“…The main causes of death are TPN-related complications, surgery-related complications, and post-transplantation complication, together with septic shock of GI origin. A variety of clinical, histological and manometric parameters have been found to be predictive of a poor clinical outcome in adult patients, including myopathy and decreased contractile activity [2,50,52,53,[55][56][57][58][59][60] . MNGIE has a particularly poor prognosis with slowly progressive evolution and death around 40 years of age [11] .…”