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1.
ObjectiveInjection of urethral bulking agents (UBA) has been used to increase bladder capacity prior to bladder neck reconstruction (BNR) or as an adjuvant therapy following BNR to improve continence. The purpose of this study was to determine the effectiveness of urethral injections in the exstrophy population.Materials and methodsA review was performed of patient characteristics, bladder capacity, and continence status of all patients with the exstrophy–epispadias complex who underwent injection of UBA between 1980 and 2008.ResultsAmong 66 patients with a median follow-up of 8 years, 41 underwent injections prior to BNR, and 25 had injections after BNR. Only 24% of patients who underwent injections prior to BNR were continent on last follow-up. Among 25 patients who underwent BNR prior to injection(s), 16 were partially continent and nine were incontinent prior to first injection. Patients who were partially continent attained social continence (dry interval greater than 3 h) at a significantly higher rate than those who were incontinent (63% vs. 13%, p = 0.047). No patient with cloacal exstrophy in either group attained urethral continence.ConclusionUBAs do not appear to have a role prior to BNR. However, they may provide benefit when given adjunctively following BNR in patients who are partially continent.  相似文献   

2.
ObjectiveTo evaluate potential predictors of voided continence among bladder exstrophy patients with a history of a failed closure.Patients and methodsThe authors reviewed all patients who underwent a bladder neck reconstruction (BNR) with a history of one or more failed exstrophy closures between 1979 and 2007. The following data were collected for each patient: number of failures, site of surgery, mode of failure, presence of osteotomy, bladder capacity, need for additional procedures, and continence status.ResultsAmong patients who underwent successful reclosure following one or more failed closures, 52 patients underwent BNR, and 24 (46%) were continent at last follow-up. Bladder capacity was the only variable predictive of voided continence. The median bladder capacity at the time of BNR differed between those who achieved continence (100 mL) and those who did not (65 mL) (p = 0.005). ROC analysis showed an optimal pre-BNR bladder capacity cutoff for predicting future BNR success of between 80 and 100 mL.ConclusionAs previously shown in patients with successful primary closure of exstrophy, these data suggest that bladder capacity also has predictive value in the success of BNR after failed exstrophy closure.  相似文献   

3.
ObjectiveWe reviewed our initial results with complete primary repair of exstrophy in regard to continence status and the need for subsequent continence procedures.Patients and methodsWe performed a retrospective review of our surgical records from 1996 to 2008 to identify all patients with bladder exstrophy managed at our center.ResultsSixteen children were closed successfully. Six patients (37.5%) experienced complications: umbilical hernias in two, transient penopubic fistula in three, and subcoronal fistula due to meatal stenosis in one. Of the 12 males, seven (58.3%) were left with a hypospadias at the time of primary closure. Two (22.2%) children required a formal bladder neck reconstruction to achieve continence. Bladder augmentation and continent catheterizable stoma was performed in four cases (44.4%), and bladder neck injection in one case (11.1%). Bladder neck closure was also performed in another child following primary closure. Three of these children are continent and void spontaneously (33.3%). The remaining six require clean intermittent catheterization four to six times a day, resulting in four (44.4%) being continent. The number of continence procedures and mean number per patient were 15 and 1.66, respectively.ConclusionOur early experience with this technique has been encouraging, with few major complications, a highly successful closure rate and a cosmetically normal result.  相似文献   

4.
ObjectiveIn a series of failed exstrophy closures, to identify determinants of successful repeat closure and the impact of failed closure on the fate of the lower urinary tract and continence status.Patients and methodsWe performed a retrospective review of operative notes and medical records of patients with a history of one or more failed exstrophy closures in 1978–2007. The primary surgical endpoints were failure rate of repeat closure attempts, mode of continence surgery and continence outcome. Continence was defined as achieving a dry interval of >3 h and voiding through the urethra.ResultsWe identified 122 patients (85 male/37 female) who had undergone repeat closure following failure. The success rate of repeat closure attempts at our institution was 98%.Of the 94 patients who had undergone successful repeat closure, definitive continence management and had their dryness evaluated, 38 were candidates for bladder neck reconstruction and 17(18%) were continent. Of the remaining patients, 90% were able to attain dryness, but at the expense of clean intermittent catheterization and continent urinary diversion.ConclusionA failed exstrophy closure has significant implications for long-term surgical outcome. Reclosure can be accomplished in the majority of cases. In comparison to patients with successful primary closure, the rates of urethral continence following successful repeat closure were lower.  相似文献   

