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Pediatric liver disease

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https://www.readbyqxmd.com/read/29775560/pediatric-nonalcoholic-fatty-liver-disease
#1
Claudio Chiesa, Gian Marco Andreoli, Lucia Pacifico
No abstract text is available yet for this article.
May 15, 2018: Jornal de Pediatria
https://www.readbyqxmd.com/read/29769027/immune-monitoring-after-pediatric-liver-transplantation-the-prospective-chilsfree-cohort-study
#2
Imeke Goldschmidt, André Karch, Rafael Mikolajczyk, Frauke Mutschler, Norman Junge, Eva Doreen Pfister, Tamara Möhring, Lorenzo d'Antiga, Patrick McKiernan, Deirdre Kelly, Dominique Debray, Valérie McLin, Joanna Pawlowska, Loreto Hierro, Kerstin Daemen, Jana Keil, Christine Falk, Ulrich Baumann
BACKGROUND: Although trough levels of immunosuppressive drugs are largely used to monitor immunosuppressive therapy after solid organ transplantation, there is still no established tool that allows for a validated assessment of functional degree of immunosuppression or the identification of clinically relevant over- or under-immunosuppression, depending on graft homeostasis. Reliable non-invasive markers to predict biopsy proven acute rejection (BPAR) do not exist. Literature data suggest that longitudinal measurements of immune markers might be predictive of BPAR, but data in children are scarce...
May 16, 2018: BMC Gastroenterology
https://www.readbyqxmd.com/read/29766815/old-and-new-treatments-for-pediatric-autoimmune-hepatitis
#3
Silvia Nastasio, Giuseppe Maggiore, Marco Sciveres, Lorenza Matarazzo
Autoimmune hepatitis is a rare inflammatory disease of the liver that most frequently affects children and young adults. It is a multifactorial disease of unknown etiology, characteristically progressive in nature, and if left untreated, may lead to cirrhosis and terminal liver failure. It has been known for several decades now that immunosuppressive treatment convincingly alters the outcome of most patients with autoimmune hepatitis and as such it should be started as soon as diagnosis is made. Primary goals of treatment are: normalization of hepatocellular function, extinction of the hepatic necroinflammatory process, and maintenance of a stable remission, thus preventing progression to cirrhosis and its complications...
May 16, 2018: Current Pediatric Reviews
https://www.readbyqxmd.com/read/29762251/prospective-assessment-of-adenovirus-infection-in-pediatric-kidney-transplant-recipients
#4
Rachel M Engen, Meei-Li Huang, Giulia E Park, Jodi M Smith, Ajit P Limaye
BACKGROUND: Adenovirus infection is associated with graft dysfunction and graft loss in pediatric cardiac, lung, and liver transplants in prior retrospective studies, but data in pediatric kidney transplant recipients is limited. METHODS: We conducted a prospective single-center cohort study of 75 consecutive pediatric kidney transplant recipients who underwent monthly screening for adenovirus viremia and symptom assessment for 2 years posttransplant. RESULTS: Adenovirus viremia was detected in 11 (14...
May 2, 2018: Transplantation
https://www.readbyqxmd.com/read/29762096/us-time-harmonic-elastography-detection-of-liver-fibrosis-in-adolescents-with-extreme-obesity-with-nonalcoholic-fatty-liver-disease
#5
Christian A Hudert, Heiko Tzschätzsch, Jing Guo, Birgit Rudolph, Hendrik Bläker, Christoph Loddenkemper, Werner Luck, Hans-Peter Müller, Daniel C Baumgart, Bernd Hamm, Jürgen Braun, Hermann-Georg Holzhütter, Susanna Wiegand, Ingolf Sack
Purpose To measure in vivo liver stiffness by using US time-harmonic elastography in a cohort of pediatric patients who were overweight to extremely obese with nonalcoholic fatty liver disease (NAFLD) and to evaluate the diagnostic value of time-harmonic elastography for differentiating stages of fibrosis associated with progressive disease. Materials and Methods In this prospective study, 67 consecutive adolescents (age range, 10-17 years; mean body mass index, 34.7 kg/m2 ; range, 21.4-50.4 kg/m2 ) with biopsy-proven NAFLD were enrolled...
