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https://www.readbyqxmd.com/read/28621023/pediatric-liver-transplantation-for-fulminant-hepatic-failure-secondary-to-intentional-iron-overdose
#1
Joanne Lai, Jaime Chu, Ronen Arnon
Acute iron poisoning may lead to life-threatening hepatotoxicity. We present the cases of two pediatric patients with hepatotoxicity following intentional iron ingestion that progressed rapidly to fulminant hepatic failure despite treatment with deferoxamine. Liver transplantation was lifesaving in both patients. These cases emphasize the need for a high index of suspicion for iron ingestion, close monitoring for liver toxicity, and timely consideration for liver transplantation.
June 15, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28583557/in-situ-posterior-graft-segmentectomy-for-large-for-size-syndrome-in-deceased-donor-liver-transplantation-in-adults-a-case-report
#2
A Nagatsu, T Yoshizumi, T Ikegami, N Harimoto, N Harada, Y Soejima, A Taketomi, Y Maehara
Large-for-size syndrome (LFSS) is controversial in pediatric living donor liver transplantation patients and is associated with a poor graft outcome. Similar situations in deceased donor liver transplantation (DDLT) in adults have not been reported frequently, and there are no official guidelines worldwide. Deceased donation is extremely limited in Japan, and when a larger liver is allocated for a very sick small recipient in Japan, transplantation with a plan to address LFSS might be necessary. The patient is a 58-year-old female patient who had acute liver failure with coma...
June 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28535186/fxr-gankyrin-axis-is-involved-in-development-of-pediatric-liver-cancer
#3
Leila Valanejad, Kyle Lewis, Mary Wright, Yanjun Jiang, Amber D'Souza, Rebekah Karns, Rachel Sheridan, Anita Gupta, Kevin Bove, David Witte, James Geller, Gregory Tiao, David L Nelson, Lubov Timchenko, Nikolai Timchenko
The development of hepatoblastoma (HBL) is associated with failure of hepatic stem cells (HSC) to differentiate into hepatocytes. Despite intensive investigations, mechanisms of the failure of HSC to differentiate are not known. We found that oncogene Gankyrin (Gank) is involved in the inhibition of differentiation of HSC via triggering degradation of tumor suppressor proteins (TSPs) Rb, p53, C/EBPα and HNF4α. Our data show that the activation of a repressor of Gank, farnesoid X receptor, FXR, after initiation of liver cancer by Diethylnitrosamine (DEN) prevents the development of liver cancer by inhibiting Gank and rescuing tumor suppressor proteins...
May 23, 2017: Carcinogenesis
https://www.readbyqxmd.com/read/28461212/risk-factors-and-outcomes-related-to-pediatric-intensive-care-unit-admission-after-hematopoietic-stem-cell-transplantation-a-single-center-experience
#4
Marta Pillon, Angela Amigoni, Annaelena Contin, Manuela Cattelan, Elisa Carraro, Emiliana Campagnano, Manuela Tumino, Elisabetta Calore, Antonio Marzollo, Chiara Mainardi, Maria Paola Boaro, Marta Nizzero, Andrea Pettenazzo, Giuseppe Basso, Chiara Messina
To describe incidence, causes, and outcomes related to pediatric intensive care unit (PICU) admission for patients undergoing hematopoietic stem cell transplantation (HSCT), we investigated the risk factors predisposing to PICU admission and prognostic factors in terms of patient survival. From October 1998 to April 2015, 496 children and young adults (0 to 23 years) underwent transplantation in the HSCT unit. Among them, 70 (14.1%) were admitted to PICU. The 3-year cumulative incidence of PICU admission was 14...
April 28, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28433098/wilson-disease-in-children
#5
Eve A Roberts, Piotr Socha
Wilson disease (WD) is an inherited disorder mainly of hepatocellular copper disposition, due to dysfunction of the Wilson ATPase, a P1B-ATPase encoded by the gene ATP7B. In children, as in older age brackets, clinical disease is highly diverse. Although hepatic disease is the common presentation in children/adolescents, neurologic, psychiatric, and hematologic clinical presentations do occur. Very young children may have clinically evident liver disease due to WD. Early diagnosis, preferably when the child/adolescent is asymptomatic, is most likely to result in near-normal longevity with generally good health so long as the patient tolerates effective medication, is adherent to the lifelong treatment regimen, and has consistent access to the medication...
