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Cholestasis in children

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https://www.readbyqxmd.com/read/28733223/sequencing-of-fic1-bsep-and-mdr3-in-a-large-cohort-of-patients-with-cholestasis-revealed-a-high-number-of-different-genetic-variants
#1
Carola Dröge, Michele Bonus, Ulrich Baumann, Caroline Klindt, Elke Lainka, Simone Kathemann, Florian Brinkert, Enke Grabhorn, Eva-Doreen Pfister, Daniel Wenning, Alexander Fichtner, Daniel N Gotthardt, Karl Heinz Weiss, Patrick McKiernan, Ratna Dua Puri, I C Verma, Stefanie Kluge, Holger Gohlke, Lutz Schmitt, Ralf Kubitz, Dieter Häussinger, Verena Keitel
BACKGROUND&AIMS: The bile salt export pump (BSEP, ABCB11), multidrug resistance protein 3 (MDR3, ABCB4) and the ATPase familial intrahepatic cholestasis 1 (FIC1, ATP8B1) mediate bile formation. Mutations in these genes underlie cholestatic liver diseases ranging from intrahepatic cholestasis of pregnancy, benign recurrent intrahepatic cholestasis or low phospholipid-associated cholelithiasis to progressive familial intrahepatic cholestasis. METHODS: Coding exons with flanking intron regions of ATP8B1, ABCB11, and ABCB4 were sequenced in cholestatic patients with assumed genetic cause...
July 18, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28731841/survey-of-nutrition-management-practices-in-centers-for-pediatric-intestinal-rehabilitation
#2
Anita M Nucci, Kipp Ellsworth, Austin Michalski, Emily Nagel, Jackie Wessel
BACKGROUND: Nutrition management of pediatric intestinal failure (IF) requires interdisciplinary coordination of parenteral nutrition (PN) and enteral nutrition (EN) support. Nutrition strategies used by specialists in pediatric intestinal rehabilitation to promote gut adaptation and manage complications have not been previously summarized. METHODS: A practice survey was distributed to members of the dietitian subgroup of the American Society for Parenteral and Enteral Nutrition Pediatric Intestinal Failure Section...
July 1, 2017: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/28712532/influence-of-different-intravenous-lipid-emulsions-on-growth-development-and-laboratory-and-clinical-outcomes-in-hospitalised-paediatric-patients-a-systematic-review
#3
REVIEW
Roy-Rosshan Edward, Jacqueline K Innes, Luise V Marino, Philip C Calder
BACKGROUND & AIMS: Fats in the form of lipid emulsions (LEs) are an integral part of intravenous nutrition. The fatty acid composition of different LEs varies. The exact composition of a LE may influence cell and tissue function and clinical outcome. Currently, it is not clear which LE might be best for paediatric patients. We conducted a systematic review of the effects of different intravenous LEs in hospitalised paediatric patients. METHODS: Randomised controlled trials published in a peer reviewed journal, written in the English language, and comparing two or more different intravenous LEs in hospitalised paediatric patients were included...
July 8, 2017: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28710680/development-of-a-novel-tool-to-assess-the-impact-of-itching-in-pediatric-cholestasis
#4
Binita M Kamath, Linda Abetz-Webb, Ciara Kennedy, Bonnie Hepburn, Martha Gauthier, Nathan Johnson, Sharon Medendorp, Alejandro Dorenbaum, Lora Todorova, Benjamin L Shneider
OBJECTIVES: The aim was to develop a clinical outcome assessment (COA) for itching in children with cholestatic pruritus. METHODS: This prospective study aimed to enroll patients aged 4-30 years with Alagille syndrome (ALGS) or progressive familial intrahepatic cholestasis type 1 and caregivers of patients aged 5 months to 14 years. Eligible patients experienced itching during ≥3 of the 7 days before enrollment and had not undergone liver transplant or surgical interruption of the enterohepatic circulation...
