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Cholestasis in children

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https://www.readbyqxmd.com/read/27898169/cross-sectional-analysis-of-progressive-familial-intrahepatic-cholestasis-in-puerto-rican-children
#1
Dellys M Soler, Antonio I Del Valle, David Fernandez-Lube, Benjamin L Shneider
OBJECTIVE: Specific inherited disorders may be more common in island communities. Prior case reports suggest that cholestatic liver diseases may constitute a group of these inherited disorders in Puerto Rico. A cross-sectional survey of liver diseases in children was conducted to assess this hypothesis. METHODS: A cross-sectional analysis was performed in patients with chronic cholestasis at "Hospital Pediátrico Universitario" in San Juan, Puerto Rico. Ten potential participants with high gamma-glutamyl transpeptidase (GGTP) cholestasis were identified...
December 2016: Puerto Rico Health Sciences Journal
https://www.readbyqxmd.com/read/27890981/neonatal-cholestasis-single-centre-experience-in-central-india
#2
Mayank Jain, Sagar Adkar, Chandrashekhar Waghmare, Jenisha Jain, Shikhar Jain, Kamna Jain, Gouri Rao Passi, Rashmi Shad Vinay, M K Soni
BACKGROUND: Neonatal cholestasis syndrome (NCS) is a major cause of morbidity and mortality in infants. The disorder has rarely been studied in centers from Central India. OBJECTIVES: To study the prevalence, clinical presentation and etiology of NCS at a tertiary referral center in Central India. MATERIALS AND METHODS: The study was carried out at a tertiary referral center in Central India. The study is a descriptive study. The records of all patients with suspected NCS treated in the Department of Pediatrics from 2007-2012 were analyzed...
October 2016: Indian Journal of Community Medicine
https://www.readbyqxmd.com/read/27882439/obstetric-and-perinatal-outcome-of-babies-born-from-sperm-selected-by-macs-from-a-randomized-controlled-trial
#3
Laura Romany, Nicolas Garrido, Ana Cobo, Belen Aparicio-Ruiz, Vicente Serra, Marcos Meseguer
PURPOSE: The purpose of this study is to assess outcomes after magnetic-activated cell sorting (MACS) technology on obstetric and perinatal outcomes compared with those achieved after swim up from randomized controlled trial. METHODS: This is a two-arm, unicentric, prospective, randomized, and triple-blinded trial and has a total of 237 infertile couples, between October 2010 and January 2013. A total of 65 and 66 newborns from MACS and control group, respectively, were described...
November 23, 2016: Journal of Assisted Reproduction and Genetics
https://www.readbyqxmd.com/read/27875503/influence-of-partial-external-biliary-diversion-on-the-lipid-profile-in-children-with-progressive-familial-intrahepatic-cholestasis
#4
Irena Jankowska, Piotr Czubkowski, Aldona Wierzbicka, Joanna Pawłowska, Piotr Kaliciński, Piotr Socha
OBJECTIVES: The concentration of bile acids is highly increased in progressive familial intrahepatic cholestasis (PFIC). Bile acids are the end products of cholesterol metabolism, and aid in the absorption of fat-soluble vitamins and dietary fat. The aim of our study was to investigate lipid metabolism in patients with PFIC with focus on the effect of partial external biliary diversion (PEBD). METHODS: In 26 patients with PFIC, who underwent PEBD surgery at the median age of 2...
December 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27843664/minimally-invasive-treatment-of-mirizzi-syndrome-a-rare-cause-of-cholestasis-in-childhood
#5
Ahmet Ali Tuncer, Sezgin Yilmaz, Mustafa Yavuz, Salih Çetinkurşun
Mirizzi syndrome is the compressive blockage of the cystic or choledochal duct caused by a biliary stone occupying the cystic canal or Hartmann's pouch. This occurrence is rare and, in English literature, three cases defined in children have been observed. In order to draw attention to this rare occurrence, we preferred a 14-year-old male patient with Mirizzi syndrome. In this case, ERCP was performed preoperatively and the diagnosis was carried out with the help of clear visualisation and identification of the tissue structures as well as the stent placed in bile duct; so we protected the patient from the possible iatrogenic injury occurring during surgery...
