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

Dimensionamento di barriere paramassi a rete

L'articolo descrive sperimentazione e modellazione di barriere paramassi a rete, ai fini di progettazione e studio del comportamento in vera grandezza delle opere di difesa dalla caduta massi

Calcification of the human heart valves: a mineralogical approach

Normal physiologic processes result in development of mineralized tissue. Bones and tooth enamel are the main example of biominerals. Pathologic processes lead to calcification of the atherosclerotic plaques, kidney and salivary stones and other pathologic deposits. Most of these seem to be constituted from a mixture of calcium phosphate phases but their formation mechanisms are not completely known. In cardiac pathology, calcification of heart valves can be advanced by a congenital malformation or an infectious process or related to the senile degeneration. Pathological mineral deposits occurring in human cardiac valves were studied using Polarizing Microscopy, Scanning Electron Microscopy (SEM-EDS), Electron Microprobe (EMPA), X-Ray Powders Diffraction (XRPD), Infrared Spectroscopy (FTIR). Samples were obtained as surgical waste from thirty patients undergoing valvular replacement in case of severe aortic and mitral stenoses. The experimental results showed that the mineral phase grown in human cardiac valves is a calcium phosphate with poor crystallinity. It develops as nodules in the organic matrix. The FT-IR spectra may be used to infer the presence of carbonate group. The carbonate bands in the infrared spectra have a saw-tooth profile similar to sample PC18, a synthetic type A-B CAp but in samples of aortic valves a-parameter is smaller and the c-parameter is greater than those of PC18 [i.e. TV12 a=9.4165(8) Å, c= 6.8951(7) Å; PC18 a=9.4803(3) Å, c=6.8853(3) Å] probably due to substitutional carbonate groups in phosphate positions which cause a shrinkage in the a-parameter. Pathological phase investigated can be considered a bioapatite as the inorganic component of bone and tooth enamel, even if it possesses unusual morphologies for a calcium phosphate and a Ca/P ratio unlike that of normal mineralized tissue

Pathological biomineralization from human aortic and mitral valve stenosis.

Samples were collected as surgical waste from patients undergoing valvular replacement because of severe aortic (n=6) and mitral (n=2) stenosis. Pathological mineral formations have been investigated with XRPD and SEM-EDS, both in high and in low vacuum conditions. Samples were not coated because of metallic coating artifacts.The a cell parameters were found to be smaller than the a parameter of human dental enamel apatite, while the c parameters were greater. High resolution images show a complex relationship between inorganic component and organic matrix as well as particular morphologies of the pathogenic biomineralization. Bioapatite appears as lamellar crystals, globular aggregated and massive; at high magnification it appears to be constituted of spherical particles of variable size. Bioapatite morphology observed in this study appears to be different from biogenic calcium phosphate crystals and from inorganically produced counterparts. The small spheres could be considered as nanobacterial-like structures (?). This attractive hypothesis has not been confirmed yet

Myosin changes in hypertrophied human atrial and ventricular myocardium. A correlated immunofluorescence and quantitative immunochemical study on serial cryosections.

Two antigenically distinct types of myosin heavy chain, referred to as alpha and beta, have been identified in autoptic and bioptic specimens of human heart using specific antimyosin antibodies. By immunofluorescence heavy chain alpha was present in all atrial myocytes and in a variable number of ventricular myocytes. Heavy chain beta was present in all ventricular myocytes and in a number of atrial myocytes. Ventricular hypertrophy in patients with aortic stenosis, systemic hypertension or tetralogy of Fallot was characterized by an almost complete absence of fibres reactive with anti-alpha. A striking decrease in alpha chain reactivity and a parallel increase in beta chain reactivity was apparent in the hypertrophied left atria of patients with mitral stenosis. To quantify these myosin changes a novel procedure was developed whereby myosin was extracted from single cryosections serial to those processed for immunofluorescence and the relative amount of alpha and beta heavy chain was determined by enzyme immunoassay. Heavy chain alpha was less than 5% in most normal ventricular specimens and disappeared completely under the effect of pressure overload. On the other hand heavy chain beta was generally undetectable in the left atrial myocardium but increased up to 90% in biopsies of hypertrophied atria

Hancock II bioprosthesis: a glance at the microscope in mid-long-term explants

Abstract BACKGROUND AND OBJECTIVES: The Hancock II bioprosthesis is a second-generation porcine valve xenograft treated with the detergent sodium dodecyl sulphate (T6) to retard calcification. The aim of this investigation was to study the gross and microscopic features in Hancock II explants to assess the structural changes occurring with time. METHODS: Among 1382 Hancock II bioprostheses (701 isolated aortic, 421 isolated mitral, 130 double) implanted from 1983 to 1997 in 1252 patients, 22 (16 mitral, 6 aortic) were removed at reoperation until 1999 and were available for pathological investigation: infective endocarditis occurred in 5 and structural deterioration in 8, whereas in the remaining 9 xenografts reoperation was performed for nonstructural valve deterioration (paravalvular leak in 4 and prophylactic replacement in 5). Morphological investigation consisted of gross examination and x-ray, histologic, immunohistochemistry, electron microscopic, and atomic absorption spectroscopic examination. RESULTS: The cause of structural valve deterioration was dystrophic calcification in 4 cases (1 aortic, 3 mitral; range of time graft was in place, 101 to 144 months), non-calcium-related tears in 3 cases (all mitral, range 121 to 163 months), and commissural dehiscence in 1 (aortic, range 156 months). Five of the nonstructural valve deterioration explants (range 42 to 122 months) showed only pinpoint mineralization at the commissures. Mean calcium content in nonstructural deterioration explants was 14.70 +/- 22.33 versus 99.11 +/- 81.52 mg/g in explants with structural valve deterioration. Electron microscopic examination showed early nuclei of mineralization mostly consisting of calcospherulae upon cell debris. Local or diffuse lipid insudation was observed in all but 2 explants and consisted of cholesterol clefts, lipid droplets, and lipid-laden macrophages featuring foam cells. The lipid insudation was the most plausible cause of tearing in 2 explants. CONCLUSIONS: These pathologic findings support the clinical results of a delayed occurrence of structural failure of Hancock II bioprostheses and a mitigation of mineralization by the anti-calcification treatment. However, other factors such as lipid insudation may come into play in the long term. IF 3.26