May 15, 2018: Radiology
https://www.readbyqxmd.com/read/29752170/treating-obstructive-sleep-apnea-and-chronic-intermittent-hypoxia-improves-the-severity-of-nonalcoholic-fatty-liver-disease-in-children
#6
Shikha S Sundaram, Ann C Halbower, Jelena Klawitter, Zhaoxing Pan, Kristen Robbins, Kelley E Capocelli, Ronald J Sokol
OBJECTIVE: To determine the effects of treating obstructive sleep apnea/nocturnal hypoxia on pediatric nonalcoholic fatty liver disease (NAFLD) severity and oxidative stress. STUDY DESIGN: Biopsy proven participants (n = 9) with NAFLD and obstructive sleep apnea/hypoxia were studied before and after treatment with continuous positive airway pressure (CPAP) for sleep disordered breathing, including laboratory testing and markers of oxidative stress, urine F(2)-isoprostanes...
May 8, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29750764/linezolid-containing-treatment-regimens-for-tuberculosis-in-children
#7
Luis M Prieto, Begoña Santiago, Teresa Del Rosal, Begoña Carazo, Ana B Jiménez, Beatriz Pérez-Gorricho, Felipe Rubio, Alfredo Tagarro, Daniel Blázquez, David Moreno-Pérez, María J Mellado, Fernando Baquero-Artigao
BACKGROUND: In recent years there is an increasing interest in the use of linezolid for the treatment of tuberculosis (TB). METHODS: Patients under 18 years of age who received linezolid within the Spanish Pediatric TB Network (pTBred) from 2001 to 2016 were retrospectively included. Treatment characteristics, adverse events (AEs) and outcomes were analyzed. RESULTS: Fifteen children were included (53% male) with a median age of 3.6 years (IQR: 1...
May 10, 2018: Pediatric Infectious Disease Journal
https://www.readbyqxmd.com/read/29744756/regression-of-fibrosis-in-pediatric-liver-diseases
#8
Vikrant Sood, Bikrant Bihari Lal, Archana Rastogi, Rajeev Khanna, Dinesh Rawat, Seema Alam
The concept of irreversibility of cirrhosis has been challenged in the recent past with literature in this regard, albeit still scarce, now being accumulated across all age groups, etiologies, and geographical regions. This small series of nine pediatric cases elegantly recapitulates the concept of regression of hepatic fibrosis/cirrhosis and paves way for further detailed studies to enable development of therapeutic anti-fibrotic modalities in future.
May 9, 2018: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/29740791/nonalcoholic-fatty-liver-disease-in-children-not-a-small-matter
#9
REVIEW
Praveen Kumar Conjeevaram Selvakumar, Mohammad Nasser Kabbany, Naim Alkhouri
The prevalence of nonalcoholic fatty liver disease (NAFLD) has increased substantially in the past two decades and NAFLD has now become the most common cause of chronic liver disease in children and adolescents. NAFLD is a broad clinicopathologic spectrum ranging from simple steatosis to varying degrees of necroinflammation called nonalcoholic steatohepatitis (NASH), leading to fibrosis and subsequently to cirrhosis. Despite the increasing prevalence and progressive nature of NAFLD even among children, therapy for NAFLD in both adults and children are limited...
May 9, 2018: Paediatric Drugs
https://www.readbyqxmd.com/read/29735029/pediatric-intestinal-transplantation
#10
REVIEW
Neslihan Celik, George V Mazariegos, Kyle Soltys, Jeffrey A Rudolph, Yanjun Shi, Geoffrey J Bond, Rakesh Sindhi, Armando Ganoza
Pediatric intestinal transplantation has moved from the theoretic to an actual therapy for children with irreversible intestinal failure who are suffering from complications of total parenteral nutrition. Owing to significant advancement in the management of intestinal failure and prevention of parenteral nutrition-related complications that have led to reduction in incidence of parenteral nutrition-associated liver disease and have improved intestinal adaptation, the indications for intestinal transplantation are evolving...
