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Pediatric liver disease

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https://www.readbyqxmd.com/read/27906803/naspghan-clinical-practice-guideline-for-the-diagnosis-and-treatment-of-nonalcoholic-fatty-liver-disease-in-children
#1
Miriam B Vos, Stephanie H Abrams, Sarah E Barlow, Sonia Caprio, Stephen R Daniels, Rohit Kohli, Marialena Mouzaki, Pushpa Sathya, Jeffrey B Schwimmer, Shikha S Sundaram, Stavra A Xanthakos
Nonalcoholic fatty liver disease (NAFLD) is a highly prevalent chronic liver disease that occurs in the setting of insulin resistance and increased adiposity. It has rapidly evolved into the most common liver disease seen in the pediatric population and is a management challenge for general pediatric practitioners, subspecialists and for health systems. In this guideline, the expert committee on NAFLD (ECON) reviewed and summarized the available literature, formulating recommendations to guide screening and clinical care of children with NAFLD...
November 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27906797/non-cirrhotic-portal-fibrosis-in-pediatric-population
#2
Vikrant Sood, Bikrant Bihari Lal, Rajeev Khanna, Dinesh Rawat, Chhagan Bihari Sharma, Seema Alam
BACKGROUND: Non-cirrhotic portal fibrosis (NCPF) has been classically described as a disease of young to middle age with limited literature regarding its occurrence, onset or clinical presentation in children. We hereby present a series of 19 patients diagnosed and managed as NCPF in pediatric age group. METHODS: A retrospective review of all the patients presenting to the pediatric hepatology department (age < 18 years) and diagnosed as NCPF was done and data was evaluated...
November 30, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27904251/pediatric-biliary-interventions-in-the-native-liver
#3
REVIEW
Lisa H Kang, Colin N Brown
Biliary disease in infants and children frequently presents diagnostic and therapeutic challenges. Pediatric interventional radiologists are often involved in the multidisciplinary teams who care for these patients. This article reviews several notable causes of biliary disease in children who have not undergone liver transplantation, describes the role of percutaneous interventional procedures in managing these conditions, and details applicable biliary interventional techniques.
December 2016: Seminars in Interventional Radiology
https://www.readbyqxmd.com/read/27895749/a-single-center-retrospective-study-of-pediatric-hepatoblastoma
#4
Yi Zhang, Weiling Zhang, Suoqin Tang, Liping Chen, You Yi, Pinwei Zhang, Aiping Liu, Tian Zhi, Dongsheng Huang
Hepatoblastoma is a malignant liver tumor generally diagnosed in infants and children <3 years old. The current retrospective study aimed to investigate the associations of tumor stage, pathological type, metastasis and chemotherapy with clinical outcomes. In the current study, a total of 102 patients with hepatoblastoma were enrolled between September 2006 and June 2014. Clinical records and follow-up information for each of patient were obtained to conduct a Kaplan-Meier survival analysis and log-rank test...
November 2016: Oncology Letters
https://www.readbyqxmd.com/read/27882046/the-role-of-genetic-and-immune-factors-for-the-pathogenesis-of-primary-sclerosing-cholangitis-in-childhood
#5
REVIEW
Priscila Menezes Ferri, Ana Cristina Simões E Silva, Soraya Luiza Campos Silva, Diego Junior Queiroga de Aquino, Eleonora Druve Tavares Fagundes, Débora Marques de Miranda, Alexandre Rodrigues Ferreira
Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by chronic inflammation of the biliary tree resulting in liver fibrosis. PSC is more common in male less than 40 years of age. The diagnosis of PSC is based on clinical, laboratory, image, and histological findings. A biochemical profile of mild to severe chronic cholestasis can be observed. Endoscopic retrograde cholangiography is the golden standard method for diagnosis, but magnetic resonance cholangiography is currently also considered a first-line method of investigation...
