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Pediatric liver disease

María José Garcés Hernández, Miguel Klünder, Nayely Garibay Nieto, Juan Carlos López Alvarenga, Jenny Vilchis Gil, Samuel Flores Huerta, Rosa Quispe Siccha, Joselin Hernandez
OBJECTIVE: Visceral adiposity index (VAI) is a mathematical model associated with cardiometabolic risk in adults, but studies on children failed to support this association. Our group has proposed a pediatric VAI model using pediatric ranges, but it has not yet been evaluated and needs further adjustments. The objective of this study was to further adjust the proposed pediatric VAI by age, creating a new pediatric metabolic index (PMI), and assess the correlation of the PMI with insulin resistance indexes and hepatic enzymes...
March 2018: Endocrine Practice
Kazuaki Tokodai, Shigehito Miyagi, Chikashi Nakanishi, Yasuyuki Hara, Wataru Nakanishi, Koji Miyazawa, Kenji Shimizu, Keigo Murakami, Hironobu Sasano, Masafumi Goto, Michiaki Unno, Takashi Kamei
The aim of this study was to evaluate the significance of post-transplant DSA as a predictor of liver fibrosis during long-term follow-up after pediatric LT. We evaluated the histological findings in 18 LT recipients who underwent liver biopsy after DSA screening. Liver fibrosis was scored based on the METAVIR fibrosis staging. Patients were divided into 2 groups based on histological findings, and clinical characteristics among patients with liver fibrosis were assessed. Of 18 patients, 7 were included in the fibrosis group...
March 14, 2018: Pediatric Transplantation
Bernadette O Erokwu, Christian E Anderson, Chris A Flask, Katherine M Dell
BACKGROUND: Autosomal Recessive Polycystic Kidney Disease (ARPKD) is associated with significant mortality and morbidity and there are currently no disease-specific treatments available for ARPKD patients. One major limitation in establishing new therapies for ARPKD is a lack of sensitive measures of kidney disease progression. Magnetic Resonance Imaging (MRI) can provide multiple quantitative assessments of disease. METHODS: We applied quantitative image analysis of high resolution (non-contrast) T2-weighted MRI techniques to study cystic kidney disease progression and response to therapy in the PCK rat model of ARPKD...
March 14, 2018: Pediatric Research
Maggie A Stanislawski, Catherine A Lozupone, Brandie D Wagner, Merete Eggesbø, Marci K Sontag, Nichole Nusbacher, Mercedes Martinez, Dana Dabelea
BACKGROUND: Recent evidence supports that the gut microbiota may be involved in the pathophysiology of non-alcoholic fatty liver disease (NAFLD), and may also offer avenues for treatment or prevention. METHODS: We investigated the associations among gut microbiota, diet and hepatic fat fraction (HFF) in 107 adolescents. Magnetic Resonance Imaging (MRI) was used to assess HFF, and 16S rRNA gene sequencing was performed on collected fecal samples. Dietary intake was assessed using Food Frequency Questionnaires...
March 14, 2018: Pediatric Research
Toshihiro Yasui, Tatsuya Suzuki, Fujio Hara, Shunsuke Watanabe, Naoko Uga, Atsuki Naoe, Yasuhiro Kondo
OBJECTIVES: In pediatric patients, renal dysfunction after living-donor liver transplant is a major issue that is difficult to evaluate. Recently, predictive equations for Japanese children have been introduced. MATERIALS AND METHODS: We conducted a retrospective study by prospectively collecting data on 26 patients under 16 years old who underwent living-donor liver transplant between June 2004 and March 2015. Serum creatinine and cystatin C levels were measured...
March 9, 2018: Experimental and Clinical Transplantation
(no author information available yet)
Trace elements are dietary elements which are needed in very minute quantities for the proper growth, development, and physiology of the organism, which play a crucial part in energy metabolism and material transformation. It's generally agreed that trace elements are essential components of parenteral nutrition. These societies for parenteral and enteral nutrition in Europe, the United States and Australia proposed that various trace elements should be provided daily from commencement for all patients receiving parenteral nutrition and published some guidelines on adult trace elements recommendations for parenteral nutrition...
March 1, 2018: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
S M Dehghani, I Shahramian, M Afshari, M Bahmanyar, M Ataollahi, A Sargazi
Background: Acute cellular rejection (ACR), a reversible process, can affect the graft survival. Objective: To evaluate the relation between ACR and clinical factors in recipients of allograft liver transplantation. Methods: 47 recipients of liver were consecutively enrolled in a retrospective study. Their information were retrieved from their medical records and analyzed. Results: Of the 47 recipients, 38 (81%) experienced acute rejection during 24 months of the transplantation...
