keyword
MENU ▼
Read by QxMD icon Read
search

Sclerosing cholangitis children

keyword
https://www.readbyqxmd.com/read/29901555/spectrum-of-pediatric-autoimmune-liver-disease-and-validation-of-its-diagnostic-scores-in-indian-children
#1
Vikrant Sood, Bikrant Bihari Lal, Dinesh Rawat, Rajeev Khanna, Archana Rastogi, Chhagan Bihari, Guresh Kumar, Seema Alam
OBJECTIVES: There is limitedliterature on the spectrum of pediatric autoimmune liver disease (AILD, encompassing both autoimmune hepatitis/AIH and autoimmune sclerosing cholangitis/ASC) in Asian populations and its diagnostic scores similarly require further validation. This work thus aimed to study the clinical characteristics, and to validate available diagnostic criteria in the local pediatric AILD cohort. METHODS: A review of all pediatric AILD cases, presenting over a 6 year (2011-2017) period was done, along with comparison of the available diagnostic scores: original (1999), simplified (2008) score and new proposed (2017) score...
June 12, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29808814/-affected-liver-biochemistry-in-children-with-rheumatic-diseases
#2
Helene Mygind Wolsk, Marianne Hørby Jørgensen, Susan Nielsen
Children with rheumatic diseases often have elevated liver biochemistry. This can be triggered by medical treatment, e.g. methotrexate can induce liver injury ranging from mild to severe. Autoimmune hepatitis and sclerosing cholangitis are also seen in conjunction with rheumatic diseases, and early diagnosis and treatment is crucial to prevent development of cirrhosis and liver transplantation. Macrophage activation syndrome is a rare but important differential diagnosis as it is a potentially fatal complication to systemic rheumatic diseases, causing liver dysfunction and multi-organ failure...
May 28, 2018: Ugeskrift for Laeger
https://www.readbyqxmd.com/read/29795465/paternal-cholestasis-exacerbates-obesity-associated-hypertension-in-male-offspring-but-is-prevented-by-paternal-ursodeoxycholic-acid-treatment
#3
Vanessa Pataia, Georgia Papacleovoulou, Vanya Nikolova, Anne-Maj Samuelsson, Stephanie Chambers, Eugene Jansen, Paul D Taylor, Lucilla Poston, Catherine Williamson
BACKGROUND: Obesity is a heterogeneous phenotype and risk associations to non-communicable diseases such as cardiovascular disease and type 2 diabetes are influenced by several factors. The paternal metabolic status at the time of conception influences offspring susceptibility to developing obesity and adiposity-associated cardiometabolic disease. Cholestatic liver diseases are characterized by raised circulating serum bile acid levels and dyslipidemia, and are commonly treated with ursodeoxycholic acid (UDCA)...
May 24, 2018: International Journal of Obesity: Journal of the International Association for the Study of Obesity
https://www.readbyqxmd.com/read/29685617/validation-of-transient-elastography-cut-points-to-assess-advanced-liver-fibrosis-in-children-and-young-adults-the-boston-children-s-hospital-experience
#4
Christine K Lee, Paul D Mitchell, Roshan Raza, Sarah Harney, Shanna M Wiggins, Maureen M Jonas
OBJECTIVE: To derive an optimal liver stiffness measurement cut point to discriminate METAVIR fibrosis stage F4 and to validate both METAVIR fibrosis stage F3-F4 and F4 cut points in a separate cohort. STUDY DESIGN: Patients at Boston Children's Hospital with liver stiffness measurement from 2006 to 2016 and liver biopsy ≤12 months before screening were eligible. Patients enrolled 2006-2011 were used to calibrate liver stiffness measurement cut points and those enrolled 2011-2016 for validation...
April 20, 2018: Journal of Pediatrics
https://www.readbyqxmd.com/read/29680189/vitamin-d-non-sufficiency-is-prevalent-in-children-with-chronic-liver-disease-in-a-tropical-country
#5
Way Seah Lee, Muhammad Yazid Jalaludin, Shin Yee Wong, Sik Yong Ong, Hee Wei Foo, Ruey Terng Ng
BACKGROUND: To determine vitamin D status in children with chronic liver disease (CLD) in a tropical country. METHODS: Cross-sectional study in Malaysian children with CLD. Factors affecting serum vitamin D level (definition: deficient < 30 nmol/L; insufficient 30-50 nmol/L; sufficient ≥ 50 nmol/L) was analyzed. RESULTS: Of the 59 children studied (males 32, 54%; median age 6.8 ± 5.3 years), the three most common causes were biliary atresia (n = 25), autoimmune hepatitis (n = 16) and sclerosing cholangitis (n = 6)...