2016: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/27878435/novel-phenotypes-and-loci-identified-through-clinical-genomics-approaches-to-pediatric-cataract
#6
Nisha Patel, Deepti Anand, Dorota Monies, Sateesh Maddirevula, Arif O Khan, Talal Algoufi, Mohammed Alowain, Eissa Faqeih, Muneera Alshammari, Ahmed Qudair, Hadeel Alsharif, Fatimah Aljubran, Hessa S Alsaif, Niema Ibrahim, Firdous M Abdulwahab, Mais Hashem, Haifa Alsedairy, Mohammed A Aldahmesh, Salil A Lachke, Fowzan S Alkuraya
Pediatric cataract is highly heterogeneous clinically and etiologically. While mostly isolated, cataract can be part of many multisystem disorders, further complicating the diagnostic process. In this study, we applied genomic tools in the form of a multi-gene panel as well as whole-exome sequencing on unselected cohort of pediatric cataract (166 patients from 74 families). Mutations in previously reported cataract genes were identified in 58% for a total of 43 mutations, including 15 that are novel. GEMIN4 was independently mutated in families with a syndrome of cataract, global developmental delay with or without renal involvement...
November 22, 2016: Human Genetics
https://www.readbyqxmd.com/read/27872411/osteoporosis-in-pediatric-liver-transplantation
#7
Walid Faraj, Ghina El Nounou, Abdallah Abou Al Naaj, Nancy Nakhoul, Ali Haydar, Mohammad Khalife
Liver transplantation provides an important, often life-saving treatment for end-stage liver disease. Osteoporosis post-liver transplantation has been described in adults; however, this has not been described in the pediatric population to date. We present a case of a 13-year-old female patient who underwent an orthotopic liver transplant for cryptogenic liver cirrhosis. Her immunosuppressants were tacrolimus and prednisone. Four months posttransplant, she started complaining of bilateral lower limb pain and limping while walking, progressing to a point where she was almost immobile...
December 2016: Progress in Transplantation
https://www.readbyqxmd.com/read/27869428/-hemolytic-anemia-as-the-first-clinical-presentation-of-wilson-disease-a-pediatric-case
#8
José A Henao C, Kathia Valverde Muñoz, María L Ávila A
: Wilson disease is an autosomal recessive disorder of the copper's hepatic metabolism; it results in toxicity due to accumulation of the mineral. The hemolytic anemia is present in 17% at some point of the disease, although it is a rare initial clinical presentation. CASE REPORT: 11 years old boy who presented with negative Coombs hemolytic anemia and elevation of liver enzymes. The possibility of Wilson disease was considered, which was confirmed with the finding of a Kayser-Fleischer ring in the eye exam...
December 1, 2016: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/27862929/effects-of-recipient-size-and-allograft-type-on-pediatric-liver-transplantation-for-biliary-atresia
#9
Sophoclis P Alexopoulos, Victor Nekrasov, Shu Cao, Susan Groshen, Navpreet Kaur, Yuri S Genyk, Lea Matsuoka
RATIONALE: The majority of pediatric patients with end stage liver disease receive a transplant with a whole liver allograft. However, smaller recipients with biliary atresia may have improved outcomes with deceased donor partial liver or living donor allografts. This study compares the national outcomes for liver transplantation in biliary atresia, with attention to the interaction between liver allograft type and recipient size. RESULTS: From 1/2/2002 to 12/30/2014, 2123 pediatric patients underwent a primary liver transplant for biliary atresia...
November 10, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/27862714/de-novo-inflammatory-bowel-disease-after-pediatric-kidney-or-liver-transplant
#10
Melissa A Fernandes, Hillary J Braun, Kim Evason, Sue Rhee, Emily R Perito
A subset of children who receive a liver and/or kidney transplant develop de novo inflammatory bowel disease-like chronic intestinal inflammation, not explained by infection or medications, following transplant. We have conducted a single-center, retrospective case series describing the unique clinical and histologic features of this IBD-like chronic intestinal inflammation following solid organ transplant. At our center, nine of 327 kidney or liver recipients developed de novo IBD following transplant (six liver, two kidney, one liver-kidney)...
