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Pediatric cholestasis

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https://www.readbyqxmd.com/read/29901549/a-bibliometric-analysis-of-clinical-and-translational-research-in-pediatric-gastroenterology-from-1970-to-2017
#1
Melanie H Schwimmer, Mary Catherine Sawh, Karen M Heskett, Nidhi P Goyal, Kimberly P Newton, Jeffrey B Schwimmer
OBJECTIVES: Pediatric gastroenterology is a clinical and research discipline principally developed over the past 50 years. Bibliometric methods provide quantitative analysis and identify research trends. Study aims were to characterize the growth and trends in pediatric gastroenterology clinical and translational research using citation analysis. METHODS: Using citations analysis software, a search strategy specific for pediatric gastroenterology was implemented for the years 1970-2017...
June 12, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29868931/bile-duct-paucity-in-childhood-spectrum-profile-and-outcome
#2
Babu Lal Meena, Rajeev Khanna, Chhagan Bihari, Archana Rastogi, Dinesh Rawat, Seema Alam
We studied the etiological spectrum, clinicolaboratory and histological profile, and outcome of infants and children under 18 years of age presenting between December 2010 and May 2016 with histological evidence of paucity of intralobular bile ducts (PILBD, bile ducts to portal tract ratio < 0.6) Post-transplant PILBD was excluded. Of 632 pediatric liver biopsies screened, 70 had PILBD-44 were infants. PILBD was classified histologically into destructive (n = 50) and non-destructive PILBD (n = 20)...
June 4, 2018: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29783821/-clinical-and-genetic-analysis-of-eleven-pediatric-patients-with-alagille-syndrome
#3
L Guo, S T Zhao, Y Cheng, M Deng, H Li, Y Z Song, X R Cai, Q Zhou
Objective: To explore the clinical and molecular genetic features of patients with Alagille syndrome (AS). Methods: The clinical data of eleven pediatric patients, who were suspected to have AS at the Department of Pediatrics in the First Affiliated Hospital of Jinan University from August 2010 to March 2017, were collected and analyzed. Genomic DNA was extracted from peripheral blood leukocytes of the patients and their parents. For 5 patients collected before March 2006, all JAG1 exons and their flanking sequences were directly sequenced...
May 2, 2018: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29729773/surgical-outcomes-in-alagille-syndrome-and-pfic-a-single-institution-s-20-year-experience
#4
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/29667721/diverse-manifestations-of-acute-sickle-cell-hepatopathy-in-pediatric-patients-with-sickle-cell-disease-a-case-series
#5
Lydia H Pecker, Nidhi Patel, Susan Creary, Anil Darbari, Emily Riehm Meier, Deepika S Darbari, Ross M Fasano
The hepatic complications of sickle cell disease (SCD) are associated with increased morbidity and mortality in adults; children usually survive but may suffer significant sequelae. Few diagnostic tools differentiate the various hepatic manifestations of SCD. Why patients exhibit one hepatic pathology versus another is unclear. We report four pediatric patients with hemoglobin SS disease with diverse manifestations of acute hepatic involvement including acute sickle hepatic crisis, hepatic sequestration, sickle cell intrahepatic cholestasis, and a non-SCD cause of hepatopathy in a patient with viral hepatitis...
April 18, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29610171/autoimmune-pancreatitis-and-ulcerative-rectocolitis-in-an-adolescent
#6
Elie Cousin, Ianis Cousin, Karim Aziz, Pascal Chailloux, Estelle Breton
Autoimmune pancreatitis (AIP) is rare in teenagers and difficult to diagnose. There are no clear and established diagnostic criteria in the pediatric population to distinguish subtype 1 and subtype 2. Here, we report the case of a 16-year-old white French teenager admitted to the pediatric emergency service with more than 1 year's history of pain originating from the epigastric and the right hypochondriac regions, with bloody diarrhea. After exclusion of pancreatic cancer and other common causes of acute pancreatitis, the diagnosis of AIP was suspected...
