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https://www.readbyqxmd.com/read/28523321/autoimmune-hepatitis-with-sclerosing-cholangitis-in-a-patient-with-thiopurine-methyltransferase-deficiency-case-presentation
#1
Sorin Claudiu Man, Cristina Nicoleta Schnell, Valentina Sas, Anca Dana Buzoianu, Dan Gheban
The association between two autoimmune diseases is known in the literature as overlap syndrome. We present the case of an 18-year-old boy, diagnosed at the age of 13 with an overlap syndrome between type I autoimmune hepatitis and sclerosing cholangitis. The response to immunosuppressant therapy was hampered by azathioprine-induced toxicity causing severe pancytopenia, as a result of thiopurine methyltransferase enzyme genetic deficiency. Treatment was replaced by mycophenolate mofetil. Although the relapse rate was reduced, the disease progressed to cirrhosis...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/28521077/clinical-case-series-of-pediatric-hepatic-angiosarcoma
#2
Kalee L Grassia, Caitlin M Peterman, Ionela Iacobas, Judith F Margolin, Ewa Bien, Bhavna Padhye, Rebecka L Meyers, Denise M Adams
Hepatic angiosarcoma is a rare, aggressive, malignant neoplasm with fewer than 50 cases reported in children. Prognosis is poor, with a minority surviving beyond 2 years after diagnosis. We report eight cases of pediatric hepatic angiosarcoma, diagnosed at a mean age of 3 years. Seven were initially diagnosed with an infantile hepatic hemangioendothelioma (IHHE) or hemangioma and the eighth with a "vascular tumor." Two patients, who received liver transplant, survived. We suggest hepatic hemangiomas can rarely transform into angiosarcomas and a subset of IHHEs (Type II) are actually a low-grade form of angiosarcoma rather than a benign lesion...
May 18, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28512555/assessment-of-obesity-and-hepatic-late-adverse-effects-in-the-egyptian-survivors-of-pediatric-acute-lymphoblastic-leukemia-a-single-center-study
#3
Farida H El-Rashedy, Mahmoud A El-Hawy, Sally M El Hefnawy, Mona M Mohammed
BACKGROUND: Childhood acute lymphoblastic leukemia (ALL) with current cure rates reaching 80% emphasizes the necessity to determine treatment-related long-term effects. The aim of this study is to estimate the prevalence of overweight, obesity, and hepatic late adverse effects in a cohort of ALL survivors treated at the Hematology and Oncology Unit, Pediatrics Department, Menoufia University, Egypt. METHODS: In this case-control study, height, weight, and body mass index (BMI) were assessed for 35 pediatric ALL survivors and 35 healthy children...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28507928/the-role-of-direct-acting-antivirals-in-the-treatment-of-children-with-chronic-hepatitis-c
#4
REVIEW
Christine Hong Ting Yang, Eric R Yoo, Aijaz Ahmed
In the United States, chronic infection with the hepatitis C virus (HCV) affects an estimated 0.1-2% of the pediatric population, who are consequently at risk for major complications, including cirrhosis, hepatocellular carcinoma, and death. The current standard of treatment for chronic hepatitis C (CHC) in children is pegylated-interferon-alpha (PEG-IFN) in combination with ribavirin. PEG-IFN/ribavirin therapy is approved for children ages 3 and older; however, it is often held from use until adulthood because of its extensive list of potential side effects and high likelihood of causing adverse symptoms...
March 28, 2017: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/28502443/hepatitis-b-and-c
#5
REVIEW
Wikrom Karnsakul, Kathleen B Schwarz
Chronic viral hepatitis is a global health threat and financial burden. Hepatitis B and C viruses (HBV and HCV) are the most common causes of chronic viral hepatitis in the United States. Most cases are asymptomatic before adulthood. Research has resulted in effective therapy for HCV and the promise of effective therapies for HBV. For HCV, therapy is pegylated interferon and ribavirin. Clinical trials with effective direct-acting antiviral agents are underway in pediatrics. For HBV, approved agents are alpha-interferon, lamivudine, adefovir, tenofovir, and entecavir...
June 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/28499592/risk-factors-for-poor-outcomes-of-children-with-acute-acalculous-cholecystitis
#6
Yi-An Lu, Cheng-Hsun Chiu, Man-Shan Kong, Han-I Wang, Hsun-Chin Chao, Chien-Chang Chen
BACKGROUND: Acute acalculous cholecystitis (AAC) is generally considered to be a mild disease in children; however, if left untreated or treated without caution, AAC can lead to severe outcomes, such as death. The objectives of this study were to present the clinical features and identify the predictors of mortality in pediatric AAC. METHODS: Patients diagnosed with AAC between 2005 and 2012 were enrolled. AAC was defined by the presence of fever and an echo-proven thickened gallbladder wall exceeding 4 mm...