April 5, 2018: Pediatrics and Neonatology
https://www.readbyqxmd.com/read/29676289/natural-history-and-outcome-of-inflammatory-bowel-diseases-in-children-in-saudi-arabia-a-single-center-experience
#6
Khalid M Alreheili, Khalid A Alsaleem, Ali I Almehaidib
Background/Aim: Inflammatory bowel disease (IBD) is a chronic gastrointestinal disorder which includes ulcerative colitis (UC), Crohn's disease (CD), and indeterminate colitis (IC). The natural history of pediatric IBDs is poorly understood and generally unpredictable. We aim to study the natural history of IBD in Saudi children including the extraintestinal manifestations, changes in diagnosis, disease behavior, medical management, and surgical outcome. Patients and Methods: A retrospective review of all the charts of children less than 14 years of age who were diagnosed as IBD and followed up in King Faisal Specialist Hospital and Research Center (KFSH and RC) from January 2001 to December 2011 was performed...
May 2018: Saudi Journal of Gastroenterology: Official Journal of the Saudi Gastroenterology Association
https://www.readbyqxmd.com/read/29470290/autoimmune-hepatitis-in-children-and-adolescents-effect-on-quality-of-life
#7
Isadora de Carvalho Trevizoli, Carolina Silva Pinedo, Vanessa Oliveira Teles, Renata Belém Pessoa de Melo Seixas, Elisa de Carvalho
OBJECTIVES: The aim of the to evaluate the effect of autoimmune hepatitis (AIH) on the quality of life of children and adolescents and to identify which variables effect health-related quality of life (HRQoL). METHODS: The Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0) was used to evaluate HRQoL. In addition, a questionnaire was applied which included signs, symptoms, and use of medications. RESULTS: A total of 43 patients (mean age, 15...
February 21, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29470284/evolving-practice-and-changing-phenotype-in-pediatric-autoimmune-liver-disease-outcomes-from-an-australian-center
#8
Harveen Singh, Fariha Balouch, Charlton Noble, Peter Lewindon
OBJECTIVES: Autoimmune liver disease (AILD) incorporates primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH), and autoimmune sclerosing cholangitis (ASC). ASC is a condition that includes overlap of AIH and PSC. We investigate changes in practice in relation to diagnosis and phenotype over 2 time periods. METHODS: Retrospective chart review was conducted from January 2000 to 2016. Data were divided into two 8-year cohorts, CI and C2. RESULTS: Data were collected in 75 children, 29 in 2000-2007 (C1) and 46 in 2008-2016 (C2)...
February 21, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29378308/symptoms-do-not-correlate-with-findings-from-colonoscopy-in-children-with-inflammatory-bowel-disease-and-primary-sclerosing-cholangitis
#9
Amanda Ricciuto, Jennifer Fish, Nicholas Carman, Thomas D Walters, Peter C Church, Bettina E Hansen, Eileen Crowley, Iram Siddiqui, Geoffrey C Nguyen, Binita M Kamath, Anne M Griffiths
BACKGROUND & AIMS: Approximately 75% of children with primary sclerosing cholangitis (PSC) also have inflammatory bowel disease (IBD). IBD in patients with PSC (PSC-IBD) often has a unique phenotype, including a mild clinical course, yet it is associated with an increased risk of colorectal cancer compared with colonic IBD without PSC. We investigated whether subclinical endoscopic and histologic inflammation could account for the increased risk of colorectal cancer in patients with PSC-IBD, and whether these patients have increased fecal levels of calprotectin, a marker of inflammation...
March 24, 2018: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29356770/diagnosis-and-management-of-pediatric-autoimmune-liver-disease-espghan-hepatology-committee-position-statement
#10
Giorgina Mieli-Vergani, Diego Vergani, Ulrich Baumann, Piotr Czubkowski, Dominique Debray, Antal Dezsofi, Björn Fischler, Girish Gupte, Loreto Hierro, Giuseppe Indolfi, Jörg Jahnel, Françoise Smets, Henkjan J Verkade, Nedim Hadžić
Paediatric autoimmune liver disease is characterized by inflammatory liver histology, circulating autoantibodies, and increased levels of IgG, in the absence of a known etiology. Three conditions have a likely autoimmune pathogenesis: autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis, and de novo AIH after liver transplantation. Two types of pediatric AIH are recognized according to seropositivity for smooth muscle and/or antinuclear antibody (AIH-1) or liver kidney microsomal type 1 and/or anti-liver cytosol type 1 antibodies (AIH-2)...