November 11, 2016: Pediatric Transplantation
https://www.readbyqxmd.com/read/27862115/pediatric-acute-liver-failure-of-undetermined-cause-a-research-workshop
#11
Estella M Alonso, Simon P Horslen, Edward M Behrens, Edward Doo
: Pediatric Acute liver failure (PALF) is a potentially devastating condition which occurs in previously healthy children of all ages and frequently leads to a rapid clinical deterioration. An identified cause for liver injury is lacking in approximately 30% of cases. Children with undetermined diagnosis have lower spontaneous survival and higher rates of transplantation and death than other diagnostic groups. A single day workshop sponsored by the National Institute of Diabetes and Digestive and Kidney Diseases brought together clinicians and basic scientists to integrate aligned research findings and develop a foundation for new mechanistic studies and future treatment trials...
November 14, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27861607/transient-elastography-based-liver-stiffness-age-dependently-increases-in-children
#12
Daisuke Tokuhara, Yuki Cho, Haruo Shintaku
BACKGROUND AND AIMS: Pediatric use of liver transient elastography (TE) is attractive for its non-invasiveness, but reference values have not been established. We aimed to determine reference values for TE in children. METHODS: In pediatric patients (1 to 18 years), TE (FibroScan®) with an M probe was used for both liver stiffness measurement (LSM) and measurement of hepatic fat deposition by using a controlled attenuation parameter (CAP). The patients were divided into three relevant age groups: preschoolers (1 to 5 years), elementary school children (6 to 11 years), and adolescents (12 to 18 years)...
2016: PloS One
https://www.readbyqxmd.com/read/27833495/postoperative-pain-management-of-liver-transplantation-in-cystic-fibrosis-is-it-time-to-start-us-guided-neuraxial-blocks
#13
M Piazza, G Martucci, A Arcadipane
Cystic fibrosis (CF) is the most common life-limiting genetic disease in Caucasians. Declining lung function is the principal cause of death, but liver involvement can lead to the need for liver transplantation. General anesthesia has detrimental effects on pulmonary function, increasing perioperative morbidity and mortality in CF patients. Regional anesthetic techniques improve outcomes by reducing anesthetic drugs and administration of opioids, and hastening extubation, awakening, and restarting respiratory of physiotherapy...
October 2016: Saudi Journal of Anaesthesia
https://www.readbyqxmd.com/read/27830542/hepatocyte-transplantation-in-special-populations-clinical-use-in-children
#14
Zahida Khan, Stephen C Strom
Orthotopic liver transplantation remains the only proven cure for end-stage liver failure. Despite significant advances in the field, the clinical demand for donor organs far outweighs the supply. Hepatocyte transplantation has been proposed as an alternative approach to whole liver transplant in select diseases. Several international centers have reported experimental trials of human hepatocyte transplantation in acute liver failure and liver-based metabolic disorders. This chapter provides an introduction to hepatocyte transplantation from both a technical and clinical perspective...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/27822768/pediatric-nonalcoholic-fatty-liver-disease-the-rise-of-a-lethal-disease-among-mexican-american-hispanic-children
#15
REVIEW
Monica M Betancourt-Garcia, Armando Arguelles, Jorge Montes, Ambrosio Hernandez, Manish Singh, R Armour Forse
Hispanic children of Mexican origin have a high incidence of NAFLD. Susceptibility has been linked to a combination of factors including an increasing epidemic of obesity in children and adolescents, an allele substitution in the PNPLA3 gene that reduces hepatic lipid catabolism, and an altered microbiome that may increase hepatic endotoxins. The combination of NAFLD and portal vein toxins secondary to an indigenous gut microbiome appear to lead to the early occurrence of NASH, which progresses to cirrhosis and early hepatocellular carcinoma...
