[Transthyretin-related hereditary amyloidosis].

Transthyretin-related hereditary amyloidosis (ATTR) is a progressive and potentially lethal genetic disorder, transmitted as an autosomal dominant trait. Tissue injury is induced by amyloid fibrils consisting of mutated transthyretin. The symptomatology and clinical course of ATTR is highly variable but typically causes peripheral polyneuropathy and autonomic dysfunction, leading to death within 10 years.

Baseline Anthropometric and Metabolic Parameters Correlate with Weight Loss in Women 1-Year After Laparoscopic Roux-En-Y Gastric Bypass.

Background: In this study, we explored in a prospective cohort of morbidly obese women undergoing laparoscopic Roux-en-Y gastric bypass (LRYGP) correlations between baseline anthropometrics, metabolic parameters, resting energy expenditure (REE), body composition, and 1-year % excess body mass index loss (%EBMIL). We also investigated risk factors for insufficient %EBMIL.

Methods: One hundred three consecutive women were prospectively evaluated at baseline (age 40.

The impact of multidisciplinary care on early morbidity and mortality after heart transplantation.

Objectives: The impact of multidisciplinary care on outcome after heart transplantation (HTx) remains unclear.

Methods: This retrospective study investigates the impact of multidisciplinary care on the primary end point 1-year all-cause mortality (ACM) and the secondary end point mean acute cellular rejection (ACR) grade within the first postoperative year.

Results: This study includes a total 140 HTx recipients (median age: 53.

Patients' preferences in transplantation from marginal donors: results of a discrete choice experiment.

To increase the donor pool, the strategy of transplantation from "marginal" donors was developed though patients' preferences about these donors were insufficiently known. The preferences of patients registered on the waiting list or already transplanted in eight transplant teams covering four main organs (i.e.

A Renal Paratransplant Hernia Causing Complete Small Bowel Obstruction: A Case Report and Review of the Literature.

A rare type of acquired internal hernia, the renal paratransplant hernia (RPTH), of which only 11 cases have been reported in the literature so far, can follow renal transplantation. We report a patient who presented with acute abdominal pain and vomiting 6 weeks after renal transplantation in the right iliac fossa. A noncontrast abdominal computed tomography scan showed signs of small bowel obstruction.

Chronic Lung Allograft Dysfunction: A Systematic Review of Mechanisms.

Chronic lung allograft dysfunction (CLAD) is the major limitation of long-term survival after lung transplantation. Chronic lung allograft dysfunction manifests as bronchiolitis obliterans syndrome or the recently described restrictive allograft syndrome. Although numerous risk factors have been identified so far, the physiopathological mechanisms of CLAD remain poorly understood.

Liver transplantation for adenomatosis: European experience.

The aim of this study was to collect data from patients who underwent liver transplantation (LT) for adenomatosis; to analyze the symptoms, the characteristics of the disease, and the recipient outcomes; and to better define the role of LT in this rare indication. This retrospective multicenter study, based on data from the European Liver Transplant Registry, encompassed patients who underwent LT for adenomatosis between January 1, 1986, and July 15, 2013, in Europe. Patients with glycogen storage disease (GSD) type IA were not excluded.

Subjective parameters markedly limit the referral of transplantation candidates to liver transplant centres.

Background & Aims: Equality of access to organ transplantation is a mandatory public health requirement. Referral from a local to a university hospital and then registration on the national waiting list are the two key steps enabling access to liver transplantation (LT). Although the latter procedure is well defined using the Model for End-stage Liver Disease score that improves equality of access, the former is mostly reliant on the practices of referring physicians.

Shaping macrophages function and innate immunity by bile acids: mechanisms and implication in cholestatic liver diseases.

The liver is selectively enriched in innate immune cells, macrophages (Kupffer cells), natural killer, and natural killer T cells. These cells release an array of mediators with cytotoxic, pro- and anti-inflammatory, angiogenic, fibrogenic, and mitogenic activity that function to fight infections, limit tissue injury, and promote wound healing. The diverse activity of macrophages is mediated by distinct subpopulations that develop in response to signals within their microenvironment.

Aspirin may reduce liver fibrosis progression: Evidence from a multicenter retrospective study of recurrent hepatitis C after liver transplantation.

Background And Aims: There is evidence for an association between thrombosis in the hepatic microcirculation and liver fibrosis. The aim of this study was to evaluate the role of daily low-dose aspirin (75 or 100mg, given for prevention of hepatic artery thrombosis) in fibrosis progression to ≥ F2 fibrosis score in liver-transplant recipients with recurrent hepatitis C virus (HCV).