February 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29316057/a-promising-medium-term-follow-up-of-pediatric-sclerosing-cholangitis-mild-phenotype-or-early-diagnosis
#11
Federica Ferrari, Giusy Ranucci, Marina Aloi, Luca Della Volpe, Franca Viola, Erasmo Miele, Salvatore Cucchiara, Raffaele Iorio
AIM: Sclerosing cholangitis (SC) is a chronic cholestatic liver disease that is being increasingly diagnosed in childhood. The long-term course and prognosis of pediatric SC are poorly described. METHODS: We reviewed data of pediatric SC patients, followed in two referral centers, during a period of up to 20 years. We aimed to evaluate long-term outcomes according to SC phenotype. RESULTS: Among 45 patients (median age, 10.4 years; male patients, 73...
January 9, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29168311/usefulness-of-serum-wisteria-floribunda-agglutinin-positive-mac-2-binding-protein-in-children-with-primary-sclerosing-cholangitis
#12
Shuichiro Umetsu, Ayano Inui, Tsuyoshi Sogo, Haruki Komatsu, Tomoo Fujisawa
BACKGROUND: Wisteria floribunda agglutinin-positive Mac-2 binding protein (WFA+ -M2BP) is a novel serum marker of hepatic fibrosis in adults with chronic hepatitis C. However, it remains unclear whether serum WFA+ -M2BP levels are associated with the progression of liver histology in primary sclerosing cholangitis (PSC). METHODS: Twenty-eight children and adolescents with pediatric-onset PSC (male : female patient ratio, 20:8; median age at diagnosis, 9 years) were enrolled in this study...
April 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29163969/ercp-in-infants-children-and-adolescents-is-feasible-and-safe-results-from-a-tertiary-care-center
#13
Jasmin Felux, Ekkehard Sturm, Andreas Busch, Emanuel Zerabruck, Florian Graepler, Dietmar Stüker, Andreas Manger, Hans-Joachim Kirschner, Gunnar Blumenstock, Nisar P Malek, Martin Goetz
Background: Indications for endoscopic retrograde cholangiopancreatography (ERCP) in children differ from adults. Paucity of data and concerns about potential lower effectiveness and more side effects limit its use even in high volume centers. We retrospectively analyzed indications, success rates, limitations, and side effects of ERCPs in children <18 years. Methods: From January 2012 to March 2015, 54 ERCPs were performed in 31 children (median age 11 (0-17) years; median weight 22 (3...
November 2017: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/29112085/diagnosis-and-management-of-paediatric-autoimmune-liver-disease-espghan-hepatology-committee-position-statement
#14
Giorgina Mieli-Vergani, Diego Vergani, Ulrich Baumann, Piotr Czubkowski, Dominique Debray, Antal Dezsofi, Björn Fischler, Girish Gupte, Loreto Hierro, Giuseppe Indolfi, Jörg Jahnel, Françoise Smets, Henkjan J Verkade, Nedim Hadzic
Paediatric autoimmune liver disease is characterised by inflammatory liver histology, circulating autoantibodies and increased levels of IgG, in the absence of a known etiology. Three conditions have a likely autoimmune pathogenesis: autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC), and de novo AIH after liver transplantation. Two types of paediatric AIH are recognized according to seropositivity for smooth muscle and/or antinuclear antibody (SMA/ANA, AIH-1) or liver kidney microsomal type 1 and/or anti-liver cytosol type 1 antibodies (anti-LKM-1/anti-LC-1; AIH-2)...
November 3, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29059700/favorable-response-to-immunosuppressive-combination-therapy-with-mizoribine-and-azathioprine-in-children-with-primary-sclerosing-cholangitis
#15
Hitoshi Tajiri, Yoh Zen, Tomoko Takano, Stephen Brooks
Primary sclerosing cholangitis (PSC), with no curative intervention, can progress to end-stage liver disease. Mizoribine, a purine antimetabolite, has never been used for the management of PSC. To evaluate the role of mizoribine with azathioprine we undertook a preliminary clinical study in children with PSC. Children with PSC and autoimmune features were simultaneously treated with mizoribine and azathioprine. Ursodeoxycholic acid or mesalazine were not regulated. The primary end-points of our study included improvement of aspartate aminotransferase, alanine aminotransferase, and γ-glutamyltransferase...
