Objective: Young adults with spina bifida (and other congenital neurologic diseases) have their own aspirations that may include education, employment, relationships and having children. As they move from pediatric to adult care, they must eventually transition to an adult healthcare team. The objective of this paper is to review the challenges and controversies in the transitional and adult care of people with congenital neurourological diseases.
View Article and Find Full Text PDFRisk Manag Healthc Policy
September 2024
Purpose: This study explores the effects of CMS reimbursement financial penalties from the Hospital-Acquired Condition Reduction Program (HACRP) on hospital-acquired infections (HAI) in hospitals across the United States.
Methods: Hospital-level data for 2896 hospitals in the United States were evaluated using multiple linear regression models with random effects analysis through a difference-in-differences study design to examine HAIs under the HACRP between hospitals that were financially penalized or not from calendar years 2013 to 2020.
Results: This study showed significant differences from the pre-program Total HAC scores to the most recent reviewed year, validating the efficacy of the HACRP, and showing a reduction of overall HAIs over the years evaluated in the study.
Healthcare-associated infections (HAIs) place a significant financial burden on United States hospitals. HAI treatments extend hospital lengths of stay and increase hospital operational costs while significantly reducing hospital profit margins. Given these challenges, the research aim of this study was to explore the association between HAIs and hospital financial performance.
View Article and Find Full Text PDFIntroduction: Women born with anorectal malformation (ARM) or Hirschsprung disease (HD) may have impaired urologic function resulting in sequelae in adulthood. This study assessed and compared self-reported urinary outcomes in adult females born with ARM or HD to a reference population.
Methods: This was an IRB approved, cross-sectional study of female-born patients with ARM or HD, who completed surveys between November 2021 and August 2022.
Cloacal exstrophy, also known as OEIS complex, is a rare condition, comprised of severe congenital anomalies. This case report describes a 24-year-old 46,XY individual who had repair of the omphalocele at birth, but has remained with an unrepaired bladder exstrophy. This case highlights the intersections between medical decision-making, individualized management of complex patients, and ethical considerations for adults lacking capacity.
View Article and Find Full Text PDFIntroduction: Bladder exstrophy and epispadias complex (BEEC) is a spectrum of congenital malformations ranging from an isolated epispadias to a full exstrophy. It is an uncommon disease and little is known on how patients cope with its implications later in life.
Objective: The goal of this study is to assess the sexual, continence and fertility outcomes of BEEC patients, who had reconstructive bladder surgery during childhood.
The objective of this scoping review is to provide a summary of the current literature regarding adolescents and young adults with histories of cloacal anomalies. Preferred Reporting Items for Systematic Reviews and Meta-analysis Extension for Scoping Reviews were used. Data were categorized into four domains-urologic, colorectal, gynecologic/obstetric, and sexual/psychosocial.
View Article and Find Full Text PDFWith continued improvements in medical care and surgical reconstruction, more patients with classic bladder exstrophy (CBE) are living into adulthood, than ever before. With improved survival, a greater emphasis on adult issues and improving quality of life (QOL) for these individuals is of increasing importance. This review aims to summarize data on long-term considerations for the adult with exstrophy and to highlight areas of future research and collaboration.
View Article and Find Full Text PDFBackground: Initial management of pediatric patients with neurogenic bladder is focused on clean intermittent catheterization and medical therapies. Those with more hostile or small capacity bladders require surgical intervention including bladder augmentation that can result in significant clinical sequelae. This study examines a rarely described approach wherein the bladder reconstruction is extraperitonealized by bringing bowel segments through a peritoneal window and then closed.
View Article and Find Full Text PDFContext: Differences of sex development (DSD) represent a wide range of conditions presenting at different ages to various health professionals. Establishing a diagnosis, supporting the family, and developing a management plan are important.
Objective: We aimed to better understand the presentation and prevalence of pediatric DSD.
Background: Little is known about fertility and pregnancy outcomes in patients with anorectal malformations (ARM), particularly those with long common channel cloaca and cloacal exstrophy who may have impaired fertility. The purpose of this study is to describe pregnancy and offspring data from a cohort of patients with ARM.
Methods: A retrospective review of female patients with ARM from our database, which includes patients operated on since 1980, was performed as well as a review of the literature.
Purpose: Long-term urinary outcomes for patients born with Hirschsprung disease (HD) and anorectal malformations (ARM) may impact their health and wellbeing into adulthood. This study describes self-reported long-term urinary outcomes in males with HD and ARM.
Methods: This was a prospective study of male patients in the Adult Colorectal Research Registry who completed surveys on urinary function between October 2019 and March 2022.
Purpose: The long-term impact of anorectal malformations (ARM) and Hirschsprung disease (HD) on sexual function is well recognized but understudied. This study evaluated self-reported sexual and fertility outcomes in adult males with ARM and HD.
Methods: This was an IRB approved, prospective study of males in the Adult Colorectal Research Registry who completed surveys between October 2019 and March 2022.
Posterior urethral valves (PUV) are the commonest cause of end-stage renal disease in children, but the genetic architecture of this rare disorder remains unknown. We performed a sequencing-based genome-wide association study (seqGWAS) in 132 unrelated male PUV cases and 23,727 controls of diverse ancestry, identifying statistically significant associations with common variants at 12q24.21 (p=7.
View Article and Find Full Text PDFHypospadias is a common condition and familiar, though sometimes challenging territory for the pediatric urologist. This review is a summary of hypospadias incidence, epidemiology, surgery, research, and complications intended for the non-specialist. It outlines the history, the principles of surgery for hypospadias, and the long-term outcomes.
View Article and Find Full Text PDFNeurogenic lower urinary tract (NLUT) dysfunction in paediatric patients can arise after congenital or acquired conditions that affect bladder innervation. With some patients, urinary tract dysfunction remains and is more difficult to treat without understanding the pathophysiology. We measured in vitro detrusor smooth muscle function of samples from such bladders and any association with altered Wnt-signalling pathways that contribute to both foetal development and connective tissue deposition.
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