2016: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/27829683/biochemical-and-molecular-characteristics-of-citrin-deficiency-in-korean-children
#6
Seak Hee Oh, Beom Hee Lee, Gu-Hwan Kim, Jin-Ho Choi, Kyung Mo Kim, Han-Wook Yoo
Mutations in SLC25A13 cause citrin deficiency, which has three phenotypes: neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD), failure to thrive and dyslipidemia caused by citrin deficiency (FTTDCD) and adult-onset type 2 citrullinemia (CTLN2). The purpose of this study was to determine the mutation spectrum and the clinical and biochemical characteristics of citrin deficiency in Korean patients. Thirty-four patients were diagnosed with citrin deficiency based on mutations in SLC25A13, as verified by direct sequencing and long PCR screening of a large transposon insertion...
November 10, 2016: Journal of Human Genetics
https://www.readbyqxmd.com/read/27776008/key-histopathologic-features-of-liver-biopsies-that-distinguish-biliary-atresia-from-other-causes-of-infantile-cholestasis-and-their-correlation-with-outcome-a-multicenter-study
#7
Pierre Russo, John C Magee, Robert A Anders, Kevin E Bove, Catherine Chung, Oscar W Cummings, Milton J Finegold, Laura S Finn, Grace E Kim, Mark A Lovell, Margret S Magid, Hector Melin-Aldana, Sarangarajan Ranganathan, Bahig M Shehata, Larry L Wang, Frances V White, Zhen Chen, Catherine Spino
The liver biopsy guides diagnostic investigation and therapy in infants with undiagnosed cholestasis. Histologic features in the liver may also have prognostic value in the patient with biliary atresia (BA). We assessed the relative value of histologic features in 227 liver needle biopsies in discriminating between BA and other cholestatic disorders in infants enrolled in a prospective Childhood Liver Disease Research Network (ChiLDReN) cohort study by correlating histology with clinical findings in infants with and without BA...
December 2016: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/27757140/bipolar-and-related-disorders-induced-by-sodium-4-phenylbutyrate-in-a-male-adolescent-with-bile-salt-export-pump-deficiency-disease
#8
Giovanni Vitale, Giulia Simonetti, Martina Pirillo, Gianfranco Taruschio, Pietro Andreone
Bile Salt Export Pump (BSEP) Deficiency disease, including Progressive Familial Intrahepatic Cholestasis type 2 (PFIC2), is a rare disease, usually leading within the first ten years to portal hypertension, liver failure, hepatocellular carcinoma. Often liver transplantation is needed. Sodium 4-phenylbutyrate (4-PB) seems to be a potential therapeutic compound for PFIC2. Psychiatric side effects in the adolescent population are little known and little studied since the drug used to treat children and infants...
September 2016: Psychiatry Investigation
https://www.readbyqxmd.com/read/27746616/progressive-familial-intrahepatic-cholestasis-pfic-in-indian-children-clinical-spectrum-and-outcome
#9
Sajan Agarwal, Bikrant Bihari Lal, Dinesh Rawat, Archana Rastogi, Kishore G S Bharathy, Seema Alam
OBJECTIVE: To study the clinical and laboratory profile of children with progressive familial intrahepatic cholestasis (PFIC) and evaluate their outcome. METHODS: The study is a retrospective review of all cases diagnosed with PFIC between January 2011 and July 2015. All children underwent histopathological examination and immunostaining. Management was done as per institute's protocol. RESULTS: There were a total of 24 PFIC cases (PFIC 1-2, PFIC 2-19, PFIC 3-3)...
September 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/27706244/a-specially-designed-multi-gene-panel-facilitates-genetic-diagnosis-in-children-with-intrahepatic-cholestasis-simultaneous-test-of-known-large-insertions-deletions
#10
Neng-Li Wang, Yu-Lan Lu, Ping Zhang, Mei-Hong Zhang, Jing-Yu Gong, Yi Lu, Xin-Bao Xie, Yi-Ling Qiu, Yan-Yan Yan, Bing-Bing Wu, Jian-She Wang
BACKGROUND AND AIMS: Large indels are commonly identified in patients but are not detectable by routine Sanger sequencing and panel sequencing. We specially designed a multi-gene panel that could simultaneously test known large indels in addition to ordinary variants, and reported the diagnostic yield in patients with intrahepatic cholestasis. METHODS: The panel contains 61 genes associated with cholestasis and 25 known recurrent large indels. The amplicon library was sequenced on Ion PGM system...
