Methylmalonic acidemia/propionic acidemia – the biochemical presentation and comparing the outcome between liver transplantation versus non-liver transplantation groups
نویسندگان
چکیده
منابع مشابه
Early Liver Transplantation for Neonatal-Onset Methylmalonic Acidemia.
With conventional dietary treatment, the clinical course of methylmalonic acidemia due to cobalamin-unresponsive methylmalonyl-CoA mutase (MCM) deficiency is characterized by the persistent risk of recurrent life-threatening decompensation episodes with metabolic acidosis, hyperammonemia, and coma. Liver transplant has been proposed as an alternative treatment and anecdotally attempted in the l...
متن کاملLiving Donor Liver Transplantation for Acute Liver Failure : Comparing Guidelines on the Prediction of Liver Transplantation.
Determining the indications for and timing of liver transplantation (LT) for acute liver failure (ALF) is essential. The King's College Hospital (KCH) guidelines and Japanese guidelines are used to predict the need for LT and the outcomes in ALF. These guidelines' accuracy when applied to ALF in different regional and etiological backgrounds may differ. Here we compared the accuracy of new (201...
متن کاملLIVING-RELATED LIVER TRANSPLANTATION IN CHILDREN: THE SHIRAZ EXPERIENCE
The liver transplantation program was established at Shiraz Nemazee Hospital in 1993. Shortage of cadaver organ supply due to various social and legal issues urged us to develop a living-related liver transplantation (LRLT) program. So far 7 (6 males, 1 female) living-related liver transplantations have been performed at this center. The mean age of patients was 8.21 years (+/- 4.16), with ...
متن کاملFecal Microbiota Transplantation in Liver Diseases and Recent Developments
Introduction: The intestine, as an important part of the human gastrointestinal tract, provides a favorable milieu for the growth and development of a diverse and large population (approximately 1000 species) of bacteria. Currently, the presence of a cohesive relationship between intestinal bacteria and the host is being well studied and identified. According to the available scientific evidenc...
متن کاملliver transplantation
however, history of liver transplantation has started in 1963, but after discovery of anti-rejection drugs such as cyclosporine in 1980s, this method has experienced. from 1984 more than 3000 patients underwent surgery and just 1680 patients in 66 liver transplantation centers have operated in 1988. every patient suffers from severe liver diseases and is in terminal stage of this disease, must ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Orphanet Journal of Rare Diseases
سال: 2019
ISSN: 1750-1172
DOI: 10.1186/s13023-019-1045-1