64 research outputs found

    Isolated Fetal Ascites Secondary to Persistent Urogenital Sinus

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    Objective. To present a case of isolated ascites secondary due to urogenital abnormalities (urogenital sinus) without any other prenatal ultrasound marker. Method. A 36-year-old woman with prenatal isolated ascites delivered a female baby, weighing 2.285 g; ascites was drained at birth and the baby underwent several episodes of urinary retention prior to undergoing X-ray investigations. Results. A voiding cystourethrogram revealed a short urogenital sinus: a vesicostomy was performed. A vaginoscopy revealed double vagina with a large posterior vagina. A posterior sagittal anorectal pull-through with genitoplasty was performed at 2 years old with 1-year follow-up. Conclusions. Though rare, a urogenital abnormality is to be suspected in fetal ascites cases with negative viral tests and no cardiac anomalies. The most common ultrasound marker of such abnormalities (fluid filled cavity) may be missing because of complete drainage of urine through the tubes into peritoneum

    Case Report Isolated Fetal Ascites Secondary to Persistent Urogenital Sinus

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    Objective. To present a case of isolated ascites secondary due to urogenital abnormalities (urogenital sinus) without any other prenatal ultrasound marker. Method. A 36-year-old woman with prenatal isolated ascites delivered a female baby, weighing 2.285 g; ascites was drained at birth and the baby underwent several episodes of urinary retention prior to undergoing X-ray investigations. Results. A voiding cystourethrogram revealed a short urogenital sinus: a vesicostomy was performed. A vaginoscopy revealed double vagina with a large posterior vagina. A posterior sagittal anorectal pull-through with genitoplasty was performed at 2 years old with 1-year follow-up. Conclusions. Though rare, a urogenital abnormality is to be suspected in fetal ascites cases with negative viral tests and no cardiac anomalies. The most common ultrasound marker of such abnormalities (fluid filled cavity) may be missing because of complete drainage of urine through the tubes into peritoneum

    Consensus review of best practice of transanal irrigation in adults

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    Study design: Review article. Objectives: To provide a consensus expert review of the treatment modality for transanal irrigation (TAI). Methods: A consensus group of specialists from a range of nations and disciplines who have experience in prescribing and monitoring patients using TAI worked together assimilating both the emerging literature and rapidly accruing clinical expertise. Consensus was reached by a round table discussion process, with individual members leading the article write-up in the sections where they had particular expertise. Results: Detailed trouble-shooting tips and an algorithm of care to assist professionals with patient selection, management and follow-up was developed. Conclusion: This expert review provides a practical adjunct to training for the emerging therapeutic area of TAI. Careful patient selection, directly supervised training and sustained follow-up are key to optimise outcomes with the technique. Adopting a tailored, stepped approach to care is important in the heterogeneous patient groups to whom TAI may be applied. Sponsorship: The review was financially supported by Coloplast A/S. Spinal Cord (2013) 51, 732–738; doi:10.1038/sc.2013.86; published online 20 August 201

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

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    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

    Effect of Dexamethasone on Postoperative Adhesion Formation

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    Typescript (photocopy).Allomyces macrogynus produces uninucleate, motile gametes by the rearrangement and cleavage of the multinucleate cytoplasm of gametangia. In other aquatic fungi, a Golgi complex made up of stacked cisternae is the source of new membrane and glycoproteins formed during cytoplasmic cleavage. Allomyces does not have stacks of Golgi cisternae but instead has individual smooth endomembrane elements, termed Golgi equivalents. The objective of this study was to use an ultrastructural, pharmacological, and biochemical approach to determine the origin of cleavage furrow membrane during gametogenesis. The microdroplet culture method developed for this study gave a 1.7 to 2.4-fold higher degree of synchrony during gametogenesis. Using this method, it was possible to construct a precise timing sequence for gamete differentiation. Straining with ZnI-OsO₄ impregnation demonstrated that the endoplasmic reticulum was the origin of the nuclear cap membrane but neither the cleavage furrows nor the membrane elements which gave rise to them strained with ZnI-OsO₄. Since acidic compartments, such as trans Golgi do not stain by this method it appeared likely that cleavage furrows might be derived from Golgi equivalents. To further test the hypothesis that Golgi equivalents were involved in cleavage, induced gametangia were treated with monensin, an ionophore which specifically affects trans Golgi cisternae. Gametangia treated with monensin did not complete cytoplasmic cleavage but, instead, released multinucleate gametes. Although swollen vacuoles were present, other gametogenic events including nuclear cap membrane formation occurred normally. Fractionation of subcellular components on sucrose density gradients demonstrated that Allomyces had both endoplasmic reticulum and Golgi equivalents with buoyant densities and marker enzyme profiles typical for these components in cells with stacked Golgi cisternae. For the first time, it was possible to demonstrate developmentally-regulated glycoproteins in vegetative hyphae and gametangia of Allomyces. Some of these glycoproteins appear in Golgi fractions, implying that the Golgi equivalents might be involved in glycosylation. In summary, Allomyces has a Golgi complex composed of individual or, perhaps, stacks of two Golgi equivalents. Although the endoplasmic reticulum was the source of the nuclear cap membrane, Golgi equivalents were the origin of cleavage furrow membranes formed during gametogenesis
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