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https://www.readbyqxmd.com/read/28437324/noncirrhotic-portal-fibrosis-in-pediatric-population
#1
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
#2
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
#3
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
#4
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
#5
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
#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
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
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...
April 17, 2017: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/28417347/-serological-markers-of-viral-hepatitis-b-and-c-in-children-with-sickle-cell-disease-monitored-in-the-pediatrics-department-at-the-university-hospital-of-bobo-dioulasso-burkina-faso
#8
S A Kissou, M Koura, A Sawadogo, A-S Ouédraogo, H Traoré, E Kamboulé, W W F Zogona, B Nacro
Viral hepatitis B and C are universal public health problems. Burkina Faso is a high endemic area for hepatitis B. Patients with sickle cell disease are at risk. The aim of this study was to investigate and quantify the serological markers of viral hepatitis B and C among pediatric patients with sickle cell disease. This was a descriptive cross-sectional study, which took place from July to November 2014 at the Department of Pediatrics, University Hospital of Bobo- Dioulasso. The study included 44 SS and 26 SC sickle cell patients...
April 17, 2017: Bulletin de la Société de Pathologie Exotique
https://www.readbyqxmd.com/read/28403051/modelling-cd4-t-cell-recovery-in-hepatitis-c-and-hiv-co-infected-children-receiving-antiretroviral-therapy
#9
Adedeji O Majekodunmi, Claire Thorne, Ruslan Malyuta, Alla Volokha, Robin E Callard, Nigel J Klein, Joanna Lewis
BACKGROUND: The effect of hepatitis C virus (HCV) coinfection on CD4 T cell recovery in treated HIV-infected children is poorly understood. OBJECTIVE: To compare CD4 T cell recovery in HIV/HCV coinfected children with recovery in HIV monoinfected children. METHOD: We studied 355 HIV monoinfected and 46 HIV/HCV coinfected children receiving antiretroviral therapy (ART) during a median follow-up period of 4.2 years (interquartile range: 2.7-5...
May 2017: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/28399447/contrast-enhanced-ct-features-of-hepatoblastoma-can-we-predict-histopathology
#10
Akshay D Baheti, A Luana Stanescu, Ning Li, Teresa Chapman
BACKGROUND: Hepatoblastoma is the most common hepatic malignancy occurring in the pediatric population. Intratumoral cellular behavior varies, and the small-cell undifferentiated histopathology carries a poorer prognosis than other tissue subtypes. Neoadjuvant chemotherapy is recommended for this tumor subtype prior to surgical resection in most cases. Early identification of tumors with poor prognosis could have a significant clinical impact. Objective The aim of this work was to identify imaging features of small-cell undifferentiated subtype hepatoblastoma that can help distinguish this subtype from more favorable tumors and potentially guide the clinical management...
April 6, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28396861/non-invasive-investigations-for-the-diagnosis-of-fontan-associated-liver-disease-in-pediatric-and-adult-fontan-patients
#11
Amyna Fidai, Frederic Dallaire, Nanette Alvarez, Yvonne Balon, Robin Clegg, Michael Connelly, Frank Dicke, Deborah Fruitman, Joyce Harder, Kimberley Myers, David J Patton, Tim Prieur, Erika Vorhies, Robert P Myers, Steven R Martin, Steven C Greenway
Fontan-associated liver disease (FALD) is a serious complication related to the chronically elevated venous pressure and low cardiac output of this abnormal circulation. However, diagnostic markers for this condition are limited. We hypothesized that specific tests for fibrosis developed for other chronic liver diseases would identify a higher prevalence of FALD than ultrasound and standard laboratory tests and that identified abnormalities would correlate with time post-Fontan. In this cross-sectional study, we assessed 19 children (average age 8...
2017: Frontiers in Cardiovascular Medicine
https://www.readbyqxmd.com/read/28391883/childhood-adult-onset-lysosomal-acid-lipase-deficiency-a-serious-metabolic-and-vascular-phenotype-beyond-liver-disease-four-new-pediatric-cases
#12
Pierre Poinsot, Sophie Collardeau Frachon, Lioara Restier, André Sérusclat, Mathilde Di Filippo, Sybil Charrière, Philippe Moulin, Alain Lachaux, Noel Peretti
BACKGROUND: The childhood/adult-onset lysosomal acid lipase deficiency (LALD; late-onset LALD) is a rare genetic disease. Children present severe fatty liver disease with early cirrhosis. Before enzyme replacement therapy, statins were the standard treatment to improve the severe dyslipidemia. However, late-onset LALD should be considered as a systemic metabolic disease: chronic hyper-low-density lipoprotein and hypo-high-density lipoprotein cholesterolemia induces early atherosclerosis in addition to the liver morbidity...
January 2017: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/28390159/the-natural-history-of-primary-sclerosing-cholangitis-in-781-children-a-multicenter-international-collaboration
#13
Mark R Deneau, Wael El-Matary, Pamela L Valentino, Reham Abdou, Khaled Alqoaer, Mansi Amin, Achiya Z Amir, Marcus Auth, Fateh Bazerbachi, Annemarie Broderick, Albert Chan, Jillian Cotter, Sylvia Doan, Mounif El-Youssef, Federica Ferrari, Katryn N Furuya, Madeleine Gottrand, Frederic Gottrand, Nitika Gupta, Matjaz Homan, M K Jensen, Binita M Kamath, Kyung Mo Kim, Kaija-Leena Kolho, Anastasia Konidari, Bart Koot, Raffaele Iorio, Oren Ledder, Cara Mack, Mercedes Martinez, Tamir Miloh, Parvathi Mohan, Niamh O'Cathain, Alexandra Papadopoulou, Amanda Ricciuto, Lawrence Saubermann, Pushpa Sathya, Eyal Shteyer, Vratislav Smolka, Atushi Tanaka, Raghu Varier, Veena Venkat, Bernadette Vitola, Miriam B Vos, Marek Woynarowski, Jason Yap
BACKGROUND: There are limited data on the natural history of primary sclerosing cholangitis (PSC) in children. We aimed to describe the disease characteristics and long-term outcomes of pediatric PSC. METHODS: We retrospectively collected all pediatric PSC cases from 36 participating institutions and conducted a survival analysis from the date of PSC diagnosis to dates of diagnosis of portal hypertensive or biliary complications, cholangiocarcinoma, liver transplantation, or death...