April 2018: Pediatrics
https://www.readbyqxmd.com/read/29601463/conjugated-hyperbilirubinemia-in-the-neonate-and-young-infant
#7
Anna K Weiss, Parag V Vora
Cholestatic jaundice in the first few weeks of life may herald potentially life-threatening pathology. It is therefore incumbent upon the pediatric practitioner to have a high index of suspicion for severe disease when investigating jaundice in a young infant. This article outlines the epidemiology, pathophysiology, differential diagnosis, and diagnostic workup for both the most common and the most severe causes of cholestasis in the neonatal period.
April 2018: Pediatric Emergency Care
https://www.readbyqxmd.com/read/29499989/clinical-pathologic-and-genetic-features-of-neonatal-dubin-johnson-syndrome-a-multicenter-study-in-japan
#8
Takao Togawa, Tatsuki Mizuochi, Tokio Sugiura, Hironori Kusano, Ken Tanikawa, Takato Sasaki, Fumio Ichinose, Seiichi Kagimoto, Takahisa Tainaka, Hiroo Uchida, Shinji Saitoh
OBJECTIVE: To clarify the clinical, pathologic, and genetic features of neonatal Dubin-Johnson syndrome. STUDY DESIGN: Ten patients with neonatal Dubin-Johnson syndrome were recruited from 6 pediatric centers in Japan between September 2013 and October 2016. Clinical and laboratory course, macroscopic and microscopic liver findings, and molecular genetic findings concerning ATP-binding cassette subfamily C member 2 (ABCC2) were retrospectively and prospectively examined...
May 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29406331/reduced-immunohistochemical-expression-of-hnf1%C3%AE-and-foxa2-in-liver-tissue-can-discriminate-between-biliary-atresia-and-other-causes-of-neonatal-cholestasis
#9
Usama F Shaalan, Noha L Ibrahim, Nermine A Ehsan, Mervat M Sultan, Ghada M Naser, Mohamed O Abd El-Fatah
Biliary atresia (BA) is a necroinflammatory occlusive cholangiopathy that affects infants. Genetic and environmental factors has been proposed for its occurrence. The objectives of this study was to investigate the protein expression of 2 important genes regulating ductal plate remodeling, hepatocyte nuclear factor 1-beta (Hnf1β) and the fork head box protein A2 (FoxA2) in liver tissue from patients with BA and to compare their expression with other causes of neonatal cholestasis (NC). This retrospective study included 60 pediatric patients, 30 with BA and 30 with NC...
February 28, 2018: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/29392752/increased-expression-of-senescence-associated-cell-cycle-regulators-in-the-progression-of-biliary-atresia-an-immunohistochemical-study
#10
Motoko Sasaki, Fang-Ying Kuo, Chao-Cheng Huang, Paul E Swanson, Chao-Long Chen, Jiin-Haur Chuang, Matthew M Yeh
AIMS: Cellular senescence plays a role in tumour suppression and in the pathogenesis of various non-neoplastic diseases, including primary biliary cholangitis and other adult cholangiopathies. Less is known about the role of cellular senescence in cholangiopathies in children. With that in mind, we examined the expression of senescence-associated cell cycle regulators in biliary atresia, the most common form of paediatric obliterative cholangiopathy. METHODS AND RESULTS: The expression of senescence-associated cell cycle regulators (p16Ink4a and p21WAF 1/Cip1 ) and a ductular reaction related marker (neural cell adhesion molecule: NCAM) was examined in bile ducts and bile ductules in liver samples taken from the patients with biliary atresia [n = 80; including 23 samples at the time of the Kasai procedure (KP) and 63 obtained from the explanted liver (LT) (six cases with samples at both surgical stages of disease)] and from appropriate controls (n = 17)...