March 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29056396/serology-in-autoimmune-hepatitis-a-clinical-practice-approach
#16
REVIEW
Benedetta Terziroli Beretta-Piccoli, Giorgina Mieli-Vergani, Diego Vergani
Serology is key to the diagnosis of autoimmune hepatitis (AIH). Clinicians need to be aware of which tests to request, how to interpret the laboratory reports, and be familiar with the laboratory methodology. If correctly tested, >95% of AIH patients show some serological reactivity. Indirect immunofluorescence on triple rodent tissue is recommended as first screening step, since it allows the detection of all liver-relevant autoantibodies, except for anti-soluble liver antigen (SLA) antibody, which needs to be detected by molecular based assays...
February 2018: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/29045346/autoimmune-hepatitis-and-autoimmune-hepatitis-overlap-with-sclerosing-cholangitis-immunophenotype-markers-in-children-and-adolescents
#17
Priscila M Ferri, Ana C Simões E Silva, Karen C L Torres, Soraya L C Silva, Diego J Q Aquino, Maria L M Ferreira, Eleonora D T Fagundes, Débora M Miranda, Alexandre R Ferreira
OBJECTIVE: The pathophysiology of autoimmune hepatitis (AIH) may involve the activation of immune cells and changes in the expression of cellular markers. The aim of the present study was to characterize the immunophenotype markers of lymphocytes and monocytes in the peripheral blood of children and adolescents with type 1 AIH and AIH overlap with sclerosing cholangitis (overlap syndrome [OS]). METHODS: This is a cross-sectional study of 20 children and adolescents diagnosed with type 1 AIH and 19 with OS...
February 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28993983/skin-manifestations-associated-with-autoimmune-liver-diseases-a-systematic-review
#18
REVIEW
Benedetta Terziroli Beretta-Piccoli, Pietro Invernizzi, M Eric Gershwin, Carlo Mainetti
Autoimmune liver diseases, which include mainly autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, and the variant syndromes, are often associated with extrahepatic autoimmune diseases. However, the association with cutaneous diseases is less well described. In the present article, we provide a systematic literature review on skin manifestations linked to each of these four autoimmune liver diseases, excluding skin manifestations of systemic diseases. The association of autoimmune hepatitis with vitiligo is well known, with a particular striking association with type 2 autoimmune hepatitis, a condition occurring almost entirely in children and adolescents, much rarer and more aggressive than type 1 autoimmune hepatitis; probable associations are also identified with alopecia areata, psoriasis, and pyoderma gangrenosum...
December 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28977113/primary-sclerosing-cholangitis-in-children-and-adolescents
#19
Eleonora Druve Tavares Fagundes, Alexandre Rodrigues Ferreira, Caroline Caldeira Hosken, Thaís Costa Nascentes Queiroz
BACKGROUND: Primary sclerosing cholangitis is a rare disease, but its prevalence has been underestimated in children and adolescents due to broad variation in clinical presentation as well as diagnostic challenges in this life period. OBJECTIVE: To evaluate children and adolescents with primary sclerosing cholangitis and to describe their clinical, laboratorial, histopathological, and cholangiography conditions. METHODS: This is an observational descriptive research that took place from 2005 to 2016 and included all the patients seen in the Outpatient Unit for Pediatric Hepatology of Hospital das Clinicas of UFMG who had been diagnosed with primary sclerosing cholangitis before the age of 18...
December 2017: Arquivos de Gastroenterologia
https://www.readbyqxmd.com/read/28953529/anti-tnf%C3%AE-treatment-in-children-and-adolescents-with-combined-inflammatory-bowel-disease-and-autoimmune-liver-disease
#20
Natalia Nedelkopoulou, Babu Vadamalayan, Diego Vergani, Giorgina Mieli-Vergani
OBJECTIVES: Inflammatory bowel disease (IBD) and autoimmune liver disease (AILD) are closely associated, the former often dictating progression of the latter. Antibodies to tumor necrosis factor alpha (anti-TNFα) are effective in the management of IBD, but may cause liver injury. METHODS: Retrospective review of medical records of patients with juvenile AILD who received anti-TNFα for IBD to evaluate the safety and efficacy of anti-TNFα. RESULTS: Eleven patients (6 boys), ages 9 to 15 years (median 13 years) were identified...
January 2018: Journal of Pediatric Gastroenterology and Nutrition
keyword
keyword
102137
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"