5.
ObjectiveReconstruction of bladder exstrophy remains a challenge. We evaluated our experience with complete primary repair in classic bladder exstrophy.MethodsA retrospective data review was conducted of bladder exstrophy patients presenting at our institution between May 2000 and September 2007. Fifty-one patients (21 females and 30 males) with classic bladder exstrophy were included. Age of presentation ranged from 24 h to 14 months. Mean follow up was 3 years (1 month–7 years). Patients were evaluated for continence, upper tract dilatation and cosmetic result.ResultsEight patients (15.6%) had failed closures and six (11.7%) had fistulae. Evaluation of continence excluded 16 patients not followed up at our center. Thirty-seven percent were continent on clean intermittent catheterization after the age of 5 years. Patients became dry only after augmentation cystoplasty. Upper tract changes were mild during our study with all patients having normal serum creatinine.ConclusionPatients may require more than one procedure for reconstruction. In our series, augmentation was required to achieve acceptable dryness. Early promising results with dry intervals in young patients did not translate to continence in older patients.  相似文献   

6.
Treatment of children with severe impairment of bladder function requires a large-volume, low-pressure reservoir combined with a continent, easily catheterizable valve. The Mitrofanoff principle (MP) appears to meet these requirements.Between 1986 and 1993, the MP was applied in 15 children (4 girls) aged 4 to 14 years. The primary diagnosis was bladder exstrophy in 8 (2 girls), neuropathic bladder in 3 (2 girls), urethral valves in 2, and rhabdomyosarcoma (RMS) in 2. In 10 patients bladder augmentation with an intestinal patch was performed in addition to a Mitrofanoff procedure; in 5 a neobladder and continent appendicostomy were made. One boy with RMS died of distant metastases with a well-functioning appendicostomy and adequate renal function. At 2 to 9 years follow-up of the other 14 patients, 12 have a good result defined as: (1) adequate reservoir capacity; (2) continence; (3) normal renal function; and (4) no hydronephrosis. In 1 exstrophy patient with preexisting impairment of renal function, further deterioration necessitated frequent catheterization and additional medical treatment. In I boy with fulgurated urethral valves, spontaneous micturition became subsequently possible, allowing closure of his appendicovesicostomy. Complications occurred in 10 patients, necessitating reintervention in 7. The MP in combination with the creation of an adequate reservoir gives good results in children with severe impairment of bladder function. Careful attention should be given to patient education regarding emptying of the reservoir. Long-term follow-up of renal function is mandatory.  相似文献   

7.
PurposeThe radical soft-tissue mobilization procedure was developed as a component of the staged closure of classical bladder exstrophy to improve continency rates without having to perform pelvic osteotomies. The authors describe complications following this procedure and discuss possible etiologies and subsequent management.Materials and methodsWe extracted from an institutionally approved exstrophy database the records of patients evaluated for complications following radical soft-tissue mobilization repair from 1999 to 2002.ResultsFour patients were referred to our institution following closure of exstrophy with the radical soft-tissue mobilization technique; two boys and two girls. Complications included ischemic penile injuries in both males, failed exstrophy closure in one female, incontinence with need for bladder neck transection and diversion in two patients, and upper tract deterioration in two patients of whom one required cystectomy and incontinent diversion.ConclusionsOmission of osteotomies when employing the radical soft-tissue mobilization repair appears to result in complications that could otherwise be prevented. Additionally, the complex dissection of the pelvic musculature, innervation and vasculature performed during radical mobilization has great potential to injure the pelvic structures and genitalia, as has been seen with the cases presented herein.  相似文献   