July 14, 2017: Patient
https://www.readbyqxmd.com/read/28699603/infants-with-extrahepatic-biliary-atresia-effect-of-follow-up-on-the-survival-rate-at-ege-university-medical-school-transplantation-center
#5
Miray Karakoyun, Maşallah Baran, Caner Turan, Murat Kılıç, Orkan Ergun, Sema Aydoğdu
BACKGROUND/AIMS: Biliary atresia (BA) is the main cause of neonatal cholestasis and the primary reason for infant liver transplants worldwide. It is an obliterative cholangiopathy observed only in children and caused by progressive inflammation and fibrosis of the bile duct. We collaborated with a liver transplantation center to investigate the effects of follow-up in patients with BA. MATERIALS AND METHODS: Medical records of 99 patients who were diagnosed with BA and monitored at our center from 1990 to 2002 (27 patients) and from 2003 to 2015 (72 patients) were analyzed retrospectively...
July 2017: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/28689603/small-bowel-dilation-in-children-with-short-bowel-syndrome-is-associated-with-mucosal-damage-bowel-derived-bloodstream-infections-and-hepatic-injury
#6
Maria Hukkinen, Annika Mutanen, Mikko P Pakarinen
BACKGROUND: Liver disease occurs frequently in short bowel syndrome. Whether small bowel dilation in short bowel syndrome could influence the risk of liver injury through increased bacterial translocation remains unknown. Our aim was to analyze associations between small bowel dilation, mucosal damage, bloodstream infections, and liver injury in short bowel syndrome patients. METHODS: Among short bowel syndrome children (n = 50), maximal small bowel diameter was measured in contrast series and expressed as the ratio to the height of the fifth lumbar vertebra (small bowel diameter ratio), and correlated retrospectively to fecal calprotectin and plasma citrulline-respective markers of mucosal inflammation and mass-bloodstream infections, liver biochemistry, and liver histology...
July 6, 2017: Surgery
https://www.readbyqxmd.com/read/28688656/notch-signaling-promotes-ductular-reactions-in-biliary-atresia
#7
Jessica A Zagory, William Dietz, Alex Park, Michael Fenlon, Jiabo Xu, Sarah Utley, Nirmala Mavila, Kasper S Wang
BACKGROUND: Biliary atresia (BA) is a congenital, progressive, fibro-obliterative disease of the extrahepatic biliary tree and the most common cause of end-stage liver disease in children. BA is characterized by extensive intrahepatic proliferating ductular reactions that may contribute to biliary fibrosis. Lineage tracing during experimental cholestasis indicates that cells within ductular reactions derive from PROM1-expressing hepatic progenitor cells. Given the role of Notch signaling in normal biliary development, we hypothesize that activated Notch signaling promotes the formation of ductular reactions in BA...
July 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/28685316/-liver-biopsy-in-children-and-adolescents-preliminary-morphological-examinations-in-diffuse-liver-disease
#8
REVIEW
C Lackner, A S Knisely
BACKGROUND: Frequencies of types of liver disease differ between adults and nonadults (NA). OBJECTIVES: The particular problems encountered in interpreting liver-biopsy findings in NA often require referral in consultation.  To permit this efficiently, we recommend specific approaches to light microscopy, with special stains and immunohistochemistry, as well as to ultrastructural study. METHODS: Prosection and the choice of special stains are described, and are discussed in relation to clinical questions...
July 2017: Der Pathologe
https://www.readbyqxmd.com/read/28655415/molecular-signature-of-active-fibrogenesis-prevails-in-biliary-atresia-after-successful-portoenterostomy
#9
Anna Kerola, Hanna Lampela, Jouko Lohi, Päivi Heikkilä, Annika Mutanen, Hannu Jalanko, Mikko P Pakarinen
BACKGROUND: In biliary atresia mechanisms of progressive liver injury leading to need of liver transplantation after successful portoenterostomy remain unknown. A better understanding is a prerequisite for development of novel therapies to extend native liver survival, and we aimed to unravel molecular characteristics of liver injury after successful portoenterostomy. METHODS: Liver biopsies obtained from 28 biliary atresia children during successful portoenterostomy and at median age 3...
June 24, 2017: Surgery
https://www.readbyqxmd.com/read/28652893/low-bone-mineral-density-and-the-severity-of-cholestasis-in-biliary-atresia
#10
Krittapak Homchan, Tawatchai Chaiwatanarat, Wanvisa Udomsinprasert, Voranush Chongsrisawat, Yong Poovorawan, Sittisak Honsawek
AIM: To investigate the prevalence of osteopenia and osteoporosis in postoperative biliary atresia (BA) children and the association of bone mineral density (BMD) and biochemical parameters in postKasai BA subjects. METHODS: A total of 70 patients with postKasai BA were enrolled in this prospective study. The patients were classified into two groups according to their jaundice status. BMD of the lumbar spine was analyzed using dual energy X-ray absorptiometry. RESULTS: The prevalence of low bone mass (osteopenia and osteoporosis) in BA patients were 51...