February 2, 2018: Histopathology
https://www.readbyqxmd.com/read/29391763/metal-stents-placement-for-refractory-pancreatic-duct-stricture-in-children
#11
In Sook Jeong, Sung Hee Lee, Seak Hee Oh, Do Hyun Park, Kyung Mo Kim
AIM: To evaluate the use of fully covered self-expandable metal stents (FCSEMSs) for pancreatic duct strictures in children with chronic pancreatitis. METHODS: Eight patients with refractory benign dominant stricture of the main pancreatic duct (MPD) were enrolled through chart reviews between December 2014 and June 2017 in a single center. Endoscopic retrograde cholangiopancreatography (ERCP) with placement of a 6-mm FCSEMS with dual flaps was performed. Endoscopic removal of FCSEMSs was performed with a snare or rat-tooth forceps...
January 21, 2018: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29364802/-infectious-mononucleosis-and-cholestatic-hepatitis-a-rare-association
#12
Catarina Salgado, Ana Margarida Garcia, Catarina Rúbio, Florbela Cunha
Infectious mononucleosis is one of the major clinical manifestations of Epstein-Barr virus infection. In this syndrome, elevation of liver transaminase levels is common but cholestasis is rare, with few cases described in the literature. We present the case of a 14-year-old female adolescent, admitted to the Emergency Room with fever, odynophagia and cervical adenomegaly. She was treated with amoxicillin and two days later he presented with jaundice. The analytical evaluation was compatible with cholestatic hepatitis and abdominal ultrasound revealed hepatosplenomegaly without dilatation of the bile ducts...
December 29, 2017: Acta Médica Portuguesa
https://www.readbyqxmd.com/read/29168375/cholestasis-and-hypercalcemia-secondary-to-panhypopituitarism-in-a-newborn
#13
Fatma Dursun, Nelgin Gerenli, Heves Kırmızıbekmez
Dursun F, Gerenli N, Kırmızıbekmez H. Cholestasis and hypercalcemia secondary to panhypopituitarism in a newborn. Turk J Pediatr 2017; 59: 100-103. Cholestatic hepatitis and hypercalcemia are rare features of hypopituitarism in newborns. So diagnosis of hypopituitarism is frequently delayed. The most frequent symptoms of congenital hypopituitarism are hypoglycemia, prolonged jaundince and micropenis. A patient with congenital hypopituitarism associated with cholestasis and hypercalcemia is reported here...
2017: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/29104077/assessment-of-atp8b1-deficiency-in-pediatric-patients-with-cholestasis-using-peripheral-blood-monocyte-derived-macrophages
#14
Hisamitsu Hayashi, Sotaro Naoi, Takao Togawa, Yu Hirose, Hiroki Kondou, Yasuhiro Hasegawa, Daiki Abukawa, Mika Sasaki, Koji Muroya, Satoshi Watanabe, Satoshi Nakano, Kei Minowa, Ayano Inui, Akinari Fukuda, Mureo Kasahara, Hironori Nagasaka, Kazuhiko Bessho, Mitsuyoshi Suzuki, Hiroyuki Kusuhara
Progressive familial intrahepatic cholestasis type 1 (PFIC1), a rare inherited recessive disease resulting from a genetic deficiency in ATP8B1, progresses to liver failure. Because of the difficulty of discriminating PFIC1 from other subtypes of PFIC based on its clinical and histological features and genome sequencing, an alternative method for diagnosing PFIC1 is desirable. Herein, we analyzed human peripheral blood monocyte-derived macrophages (HMDM) and found predominant expression of ATP8B1 in interleukin-10 (IL-10)-induced M2c, a subset of alternatively activated macrophages...
January 2018: EBioMedicine
https://www.readbyqxmd.com/read/29080241/next-generation-sequencing-in-pediatric-hepatology-and-liver-transplantation
#15
REVIEW
Emanuele Nicastro, Lorenzo D'Antiga
Next generation sequencing (NGS) has revolutionized the analysis of human genetic variations, offering a highly cost-effective way to diagnose monogenic diseases (MDs). Because nearly half of the children with chronic liver disorders have a genetic cause and approximately 20% of pediatric liver transplantations are performed in children with MDs, NGS offers the opportunity to significantly improve the diagnostic yield in this field. Among the NGS strategies, the use of targeted gene panels has proven useful to rapidly and reliably confirm a clinical suspicion, whereas the whole exome sequencing (WES) with variants filtering has been adopted to assist the diagnostic workup in unclear clinical scenarios...