2018: International Journal of Organ Transplantation Medicine
C Ricardo Ramirez, M L Álvarez Gómez, C A Agudelo Vélez, S Zuluaga Penagos, R A Consuegra Peña, K Uribe Hernández, I C Mejía Gil, E M Cano Londoño, M Elorza Parra, J G Franco Vásquez
OBJECTIVE: To evaluate the clinical characteristics, prevalence and factors associated with delirium in critical patients from 5 to 14 years of age. DESIGN: An analytical, cross-sectional observational study was made. Delirium was assessed with the Pediatric-Confusion Assessment Method for the Intensive Care Unit (pCAM-ICU) and motor classification was established with the Delirium Rating Scale Revised-98. SETTING: A pediatric Intensive Care Unit...
March 9, 2018: Medicina Intensiva
Jagannath M Sherigar, Joline De Castro, Yong Mei Yin, Debra Guss, Smruti R Mohanty
Glycogenic hepatopathy (GH) is a rare complication of the poorly controlled diabetes mellitus characterized by the transient liver dysfunction with elevated liver enzymes and associated hepatomegaly caused by the reversible accumulation of excess glycogen in the hepatocytes. It is predominantly seen in patients with longstanding type 1 diabetes mellitus and rarely reported in association with type 2 diabetes mellitus. Although it was first observed in the pediatric population, since then, it has been reported in adolescents and adults with or without ketoacidosis...
February 27, 2018: World Journal of Hepatology
Chetan Mandelia, Mohammad Nasser Kabbany, Praveen Kumar Conjeevaram Selvakumar, Naim Alkhouri
Nonalcoholic fatty liver disease (NAFLD) is the hepatic manifestation of the obesity epidemic. Recent studies have clearly shown that the stage of fibrosis in adults with NAFLD is the most important histological feature in long-term outcomes and the development of liver-related complications. Despite the paucity of data regarding the natural history of pediatric NAFLD, its progression to cirrhosis and end-stage liver disease requiring liver transplantation is well documented. Given the high prevalence of NAFLD in children and adults, there is an urgent need to find safe and cost-effective alternatives to biopsy to determine the stage of liver fibrosis...
March 8, 2018: Biomarkers in Medicine
Karoly Toth, Jacqueline F Spencer, Baoling Ying, Ann E Tollefson, Caroll B Hartline, Eric T Richard, Jiajun Fan, Jinglei Lyu, Boris A Kashemirov, Cheryl Harteg, Dawn Reyna, Elke Lipka, Mark N Prichard, Charles E McKenna, William S M Wold
Human adenoviruses (AdV) cause generally mild infections of the respiratory and GI tracts as well as some other tissues. However, AdV can cause serious infection in severely immunosuppressed individuals, especially pediatric patients undergoing allogeneic hematopoietic stem cell transplantation, where mortality rates are up to 80% with disseminated disease. Despite the seriousness of AdV disease, there are no drugs approved specifically to treat AdV infections. We report here that USC-087, an N-alkyl tyrosinamide phosphonate ester prodrug of HPMPA, the adenine analog of cidofovir, is highly effective against multiple AdV types in cell culture...
March 3, 2018: Antiviral Research
Sheryl Tulin-Silver, Chrystal Obi, Nishita Kothary, Matthew Lungren
PURPOSE: The aim of the study was to compare safety and efficacy of transjugular liver biopsy (TJLB) and percutaneous liver biopsy (PLB) with tract embolization in pediatric patients with liver disease. MATERIALS AND METHODS: TJLB and PLB between December 2009 and October 2015 were retrospectively reviewed. Primary endpoints were adequate sampling and complication rate. Patient age, weight, coagulation factors, ascites, blood transfusions, adequacy of biopsy sample, number of biopsy samples, and complications were compared...
March 5, 2018: Journal of Pediatric Gastroenterology and Nutrition
Ellen Mitchell, Kathleen M Loomes, Robert H Squires, David Goldberg
BACKGROUND: Recent data has suggested that pediatric patients waitlisted for a liver transplant frequently have liver offers declined. However, factors associated with liver offer decisions and center-level variability in practice patterns have not been explored. METHODS: We evaluated UNOS data on all match runs from 5/10/07-12/31/15 in which: 1) the liver was offered to ≥1 pediatric patient; 2) the transplant recipient was ranked in the first 40 positions for the organ offer; and 3) the donor was brain-dead and <50 years of age...
March 5, 2018: Liver Transplantation
Syeda Sara Batool Hamdani, Huma Arshad Cheema, Anjum Saeed, Hassan Suleman Malik, Tayaba Sehar
BACKGROUND: Wilson disease (WD) is one of the most common metabolic liver diseases in older children. It has a strong genetic background with autosomal recessive inheritance. WD is a multisystem disorder with predominant hepatic and neurological manifestations and variable age of presentation. The data on cardiac manifestations in children is very limited and only few adult studies are available in the literature. This study was planned to determine the frequency and spectrum of Electrocardiographic (ECG) changes in pediatric WD...