2017: Handbook of Clinical Neurology
https://www.readbyqxmd.com/read/28422392/hepatic-artery-reconstruction-in-living-donor-liver-transplantation-using-surgical-loupes-achieving-low-rate-of-hepatic-arterial-thrombosis-in-741-consecutive-recipients-tips-and-tricks-to-overcome-the-poor-hepatic-arterial-flow
#6
Ping-Chun Li, Ashok Thorat, Long-Bin Jeng, Horng-Ren Yang, Ming-Li Li, Chun-Chieh Yeh, Te-Hung Chen, Shih-Chao Hsu, Kin-Shing Poon
The reconstruction of the hepatic artery (HA) is the most complex step in living donor liver transplantation (LDLT) because of the smaller diameter of the artery and the increased risk of HA-related complications. Because of the smaller diameter of the HA, many centers use a microsurgical technique with interrupted sutures for arterial anastomosis. The aim of our study was to retrospectively investigate the outcomes after HA reconstruction performed under magnifying loupes using the "parachute technique." From August 1, 2002 to August 31, 2016, LDLT was performed in 766 recipients...
July 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28419061/functional-status-of-neonatal-and-pediatric-patients-after-extracorporeal-membrane-oxygenation
#7
Katherine Cashen, Ron Reeder, Heidi J Dalton, Robert A Berg, Thomas P Shanley, Christopher J L Newth, Murray M Pollack, David Wessel, Joseph Carcillo, Rick Harrison, J Michael Dean, Tammara Jenkins, Kathleen L Meert
OBJECTIVES: To describe functional status at hospital discharge for neonatal and pediatric patients treated with extracorporeal membrane oxygenation, and identify factors associated with functional status and mortality. DESIGN: Secondary analysis of observational data collected by the Collaborative Pediatric Critical Care Research Network between December 2012 and September 2014. SETTING: Eight hospitals affiliated with the Collaborative Pediatric Critical Care Research Network...
June 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28367603/mr-elastography-high-rate-of-technical-success-in-pediatric-and-young-adult-patients
#8
Madalsa Joshi, Jonathan R Dillman, Alexander J Towbin, Suraj D Serai, Andrew T Trout
BACKGROUND: Magnetic resonance (MR) elastography allows the noninvasive assessment of liver stiffness, which is a surrogate for fibrosis. OBJECTIVE: The purpose of this study was to describe our experience using liver MR elastography in a large pediatric population with attention to the frequency and causes of exam failure. MATERIALS AND METHODS: Imaging records were searched for patients ≤18 years of age who underwent 2-D gradient recalled echo (GRE) MR elastography of the liver between September 2011 and August 2015 on one of two 1...
April 3, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28333693/pediatric-intestinal-failure-associated-liver-disease
#9
Cathleen M Courtney, Brad W Warner
PURPOSE OF REVIEW: The goal of this review is to provide updates on the definition, pathophysiology, treatment, and prevention of intestinal failure-associated liver disease (IFALD) that are relevant to care of pediatric patients. RECENT FINDINGS: Current literature emphasizes the multifactorial nature of IFALD. The pathogenesis is still largely unknown; however, molecular pathways have been identified. Key to these pathways are proinflammatory cytokines involved in hepatic inflammation and bile acids synthesis such as Toll-like receptor 4 and farnesoid X receptor, respectively...
June 2017: Current Opinion in Pediatrics
https://www.readbyqxmd.com/read/28318185/neonatal-acute-liver-failure-a-diagnosis-challenge
#10
Mirta Ciocca, Fernando Álvarez
Neonatal acute liver failure is a rare, very severe disease with a high rate of mortality. It is clinically and etiologically different from acute liver failure seen in older children and adults. Coagulopathy with an international normalized ratio ≥ 3 is the critical parameter that defines it. The most common causes are fetal alloimmune hepatitis, previously called neonatal hemochromatosis, viral infections, metabolic disorders, and hemophagocytic lymphohistiocytosis. There is a group of treatable diseases that require a very early diagnosis for the prescription of an adequate treatment...