April 8, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28367603/mr-elastography-high-rate-of-technical-success-in-pediatric-and-young-adult-patients
#14
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/28366562/traumatic-colorectal-injuries-in-children-the-national-trauma-database-experience
#15
Pamela M Choi, Michael Wallendorf, Martin S Keller, Adam M Vogel
PURPOSE: We sought to utilize a nationwide database to characterize colorectal injuries in pediatric trauma. METHODS: The National Trauma Database (NTDB) was queried for all patients (age≤14years) with colorectal injuries from 2013 to 2014. We stratified patients by demographics and measured outcomes. We analyzed groups based on mechanism, colon vs rectal injury, as well as colostomy creation. Statistical analysis was conducted using t-test and ANOVA for continuous variables as well as chi-square for continuous variables...
March 27, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28364277/pediatric-choledochal-cysts-diagnosis-and-current-management
#16
REVIEW
Kevin C Soares, Seth D Goldstein, Mounes A Ghaseb, Ihab Kamel, David J Hackam, Timothy M Pawlik
Choledochal cysts are rare congenital disorders first described by Vater and Ezler in 1723. Their exact etiology remains incompletely understood; however, an anomalous pancreaticobiliary union (APBDU) and subsequent reflux of biliary contents into the biliary tree are thought to play a role. Accordingly, APBDU-associated choledochal cyst patients are significantly more likely to have evidence of hepatitis, cholangitis or pancreatitis and pathologically confirmed inflammation. In 1977, Todani and colleagues modified the original Alonso-Lej classification to include five types of CC...
March 31, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28364132/challenges-in-establishing-genotype-phenotype-correlations-in-arpkd-case-report-on-a-toddler-with-two-severe-pkhd1-mutations
#17
Kathrin Ebner, Claudia Dafinger, Nadina Ortiz-Bruechle, Friederike Koerber, Bernhard Schermer, Thomas Benzing, Jörg Dötsch, Klaus Zerres, Lutz Thorsten Weber, Bodo B Beck, Max Christoph Liebau
BACKGROUND: Autosomal recessive polycystic kidney disease (ARPKD) constitutes an important cause of pediatric end stage renal disease and is characterized by a broad phenotypic variability. The disease is caused by mutations in a single gene, Polycystic Kidney and Hepatic Disease 1 (PKHD1), which encodes a large transmembrane protein of poorly understood function called fibrocystin. Based on current knowledge of genotype-phenotype correlations in ARPKD, two truncating mutations are considered to result in a severe phenotype with peri- or neonatal mortality...
March 31, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28362694/the-challenge-of-treating-children-with-hepatitis-c-virus-infection
#18
Giuseppe Indolfi, Claire Thorne, Manal H El-Sayed, Carlo Giaquinto, Regino P Gonzalez-Peralta
The development of oral hepatitis C virus direct acting antivirals has revolutionized the therapeutic field. Nowadays, multiple safe and highly effective antiviral regimens are commercially available to treat adults with hepatitis C infection. These new regimens for the first time, genuinely raise the prospects of eradicating hepatitis C virus. However, many challenges remain: from identifying infected individuals to optimizing treatment and ensuring global access to antiviral therapy to all population groups, including children...
March 30, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28355206/hepatic-mdr3-expression-impacts-lipid-homeostasis-and-susceptibility-to-inflammatory-bile-duct-obstruction-in-neonates
#19
Alexandra N Carey, Wujuan Zhang, Kenneth D R Setchell, Julia E Simmons, Tiffany Shi, Celine S Lages, Mary Mullen, Kaitlin Carroll, Rebekah Karns, Kazuhiko Bessho, Rachel Sheridan, Xueheng Zhao, Susanne N Weber, Alexander G Miethke
BACKGROUND: Heterozygous mutations in the gene ABCB4, encoding the phospholipid floppase MDR3 (Mdr2 in mice), are associated with various chronic liver diseases. Here, we hypothesize that reduced ABCB4 expression predisposes to extrahepatic biliary atresia (EHBA). METHODS: Livers from neonatal wildtype (wt) and heterozygous Mdr2-deficient mice were subjected to mass-spectrometry based lipidomics and RNAseq studies. Following postnatal infection with rhesus rotavirus (RRV), liver immune responses and EHBA phenotype were assessed...
March 29, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28337087/medication-dosage-in-overweight-and-obese-children
#20
Kelly L Matson, Evan R Horton, Amanda C Capino
Approximately 31.8% of U.S. children ages 2 to 19 years are considered overweight or obese. This creates significant challenges to dosing medications that are primarily weight based (mg/kg) and in predicting pharmacokinetics parameters in pediatric patients. Obese individuals generally have a larger volume of distribution for lipophilic medications. Conversely, the Vd of hydrophilic medications may be increased or decreased due to increased lean body mass, blood volume, and decrease percentage of total body water...
January 2017: Journal of Pediatric Pharmacology and Therapeutics: JPPT: the Official Journal of PPAG
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