Abstract:
PURPOSE:To present the long-term outcomes of appendicovesicostomy using the Mitrofanoff principle for end-stage Wolfram bladder dysfunction as an alternative to clean intermittent self-catheterization (CIC) per urethra mainly following blindness. METHODS:Twelve Wolfram patients presenting with bilateral hydroureteronephrosis and advanced bladder dysfunction were included in this study. All patients were managed initially by CIC per urethra. All of these patients became blind during follow-up and were unable to perform urethral CIC independently. Out of these patients, six patients agreed to proceed to appendicovesicostomy. Appendicovesicostomy urinary diversion using the Mitrofanoff principle was performed in these six blind patients. The rest of the patients stopped CIC or performed CIC irregularly. RESULTS:Severe hydroureteronephrosis and large bladders were found in all patients prior to intervention. All patients were able to conduct CIC independently through the stoma and maintained overnight bladder free drainage. In all patients with urinary diversion and CIC, the hydroureteronephrosis was reduced and renal function returned to normal. However, the non-intervention group ended with different degrees of progressive renal failure with three mortalities during the follow-up. CONCLUSIONS:We suggest appendicovesicostomy as a safe and lifesaving procedure for long-term management of bladder dysfunction in Wolfram syndrome particularly after progression to blindness.
journal_name
J Pediatr Surgjournal_title
Journal of pediatric surgeryauthors
Mozafarpour S,Kajbafzadeh AM,Mojtahed A,Mojtahed M,Mahboubi H,Shalileh Kdoi
10.1016/j.jpedsurg.2014.09.058subject
Has Abstractpub_date
2015-07-01 00:00:00pages
1201-4issue
7eissn
0022-3468issn
1531-5037pii
S0022-3468(14)00592-2journal_volume
50pub_type
杂志文章abstract:BACKGROUND/PURPOSE:The Institute of Medicine has identified medical error as a leading cause of death and injury, with deaths resulting from medical error exceeding those caused by motor vehicle collisions, breast cancer, or AIDS. The authors examined the incidence and sources of medical error in relation to adverse ev...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/s0022-3468(03)00396-8
更新日期:2003-09-01 00:00:00
abstract:BACKGROUND:The benefit of Ladd's procedure for malrotation at a Children's Hospital (CH) has not previously been established. Our aim was to characterize the potential variations in management and outcomes between CH and Non-Children's Hospitals (NCH) in the treatment of malrotation with Ladd's procedure. METHODS:Ther...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/j.jpedsurg.2014.08.011
更新日期:2015-03-01 00:00:00
abstract::Patients with advanced-stage favorable-histology (FH) Wilms' tumor have a 4-year relapse-free survival rate of 70% to 90% after resection and chemotherapy of actinomycin D, vincristine, and doxorubicin. These three agents are actively pumped out of cells by P-glycoprotein (Pgp). The authors studied whether Wilms' tumo...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(94)90283-6
更新日期:1994-08-01 00:00:00
abstract::C1300 is a murine neuroblastoma that arose spontaneously in an A/JAX mouse, and from which a clone termed TBJ was subsequently derived. C1300 is a slowly growing and poorly metastasizing tumor, whereas TBJ shows early systemic metastasis as well as aggressive local growth. Compared with TBJ cells, C1300 cells are high...
journal_title:Journal of pediatric surgery
pub_type: 杂志文章
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abstract:PURPOSE:To present the clinical and histological features of prepubertal testicular tumors (PTTs), the long-term experience of a single institution was reviewed. MATERIALS AND METHODS:A total of 62 prepubertal children who were treated for testicular tumors at Kanagawa Children's Medical Center from 1971 to 2014 were ...
journal_title:Journal of pediatric surgery
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更新日期:2016-08-01 00:00:00
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journal_title:Journal of pediatric surgery
pub_type: 杂志文章
doi:10.1016/0022-3468(75)90388-7
更新日期:1975-10-01 00:00:00
abstract::Pulmonary failure is the most frequent cause of mortality in newborns, accounting for 15,000 deaths yearly. It may be the result of the respiratory distress syndrome (RDS), meconium aspiration syndrome (MAS), or persistent fetal circulation (PFC), including infants with congenital diaphragmatic hernia (CDH). Early ide...
journal_title:Journal of pediatric surgery
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journal_title:Journal of pediatric surgery
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更新日期:1976-02-01 00:00:00
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journal_title:Journal of pediatric surgery
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journal_title:Journal of pediatric surgery
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journal_title:Journal of pediatric surgery
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journal_title:Journal of pediatric surgery
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doi:10.1016/j.jpedsurg.2008.10.095
更新日期:2009-02-01 00:00:00
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journal_title:Journal of pediatric surgery
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journal_title:Journal of pediatric surgery
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更新日期:1995-02-01 00:00:00
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journal_title:Journal of pediatric surgery
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更新日期:1980-08-01 00:00:00
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journal_title:Journal of pediatric surgery
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journal_title:Journal of pediatric surgery
pub_type: 临床试验,杂志文章,随机对照试验
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更新日期:1999-12-01 00:00:00
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journal_title:Journal of pediatric surgery
pub_type: 杂志文章,多中心研究
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更新日期:1999-05-01 00:00:00
abstract:PURPOSE:An artificial placenta (AP) utilizing extracorporeal life support (ECLS) could avoid the harm of mechanical ventilation (MV) while allowing the lungs to develop. METHODS:AP lambs (n = 5) were delivered at 118 days gestational age (GA; term = 145 days) and placed on venovenous ECLS (VV-ECLS) with jugular draina...
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journal_title:Journal of pediatric surgery
pub_type: 杂志文章
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更新日期:2000-06-01 00:00:00
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journal_title:Journal of pediatric surgery
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更新日期:2001-05-01 00:00:00
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journal_title:Journal of pediatric surgery
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更新日期:1999-01-01 00:00:00
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journal_title:Journal of pediatric surgery
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更新日期:1996-03-01 00:00:00
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journal_title:Journal of pediatric surgery
pub_type: 杂志文章
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更新日期:1982-02-01 00:00:00
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journal_title:Journal of pediatric surgery
pub_type: 杂志文章
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更新日期:2002-05-01 00:00:00
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journal_title:Journal of pediatric surgery
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更新日期:2001-02-01 00:00:00
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journal_title:Journal of pediatric surgery
pub_type: 杂志文章
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更新日期:2004-10-01 00:00:00
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journal_title:Journal of pediatric surgery
pub_type: 杂志文章
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更新日期:1988-03-01 00:00:00