Methods: All HCV-positive patients who had undergone liver transplantation (LT) between 2000 and 2010 were included.

CD49b, a major marker of regulatory T-cells type 1, predicts the response to antiviral therapy of recurrent hepatitis C after liver transplantation.

The TRANSPEG study was a prospective study to assess the efficacy of antiviral therapy in patients with a recurrent hepatitis C virus (HCV) after liver transplantation. The influence of regulatory T-cells (Tregs) on the response to antiviral therapy was analyzed. Patients were considered as a function of their sustained virological response (SVR) at 18 months after treatment initiation.

[An interdisciplinary approach of the organ donation decision: the Lausanne's experience].

Since 2007, the Interdisciplinary Ethics Platform (Ethos) of the University of Lausanne is leading an interdisciplinary reflection on the organ donation decision. On this basis, the project "Organ transplantation between the rhetoric of the gift and a biomedical view of the body" studies the logics at stake in the organ donation decision-making process. Results highlight many tensions within practices and public discourses in the field of organ donation and transplantation and suggest lines of inquiry for future adjustments.

[Prevention of infection in immunocompromised travelers].

Every year there are more immunocompromised patients with a better quality of life and, therefore, that travel more frequently. While traveling, patients may be exposed to several infections, such as traveler's diarrhea or malaria, which can be associated with a high rate of complications in this population. An appropriate strategy for the prevention of travel-related infections is essential, including education about hygiene measures, vaccinations and prescription of a tailored antimicrobial prophylaxis/stand-by treatment, according to the type of immunosuppression.

[Renal glucosuria].

The occurrence of glucosuria in the absence of hyperglycemia is distinctive for renal glucosuria. SGLT2 mutations provoke familial renal glucosuria characterized by persistent glucosuria in the absence of any other renal tubular dysfunction. Renal glucosuria associated with others proximal tubular dysfunctions points to Fanconi syndrome.

Absence of mTOR Inhibitor Effect on Hepatic Cyst Growth: A Case Report of a Kidney Transplant Recipient with Autosomal Dominant Polycystic Kidney Disease.

Some experimental studies have suggested a beneficial effect of the mammalian target of rapamycin (mTOR) inhibitor use on hepatic and renal cyst growth in patients with autosomal dominant polycystic kidney disease (ADPKD). However, the results of clinical studies are conflicting and the role of mTOR inhibitors is still uncertain. We report the case of a patient with ADPKD who underwent deceased kidney transplantation because of an end-stage renal disease.

Overexpression of Regulatory T Cells Type 1 (Tr1) Specific Markers in a Patient with HCV-Induced Hepatocellular Carcinoma.

Hepatitis C virus (HCV) is an important causative agent of liver disease, but factors that determine the resolution or progression of infection are poorly understood. In this study, we suggested that existence of immunosuppressive mechanisms, supported by regulatory T cells and especially the regulatory T cell 1 subset (Tr1), may explain the impaired immune response during infection and thus the fibrosis aggravation to hepatocellular carcinoma (HCC). Using quantitative real-time PCR, we investigated the intra-hepatic presence of Tr1 cells in biopsies from a genotype 1b infected patient followed for an 18-year period from cirrhosis to HCC.

The pig as a preclinical model for intestinal ischemia-reperfusion and transplantation studies.

Small bowel transplantation has become an established procedure for treatment of irreversible intestinal failure. In this procedure, primary ischemia and reperfusion is inevitable and will lead to some level of tissue injury. Both clinical and experimental data demonstrate that events occurring at the time of transplantation, called ischemia reperfusion injury (IRI), may have deleterious short- and long-term effects, manifesting as increased episodes of acute rejection and chronic allograft dysfunction.

Wernicke's encephalopathy after laparoscopic Roux-en-Y gastric bypass: a misdiagnosed complication.

Malabsorptive syndromes and micronutrient deficiencies represent well-known long-term complications of bariatric surgery. Wernicke's encephalopathy (WE), a neurologic manifestation of thiamine deficiency, has been classically associated with alcoholism or severe malnutrition, but rarely reported after bariatric surgery. Herein, we describe the case of a 27-year-old woman that developed WE 10 months after laparoscopic Roux-en-Y gastric bypass for morbid obesity that was initially misdiagnosed with a consequent retard in the appropriate treatment.