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Intestinal Transplantation
Published in John K. DiBaise, Carol Rees Parrish, Jon S. Thompson, Short Bowel Syndrome Practical Approach to Management, 2017
Sherilyn Gordon Burroughs, Douglas G. Farmer
The University of California at Los Angeles evaluation protocol is described in Table 24.4. Studies were designed to assist with operative planning, including the type of transplant to be performed, target vascular access sites, and inflow and outflow vessels for the graft. Diseased organs to be resected and the potential sites of GI reconstruction are included. Risk of immune complications is determined via measurement of panel of reactive antibodies (PRA) and human leukocyte antigen (HLA) typing. Highly sensitized candidates (e.g., multiparous women, retransplant candidates, or recipients of multiple blood products) may require desensitization prior to acceptance. Once deemed a suitable ITx candidate, patients are listed with UNOS as a function of urgency: status 1, urgent; status 2, nonurgent; or status 7, temporarily inactive. Separate listing according to the Model for End Stage Liver Disease (MELD, adult) or Pediatric End Stage Liver Disease (PELD, pediatric) system is required if concomitant liver transplantation is necessary [32]. If pancreatic transplantation is also required, a third separate listing is initiated.
Liver transplantation
Published in Prem Puri, Newborn Surgery, 2017
Alastair J. W. Millar, CWN Spearman
Children with chronic liver disease are prioritized for liver transplantation according to the PELD (Pediatric End-Stage Liver Disease) (under 12 years) or MELD (Model for End-Stage Liver Disease) (12 years and older) score. Children presenting with acute liver failure are listed as UNOS Status 1a (this includes children <18 years old presenting with fulminant liver failure, primary graft nonfunction, hepatic artery thrombosis, acute decompensated Wilson’s disease, and nonmetastatic hepatoblastoma). Children with chronic liver disease (PELD > 25) who are in the ICU and who have at least one of the following: on ventilator; gastrointestinal bleeding requiring at least 30 mL/kg packed red cell transfusion in previous 24 h; renal failure requiring dialysis; Glasgow coma scale < 10 are classified as UNOS Status 1b.
Liver Transplantation
Published in John F. Pohl, Christopher Jolley, Daniel Gelfond, Pediatric Gastroenterology, 2014
In 2011, 6341 liver transplants were performed in the United States, making it the second most commonly transplanted organ after the kidney. In 2011, 536 pediatric liver transplants were performed, with 59 from living donors (United Network for Organ Sharing [UNOS] data). Currently, there are approximately 900 children age 18 years and younger on the UNOS waiting list for liver transplant. The most common indications for pediatric liver transplantation are outlined in the Table 44.1. Allocation of organs available for transplantation is currently based on the designated adult and pediatric scoring systems. The Model for End-Stage Liver disease (MELD) and Pediatric End-Stage Liver Disease (PELD) scoring systems were designed to prioritize listing by severity of illness rather than waiting time. These scores have been shown to predict the risk of death within 3 months. The PELD scoring system is used in children aged 12 years younger, and is based on a child’s age, extent of growth failure, serum bilirubin, international normalized ratio (INR), and serum albumin. For children over 12 years and adults, the MELD scoring is used. MELD only takes into account bilirubin, serum creatinine, and INR.
Minimally invasive assessment of hepatic function in children with indocyanine green elimination: a validation study
Published in Scandinavian Journal of Gastroenterology, 2019
Jon Nielsen, Nikolaj Nerup, Søren Møller, Robin de Nijs, Allan Rasmussen, Lars Bo Svendsen, Mette Skalshøi Kjaer, Vibeke Brix Christensen, Lise Borgwardt
The present study has certain limitations. The study population might not represent the true PLD population. Yet, patients were included across disease etiologies, ages and clinical presentation without selection, and as such represent a broad category of PLD patients. Our aim was to validate the use of minimally invasive measurement of ICG elimination in PLD patients against the traditional blood sample-based method. Hence evaluation of the clinical implication, suitability, pitfalls, and predictive value of ICG elimination results in different liver diseases were beyond the scope of this paper. Whether ICG elimination per se is a reliable measure of liver function in specific PLD conditions should be addressed separately in other studies. Nevertheless, despite differences in anthropometry, disease severity and bilirubin levels, minimally invasive assessment of ICG elimination was obtained and interpreted according to traditional blood sample-based results. The robust correlation across disease severities, ages and etiologies, with a relative bias that can be easily corrected for offers interesting future possibilities. Investigation of ICG elimination in different pediatric patient categories, previously omitted due to issues of repeated blood tests, including relation to known scoring systems like Pediatric End-stage Liver Disease [28], Model for End-stage Liver Disease including sodium [29], and Enhanced Liver Fibrosis [30] along with findings like fibrosis grade on biopsy, frailty, and sarcopenia seems appealing. Furthermore, this prospective study was conducted in a single center and results should be validated by other centers. Finally, clinical parameters on hemodynamic state and peripheral circulation were not registered for all patients and differences could potentially affect obtained measures of ICG.
Coexistence of Three Different Mutations in a Male Infant: neurofibromatosis Type 1, Progressive Familial Intrahepatic Cholestasis Type 2 and LPIN3
Published in Fetal and Pediatric Pathology, 2022
Derya Altay, Orhan Gorukmez, Duran Arslan
Although the patient’s calorie intake was 150 kcal/kg/day, weight gain of the patient was not sufficient; at 12 months old the weight was only 6000 gr (-3.97 SDS). The patient received an living-related liver transplantation due to hepatic-based coagulopathy, direct hyperbilirubinemia with a Pediatric End-stage Liver Disease score of 17, severe itching, and marked hepatosplenomegaly. Six months posttransplantation, at 18 months old the patient's weight was 8100 gr (-2.87 SDS).
Diagnostic value of soluble programmed cell death protein-1 in type-1 autoimmune hepatitis in Egyptian children
Published in Scandinavian Journal of Clinical and Laboratory Investigation, 2020
Ola G. Behairy, Eman G. Behiry, Manal S. El Defrawy, Ahmed N. El Adly
Assess disease severity by Child-Pugh score [8], the model for end-stage liver disease (MELD) score for adolescents older than 12 years of age and pediatric end-stage liver disease (PELD) score for participants younger than 12 years of age [9].