8.
ObjectiveMany changes have occurred in the treatment of bladder exstrophy over the last few years and several repairs are now offered, but there is a lack of long-term follow-up data. The purpose of this study was to evaluate long-term outcomes in a select group of female patients in whom modern staged repair was undertaken.Patients and methodsFrom an institutionally approved database 41 patients were identified. All had undergone primary bladder exstrophy closure in 1988–2005, at a mean age of 2 months (range 4 h to 3 months), with or without an osteotomy by a single surgeon, and all were followed up for a minimum of 5 years. Twelve patients underwent osteotomy at the time of primary closure. Eight had a classic transverse innominate and vertical iliac osteotomy, and four a transverse innominate only. Mean age at the time of bladder neck repair (BNR) was 4.2 years (39–65 months). Mean measured bladder capacity under gravity cystograms at the time of repair was 109 cc (80–179 cc).ResultsThirty patients (74%) were continent day and night, and voiding per urethra without augmentation or intermittent catheterization. Social continence, defined as dry for more than 3 h during the day but damp at night, was found in a further four cases (10%). Seven patients are completely incontinent with dry intervals of less than 1 h day and night. The mean time to daytime continence was 12 months (4–16 months) and to night-time continence was 19 months (10–28 months). Patients with a mean capacity greater than 100 cc had better outcomes. Six of the 30 patients achieved dryness after primary closure only, and all six had transverse innominate and vertical iliac osteotomy at the time of primary closure.ConclusionsFemale classic exstrophy patients with a good template who develop adequate capacity after a successful primary closure can achieve acceptable continence without bladder augmentation and intermittent catheterization. A select group will develop continence with closure alone without the need for bladder neck repair.  相似文献   

9.
ObjectiveTo report the results of recto sigmoid bladder reservoir (RSBR), a variant of ureterosigmoidostomy (US) in patients with bladder exstrophy (BE) using a three dimensional evaluation system.Patients and methodsThis was a predominantly retrospective evaluation for all patients of BE undergoing RSBR who had completed minimum of 1 year follow-up. The patients were evaluated using a three dimensional system which included evaluation of urinary continence per rectally, status of upper tracts and health related quality of life (HRQOL).ResultsA total of 19 patients (four females) were evaluated. Mean age was 8.4 years, and mean duration of follow-up was 3.4 years. Mean age at RSBR was 5.1 years. Ten patients had good rectal continence. There was occasional minimal urine leak while passing flatus or on straining in nine. All patients had preserved renal function except one. The mean HRQOL score was 129.7 out of maximum of 150..ConclusionsRSBR gives marked improvement in quality of life in patients of BE. In the literature there is a non-uniformity in the results reporting outcome of US. The presented three dimensional assessment provides a comprehensive way to report the results in the short/long term. If used by other surgeons it would make comparison across centers feasible.  相似文献   

10.
ObjectiveTo report our experience in the management of adult classic bladder exstrophy.Patients and methodsDuring 1977‒2006 we treated five adult males presenting with classic exstrophy–epispadias complex. Patient age at presentation ranged from 17 to 30 with a mean age of 23 years. Four patients had received no previous treatment and one underwent previous ureterosigmoidostomy. Work-up included evaluation of upper tract and bladder biopsy. Bladder patch condition was variable. Surgery involved bladder preservation in the three patients who underwent primary repair, including bladder closure, bladder neck reconstruction and epispadias repair; two of them also had augmentation ileocystoplasty. The remaining two patients underwent ureterosigmoidostomy, cystectomy and epispadias repair. Abdominal wall closure was by fasciocutaneous M-plasty. Osteotomy was not done in any case.ResultsIn patients with bladder preservation, one patient was continent (>3 h) and voided normally whereas the other two showed day and night continence (2–3 h) with mild stress incontinence. Patients were satisfied with functional outcome. Ultrasound and intravenous pyelography showed preservation of upper tract. Follow-up period ranged from 1 to 8 years.ConclusionPatients with bladder exstrophy presenting in adulthood should not be denied the opportunity of primary reconstruction with bladder preservation in the absence of significant histological changes in the bladder mucosa.  相似文献   

11.
ObjectiveDelayed exstrophy repair (DER) represents an alternative to early neonatal bladder closure. This study aims to define the consequence of DER on bladder growth in bladder exstrophy patients who underwent routine DER, compared with those who underwent immediate postnatal reconstruction.MethodsBetween 2000 and 2005, classic bladder exstrophy patients referred to the authors' institution underwent early neonatal bladder closure (group 1). Subsequently, classic bladder exstrophy patients referred to the authors' institution were treated with an elective DER (group 2). Bladder capacity was assessed between the age of 1 and 4 years with an unconscious cystogram. When dilating VUR was present, the volume of the contrast migrated into the ureter was calculated and subtracted.ResultsSixty patients were treated between 2000 and 2012. Complete follow-up data were available for 45 patients and they were included in the study: 21 in group 1 (11 males) and 24 in group 2 (14 males). The mean (SD) bladder volumes were 72.85 (28.5) ml in group 1 and 72.87 (34.9) in group 2 (p = 0.99).ConclusionIn the authors' experience, DER does not reduce the subsequent bladder capacities compared with neonatal exstrophy closure.  相似文献   