June 8, 2017: World Journal of Hepatology
https://www.readbyqxmd.com/read/28649563/symptomatic-congenital-cytomegalovirus-infection-in-children-of-seropositive-women
#11
Ines Mack, Marie-Anne Burckhardt, Ulrich Heininger, Friederike Prüfer, Sven Schulzke, Sven Wellmann
Cytomegalovirus (CMV) is the most frequent congenital virus infection worldwide. The risk of congenital CMV (cCMV) transmission is highest in seronegative women who acquire primary CMV infection during pregnancy. A growing body of evidence indicates that secondary CMV infections in pregnant women with preconceptual immunity (either through reactivation of latent virus or re-infection with a new strain of CMV) contribute to a much greater proportion of symptomatic cCMV than was previously thought. Here, we describe a case of symptomatic cCMV infection in the newborn of a woman with proven immunity prior to pregnancy...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28644566/clinical-characteristics-and-outcome-of-liver-transplantation-for-alagille-syndrome-in-children
#12
Tao Zhou, Jianjun Zhang, Yi Luo, Yuan Liu, Sy Zhuang, Feng Xue, Longzhi Han, Qiang Xia
AIM: The aim of this study was to analyze clinical characteristics and outcome of liver transplantation for Alagille syndrome in children. METHOD: By retrospectively reviewing the medical records of 9 Alagille syndrome patients(AGS) with liver transplantation(LT) in Renji Hospital between 2006 and 2015,After operative,the height and weight Z score were compared with preoperative. RESULT: There were 9 patients included in the study; cholestasis and peculiar faces were seen in all of the patients (100%), heart defect in 8 patients (88...
June 23, 2017: Journal of Digestive Diseases
https://www.readbyqxmd.com/read/28603204/a-6-year-old-girl-diagnosed-with-mevalonate-kinase-deficiency-who-had-hydrops-fetalis-and-neonatal-onset-cholestasis
#13
Yuriko Yamashita, Shinsuke Matsumoto, Ryugo Hiramoto, Isao Komori, Takayuki Tanaka, Ryuta Nishikomori, Toshio Heike, Shuichiro Umetsu, Ayano Inui
  We experienced a 6-year-old girl diagnosed with mevalonate kinase deficiency (MKD) who had cholestasis, anemia, and elevated inflammatory markers in neonatal period. She was admitted to our hospital because of fever and elevated inflammatory markers at 5 years 11months of age. Without using antibiotics, the fever and the inflammatory markers were spontaneously resolved. MKD was suspected from elevated serum IgD level and the recurrent febrile attacks. The genetic test revealed heterozygous mutation of p...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28583778/-physiopathology-and-management-of-cholestatic-pruritus-in-children
#14
A Thébaut, D Debray, E Gonzales
Pruritus is a disabling symptom accompanying chronic cholestasis. In some cases, refractory pruritus may require invasive therapies including liver transplantation. The pathogenesis of pruritus in cholestatic disease is poorly understood. It may involve a specific neural pathway and several pruritogenic substances such as bile acids, opioids, serotonin, and the more recently identified lysophosphatidic acid. While the therapeutic management of cholestatic pruritus is well established in adult patients, there is no consensus in children, given the difficulty in conducting controlled clinical studies...
June 2, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28521607/intravenous-fish-oil-and-pediatric-intestinal-failure-associated-liver-disease-changes-in-plasma-phytosterols-cytokines-and-bile-acids-and-erythrocyte-fatty-acids
#15
Kara L Calkins, Andrea DeBarber, Robert D Steiner, Martiniano J Flores, Tristan R Grogan, Susanne M Henning, Laurie Reyen, Robert S Venick
BACKGROUND: Soybean oil (SO) emulsions are associated with intestinal failure-associated liver disease (IFALD); fish oil (FO) emulsions are used to treat IFALD. SO and FO differ with respect to their fatty acid and phytosterol content. In children with IFALD whose SO was replaced with FO, we aimed to (1) quantify changes in erythrocyte fatty acids and plasma phytosterols, cytokines, and bile acids and (2) correlate these changes with direct bilirubin (DB). DESIGN: This study enrolled IFALD children who received 6 months of FO...