June 2018: Gastroenterology Clinics of North America
https://www.readbyqxmd.com/read/29729773/surgical-outcomes-in-alagille-syndrome-and-pfic-a-single-institution-s-20-year-experience
#11
Celia D Flores, Yangyang R Yu, Tamir A Miloh, John Goss, Mary L Brandt
BACKGROUND: Alagille Syndrome (AGS) and Progressive Familial Intrahepatic Cholestasis (PFIC) are rare pediatric biliary disorders that lead to progressive liver disease. This study reviews our experience with the surgical management of these disorders over the last 20years. METHODS: We retrospectively reviewed the records of children diagnosed with AGS or PFIC from January 1996 to December 2016. Data collected included demographics, surgical intervention (liver transplant or biliary diversion), and complications...
May 2018: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/29728744/free-breathing-quantification-of-hepatic-fat-in-healthy-children-and-children-with-nonalcoholic-fatty-liver-disease-using-a-multi-echo-3-d-stack-of-radial-mri-technique
#12
Tess Armstrong, Karrie V Ly, Smruthi Murthy, Shahnaz Ghahremani, Grace Hyun J Kim, Kara L Calkins, Holden H Wu
BACKGROUND: In adults, noninvasive chemical shift encoded Cartesian magnetic resonance imaging (MRI) and single-voxel magnetic resonance (MR) spectroscopy (SVS) accurately quantify hepatic steatosis but require breath-holding. In children, especially young and sick children, breath-holding is often limited or not feasible. Sedation can facilitate breath-holding but is highly undesirable. For these reasons, there is a need to develop free-breathing MRI technology that accurately quantifies steatosis in all children...
May 4, 2018: Pediatric Radiology
https://www.readbyqxmd.com/read/29723692/a-learning-collaborative-approach-increases-specificity-of-diagnosis-of-acute-liver-failure-in-pediatric-patients
#13
Michael R Narkewicz, Simon Horslen, Regina M Hardison, Benjamin L Shneider, Norberto Rodriguez-Baez, Estella M Alonso, Vicky L Ng, Mike A Leonis, Kathleen M Loomes, David A Rudnick, Philip Rosenthal, Rene Romero, Girish C Subbarao, Ruosha Li, Steven H Belle, Robert H Squires
BACKGROUND & AIMS: Many pediatric patients with acute liver failure (PALF) do not receive a specific diagnosis (such as herpes simplex virus or Wilson disease or fatty acid oxidation defects)-they are left with an indeterminate diagnosis and are more likely to undergo liver transplantation, which is contraindicated for some disorders. Strategies to facilitate complete diagnostic testing should increase identification of specific liver diseases and might reduce liver transplantation...
April 30, 2018: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29713608/investigation-of-blood-betatrophin-levels-in-obese-children-with-non-alcoholic-fatty-liver-disease
#14
Fatih Battal, Hakan Türkön, Nilüfer Aylanç, Hakan Aylanç, Şule Yıldırım, Nazan Kaymaz, Sema Uysal
Purpose: The prevalence of obesity has significantly increased among children and adolescents worldwide and is becoming an important health care problem in parallel with the increased prevalence of obesity pediatric non-alcoholic fatty liver disease. Betatrophin is a newly define hormone that is commonly secreted by liver and plays role in glucose tolerance. This study aimed to investigate the relationship between serum betatrophin levels and non-alcoholic fatty liver disease in obese children...
April 2018: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/29707848/assessment-of-a-high-snr-chemical-shift-encoded-mri-with-complex-reconstruction-for-proton-density-fat-fraction-pdff-estimation-overall-and-in-the-low-fat-range
#15
Charlie C Park, Catherine Hooker, Jonathan C Hooker, Emily Bass, William Haufe, Alexandra Schlein, Yesenia Covarrubias, Elhamy Heba, Mark Bydder, Tanya Wolfson, Anthony Gamst, Rohit Loomba, Jeffrey Schwimmer, Diego Hernando, Scott B Reeder, Michael Middleton, Claude B Sirlin, Gavin Hamilton
BACKGROUND: Improving the signal-to-noise ratio (SNR) of chemical-shift-encoded MRI acquisition with complex reconstruction (MRI-C) may improve the accuracy and precision of noninvasive proton density fat fraction (PDFF) quantification in patients with hepatic steatosis. PURPOSE: To assess the accuracy of high SNR (Hi-SNR) MRI-C versus standard MRI-C acquisition to estimate hepatic PDFF in adult and pediatric nonalcoholic fatty liver disease (NAFLD) using an MR spectroscopy (MRS) sequence as the reference standard...