12.
PurposeReconstruction of bladder exstrophy remains a challenge. We evaluated our experience with different techniques in different age groups.Material and MethodsA retrospective data review was conducted of bladder exstrophy patients presenting at our institution between May 2000 and September 2007. 61 patients (21 females and 40 males) with classic bladder exstrophy were included. Age of presentation ranged from 24 hours to 14 months. 51 patients underwent complete primary repair and 11 patients had staged repairs. Mean follow up was 3 years (1 month to 7 years). Mortality involved one male & one female (3.2%). 29 patients underwent anterior innominate osteotomy and 9 underwent posterior osteotomy. Patients were evaluated for continence, upper tract dilatation and cosmetic result.ResultsIn the primary repair group, 8 (15.6%) had failed closures and 5 (9.8%) had fistulas. In the staged repair group, 1 (0.9%) had a failed closure and 1 had an epispadic fistula. Evaluation of continence excluded 9 patients recently operated on and 17 patients not followed up at our center. The primary repair group had 15 of 25 dry patients (11 females and 4 males). In the staged repair group, 4 of 10 patients were continent. 4 of our staged cases became dry following augmentation cystoplasty. Upper tract changes were mild during our study (44% of complete repair group and 12.5% in the staged group) with all patients having normal serum creatinine.ConclusionsPatients may require more than one procedure for reconstruction. Continence was better in females than males with primary or staged repairs.  相似文献   

13.
Husmann DA  Rathbun SR 《Journal of pediatric urology》2008,4(5):381-5; discussion 386
ObjectiveTo determine the risk of bladder cancer following enteric bladder augmentation.Materials and methodsPatients followed for care after an enteric bladder augmentation have been entered into a registry; individuals followed for a minimum of 10 years were evaluated.ResultsThe study criteria were met by 153 patients. Indications for bladder augmentation were neurogenic bladder in 97, exstrophy in 38 and posterior urethral valves in 18. There was a median follow-up interval of 27 years (range 10–53). A total of seven cases of malignancy developed. Median time to tumor development following augmentation was 32 years (range 22–52). Two patients with neurogenic bladder developed transitional cell carcinoma; both were heavy smokers (>50 pack per year history). Two patients with a history of posterior urethral valves and renal transplantation developed adenocarcinoma of the enteric augment. Three patients with bladder exstrophy developed multifocal adenocarcinoma of the augmented bladder. Two patients remain alive, 5 and 6 years following radical cystoprostatectomy; five died of cancer-specific causes.ConclusionsMalignancy following enteric bladder augmentation arose in 4.5% (7/153) of our patients and was associated with coexisting carcinogenic stimuli (prolonged tobacco/chronic immunosuppressive exposure), or alternatively with the inherent risk of malignancy existing with bladder exstrophy.  相似文献   

14.
PurposeIt is controversial whether osteotomy by restoring a more normal pelvic anatomy might improve the final outcome of bladder exstrophy (BE) repairs. We compared the functional orthopaedic and urological outcome in BE patients treated with and without osteotomy.Material and MethodsOrthopaedic and urological outcome was compared in 8 BE patients treated with osteotomy and 6 BE patients treated without osteotomy. Orthopaedic evaluation included an assessment of pubic bones dissymmetry, bending of the spine, presence of Trendelemburg or Thomas sign, and presence of out-toeing. Pubic diastasis was ruled out with a plain x-ray of the pelvis. A Pediatric Orthopedic Society of North America (POSNA) questionnaire was administered to every child or his/her caregiver to assess functional outcome. Urological evaluation included an assessment of contemporary continence status and required continence surgery.ResultsAll patients presented a pubic diastasis. This was in median 49 (24 – 66) mm in patients treated without osteotomy and 42 (25 – 101) mm in those treated with osteotomy (p = 0.3). There was no difference either in the orthopaedic outcome or in any features of the POSNA questionnaire. Nor was there a difference in the final continence rate or in the number of additional continence procedures required.ConclusionsAlthough osteotmy is an essential step in the treatment of many BE patients in order to achieve a tension-free closure of the bladder and the abdominal wall, our preliminary RESULTS suggest that it does not improve the eventual orthopaedic or urological outcome of BE.  相似文献   