May 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28452736/-action-of-l-carnitine-corvitin-and-their-combination-on-functional-state-of-liver-in-experimental-model-of-reye-syndrome-in-rats
#16
M Ghonghadze, N Antelava, K Liluashvili, M Okujava, K Pachkoria
Administration of Aacetylsalicylic acid in children with viral infections (influence B, chickenpox) can be related with development of Reye syndrome - severe encephalopathy and liver insufficiency with mortality in 50% of cases. During Reye syndrome most important is deficiency of carnitine and hepatocyte damage. Decreased amount of carnitine impairs the energy function of mitochondria and gluconeogenesis as well as production of urea. As a result develops toxic encephalopathy and liver insufficiency. The goal of the research was assessment of efficacy of L-Carnitine, Corvitin and their combination on functional state of liver in experimental model of Reye Syndrome in rats...
February 2017: Georgian Medical News
https://www.readbyqxmd.com/read/28426902/hepatic-steatosis-after-pediatric-liver-transplant
#17
Emily R Perito, Tabitha Vase, Rageshree Ramachandran, Andrew Phelps, Kuang-Yu Jen, Robert H Lustig, Vickie A Feldstein, Philip Rosenthal
Hepatic steatosis develops after liver transplantation (LT) in 30% of adults, and nonalcoholic fatty liver disease (NAFLD) is the most common chronic liver disease in nontransplanted children. However, posttransplant steatosis has been minimally studied in pediatric LT recipients. We explored the prevalence, persistence, and association with chronic liver damage of hepatic steatosis in these children. In this single-center study of pediatric patients transplanted 1988-2015 (n = 318), 31% of those with any posttransplant biopsy (n = 271) had ≥ 1 biopsy with steatosis...
July 2017: Liver Transplantation
https://www.readbyqxmd.com/read/28377061/respiratory-gated-bilateral-pulmonary-radiotherapy-for-ewing-s-sarcoma-and-nephroblastoma-in-children-and-young-adults-dosimetric-and-clinical-feasibility-studies
#18
C Demoor-Goldschmidt, S Chiavassa, S Josset, M-A Mahé, S Supiot
PURPOSE: Bilateral pulmonary radiotherapy in children and young adults aims to reduce the recurrence of lung metastases. The radiation field includes liver tissue, which is sensitive to even low radiation doses. We investigated the feasibility of respiratory gating radiotherapy using voluntary deep inspiration breath hold and its toxicity in these patients. PATIENTS AND METHOD: A retrospective clinical review was conducted for all patients who had undergone bilateral pulmonary radiotherapy, with or without deep inspiration breath hold, treated in our institution between October 1999 and May 2012...
April 2017: Cancer Radiothérapie: Journal de la Société Française de Radiothérapie Oncologique
https://www.readbyqxmd.com/read/28195083/progressive-familial-intrahepatic-cholestasis-a-comprehensive-review-of-a-challenging-liver-disease
#19
REVIEW
Kavita Gaur, Puja Sakhuja
Cholestatic liver disease in children represents a diagnostic and therapeutic challenge. The requirement of a multidisciplinary approach, high levels of professional expertise, and the costs of genetic testing are a few of the reasons why such patients may suffer for want of an accurate diagnosis. Progressive familial intrahepatic cholestasis (PFIC) is a hereditary cholestatic liver disease, afflicted children often progressing to liver failure. Despite its potential to cause significant morbidity, it has seldom been studied in India...
January 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28027587/analysis-of-surgical-interruption-of-the-enterohepatic-circulation-as-a-treatment-for-pediatric-cholestasis
#20
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 nontransplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 with Alagille syndrome (ALGS), 16 with familial intrahepatic cholestasis-1 (FIC1), 18 with bile salt export pump (BSEP) disease, and 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 (PEBDs), 11 ileal exclusions (IEs), and seven gallbladder-to-colon (GBC) diversions...
May 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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