January 2018: Journal of Ayub Medical College, Abbottabad: JAMC
Akash Deep, Romit Saxena, Bipin Jose
Acute kidney injury (AKI) is a common accompaniment in patients with liver disease. The causes, risk factors, manifestations and management of AKI in these patients vary according to the liver disease in question (acute liver failure, acute-on-chronic liver failure, post-liver transplantation or metabolic liver disease). There are multiple causes of AKI in patients with liver disease-pre-renal, acute tubular necrosis, post-renal, drug-induced renal failure and hepatorenal syndrome (HRS). Definitions of AKI in liver failure are periodically revised and updated, but pediatric definitions have still to see the light of the day...
March 1, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
Jingtao Cui, Wenjuan Yan, Shaoxia Xu, Qiaofeng Wang, Weihong Zhang, Wenjing Liu, Anping Ni
BACKGROUND: Epstein-Barr virus (EBV) is associated with nasopharyngeal carcinoma (NPC) which is prevalent in South China, and its association with systemic lupus erythematosus (SLE) or other autoimmune diseases has not been studied in the mainland of China. The EBV serological tests have been performed on patients with various diseases or manifestations for years at our institution and their values need to be evaluated. METHODS: For routine medical purposes, anti-EB viral capsid antigen (VCA) IgG, IgA and IgM antibodies, anti-EBV diffuse early antigen (EA-D) IgA antibodies, and anti-EBV nuclear antigen-1(EBNA-1) IgG antibodies were tested with commercial enzyme-linked immunosorbent assay (ELISA) in patients visiting Peking Union Medical College Hospital between 2013 and 2017...
2018: PloS One
C M Jaramillo-Esparza, A Consuelo-Sánchez, C P Acosta-Rodríguez-Bueno, G Ramón-García, S W Sadowinski-Pine, M A Escobar-Sánchez, I Castorena-Villa, F Gaytán-Morales, R Vázquez-Frias
INTRODUCTION AND AIMS: Graft-versus-host disease (GVHD) is a common multisystemic complication of allogeneic hematopoietic cell transplantation. The most frequent presentations of graft-versus-host disease involve the skin, the gastrointestinal tract, and the liver. The aim of the present study was to know the frequency of gastrointestinal tract and liver GVHD and the characteristics of disease presentation in pediatric patients that underwent hematopoietic stem cell transplantation (HSCT) at a tertiary care hospital center in Mexico City...
February 24, 2018: Revista de Gastroenterología de México
Ida Giardino, Maria D'Apolito, Michael Brownlee, Angela Bruna Maffione, Anna Laura Colia, Michele Sacco, Pietro Ferrara, Massimo Pettoello-Mantovani
Chronic kidney disease in children is an irreversible process that may lead to end-stage renal disease. The mortality rate in children with end-stage renal disease who receive dialysis increased dramatically in the last decade, and it is significantly higher compared with the general pediatric population. Furthermore, dialysis and transplant patients, who have developed end-stage renal disease during childhood, live respectively far less as compared with age/race-matched populations. Different reports show that cardiovascular disease is the leading cause of death in children with end-stage renal disease and in adults with childhood-onset chronic kidney disease, and that children with chronic kidney disease are in the highest risk group for the development of cardiovascular disease...
December 2017: Türk Pediatri Arşivi
Eberhard Lurz, Claudia Quammie, Michael Englesbe, Estella M Alonso, Henry C Lin, Evelyn K Hsu, Katryn N Furuya, Nitika A Gupta, Veena L Venkat, James F Daniel, Mike A Leonis, Tamir Miloh, Grzegorz W Telega, Jason Yap, Jerome Menendez, Linda S Book, Ryan W Himes, Shikha S Sundaram, Rulan Parekh, Chris Sonnenday, John Bucuvalas, Vicky L Ng, Binita M Kamath
OBJECTIVE: To assess frailty, a measure of physiologic declines in multiple organ systems, in children with chronic liver disease using a novel pediatric frailty tool. STUDY DESIGN: We performed a prospective cross-sectional multicenter study at 17 liver transplantation (LT) centers. 71 children (5-17 years of age), 36 with compensated chronic liver disease (CCLD) and 35 with end-stage liver disease (ESLD) and listed for LT, were assessed for frailty using validated pediatric tools to assess the 5 classic Fried Frailty Criteria-slowness, weakness, exhaustion, diminished physical activity, and shrinkage...
March 2018: Journal of Pediatrics
Nancy L Van Buren, Jed B Gorlin, Robyn C Reed, Jerome L Gottschall, Stephen C Nelson
BACKGROUND: Acute splenic sequestration crisis is a complication of sickle cell disease (SCD) occurring when intrasplenic red blood cell (RBC) sickling prevents blood from leaving the spleen, causing acute splenic enlargement. Although typically seen in young children, it has been reported in older children with hemoglobin (Hb)SC disease, eventually resulting in functional asplenia. Ceftriaxone is a frequently used antibiotic of choice for children with SCD, because of its efficacy against invasive pneumococcal disease...
February 22, 2018: Transfusion
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