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Pediatric cholestasis

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https://www.readbyqxmd.com/read/28027587/analysis-of-surgical-interruption-of-the-enterohepatic-circulation-as-a-treatment-for-pediatric-cholestasis
#1
Kasper S Wang, Greg Tiao, Lee M Bass, Paula M Hertel, Douglas Mogul, Nanda Kerkar, Matthew Clifton, Colleen Azen, Laura Bull, Philip Rosenthal, Dylan Stewart, Riccardo Superina, Ronen Arnon, Molly Bozic, Mary L Brandt, Patrick A Dillon, Annie Fecteau, Kishore Iyer, Binita Kamath, Saul Karpen, Frederick Karrer, Kathleen M Loomes, Cara Mack, Peter Mattei, Alexander Miethke, Kyle Soltys, Yumirle P Turmelle, Karen West, Jessica Zagory, Cat Goodhue, Benjamin L Shneider
: To evaluate the efficacy of non-transplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 Alagille syndrome (ALGS), 16 Familial Intrahepatic Cholestasis-1 (FIC1), 18 Bile Salt Export Pump (BSEP) disease, & 4 others with low γ-glutamyl transpeptidase disease (levels <100 U/L), were identified across 14 Childhood Liver Disease Research Network (ChiLDReN) centers. Data were collected retrospectively from individuals who collectively had 39 partial external biliary diversions (PEBD), 11 ileal exclusions (IE) and 7 gallbladder-to-colon diversion (GBC)...
December 27, 2016: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28003725/neonatal-cholestasis-as-initial-presentation-of-portosystemic-shunt-a-case-report
#2
Güzide Doğan, Fatih Düzgün, Serdar Tarhan, Yeliz Çağan Appak, Erhun Kasırga
Congenital intrahepatic portosystemic shunts are rare in children. Portosystemic venous malformations are characterized by extreme clinical variability. We report a full-term 33-day-old male infant presenting with neonatal jaundice. On physical examination, he had generalized icterus and the liver was palpable 3.5 cm below the right costal margin. He had no other symptoms. Laboratory tests showed AST 632 U/L, ALT 198 U/L, total bilirubin 12.1 mg/dL, conjugated bilirubin 10.2 mg/dL, alkaline phosphatase 753 U/L, GGT 47 U/L and glucose 67 U/L...
December 2016: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/27975109/cholestasis-in-infancy
#3
Melissa G Andrianov, Ruba K Azzam
Jaundice is a key manifestation of hepatobiliary disease in all age groups. Jaundice is a common finding in the first 2 weeks after birth, occurring in 2.4% to 15% of newborns. The neonatal liver is at increased susceptibility to cholestasis, with an incidence ranging from 1 in 2,500 to 1 in 5,000 live births. Etiologies vary, but the most common is biliary atresia. In 2004, the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition published guidelines for the evaluation of cholestasis that clearly stated any infant with jaundice persisting beyond age 2 weeks (3 weeks in breast-fed infants with an otherwise normal history and physical examination) should be evaluated with a fractionated serum bilirubin level...
December 1, 2016: Pediatric Annals
https://www.readbyqxmd.com/read/27890981/neonatal-cholestasis-single-centre-experience-in-central-india
#4
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/27882046/the-role-of-genetic-and-immune-factors-for-the-pathogenesis-of-primary-sclerosing-cholangitis-in-childhood
#5
REVIEW
Priscila Menezes Ferri, Ana Cristina Simões E Silva, Soraya Luiza Campos Silva, Diego Junior Queiroga de Aquino, Eleonora Druve Tavares Fagundes, Débora Marques de Miranda, Alexandre Rodrigues Ferreira
Primary sclerosing cholangitis (PSC) is a rare cholestatic liver disease characterized by chronic inflammation of the biliary tree resulting in liver fibrosis. PSC is more common in male less than 40 years of age. The diagnosis of PSC is based on clinical, laboratory, image, and histological findings. A biochemical profile of mild to severe chronic cholestasis can be observed. Endoscopic retrograde cholangiography is the golden standard method for diagnosis, but magnetic resonance cholangiography is currently also considered a first-line method of investigation...