April 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28296681/adenovirus-hepatitis-clinicopathologic-analysis-of-12-consecutive-cases-from-a-single-institution
#11
Kurt B Schaberg, Neeraja Kambham, Richard K Sibley, John P T Higgins
Adenoviruses are common pathogens that usually cause self-limited infections. However, in the immunocompromised host they can cause severe infections involving multiple organs including the liver. A search of the pathology database at Stanford University Medical Center (1995 to 2016) identified 12 cases of adenovirus hepatitis including biopsy and autopsy specimens. There were 8 pediatric patients, 7 of which had received orthotropic liver transplants and 1 of which was receiving chemotherapy for lymphoblastic leukemia...
June 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28285079/defibrotide-for-patients-with-hepatic-veno-occlusive-disease-sinusoidal-obstruction-syndrome-interim-results-from-a-treatment-ind-study
#12
Paul G Richardson, Angela R Smith, Brandon M Triplett, Nancy A Kernan, Stephan A Grupp, Joseph H Antin, Leslie Lehmann, Tsiporah Shore, Massimo Iacobelli, Maja Miloslavsky, Robin Hume, Alison L Hannah, Bijan Nejadnik, Robert J Soiffer
Hepatic veno-occlusive disease, or sinusoidal obstruction syndrome (VOD/SOS), is a serious and potentially fatal complication of conditioning for hematopoietic stem cell transplantation (HSCT) or of chemotherapy regimens alone. Defibrotide is a complex mixture of single-stranded polydeoxyribonucleotides that is approved in the United States for treating hepatic VOD/SOS with renal or pulmonary dysfunction post-HSCT and in the European Union, Israel, and South Korea for treating severe hepatic VOD/SOS post-HSCT...
March 8, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28248832/pathophysiology-of-pediatric-multiple-organ-dysfunction-syndrome
#13
Joseph A Carcillo, Bradley Podd, Rajesh Aneja, Scott L Weiss, Mark W Hall, Timothy T Cornell, Thomas P Shanley, Lesley A Doughty, Trung C Nguyen
OBJECTIVE: To describe the pathophysiology associated with multiple organ dysfunction syndrome in children. DATA SOURCES: Literature review, research data, and expert opinion. STUDY SELECTION: Not applicable. DATA EXTRACTION: Moderated by an experienced expert from the field, pathophysiologic processes associated with multiple organ dysfunction syndrome in children were described, discussed, and debated with a focus on identifying knowledge gaps and research priorities...
March 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28160044/clinical-characteristics-of-pediatric-hiv-1-patients-treated-with-first-line-antiretroviral-therapy-in-vietnam-a-nested-case-control-study
#14
Minh Diem Dang, Duc Minh Nguyen, Huu Bich Tran, Viet Hung Pham, Daryl Spak, Linh Chi Pham, Thi Quynh Phan, Thi Thanh Dinh, Thi Kim Anh Le, Van Lam Nguyen, Thanh Hai Le, Son Ngoc Hoang, Vu Phuong Linh Dang
OBJECTIVES: Over the past decades, Vietnam has made great strides in reducing the rate of mortality in HIV-related deaths, due to increased access of antiretroviral therapy (ART); however, given the significantly high level of treatment failure (TF), it is essential to identify markers that describe the failure of ART in HIV-1 infected children. METHODS: A nested case-control study was conducted with clinical data collected from 101 HIV-infected children [26 TF and 75 treatment success (TS)] at National Hospital of Pediatrics, Vietnam (2008-2012)...
February 2017: International Journal of Public Health
https://www.readbyqxmd.com/read/28124483/mutations-in-ttc21b-cause-different-phenotypes-in-two-childhood-cases-in-china
#15
Hongwen Zhang, Baige Su, Xiaoyu Liu, Huijie Xiao, Jie Ding, Yong Yao
AIM: The TTC21B gene is now known as causative of nephronophthisis-related ciliopathies (NPHP-RC). We reported two Chinese pediatric cases with end-stage renal disease and other phenotypes caused by the TTC21B gene mutations. METHODS: The clinical features of Chinese pediatric cases with NPHP-RC were summarized. Mutation analysis of the TTC21B gene was performed using next-generation sequencing. RESULTS: The two cases both had nephrotic proteinuria, renal failure, hypertension and abnormal liver function (or hepatic fibrosis)...