February 2018: Liver Transplantation
https://www.readbyqxmd.com/read/28947328/outcomes-of-biliary-atresia-in-the-nordic-countries-a-multicenter-study-of-158-patients-during-2005-2016
#16
Mikko P Pakarinen, Lars Søndergaard Johansen, Jan F Svensson, Kristin Bjørnland, Vladimir Gatzinsky, Pernilla Stenström, Antti Koivusalo, Nina Kvist, Markus Almström, Ragnhild Emblem, Sigurdur Björnsson, Torbjörn Backman, Runar Almaas, Hannu Jalanko, Björn Fischler, Jørgen Thorup
BACKGROUND/PURPOSE: Biliary atresia is the most common reason for newborn cholestasis and pediatric liver transplantation. Even after normalization of serum bilirubin after portoenterostomy, most patients require liver transplantation by adulthood due to expanding fibrosis. We addressed contemporary outcomes of biliary atresia in the Nordic countries. METHODS: Data on center and patients characteristics, diagnostic practices, surgical treatment, adjuvant medical therapy after portoenterostomy, follow-up and outcomes were collected from all the Nordic centers involved with biliary atresia care during 2005-2016...
September 5, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28947327/understanding-the-relationship-between-hospital-volume-and-patient-outcomes-for-infants-with-gastroschisis
#17
Genia Dubrovsky, Greg D Sacks, Scott Friedlander, Steven Lee
BACKGROUND: For many surgical operations, there is a well-established relationship between surgical volume and outcome. We investigated whether this relationship exists for infants with gastroschisis. METHODS: Using the Kids' Inpatient Database for years 2003, 2006, 2009, and 2012, we identified all patients undergoing gastroschisis repair. Controlling for patient characteristics and complexity of disease (comorbid intestinal atresia/perforation, necrotizing enterocolitis, and respiratory distress syndrome), we compared surgical outcomes (mortality, length of stay, and incidence of TPN cholestasis) by hospital volume based on quartile for gastroschisis cases treated per year...
December 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28838453/cholestasis-after-pediatric-liver-transplantation-recurrence-of-a-progressive-familial-intrahepatic-cholestasis-phenotype-as-a-rare-differential-diagnosis-a-case-report
#18
B Prusinskas, S Kathemann, D Pilic, B Hegen, P Küster, V Keitel, D Häussinger, R Büscher, H A Baba, P F Hoyer, E Lainka
INTRODUCTION: Nonobstructive cholestasis after pediatric liver transplantation is a common diagnostic and therapeutic dilemma. We describe a girl with neonatal cholestasis because of progressive familial intrahepatic cholestasis 2 (PFIC-2) and presence of a homozygous splice site mutation in the ABCB11 gene. Liver transplantation was performed because of end-stage liver disease at the age of 6. Cholestasis with normal gamma-glutamyl transferase (GGT) developed 8 years after liver transplantation...
September 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28809725/clinical-characteristics-and-complications-of-pediatric-liver-biopsy-a-single-centre-experience
#19
Patricia Almeida, Richard A Schreiber, Jennifer Liang, Quais Mujawar, Orlee R Guttman
INTRODUCTION: Percutaneous liver biopsy (LB) is the gold standard method for evaluation and management of patients with liver disease. The purpose of this study was to characterize pediatric patients undergoing LB at British Columbia Children's Hospital, and to determine the rate and timing of complications following the procedure. MATERIAL AND METHODS: The medical records of all pediatric patients who underwent LB during a six-year retrospective study were reviewed to collect demographic and procedure-related data...
September 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28731841/survey-of-nutrition-management-practices-in-centers-for-pediatric-intestinal-rehabilitation
#20
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
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