March 25, 2017: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/28494529/nash-therapy-omega-3-supplementation-vitamin-e-insulin-sensitizers-and-statin-drugs
#7
Stephen Caldwell
Non-alcoholic steatohepatitis (NASH) is the more aggressive form of non-alcoholic fatty liver disease (NAFLD). NASH can progress to hepatic fibrosis, cirrhosis, portal hypertension and primary liver cancer. Therapy is evolving with a substantial number of trials of promising new agents now in progress. In this article however, we will examine data for several older forms of therapy which have been fairly extensively studied over the years: Polyunsaturated Fatty Acid (PUFA) supplements, vitamin E, insulin sensitizing agents with a focus on pioglitazone and statin agents...
May 10, 2017: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/28493388/magnetic-resonance-elastography-measured-shear-stiffness-as-a-biomarker-of-fibrosis-in-pediatric-nonalcoholic-fatty-liver-disease
#8
Jeffrey B Schwimmer, Cynthia Behling, Jorge Eduardo Angeles, Melissa Paiz, Janis Durelle, Jonathan Africa, Kimberly P Newton, Elizabeth M Brunt, Joel E Lavine, Stephanie H Abrams, Prakash Masand, Rajesh Krishnamurthy, Kelvin Wong, Richard L Ehman, Meng Yin, Kevin J Glaser, Bogdan Dzyubak, Tanya Wolfson, Anthony C Gamst, Jonathan Hooker, William Haufe, Alexandra Schlein, Gavin Hamilton, Michael S Middleton, Claude B Sirlin
Magnetic Resonance Elastography (MRE) is promising for non-invasive assessment of fibrosis, a major determinant of outcome in nonalcoholic fatty liver disease (NAFLD). However, data in children are limited. Study aims were to determine accuracy of MRE for detection of fibrosis and advanced fibrosis in children with NAFLD, and to assess agreement between manual and novel automated reading methods. We performed a prospective, multi-center study of 2D-MRE in children with NAFLD. MR-elastograms were analyzed manually at 2 reading centers and using a new automated technique...
May 11, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28473302/glassy-droplet-inclusions-within-the-cytoplasm-of-kupffer-cells-a-novel-ultrastructural-feature-for-the-diagnosis-of-pediatric-autoimmune-hepatitis
#9
Joanna Maria Lotowska, Maria Elzbieta Sobaniec-Lotowska, Urszula Daniluk, Dariusz Marek Lebensztejn
Since Kupffer cells/macrophages (KCs/MPs) may be involved in the pathogenesis of autoimmune hepatitis (AIH), this pioneer study was undertaken to evaluate KCs/MPs in pediatric AIH in transmission-electron microscope. METHODS: Ultrastructural analyses were performed using liver biopsies from 14 children with clinicopathologically diagnosed AIH. RESULTS: In all AIH children, ultrastructural findings revealed changes in the cells lining sinusoidal vessels, especially KCs/MPs and endothelial cells...
April 12, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28472948/noninvasive-assessment-of-liver-steatosis-in-children-the-clinical-value-of-controlled-attenuation-parameter
#10
Giovanna Ferraioli, Valeria Calcaterra, Raffaella Lissandrin, Marinella Guazzotti, Laura Maiocchi, Carmine Tinelli, Annalisa De Silvestri, Corrado Regalbuto, Gloria Pelizzo, Daniela Larizza, Carlo Filice
BACKGROUND: To assess the clinical validity of controlled attenuation parameter (CAP) in the diagnosis of hepatic steatosis in a series of overweight or obese children by using the imperfect gold standard methodology. METHODS: Consecutive children referred to our institution for auxological evaluation or obesity or minor elective surgery were prospectively enrolled. Anthropometric and biochemical parameters were recorded. Ultrasound (US) assessment of steatosis was carried out using ultrasound systems...
May 4, 2017: BMC Gastroenterology
https://www.readbyqxmd.com/read/28469406/review-of-current-applications-of-immunohistochemistry-in-pediatric-nonneoplastic-gastrointestinal-hepatobiliary-and-pancreatic-lesions
#11
REVIEW
Joseph de Nanassy, Dina El Demellawy
Immunohistochemical (IHC) stains are widely used by pathologists for a variety of considerations in the diagnostic workup of pediatric nonneoplastic lesions in gastrointestinal (GI), hepatic, biliary, and pancreatic lesions. The pathologic changes cover a wide range and types of presentations, including inflammatory (bacterial and viral), metaplastic, posttransplant lymphoproliferative, autoimmune, metabolic, degenerative, developmental, and genetic conditions, among others. The everyday practical value of IHC stains covers primary identification, confirmation, differential, and/or exclusionary roles in the hands and eyes and minds of the practitioners...
