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Cholestasis children

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...
October 21, 2016: American Journal of Surgical Pathology
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
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
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
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...
September 20, 2016: Journal of Pediatrics
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
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
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
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
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
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
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
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
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
BACKGROUND/AIMS: 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: 274 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...
July 16, 2016: Journal of Pediatric Gastroenterology and Nutrition
Aysel Ünlüsoy Aksu, Sinan Sarı, Güldal Yılmaz, Ödül Eğritaş Gürkan, Zeliha Demirtaş, Buket Dalgıç
To assess the relationship between aspartate aminotransferase-platelet ratio index (APRI) and liver fibrosis in children with chronic hepatocellular and biliary cholestatic liver diseases. A retrospective review of one hundred children's demographic and laboratory findings concurrent with liver biopsy, who were followed-up with prolonged cholestasis. The diagnostic accuracy of the APRI was assessed by receiver operating characteristic (ROC) curves. Advanced fibrosis was more common in the hepatocellular group...
September 2015: Turkish Journal of Pediatrics
Ashley Mehl, Humberto Bohorquez, Maria-Stella Serrano, Gretchen Galliano, Trevor W Reichman
Progressive familial intrahepatic cholestasis (PFIC) is a constellation of inherited disorders that result in the impairment of bile flow through the liver that predominantly affects children. The accumulation of bile results in progressive liver damage, and if left untreated leads to end stage liver disease and death. Patients often present with worsening jaundice and pruritis within the first few years of life. Many of these patients will progress to end stage liver disease and require liver transplantation...
June 24, 2016: World Journal of Transplantation
Simon Kargl, Roland Gitter, Wolfgang Pumberger
BACKGROUND: An association of heart disease and its treatment with biliary calculi is popularly accepted. We sought determine the prevalence and risk factors of paediatric gallstone disease in the presence of CHD and analyse the treatment options. We evaluated the role of open-heart surgery in the development of gallstones in patients with CHD. Patients and methods In a 10-year, retrospective, chart review (2005-2014), patients with CHD and cholelithiasis were identified and reviewed...
June 20, 2016: Cardiology in the Young
M L Gao, X M Zhong, X Ma, H J Ning, D Zhu, J Z Zou
OBJECTIVE: To make genetic diagnosis of Alagille syndrome (ALGS) patients using target gene sequence capture and next generation sequencing technology. METHOD: Target gene sequence capture and next generation sequencing were used to detect ALGS gene of 4 patients. They were hospitalized at the Affiliated Hospital, Capital Institute of Pediatrics between January 2014 and December 2015, referred to clinical diagnosis of ALGS typical and atypical respectively in 2 cases...
June 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
Dilek Yılmaz-Çiftdoğan, Engin Köse, Selda Aslan, Efgan Gayyurhan
Hepatitis A virus (HAV) infection has several atypical manifestations, including fulminant hepatitis, relapsing hepatitis, prolonged cholestasis and extrahepatic manifestations. In this study, we aimed to describe the characteristics and outcomes of the pediatric patients with atypical manifestations of HAV infection. Four hundred twelve children with symptomatic hepatitis A infection admitted to Gaziantep Children's Hospital Department of Pediatric Infectious Disease between August 2011 and January 2013 were analyzed retrospectively...
July 2015: Turkish Journal of Pediatrics
Mehmet Hanifi Okur, Yücel Yankol, Nesimi Mecit, Gültekin Hoş, Gökhan Ertugrul, Turan Kanmaz, Koray Acarli, Münci Kalayoglu
OBJECTIVES: Diaphragmatic hernia is a rare complication after pediatric liver transplant. This report presents occurrences of diaphragmatic hernia after living-donor liver transplants in 2 children. MATERIALS AND METHODS: In 1 of the 2 patients, a right-sided diaphragmatic hernia developed after a living-donor liver transplant due to progressive familial intrahepatic cholestasis where a left lateral segment graft was used. In the other patient, a left-sided diaphragmatic hernia developed after a living-donor liver transplant due to biliary atresia following Kasai portoenterostomy where a left lateral segment graft was used...
April 20, 2016: Experimental and Clinical Transplantation
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