A case of Incontinentia Pigmenti associated with congenital absence of portal vein system and nodular regenerative hyperplasia

Congenital absence of portal vein system (CAPVS) is a rare condition in which portal perfusion is bypassed by portosystemic shunt leading to the development of portal hypertension (PH) or porto‐systemic encephalopathy (PSE). Visceral anomalies and liver cancer can be associated with CAPVS1.Thanks to the advances in imaging, the number of CAPVS cases detected has increased. Incontinentia Pigmenti (IP) (OMIM #308300) also represents a rare condition, characterized by skin, teeth, hair, nails, eyes and central nervous system alterations, due to mutations of NEMO/IKBKG gene. We report on the first case of IP associated with CAPVS and nodular regenerative hyperplasia (NRH) of the liver, in a patient with facial dysmorphisms and speech delay. Although rare, this finding may support the role of NEMO in liver homeostasis

Solid Organ Transplantation During COVID-19 Pandemic: An International Web-based Survey on Resources’ Allocation

Background. Solid organ transplants (SOTs) are life-saving interventions, recently challenged by coronavirus disease 2019 (COVID-19). SOTs require a multistep process, which can be affected by COVID-19 at several phases. Methods. SOT-specialists, COVID-19-specialists, and medical ethicists designed an international survey according to CHERRIES guidelines. Personal opinions about continuing SOTs, safe managing of donors and recipients, as well as equity of resources' allocation were investigated. The survey was sent by e-mail. Multiple approaches were used (corresponding authors from Scopus, websites of scientific societies, COVID-19 webinars). After the descriptive analysis, univariate and multivariate ordinal regression analysis was performed. Results. There were 1819 complete answers from 71 countries. The response rate was 49%. Data were stratified according to region, macrospecialty, and organ of interest. Answers were analyzed using univariate- multivariate ordinal regression analysis and thematic analysis. Overall, 20% of the responders thought SOTs should not stop (continue transplant without restriction); over 70% suggested SOTs should selectively stop, and almost 10% indicated they should completely stop. Furthermore, 82% agreed to shift resources from transplant to COVID-19 temporarily. Briefly, main reason for not stopping was that if the transplant will not proceed, the organ will be wasted. Focusing on SOT from living donors, 61% stated that activity should be restricted only to "urgent"cases. At the multivariate analysis, factors identified in favor of continuing transplant were Italy, ethicist, partially disagreeing on the equity question, a high number of COVID-19- related deaths on the day of the answer, a high IHDI country. Factors predicting to stop SOTs were Europe except-Italy, public university hospital, and strongly agreeing on the equity question. Conclusions. In conclusion, the majority of responders suggested that transplant activity should be continued through the implementation of isolation measures and the adoption of the COVID-19-free pathways. Differences between professional categories are less strong than supposed

Pediatric post-transplant metabolic syndrome: new clouds on the horizon

Liver transplantation (LT) is a standard treatment for children with end-stage liver disease, standing at more than 90% survival rate after one yr, and at over a 70% survival rate after five yr. The majority of transplanted children enjoy an excellent quality of life but complications can occur in the long term, and can develop subclinically in otherwise well children; there are various underestimated nutritional and metabolic aspects, including the so-called post-transplant metabolic syndrome (PTMS). During the post-transplant period, the use of immunosuppressants, corticosteroids, calcineurin inhibitors, and the presence of risk factors, including non-alcoholic fatty liver disease (NAFLD), and kidney and bone complications have been largely implicated in PTMS development. Strategies to reduce the progression of PMTS should include careful screening of patients for diabetes, dyslipidemia, and obesity, and to support weight reduction with a carefully constructed program, particularly based on diet modification and exercise. With early identification and appropriate and aggressive management, excellent long-term health outcomes and acceptable graft survival can be achieved

A new strategy for analysis of erection stages including an efficient method for creep analysis

International audienc

Metabolic syndrome and nonalcoholic steatohepatitis recurrence after liver transplantation in children

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Steatosis and fibrosis in paediatric liver transplant: Insidious graft's enemies - A call for clinical studies and research.

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Risk factors for death and graft loss after small bowel transplantation

Small bowel transplantation is generally reserved for patients with life-threatening complications related to the administration of parenteral nutrition, which includes multiple venous thromboses impeding the placement of feeding catheters and progressive liver disease. Approximately two thirds of recipients are children, who are especially at risk of hepatic complications related to parenteral nutrition. Many patients are in poor condition preoperatively according to the Intestinal Transplant Registry (available at: http://[email protected]), with 51% chronically hospitalized and up to 50% dying before donor organs can be provided. The main causes of graft loss are related to the complex surgery undertaken (combined liver and bowel transplants may be complicated by bile leaks or obstruction and small bowel perforation) and the necessity to treat patients with high dose immune suppression because of the highly immunogenic nature of small bowel allografts. Even so, uncontrolled rejection was reason for graft removal in 57% of cases (and death in 9%), but the main cause of death after small bowel transplantation is infection (56.1%). Fatal infectious episodes ensue from common respiratory pathogens such as parainfluenzae, intestinal viruses such as adenovirus and opportunistic pathogens such as vancomycin resistant enterococcus, pneumocystic carinii and Epstein-Barr virus. Lymphoma caused by Epstein-Barr virus is a particular risk in pediatric recipients who are usually naive to the virus, and up to 50% of infants can become infected by the graft at the time of transplant or shortly after being discharged. Treatment toxicity also contributes to mortality after transplantation: tacrolimus, mycophenolate, and sirolimus can all produce profound bone marrow suppression; nephrotoxicity also complicates the use of tacrolimus, and impaired wound healing is associated with steroids and sirolimus. New strategies are being developed to reduce the mortality after small bowel transplantation, including the use of interleukin-2 inhibitors, early detection and treatment of viruses such as Epstein-Barr virus and adenovirus, monitoring the immune responsiveness of patients, and technical improvements to the transplant operation

Standardized quick en bloc technique for procurement of cadaveric liver grafts for pediatric liver transplantation.

This paper describes a quick procedure for cadaveric liver graft retrieval during multiple organ harvesting. The technique is based on minimal preliminary dissection, absence of in situ direct portal perfusion, and en bloc removal of the liver and pancreas, with an aortic patch encompassing the coeliac trunk and superior mesenteric artery. The results of 110 pediatric liver transplantations with 109 organs harvested using this technique are reported. There were no graft harvesting injuries. The liver graft primary nonfunction rate was 4.5% (5/110). The 3-month retransplantation rate was 10%. The actual patient survival rates were 93% at 3 months and 90% at 1 year; actual graft survival rates were 85.5% and 78%, respectively. The technique described was at least as safe as conventional procedures. A major advantage of the procedure is its flexibility, which allows for the easily combined procurement of other organs (whole pancreas and intestine)