Transitional Care for Patients with Congenital Colorectal Diseases: An EUPSA Network Office, ERNICA, and eUROGEN Joint Venture

Background: Transition of care (TOC; from childhood into adulthood) of patients with anorectal malformations (ARM) and Hirschsprung disease (HD) ensures continuation of care for these patients. The aim of this international study was to assess the current status of TOC and adult care (AC) programs for patients with ARM and HD. Methods: A survey was developed by members of EUPSA, ERN eUROGEN, and ERNICA, including patient representatives (ePAGs), comprising of four domains: general information, general questions about transition to adulthood, and disease-specific questions regarding TOC and AC programs. Recruitment of centres was done by the ERNs and EUPSA, using mailing lists and social media accounts. Only descriptive statistics were reported. Results: In total, 82 centres from 21 different countries entered the survey. Approximately half of them were ERN network members. Seventy-two centres (87.8%) had a self-reported area of expertise for both ARM and HD. Specific TOC programs were installed in 44% of the centres and AC programs in 31% of these centres. When comparing centres, wide variation was observed in the content of the programs. Conclusion: Despite the awareness of the importance of TOC and AC programs, these programs were installed in less than 50% of the participating centres. Various transition and AC programs were applied, with considerable heterogeneity in implementation, content and responsible caregivers involved. Sharing best practice examples and taking into account local and National Health Care Programs might lead to a better continuation of care in the future. Level of Evidence: III

Tratamiento del reflujo vesicoureteral en niños con mielomeningocele

OBJECTIVES: To perform a bibliographic review on the topic of vesicoureteral reflux (VUR) in children with myelomeningocele. METHODS: PubMed search using the terms: vesicoureteral reflux and myelomeningocele. RESULTS: Between 1981 and 2007 340 articles were found, 61 of which were considered adequate, and 48 were used as references for this monographic issue. We discuss the pathogenesis of VUR in neurogenic bladder, the efficacy of conservative treatment with clean intermittent catheterization and anticholinergic drugs, as well as the indications and efficacy of various surgical procedures including ureteral reimplantation, subureteral injection of various agents, bladder augmentation, cutaneous vesicostomy, urethral dilation, transureteral-ureterostomy and botulin toxin injection. CONCLUSIONS: Vesicoureteral reflux plays an important role in the development of renal damage in infants and children with congenital myelomeningocele. Nephropathy may be prevented with adequate evaluation and follow-up and timely therapeutic intervention. Renal failure in these patients always reflects the failure of medical care or social/socioeconomic conditions

Handbook of Urological Diseases in Children

Handbook of Urological Diseases in Children - This definitive manual provides a quick reference on pediatric urologic problems for medical students and physicians in training. Presented in two clearly outlined sections, part one introduces, in 12 chapters, the common forms of presentation of urological conditions in children, with emphasis on anamnesis, differential diagnosis and the appropriate use of diagnostic tools. Treatment modalities are also briefly discussed. Part two consists of 11 chapters that discuss the common pediatric urological conditions. The emphasis for part two is the provision of practical and up-to-date information in a standardized, easy-to-read format. There is extensive cross-referencing between parts one and two. Finally, an appendix presents practical information such as renal length, grading of reflux, etc

Importance of urinary flow studies after hypospadias repair: a systematic review

A systematic review was performed of publications relating to the results of urinary flow studies after hypospadias repair dating back to 1978, when what appears to be the first publication on this topic was found. The literature search was performed using the key words "hypospadias" combined with "urinary flow", "urine flow", "uroflow", "uroflowmetry", and "long-term". We also reviewed the abstracts and full-length articles cited in the reference list of selected articles. Criteria for inclusion in the present systematic review included descriptions of patient selection, surgical technique, the severity of disease (proximal vs distal), and the method used to determine uroflow, as well as a definition of urethral obstruction. In all, 339 article titles were found. Of these, 25 abstracts appeared relevant and the full text of these articles were reviewed, with 22 of the papers included in this review. Sixteen reports had appeared in the literature since 2001, compared with six between 1978 and 2000, suggesting an increasing interest in this topic. On the basis of the results of the present systematic review, we recommend that asymptomatic children operated on for hypospadias in infancy should have one flow study after toilet training, with that study repeated if the results are abnormal. It appears that early abnormal flows improve spontaneously, so that children operated on after toilet training who are asymptomatic should undergo their first uroflow measurement 1 year after surgery. Children with obstructed flow parameters or borderline flows should be followed until adulthood, until long-term follow up studies clarify the significance of abnormal flow parameters. Given the present findings, we anticipate that in the next two decades urologists will need to treat a number of men with strictures resulting from hypospadias repairs performed in childhood

The long-term culture of porcine urothelial cells and induction of urothelial stratification.

OBJECTIVE: To assess porcine urothelial cell cultures and the in vitro induction of urothelial stratification in long-term cultures, to study their morphological, functional and genetic behaviour, and thus provide potential autologous urothelium for tissue-engineered substitutes for demucosalized gastric or colonic tissue. MATERIALS AND METHODS: Primary cultures of porcine urothelium were established and the cells passaged thereafter. Cell specificity was confirmed by cytokeratin analysis, cell membrane stability assessed using lactate dehydrogenase leakage, cell de-differentiation by gamma-glutamyl transferase activity and genomic stability by karyotype investigations. Histology and scanning electron microscopy were performed to study the cultured cells and the stratified constructs. Furthermore, collagen matrices were tested as cell scaffolds. RESULTS: The cells were cultured for 180 days; 10 subcultures were established during this period. Stratification was induced in a culture flask and on a collagen matrix. Cytokeratins 7, 8, 17 and 18 were expressed in all cultures, and cell membranes were stable, with no evident de-differentiation. The cultures were stable in their genotype and no chromosomal aberrations were found. The histology and immunohistochemistry of the stratified porcine constructs, and cell membrane stability and cell de-differentiation, were compared with those in the human system. CONCLUSION: Pig and human urothelial cells can be cultured over a long period with no signs of senescence. Urothelial stratification can be induced in vitro. The collagen matrix seems to be an excellent scaffold, allowing cell adherence and growth