2017: Analytical Chemistry Insights
https://www.readbyqxmd.com/read/28468559/pediatric-mesenchymal-hamartomas-of-the-liver-can-show-both-foregut-and-hindgut-phenotype
#12
Hao Wu, William Ferguson, Eumenia Castro, Milton Finegold, Kalyani Patel
Cystic epithelium in mesenchymal hamartoma (MH) is typically biliary type. Mucinous differentiation of the epithelium and increased hepatocellular component may pose a diagnostic challenge. We studied MH in 7 children (6 M, 1 F; age 4 months to 8 years, median 1 year). Resected tumors varied from 3.0 to 17.0 cm. All tumors showed biliary epithelium in the cystic component with strong and diffuse reactivity for CK7 and CK19. Expression of CK20 and CDX2 was additionally seen in 2 tumors, rare/focal in 1, and diffuse with mucinous differentiation in the other...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/28461212/risk-factors-and-outcomes-related-to-pediatric-intensive-care-unit-admission-after-hematopoietic-stem-cell-transplantation-a-single-centre-experience
#13
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). To investigate 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 (0-23 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 14.3%. The main causes of PICU admission were: respiratory failure (36%), multiple organ failure (16%) and septic shock (13%)...
April 28, 2017: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/28447322/successful-hepatectomy-for-hepatic-abscess-with-chronic-granulomatous-disease-a-case-report
#14
Ryo Muranushi, Makoto Suzuki, Kenichiro Araki, Norio Kubo, Sayaka Otake, Yutaka Nishida, Takashi Ishige, Hirokazu Arakawa, Hiroyuki Kuwano, Ken Shirabe
BACKGROUND: Chronic granulomatous disease (CGD), a rare inherited disorder, is characterized by impaired ability of phagocytic cells to kill certain bacteria and fungi. Although liver abscess is a common manifestation of CGD, its optimal management in these patients is unknown. Here, we present a case of successful hepatectomy for hepatic abscess in a patient with CGD. CASE PRESENTATION: An adolescent patient with previously diagnosed CGD presented to the pediatrics department of our institution with fever...
December 2017: Surgical Case Reports
https://www.readbyqxmd.com/read/28437324/noncirrhotic-portal-fibrosis-in-pediatric-population
#15
Vikrant Sood, Bikrant B Lal, Rajeev Khanna, Dinesh Rawat, Chhagan Bihari, Seema Alam
OBJECTIVES: Noncirrhotic 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 were evaluated...
May 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28433098/wilson-disease-in-children
#16
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/28426902/hepatic-steatosis-after-pediatric-liver-transplant
#17
Emily R Perito, Tabitha Vase, Rageshree Ramachandran, Andrew Phelps, Kuang-Yu Jen, Robert H Lustig, Vickie A Feldstein, Philip Rosenthal
RATIONALE: Hepatic steatosis develops after liver transplant in 30% of adults, and non-alcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in non-transplanted children. However, post-transplant steatosis has been minimally studied in pediatric liver transplant recipients. We explored the prevalence, persistence, and association with chronic liver damage of hepatic steatosis in these children. RESULTS: In this single-center study of pediatric patients transplanted 1988-2015 (n=318), 31% of those with any post-transplant biopsy (n=271) had ≥1 biopsy with steatosis...
April 20, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28425257/role-of-hygienic-factors-in-the-etiology-of-allergic-disorders-in-children
#18
Şule Yeşil, Ahmet Kan, Olkar Abdulmajed, Arzu Bakirtaş, Nedim Sultan, Sadık M Demirsoy
BACKGROUND/AIM: We investigated the role of body flora and chronic inflammatory infections in the etiology of allergic disorders in Turkish children. MATERIALS AND METHODS: Forty pediatric asthma patients with positive skin prick tests and 40 age-matched healthy subjects with negative skin prick tests were enrolled in this cross-sectional study. Serum H. pylori IgG, viral hepatitis serology, IL-10, and TGF-beta levels were measured. Stool and throat cultures were taken and tested for occurrence of microorganisms...
April 18, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/28425089/high-diagnostic-yield-of-clinically-unidentifiable-syndromic-growth-disorders-by-targeted-exome-sequencing
#19
Yoo-Mi Kim, Yun-Jin Lee, Jae Hong Park, Hyoung-Doo Lee, Chong Kun Cheon, Su-Young Kim, Jae-Yeon Hwang, Ja-Hyun Jang, Han-Wook Yoo
As syndromic short stature and overgrowth are heterogeneous and the list of causative genes is rapidly expanding, there is an unmet need for identifying genetic causes based on conventional gene testing or karyotyping. Early diagnosis leads to the proper management of the patient and providing genetic counseling for family members at risk in a timely manner. We conducted targeted exome sequencing to identify the genetic causes of undiagnosed syndromic short stature or overgrowth in 15 pediatric patients from 13 families in Korea...
April 20, 2017: Clinical Genetics
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-consecutive-741-recipients-tips-and-tricks-to-overcome-the-poor-hepatic-arterial-flow
#20
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
BACKGROUND: The reconstruction of hepatic artery (HA) is most complex step in living donor liver transplantation (LDLT) due to smaller diameter of the artery and increased risk of HA related complications. Due to smaller diameter of the HA, many centres use microsurgical technique with interrupted sutures for arterial anastomosis. Aim of our study was to retrospectively investigate the outcomes after HA reconstruction performed under magnifying loupes using "parachute technique". MATERIALS AND METHODS: From August 2002 to 31(st) August 2016, LDLT was performed in 766 recipients...
April 19, 2017: Liver Transplantation
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