2016: PloS One
https://www.readbyqxmd.com/read/27663215/complications-of-endoscopic-retrograde-cholangiopancreatography-in-pediatric-patients-a-systematic-literature-review-and-meta-analysis
#11
Danielle Usatin, Melissa Fernandes, Isabel E Allen, Emily R Perito, James Ostroff, Melvin B Heyman
OBJECTIVES: To systematically review risks and summarize reported complication rates associated with the performance of endoscopic retrograde cholangiopancreatography (ERCP) in children during the past 2 decades. STUDY DESIGN: A systematic literature search of MEDLINE, Embase, and Web of Science from January 1995 to January 2016 was conducted for observational studies published in English. Studies reporting ERCP complications in patients <21 years without history of liver transplant or cholecystectomy were included...
December 2016: Journal of Pediatrics
https://www.readbyqxmd.com/read/27566421/progress-in-pediatrics-in-2015-choices-in-allergy-endocrinology-gastroenterology-genetics-haematology-infectious-diseases-neonatology-nephrology-neurology-nutrition-oncology-and-pulmonology
#12
EDITORIAL
Carlo Caffarelli, Francesca Santamaria, Dora Di Mauro, Carla Mastrorilli, Virginia Mirra, Sergio Bernasconi
This review focuses key advances in different pediatric fields that were published in Italian Journal of Pediatrics and in international journals in 2015. Weaning studies continue to show promise for preventing food allergy. New diagnostic tools are available for identifying the allergic origin of allergic-like symptoms. Advances have been reported in obesity, short stature and autoimmune endocrine disorders. New molecules are offered to reduce weight gain and insulin-resistance in obese children. Regional investigations may provide suggestions for preventing short stature...
August 27, 2016: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/27557426/sertraline-as-an-additional-treatment-for-cholestatic-pruritus-in-children
#13
Alice Thébaut, Dalila Habes, Frédéric Gottrand, Christine Rivet, Joseph Cohen, Dominique Debray, Emmanuel Jacquemin, Emmanuel Gonzales
BACKGROUNDS/AIM: Pruritus is a severe symptom accompanying chronic cholestasis. It can be debilitating and difficult to control. In children, first-line treatments are ursodeoxycholic acid and rifampicin. Refractory pruritus may require invasive therapies including liver transplantation. Clinical trials based on small samples of adult patients suggest that serotonin reuptake inhibitors can improve pruritus in cholestatic or uremic disease. We performed a prospective, multicenter study to assess efficiency and safety of the serotonin reuptake inhibitor sertraline in treating children with refractory cholestatic pruritus...
August 24, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27540711/hepatic-inflammation-may-influence-liver-stiffness-measurements-by-transient-elastography-in-children-and-young-adults
#14
Aileen Raizner, Nick Shillingford, Paul D Mitchell, Sarah Harney, Roshan Raza, Jessica Serino, Maureen M Jonas, Christine K Lee
OBJECTIVES: Transient elastography (TE) measures liver stiffness to assess fibrosis. Studies in adults have shown that inflammation increases stiffness, leading to an overestimation of fibrosis. We investigated the contribution of inflammation to liver stiffness measurements (LSM) in children/ young adults. METHODS: This was a cohort analysis of children/ young adults who underwent TE within 1 year of liver biopsy. ALT was obtained within 30d of the biopsy and LSM...
August 18, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27540004/potential-hepatotoxicities-of-intravenous-fat-emulsions-in-infants-and-children
#15
REVIEW
T Hang Nghiem-Rao
Infants and children who depend on parenteral nutrition are among the most vulnerable to developing potentially devastating intestinal failure-associated liver disease. While the pathogenesis of intestinal failure-associated liver disease remains unclear, evidence for the contribution of fat emulsions to cholestasis and liver injury has rapidly increased in recent years. Data demonstrating the interaction among phytosterols, fatty acids, and antioxidants in cellular pathways that mediate bile flow and hepatic injury have led to the development of newer alternative fat emulsions...