January 26, 2017: Nephrology
https://www.readbyqxmd.com/read/28115278/the-role-of-thromboelastography-in-pediatric-patients-with-sinusoidal-obstructive-syndrome-receiving-defibrotide
#16
Joanna L Gendreau, Christine Knoll, Roberta H Adams, Leon L Su
Sinusoidal obstructive syndrome (SOS) is a potentially fatal form of hepatic injury after hematopoietic stem cell transplantation. Patients can develop liver dysfunction, portal hypertension, ascites, coagulopathies, and multisystem organ failure. The mortality rate of severe SOS has been reported as high as 98% by day 100 after transplantation. Defibrotide, which is now approved for the treatment of SOS, has significantly decreased mortality. Defibrotide is a polynucleotide with profibrinolytic, anti-ischemic, and anti-inflammatory activity...
April 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28031453/acute-liver-failure-meets-soph-syndrome-a-case-report-on-an-intermediate-phenotype
#17
Fanny Kortüm, Iris Marquardt, Malik Alawi, Georg Christoph Korenke, Stephanie Spranger, Peter Meinecke, Kerstin Kutsche
Acute liver failure (ALF) is a life-threatening condition in the absence of preexisting liver disease in children. The main clinical presentation comprises hepatic dysfunction, elevated liver biochemical values, and coagulopathy. The etiology of ALF remains unclear in most affected children; however, the recent identification of mutations in the neuroblastoma amplified sequence (NBAS) gene in autosomal recessively inherited ALF has shed light on the cause of a subgroup of fever-triggered pediatric ALF episodes...
January 2017: Pediatrics
https://www.readbyqxmd.com/read/28030425/features-of-hepatitis-in-hepatitis-associated-aplastic-anemia-clinical-and-histopathologic-study
#18
Kalyani R Patel, Alison Bertuch, Ghadir S Sasa, Ryan W Himes, Hao Wu
OBJECTIVES: Hepatitis-associated aplastic anemia (HAA) is a rare variant of aplastic anemia in which patients present with severe pancytopenia after an episode of acute hepatitis. The marrow failure is often rapid, severe, and usually fatal if untreated. The preceding hepatitis is largely under-studied. METHODS: Retrospective study of the clinical and histopathologic features of hepatitis in pediatric patients who subsequently developed aplastic anemia and comparison with consecutive cases of acute liver failure and random cases of autoimmune hepatitis during the same time frame...
January 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#19
Güzide Doğan, Fatih Düzgün, Serdar Tarhan, Yeliz Çağan Appak, Erhun Kasırga
Congenital intrahepatic portosystemic shunts are rare in children. Portosystemic venous malformations are characterized by extreme clinical variability. We report a full-term 33-day-old male infant presenting with neonatal jaundice. On physical examination, he had generalized icterus and the liver was palpable 3.5 cm below the right costal margin. He had no other symptoms. Laboratory tests showed AST 632 U/L, ALT 198 U/L, total bilirubin 12.1 mg/dL, conjugated bilirubin 10.2 mg/dL, alkaline phosphatase 753 U/L, GGT 47 U/L and glucose 67 U/L...
December 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/27976737/altered-systemic-bile-acid-homeostasis-contributes-to-liver-disease-in-pediatric-patients-with-intestinal-failure
#20
Yong-Tao Xiao, Yi Cao, Ke-Jun Zhou, Li-Na Lu, Wei Cai
Intestinal failure (IF)-associated liver disease (IFALD), as a major complication, contributes to significant morbidity in pediatric IF patients. However, the pathogenesis of IFALD is still uncertain. We here investigate the roles of bile acid (BA) dysmetabolism in the unclear pathogenesis of IFALD. It found that the histological evidence of pediatric IF patients exhibited liver injury, which was characterized by liver bile duct proliferation, inflammatory infiltration, hepatocyte apoptosis and different stages of fibrosis...
December 15, 2016: Scientific Reports
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