2016: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/27774663/liver-and-serum-expression-of-matrix-metalloproteinases-in-asymptomatic-pediatric-liver-transplant-recipients
#6
Silja H Voutilainen, Silja K Kosola, Taina I Tervahartiala, Timo A Sorsa, Hannu J Jalanko, Mikko P Pakarinen
We related hepatic gene and serum expression of matrix metalloproteinases (MMP) and their tissue inhibitors (TIMP) to liver histology in pediatric LT recipients. Liver biopsies and serum samples were obtained from 52 patients 10.6 years post-LT and age-matched controls for analyses of MMPs and TIMPs. Patients with fibrosis had significantly higher hepatic gene expression of MMP-2, MMP-9, MMP-14, TIMP-1, and TIMP-2 than patients without. Expression of these genes correlated with graft Metavir fibrosis stage (r = 0...
October 23, 2016: Transplant International: Official Journal of the European Society for Organ Transplantation
https://www.readbyqxmd.com/read/27663215/complications-of-endoscopic-retrograde-cholangiopancreatography-in-pediatric-patients-a-systematic-literature-review-and-meta-analysis
#7
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
#8
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/27510535/predictors-of-failure-of-fish-oil-therapy-for-intestinal-failure-associated-liver-disease-in-children
#9
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/27429428/guideline-for-the-evaluation-of-cholestatic-jaundice-in-infants-joint-recommendations-of-the-north-american-society-for-pediatric-gastroenterology-hepatology-and-nutrition-and-the-european-society-for-pediatric-gastroenterology-hepatology-and-nutrition
#10
Rima Fawaz, Ulrich Baumann, Udeme Ekong, Björn Fischler, Nedim Hadzic, Cara L Mack, Valérie A McLin, Jean P Molleston, Ezequiel Neimark, Vicky L Ng, Saul J Karpen
Cholestatic jaundice in infancy affects approximately 1 in every 2500 term infants and is infrequently recognized by primary providers in the setting of physiologic jaundice. Cholestatic jaundice is always pathologic and indicates hepatobiliary dysfunction. Early detection by the primary care physician and timely referrals to the pediatric gastroenterologist/hepatologist are important contributors to optimal treatment and prognosis. The most common causes of cholestatic jaundice in the first months of life are biliary atresia (25%-40%) followed by an expanding list of monogenic disorders (25%), along with many unknown or multifactorial (eg, parenteral nutrition-related) causes, each of which may have time-sensitive and distinct treatment plans...
January 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27405544/molecular-diagnosis-of-pediatric-patients-with-citrin-deficiency-in-china-slc25a13-mutation-spectrum-and-the-geographic-distribution
#11
Wei-Xia Lin, Han-Shi Zeng, Zhan-Hui Zhang, Man Mao, Qi-Qi Zheng, Shu-Tao Zhao, Ying Cheng, Feng-Ping Chen, Wang-Rong Wen, Yuan-Zong Song
Citrin deficiency (CD) is a Mendelian disease due to biallelic mutations of SLC25A13 gene. Neonatal intrahepatic cholestasis caused by citrin deficiency (NICCD) is the major pediatric CD phenotype, and its definite diagnosis relies on SLC25A13 genetic analysis. China is a vast country with a huge population, but the SLC25A13 genotypic features of CD patients in our country remains far from being well clarified. Via sophisticated molecular analysis, this study diagnosed 154 new CD patients in mainland China and identified 9 novel deleterious SLC25A13 mutations, i...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27256232/-target-gene-sequence-capture-and-next-generation-sequencing-technology-to-diagnose-four-children-with-alagille-syndrome
#12
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
https://www.readbyqxmd.com/read/27186695/atypical-clinical-manifestations-of-hepatitis-a-among-children-aged-1-16-years-in-south-eastern-region-of-turkey
#13
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
https://www.readbyqxmd.com/read/27154793/severe-direct-hyperbilirubinemia-as-a-consequence-of-right-heart-failure-in-congenital-heart-disease
#14
Joseph Philip, Ravi S Samraj, Dalia Lopez-Colon, Regino Gonzalez-Peralta, Arun Chandran, Mark S Bleiwies
Adult literature documents increased cholestasis in right heart failure yet is poorly documented in the pediatric population. We describe three infants with congenital heart disease who developed significantly elevated direct bilirubin levels of 43, 23, and 12 mg/dL, respectively, in the absence of hepatic dysfunction. The common hemodynamic pathophysiology in these infants is right heart dysfunction with moderate to severe tricuspid regurgitation in the setting of low perfusion state. Right heart dysfunction in infants can result in severe conjugated bilirubin, likely as a consequence of venous congestion and can be used as an indirect marker of right heart dynamics...
