.
Journal of pediatric urologyThe inadequate bladder template: Its effect on outcomes in classic bladder exstrophy.
Journal of pediatric urologyNovel Anatomical Observations of the Prostate, Prostatic Vasculature and Penile Vasculature in Classic Bladder Exstrophy Using Magnetic Resonance Imaging.
The Journal of urologyCombined Bladder Neck Reconstruction and Continent Stoma Creation as a Suitable Alternative for Continence in Bladder Exstrophy: A Preliminary Report.
UrologyNovel Observations of Female Genital Anatomy in Classic Bladder Exstrophy Using 3-Dimensional Magnetic Resonance Imaging Reconstruction.
The Journal of urologyThe Role of Human Acellular Dermis in Preventing Fistulas After Bladder Neck Transection in the Exstrophy-epispadias Complex.
UrologyComplete Primary Repair of Bladder Exstrophy: Critical Analysis of the Long-term Outcome.
UrologyHow to close classic bladder exstrophy: Are subspecialty training and technique important?
Journal of pediatric urologyPelvic Organ Prolapse and Pregnancy in the Female Bladder Exstrophy Patient.
Current urology reportsBladder Re-augmentation in Classic Bladder Exstrophy: Risk Factors and Prevention.
UrologyBladder exstrophy closure in the newborn period with external pelvic fixation performed without osteotomy: A preliminary report.
Journal of pediatric urologyIsl1 mediates mesenchymal expansion in the developing external genitalia via regulation of Bmp4, Fgf10 and Wnt5a.
Human molecular geneticsRe: Use of Human Acellular Dermal Matrix during Classic Bladder Exstrophy Repair.
The Journal of urologyCystectomy in the Pediatric Exstrophy Population: Indications and Outcomes.
UrologyRole of the LF-SINE-Derived Distal ISL1 Enhancer in Patients with Classic Bladder Exstrophy.
Journal of pediatric geneticsLong-term fate of the upper tracts following complete primary repair of bladder exstrophy.
Journal of pediatric urologyRepair of Vesicocutaneous and Urethrocutaneous Fistulae with Rectus Muscle Flap in a Bladder Exstrophy Patient.
Urology case reportsLong-term sexual health outcomes in men with classic bladder exstrophy.
BJU internationalRe: Peri-Operative Transfusion Risk in Classic Bladder Exstrophy Closure: Results from a National Database Review.
The Journal of urologyISL1 is a major susceptibility gene for classic bladder exstrophy and a regulator of urinary tract development.
Scientific reportsContinent Anal Urinary Diversion in Classic Bladder Exstrophy: 45-Year Experience.
UrologyFailed Primary Bladder Exstrophy Closure with Osteotomy: Multivariable Analysis of a 25-Year Experience.
The Journal of urologyThe long-term outcomes after staged repair of exstrophy-epispadias complex.
Journal of Indian Association of Pediatric SurgeonsSpontaneous bladder rupture in non-augmented bladder exstrophy.
Journal of pediatric urologyCongenital renal anomalies in cloacal exstrophy: Is there a difference?
Journal of pediatric urologyPeri-operative transfusion risk in classic bladder exstrophy closure: Results from a national database review.
Journal of pediatric urologyA critical appraisal of continence in bladder exstrophy: Long-term outcomes of the complete primary repair.
Journal of pediatric urologyImpact of concomitant hernia repair at the time of complete primary repair of bladder exstrophy.
Journal of pediatric urologyIs 1p36 deletion associated with anterior body wall defects?
American journal of medical genetics. Part AUse of muscle pedicle flaps for failed bladder neck closure in the exstrophy spectrum.
Journal of pediatric urologyIliac wing osteotomy for the closure of pelvis in the treatment of bladder exstrophy in the neonate.
Journal of pediatric orthopedics. Part BCommentary to "Use of human acellular dermal matrix during classic bladder exstrophy repair".
Journal of pediatric urologySexual Function and Fertility of Women with Classic Bladder Exstrophy and Continent Urinary Diversion.
The Journal of urologyUse of human acellular dermal matrix during classic bladder exstrophy repair.
Journal of pediatric urologyDuplicated Renal Collecting System With Ectopic Ureter in Female Bladder Exstrophy: A Case Report.
UrologyThe cost of failure: The economic impact of failed primary closure in classic bladder exstrophy.
Journal of pediatric surgeryRe: Sexual Function and Health-Related Quality of Life in Women with Classic Bladder Exstrophy.
The Journal of urologyExstrophy-Epispadias Complex in a Newborn: Case Report and Review of the Literature.
AJP reportsSexual Function, Social Integration and Paternity of Males with Classic Bladder Exstrophy following Urinary Diversion.
The Journal of urologyThe radical soft-tissue mobilization (Kelly repair) for bladder exstrophy.
Journal of pediatric urologyMedicinal Leech Therapy for Glans Penis Congestion After Primary Bladder Exstrophy-Epispadias Repair in an Infant: A Case Report.
UrologyNewborn exstrophy closure without osteotomy: Is there a role?
Journal of pediatric urologyThe Tunica Vaginalis Flap as an Adjunct to Epispadias Repair: A Preliminary Report.
UrologyProspective study on the incidence of bladder/cloacal exstrophy and epispadias in Europe.
Journal of pediatric urologyCan Classic Bladder Exstrophy be Safely and Successfully Reconstructed at a Low Volume Center?
The Journal of urologyIncidence of Hip Dysplasia Associated With Bladder Exstrophy.
Journal of pediatric orthopedicsSexual function in adult patients with classic bladder exstrophy: A multicenter study.
Journal of pediatric urologySecondary reclosure in classic bladder exstrophy: challenges and outcomes.
UrologyPsychosocial screening at paediatric BEEC clinics: a pilot evaluation study.
Journal of pediatric urologyGenome-wide association study and meta-analysis identify ISL1 as genome-wide significant susceptibility gene for bladder exstrophy.
PLoS geneticsIntra thoracic migration of ureteric stent after exstrophy bladder closure: Unusual complication.
African journal of paediatric surgery : AJPSAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Comparison of internal and external rotation of corpora cavernosa in classic bladder exstrophy: A quantitative analysis of urethral coverage.
- Update and mid-term follow up of classic bladder exstrophy managed by the Toronto approach (Modified staged repair): Continence status, sexual function and upper tract functional outcomes.
- Early findings on OnabotulinumtoxinA for postoperative pain control in bladder exstrophy.
- Letter to the Editor re: "Long-term sexual outcomes in classic bladder exstrophy: Insights from standardized scores".
- Response to Letter to the Editor re: "Long-term sexual outcomes in classic bladder exstrophy: Insights from standardized scores".
- Response to Commentary on "Update and mid-term follow up of classic bladder exstrophy managed by the Toronto Approach (Modified Staged Repair): Continence status, sexual function and upper tract functional outcomes".
- Long-term sexual outcomes in classic bladder exstrophy: Insights from standardized scores.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:93930(Orphanet)
- MONDO:0010805(MONDO)
- GARD:6398(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q258858(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