15.
ObjectiveAfter posterior urethral valve ablation, some boys are still have a hostile bladder. We conducted a prospective randomized study to determine if Botox injection at the bladder neck will help improve vesical dysfunction in this subgroup of boys or not.Patients and methodsTwenty boys with history of posterior urethral valve ablation and severe bladder dysfunction with a mean age of 16 months were studied. Cases were further randomized into two groups. Group I (study group) had endoscopic injection of a single dose of 100 IU of Botox into the hypertrophied bladder neck at 3, 6, and 9 o’clock. Group II (control group) patients with the same parameters had urethroscopy to exclude residual valves. Both groups had the standard conservative treatment. Cases were followed after 6 months of initiating the management protocol. This includes laboratory studies (urine culture and sensitivity, blood urea nitrogen, serum creatinine), ultrasound of the urinary tract. Voiding cysto-urethrogram and urodynamic study (pressure flow study).ResultsThere was no statistical difference in both groups regarding rate of urinary tract infection, improvement of hydronephrosis, resolution of vesico-ureteral reflux, creatinine level at the start or at the end of the study. Urodynamic parameters revealed an increase in cystometric capacity in both groups at the end of the study but without statistical difference. The mean voiding pressure reduced significantly in both groups but without statistical difference.ConclusionsTemporarily abolishing the effect of bladder neck by Botox injection does not seem to improve the outcome of those boys who had a severe voiding dysfunction after valve ablation.  相似文献   

16.
ObjectiveIt is accepted that the length of the anterior segment of the pelvis in classic bladder exstrophy is shorter than that of controls. However, studies performed involve children with a wide range of ages. By studying children with classic bladder exstrophy under the age of 1 year, the authors aim to ascertain if there is a congenital pubic bone length discrepancy associated with bladder exstrophy.Material and methodsFourteen classic bladder exstrophy patients under 1 year of age and 14 age-matched controls were identified. Three-dimensional (3D) computerized tomography was used to measure the length of the superior pubic ramus bilaterally. In the exstrophy group, scans were performed before bladder closure. The Mann–Whitney U test was used and p < 0.05 was considered significant.ResultsThere was no significant difference (p = 0.76) in the median age in cases (72 days) and controls (64 days). The median superior pubic ramus length was 19.1 mm in the exstrophy group and 20 mm in the control group (p = 0.99).ConclusionsSuperior pubic ramus length in children under 1 year of age with classic bladder exstrophy is not statistically different from that in control subjects. Therefore, the authors hypothesize that the previously described shortening of the pubic bones is an acquired phenomenon, which may develop after the first year of life.  相似文献   

17.
ObjectiveWe report our experience with the Indiana pouch (continent urinary reservoir) in 12 consecutive children over the last 15 years and report their follow-up.Material and methodsTwelve consecutive children, who underwent the continent urinary reservoir procedure in the form of an Indiana pouch, were prospectively enrolled in the study. All consecutive children who were referred to our service with multiple failed surgeries for exstrophy–epispadias repair, cloacal exstrophy, genitourinary rhabdomyosarcoma with residual disease in the trigonal area not amenable to partial cystectomy, and neuropathic bladder with nephrogenic metaplasia were included over the period 1997–2012. All these children were offered the same form of bladder substitution (Indiana pouch) as described by the Indiana group many years ago. Postoperative care was on a fixed protocol, and follow-up details recorded over the years. They were followed up for dry interval with clean intermittent catheterisation, social acceptance, and early and late complications.ResultsOut of these 12 patients (7 males and 5 females), eight patients had exstrophy–epispadias with multiple failed operations carried out elsewhere, cloacal exstrophy (2), residual rhabdomyosarcoma in the trigonal area with incontinence following chemotherapy (1), and neuropathic bladder with recurrent diffuse neoplastic polyposis (1). In the follow-up period of 1–15 years (median 24 months) all the patients had a dry interval of 4 h or more with clean intermittent catheterisation. One patient had wound dehiscence during the postoperative period and another required stomal revision 1 year after initial surgery.ConclusionsThe Indiana pouch is a reliable, safe, and effective form of bladder substitution. It can be reconstructed in a wide range of lower urinary tract disorders. In the vast majority of children with multiple failed surgical procedures for exstrophy–epispadias, the Indiana pouch is a safe, reliable, and reproducible procedure to provide a socially acceptable quality of life with a dry interval of 4 h.  相似文献   