May 6, 2016: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/27143191/diaphragmatic-hernia-after-liver-transplant-in-children-report-of-2-cases
#15
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
https://www.readbyqxmd.com/read/27083757/the-prevalence-of-congenital-heart-defects-in-infants-with-cholestatic-disorders-of-infancy-a-single-centre-study
#16
Aya M Fattouh, Engy A Mogahed, Nehal Abdel Hamid, Rodina Sobhy, Noha Saber, Hanaa El-Karaksy
BACKGROUND: There is deficiency of data about congenital heart defects (CHDs) in cholestatic disorders of infancy other than Alagille syndrome (AGS). We aimed to define the prevalence and types of CHDs in infants with various causes of cholestatic disorders of infancy. METHODS: This cross-sectional study was conducted on 139 infants presenting with cholestasis whether surgical or non-surgical. The study was carried out at the Pediatric Hepatology Unit, Cairo University Children's Hospital, Egypt...
September 2016: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/27066444/cholestasis-beyond-the-neonatal-and-infancy-periods
#17
REVIEW
Racha Khalaf, Claudia Phen, Sara Karjoo, Michael Wilsey
Cholestasis results from impairment in the excretion of bile, which may be due to mechanical obstruction of bile flow or impairment of excretion of bile components into the bile canaliculus. When present, cholestasis warrants prompt diagnosis and treatment. The differential diagnosis of cholestasis beyond the neonatal period is broad and includes congenital and acquired etiologies. It is imperative that the clinician differentiates between intrahepatic and extrahepatic origin of cholestasis. Treatment may be supportive or curative and depends on the etiology...
March 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/27003131/expression-of-fibroblast-growth-factor-21-in-patients-with-biliary-atresia
#18
Dawei Li, Tianfei Lu, Conghuan Shen, Yuan Liu, Jiang Zhang, Yuhua Shan, Yi Luo, Zhifeng Xi, Bijun Qiu, Qimin Chen, Jianjun Zhang, Qiang Xia
Fibroblast growth factor 21 is a critical circulating adipokine involving in metabolic disorders and various liver diseases. This study was performed to investigate whether FGF21 is also associated with the pathophysiology of biliary atresia. Serum FGF21 levels were measured in 57 BA patients and 20 age matched healthy controls. We also examined hepatic FGF21 mRNA expression and FGF21 protein levels in liver tissues obtained from 15 BA patients undergoing liver transplantation and 5 cases of pediatric donation after cardiac death donor without liver diseases by RT-PCR and Western blotting...
July 2016: Cytokine
https://www.readbyqxmd.com/read/26992434/hypoxia-inducible-factor-2alpha-promotes-hepatocyte-apoptosis-during-cholestasis
#19
Xiangwei Hua, Tianfei Lu, Jiang Zhang, Qi Miao, Zhaolian Bian, Haiyan Zhang, Shanshan Huang, Weiwei Lin, Zhifeng Xi, Ming Zhang, Qimin Chen, Xiong Ma, Jianjun Zhang, Qiang Xia
AIM: Hypoxia-inducible factor-2alpha(HIF-2α) has been reported to play an important role in a host of pathophysiological processes including cellular survival. The current work is to explore the role of HIF-2α in cholestasis-mediated hepatocyte apoptosis. METHODS: HIF-2α expression was measured by immunohistochemistry and confocal microscopy. Hepatic apoptosis was detected by terminal deoxynucleotidyl transferase-mediated dUTP-digoxigenin nick-end labeling (TUNEL)...
March 18, 2016: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/26959834/increased-pr-interval-in-fetuses-of-patients-with-intrahepatic-cholestasis-of-pregnancy
#20
Marcelo Rodríguez, Jose Moreno, Rolando Márquez, Ricardo Eltit, Felipe Martinez, Alvaro Sepúlveda-Martínez, Mauro Parra-Cordero
OBJECTIVE: To evaluate the fetal mechanical PR interval in fetuses from pregnancies with intrahepatic cholestasis of pregnancy (ICP). METHODS: A case-control study was conducted in the Maternal-Fetal Medicine Unit at Hospital Carlos Van Buren between 2011 and 2013. Fetal echocardiography was performed in patients with ICP and normal pregnancies. Demographic and clinical characteristics were compared using the Mann-Whitney U test for continuous variables. A p value <0...
2016: Fetal Diagnosis and Therapy
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