April 29, 2018: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/29705277/endotoxin-metabolism-reflects-hepatic-functional-reserve-in-end-stage-liver-disease
#16
N Okada, Y Sanada, T Urahashi, Y Ihara, N Yamada, Y Hirata, T Katano, S Otomo, K Ushijima, K Mizuta
BACKGROUND: The hepatic clearance of endotoxin (Et) may reflect hepatic functional reserve and ischemic injury to hepatocytes. Therefore, we examined the relationships between Et activity (EA) and the metrics Pediatric End-Stage Liver Disease (PELD)/Model of End-Stage Liver Disease (MELD) score and alanine transaminase (ALT) levels in the postoperative period. METHODS: We performed 8 living-donor liver transplantations (LDLTs) for biliary atresia at our center from April 2012 to December 2012...
April 25, 2018: Transplantation Proceedings
https://www.readbyqxmd.com/read/29701873/children-with-intestinal-failure-undergoing-intestinal-rehabilitation-are-at-risk-for-essential-fatty-acid-deficiency
#17
Riikka Gunnar, Mirka Lumia, Mikko Pakarinen, Laura Merras-Salmio
BACKGROUND: Essential fatty acid (EFA) status may be compromised during the intestinal failure (IF) rehabilitation. Parenteral lipid restriction is used to treat intestinal failure associated liver disease (IFALD), while the enteral fatty acid (FA) absorption remains limited. We analyzed the FA status among pediatric IF and intestinal insufficiency patients. METHODS: We evaluated 49 patients aged 0-18 years attending our nationwide IF referral center. Their serum FA fractions were determined and examined against previous nutrition, parenteral lipid emulsion, and intestinal anatomy data...
April 27, 2018: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29701871/pediatric-intestinal-failure-the-key-outcomes-for-the-first-100-patients-treated-in-a-national-tertiary-referral-center-during-1984-2017
#18
Laura Merras-Salmio, Annika Mutanen, Elisa Ylinen, Risto Rintala, Antti Koivusalo, Mikko P Pakarinen
BACKGROUND: Pediatric-onset intestinal failure (IF) remains a severe illness with life-threatening consequences. In this study, we analyzed a single center's outcomes of IF over 3 decades. METHODS: All children with IF who required parenteral nutrition (PN) >2 months or small-intestinal resection ≥50% managed since 1984 were included for retrospective outcome analyses. RESULTS: In total, 100 patients with median PN duration of 1.2 (interquartile range, 0...
April 27, 2018: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/29699915/dyslipidemia-is-associated-with-pediatric-nonalcoholic-fatty-liver-disease
#19
Shima Dowla, Stella Aslibekyan, Amy Goss, Kevin Fontaine, Ambika P Ashraf
BACKGROUND: With the increasing prevalence of childhood obesity, nonalcoholic fatty liver disease (NAFLD) has emerged as the most common cause of pediatric chronic liver disorder. Factors underlying the pathophysiology of NAFLD remain poorly defined. OBJECTIVE: This study aimed to describe the metabolic characteristics of children with NAFLD differing in race/ethnicity and to test associations between dyslipidemia and NAFLD. METHODS: A retrospective chart review was conducted at a tertiary referral university hospital among 309 children with a diagnosis of NAFLD...
April 3, 2018: Journal of Clinical Lipidology
https://www.readbyqxmd.com/read/29693762/growth-body-composition-and-bone-density-following-pediatric-liver-transplantation
#20
Amin Sheikh, Tim Cundy, Helen Maria Evans
Patients transplanted for cholestatic liver disease are often significantly fat-soluble vitamin deficient and malnourished pretransplant, with significant corticosteroid exposure post-transplant, with increasing evidence of obesity and metabolic syndrome post-LT. Our study aimed to assess growth, body composition, and BMD in patients post-pediatric LT. Body composition and bone densitometry scans were performed on 21 patients. Pre- and post-transplant anthropometric data were analyzed. Bone health was assessed using serum ALP, calcium, phosphate, and procollagen-1-N-peptide levels...
April 24, 2018: Pediatric Transplantation
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