November 7, 2016: Obesity Surgery
https://www.readbyqxmd.com/read/27817193/effect-of-microrna-155-on-the-interferon-gamma-signaling-pathway-in-biliary-atresia
#16
Yu-An Hsu, Chih-Hung Lin, Hui-Ju Lin, Chi-Chun Huang, Hsiu-Chu Lin, Ying-Chi Chen, Ching-Yao Chang, Su-Hua Huang, Jane-Ming Lin, Kuan-Rong Lee, Lei Wan
MicroRNAs (miRNAs) are ~22-nucleotide long RNAs that negatively regulate gene expression and inflammatory responses in eukaryotes. The aim of this work was to evaluate the roles of miRNA (miR)-155 on the interferon-γ (IFN-γ)-induced response in biliary atresia (BA), which is the most common form of pediatric chronic liver disease and a leading indication for pediatric liver transplantation. The expression of miR-155 and the suppressor of cytokine signaling 1 (SOCS1) gene in human and mice liver tissues of BA and healthy controls was evaluated...
December 31, 2016: Chinese Journal of Physiology
https://www.readbyqxmd.com/read/27805526/histologic-changes-in-bone-marrow-biopsies-from-liver-transplant-patients
#17
Pelin Börcek, Binnaz Handan Özdemir, Çiğdem Sercan, Eda Yılmaz Akçay, Sema Karakuş, Mehmet Haberal
OBJECTIVES: Liver transplant may complicated by various hematologic conditions, resulting in indication for bone marrow biopsy. Immunosuppressive therapies, specific infections, and secondary neoplasms affect bone marrow. In the present study, we evaluated the histologic spectrum of bone marrow findings in liver allograft recipients. MATERIALS AND METHODS: Of 338 patients who received liver transplants and were followed at the Başkent University, Faculty of Medicine, 44 patients underwent bone marrow biopsy...
November 2016: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/27783314/space-occupying-lesions-in-the-liver
#18
REVIEW
Manigandan Subramanyam Thyagarajan, Khalid Sharif
Space occupying liver lesions usually present with abdominal pain or abnormal physical findings, such as a palpable abdominal mass or distention. Liver lesions identified in children include benign and malignant neoplasms, inflammatory masses, cysts and metastatic lesions. Two-thirds of liver lesions in children are malignant. Hepatoblastoma accounts for two-thirds of malignant liver tumors in children. Benign lesions of the liver in children include vascular lesions, hamartomas, adenomas, and focal nodular hyperplasia...
October 25, 2016: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/27782894/etiologies-outcomes-and-prognostic-factors-of-pediatric-acute-liver-failure-a-single-center-s-experience-in-turkey
#19
Figen Özçay, Eda Karadağ Öncel, Zeren Barış, Oğuz Canan, Gökhan Moray, Mehmet Haberal
BACKGROUND/AIMS: Our aim was to determine the etiologies, outcomes, and prognostic indicators in children with acute liver failure. MATERIALS AND METHODS: Ninety-one patients who were followed for pediatric acute liver failure (PALF) over a 15-year period were included. Patients who survived with supportive therapy were designated as Group 1, while those who died or underwent liver transplantation were designated as Group 2. RESULTS: There were 37 (40...
September 2016: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/27781375/multidisciplinary-management-of-hepatoblastoma-in-children-experience-from-a-developing-country
#20
Naresh Shanmugam, Julius Xavier Scott, Vimal Kumar, Mukul Vij, Priya Ramachandran, Gomathy Narasimhan, Mettu Srinivas Reddy, Venugopal Kota, Deenadayalan Munirathnam, Chayarani Kelgeri, Karthick Sundaram, Mohamed Rela
BACKGROUND: Advances in chemotherapy, liver resection techniques, and pediatric liver transplantation have vastly improved survival in children with hepatoblastoma (HB). These are best managed by a multidisciplinary team (MDT) in a setting where all treatment options are available. Until recently, this was difficult to achieve in India. METHODS: All children (<16 years) with HB treated in a pediatric liver surgery and transplantation unit between January 2011 and July 2016 were reviewed...
October 26, 2016: Pediatric Blood & Cancer
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