October 2016: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/27532546/myo5b-mutations-cause-cholestasis-with-normal-serum-gamma-glutamyl-transferase-activity-in-children-without-microvillous-inclusion-disease
#16
Emmanuel Gonzales, Sarah A Taylor, Anne Davit-Spraul, Alice Thébaut, Nadège Thomassin, Catherine Guettier, Peter F Whitington, Emmanuel Jacquemin
: Some patients with microvillus inclusion disease due to myosin 5B (MYO5B) mutations may develop cholestasis characterized by a progressive familial intrahepatic cholestasis-like phenotype with normal serum gamma-glutamyl transferase activity. So far MYO5B deficiency has not been reported in patients with such a cholestasis phenotype in the absence of intestinal disease. Using a new-generation sequencing approach, we identified MYO5B mutations in five patients with progressive familial intrahepatic cholestasis-like phenotype with normal serum gamma-glutamyl transferase activity without intestinal disease...
August 17, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/27510535/predictors-of-failure-of-fish-oil-therapy-for-intestinal-failure-associated-liver-disease-in-children
#17
Prathima Nandivada, Meredith A Baker, Paul D Mitchell, Alison A O'Loughlin, Alexis K Potemkin, Lorenzo Anez-Bustillos, Sarah J Carlson, Duy T Dao, Gillian L Fell, Kathleen M Gura, Mark Puder
BACKGROUND: Parenteral fish-oil (FO) therapy is a safe and effective treatment for intestinal failure-associated liver disease (IFALD). Patients whose cholestasis does not resolve with FO may progress to end-stage liver disease. OBJECTIVE: We sought to identify factors associated with the failure of FO therapy in treating IFALD to guide prognostication and referral guidelines. DESIGN: Prospectively collected data for patients treated with FO at Boston Children's Hospital from 2004 to 2014 were retrospectively reviewed...
September 2016: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/27499927/increased-mmp-7-expression-in-biliary-epithelium-and-serum-underpins-native-liver-fibrosis-after-successful-portoenterostomy-in-biliary-atresia
#18
Anna Kerola, Hanna Lampela, Jouko Lohi, Päivi Heikkilä, Annika Mutanen, Jaana Hagström, Taina Tervahartiala, Timo Sorsa, Caj Haglund, Hannu Jalanko, Mikko P Pakarinen
The molecular mechanisms underlying progressive liver fibrosis following surgical treatment of biliary atresia (BA) remain unclear. Our aim was to address hepatic gene and protein expression and serum levels of matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs) after successful portoenterostomy (PE), and relate them to histological signs of liver injury, clinical follow-up data and biochemical markers of hepatic function. LIver biopsies and serum samples were obtained from 25 children after successful PE at median age of 3...
July 2016: Journal of Pathology. Clinical Research
https://www.readbyqxmd.com/read/27484488/higher-doses-of-fish-oil-based-lipid-emulsions-used-to-treat-inadequate-weight-gain-and-rising-triene-tetraene-ratio-in-a-severely-malnourished-infant-with-intestinal-failure-associated-liver-disease
#19
Mary Riedy, Brittany DePaula, Mark Puder, Kathleen M Gura, Kevin A Sztam
Fish oil-based lipid emulsions (FOLEs) have been used to treat cholestasis in children with intestinal failure-associated liver disease (IFALD). When FOLEs are dosed at 1 g/kg/d, essential fatty acid (EFA) deficiency typically does not occur. We describe the clinical course of a severely malnourished parenteral nutrition-dependent infant with IFALD. Baseline EFA panels were normal upon starting FOLE at 1 g/kg/d. Despite biochemical improvement in IFALD, weight velocity was below target and biochemical EFA status worsened, even after correction for other factors affecting weight...
August 1, 2016: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/27429423/oral-tocofersolan-corrects-or-prevents-vitamin-e-deficiency-in-children-with-chronic-cholestasis
#20
Alice Thébaut, Antal Nemeth, Jeannie Le Mouhaër, René Scheenstra, Ulrich Baumann, Bart Koot, Fredéric Gottrand, Roderick Houwen, Laure Monard, Sylvie Lafaye de Micheaux, Dalila Habes, Emmanuel Jacquemin
OBJECTIVES: D-Alpha-tocopheryl polyethylene glycol 1000 succinate (Tocofersolan, Vedrop), has been developed in Europe to provide an orally bioavailable source of vitamin E in children with cholestasis. The aim was to analyze the safety/efficacy of Vedrop in a large group of children with chronic cholestasis. METHODS: Two hundred seventy-four children receiving Vedrop for vitamin E deficiency or for its prophylaxis were included from 7 European centers. Median age at treatment onset was 2 months and median follow-up was 11 months...
December 2016: Journal of Pediatric Gastroenterology and Nutrition
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