18.
PurposeTo present the long-term RESULTS of bladder autoaugmentation in children with low compliant neurogenic bladders comparing the usual technique with a modified technique using an intra vesical silicon inflatable balloon device.Material and MethodsTwenty-two patients with low-compliant neurogenic bladders and incontinence confirmed urodynamically were divided in two groups. Group I (12 patients) underwent autoaugmentation using the classical detrusorectomy approach. Group II (10 patients) underwent autoaugmentation associated to an intra vesical silicon inflatable device filled with saline solution customized to allow urine elimination and sustain partial bladder distention.ResultsFollow up ranged from 2 to 11 years (mean 4 years). Patients in Group I showed unsatisfactory RESULTS, with only one patient achieving continence using clean intermittent catheterization (CIC). Four patients showed mild improve on urodynamic parameters. Patients in group II showed encouraging RESULTS. Six patients achieved continence with CIC. Two patients achieved continence using CIC and anticholinergic agents. Bladder capacity had a mean increase of approximately 71% and bladder compliance mean increased from 15.6 ± 16.8 ml/cmH2O to 34.3 ± 22.8 ml/cmH2O (p = 0.02). Two patients with previously dilated upper urinary tract showed a decrease in the grade of dilatation.ConclusionsBladder autoaugmentation using an intra vesical silicon inflatable balloon device showed better RESULTS compared to the usual autoaugmentation technique. The presented device didn't add morbidity to the surgical procedure. This technique can be used in selected patients in order to improve urodynamic parameters and continence without the potential disadvantages of using bowel segments for bladder augmentation.  相似文献   

19.
目的 评价双侧前外侧骨盆截骨在治疗小儿膀胱外翻中的应用.方法 1997年1月至2007年12月13例膀胱外翻患儿在本院行双侧前外侧骨盆截骨术合拢骨盆环,膀胱内翻关闭术,年龄6个月~13岁.其中3例是曾在外院作过膀胱外翻关闭手术失败的病例.骨盆截骨采用从髂前上棘髂前下棘之间到坐骨大切迹的前外截面.结果 平均随访5年(6个月~10年).I期修复膀胱外翻,术后无腹壁伤口裂开,耻骨合拢处分离不超过2 cm,步态正常.其中3例男性患儿术后随访排尿得到了良好控制.结论 双侧前外侧骨盆截骨在术中不需要改变体位,截骨后使耻骨尽町能合拢,且可用于膀胱外翻关闭手术失败再次截骨,较传统的后侧骨盆截骨术治疗膀胱外翻更优越.骨盆截骨手术有助于膀胱外翻患儿今后控制排尿.  相似文献   

20.
PurposePelvic osteotomies have been shown to enhance success rates for classic exstrophy patients when closed primarily or secondarily after initial failure. Primary closure of cloacal exstrophy also benefits from osteotomy but this has yet to be shown for re-closure of cloacal exstrophy failures. This study looks at the applications, complications, and long-term success rates in this very select group of patients.MethodsWe extracted from an institutionally approved exstrophy database 15 patients who had undergone repeat pelvic osteotomy and analyzed patient history, complications and orthopedic outcomes.ResultsAll patients who underwent reclosure at our institution remain closed. Major complications were seen in two patients and minor complications in four patients. Urinary continence was achieved in 10 patients with augmentation and continent stoma formation, urinary diversion was performed in two patients and three patients await a continence procedure.ConclusionsRepeat pelvic osteotomy in cloacal exstrophy is successful and the complication rate is low. Pelvic osteotomy is associated with enhanced success rates of primary and secondary closure with better cosmesis of the abdominal wall and genitalia. Intrasymphyseal plates along with gradual reduction of the extreme diastasis utitlizing an external fixation device can be beneficial prior to further genitourinary surgery.  相似文献   

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