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Chronic liver disease children

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https://www.readbyqxmd.com/read/29456414/high-prevalence-of-hepatitis-b-antibody-loss-and-a-case-report-of-de-novo-hepatitis-b-virus-infection-in-a-child-after-living-donor-liver-transplantation
#1
Palittiya Sintusek, Nawarat Posuwan, Piyaporn Wanawongsawad, Suttiruk Jitraruch, Yong Poovorawan, Voranush Chongsrisawat
AIM: To assess the seroprevalence of hepatitis B virus (HBV) immunity among previously vaccinated pediatric liver transplant recipients and present a case report of de novo hepatitis B infection after liver transplantation. METHODS: This study focused on children with chronic liver diseases who received primary hepatitis B immunization and had a complete dataset of anti-HBs before and after liver transplantation between May 2001 and June 2017. Medical records were retrospectively reviewed for potential factors relating to HBV immunity loss...
February 14, 2018: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29451227/statural-growth-and-prevalence-of-endocrinopathies-in-relation-to-liver-iron-content-lic-in-adult-patients-with-beta-thalassemia-major-btm-and-sickle-cell-disease-scd
#2
Mohamed A Yassin, Ashraf T Soliman, Vincenzo De Sanctis, Mohammad Aj Abdula, Lubna M Riaz, Firdous F Ghori, Anil Yousaf, Abdulqadir J Nashwan, Sandara Abusamaan, Abbas Moustafa, Samah Kohla, Dina S Soliman
Despite regular blood transfusion and iron chelation therapy, growth impairment and pubertal delay are commonly seen in children and adolescents with transfusion-dependent Beta thalassaemia major (BTM) and sickle cell disease (SCD). We evaluated growth parameters and endocrine disorders in relation to the liver iron concentration (LIC) assessed by the Ferriscan® method in a cohort of adults with SCD (n =40) and BTM (n = 52) receiving blood transfusions and iron chelation therapy since early childhood. Before transfusion, hemoglobin concentration had not been less than 9 g/dl in the past 12 years; subcutaneous daily desferrioxamine was administered for all of them since early childhood (2- 5 years of age)...
February 16, 2018: Acta Bio-medica: Atenei Parmensis
https://www.readbyqxmd.com/read/29446799/autoimmune-hepatitis-in-children
#3
Saumya Pathak, Deepak Kamat
Autoimmune hepatitis (AIH) is an immune-mediated, inflammatory liver disease. Clinical presentation of AIH in children is highly variable. It can present acutely, chronically, or silently. There are two main types of AIH-type 1 and type 2, which are differentiated and defined by the presence of specific autoantibodies. AIH eventually progresses to cirrhosis when left untreated, and occasionally even with treatment. AIH must be suspected and excluded in all children presenting with signs of acute, prolonged, or severe liver disease...
February 1, 2018: Pediatric Annals
https://www.readbyqxmd.com/read/29423877/nhlrc2-variants-identified-in-patients-with-fibrosis-neurodegeneration-and-cerebral-angiomatosis-finca-characterisation-of-a-novel-cerebropulmonary-disease
#4
Johanna Uusimaa, Riitta Kaarteenaho, Teija Paakkola, Hannu Tuominen, Minna K Karjalainen, Javad Nadaf, Teppo Varilo, Meri Uusi-Mäkelä, Maria Suo-Palosaari, Ilkka Pietilä, Anniina E Hiltunen, Lloyd Ruddock, Heli Alanen, Ekaterina Biterova, Ilkka Miinalainen, Annamari Salminen, Raija Soininen, Aki Manninen, Raija Sormunen, Mika Kaakinen, Reetta Vuolteenaho, Riitta Herva, Päivi Vieira, Teija Dunder, Hannaleena Kokkonen, Jukka S Moilanen, Heikki Rantala, Lawrence M Nogee, Jacek Majewski, Mika Rämet, Mikko Hallman, Reetta Hinttala
A novel multi-organ disease that is fatal in early childhood was identified in three patients from two non-consanguineous families. These children were born asymptomatic but at the age of 2 months they manifested progressive multi-organ symptoms resembling no previously known disease. The main clinical features included progressive cerebropulmonary symptoms, malabsorption, progressive growth failure, recurrent infections, chronic haemolytic anaemia and transient liver dysfunction. In the affected children, neuropathology revealed increased angiomatosis-like leptomeningeal, cortical and superficial white matter vascularisation and congestion, vacuolar degeneration and myelin loss in white matter, as well as neuronal degeneration...
February 8, 2018: Acta Neuropathologica
https://www.readbyqxmd.com/read/29404465/nonalcoholic-fatty-liver-disease-and-the-ongoing-role-of-liver-biopsy-evaluation
#5
REVIEW
Elizabeth M Brunt
Nonalcoholic fatty liver disease (NAFLD) is one of the most common underlying causes of chronically elevated liver tests and liver disease in adults and children worldwide and may be strongly suspected if not diagnosed by ever evolving and available serologic and imaging-based noninvasive tests. However, the definitive diagnosis of the most progressive form of NAFLD, nonalcoholic steatohepatitis, and the identification of fibrosis stage still require liver biopsy evaluation as noninvasive testing has not replaced some of the specifics or the totality of information obtainable from liver biopsy...
July 2017: Hepatology Communications
https://www.readbyqxmd.com/read/29396050/association-of-interleukin-il-4-variable-number-of-tandem-repeats-vntrs-and-il-4-590-promoter-polymorphisms-with-susceptibility-to-pediatric-autoimmune-hepatitis-type-1
#6
Amira Ibrahim Mansour, Ola Galal Behairy, Eman Rateb Abd Almonaem, Rasha Mahmoud Abd-Rabuh, Inas Abd Elmonem Ahmed
OBJECTIVE: Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease mediated by an autoimmune reaction to hepatocytes, the present study aimed to assess the possible associations between interleukin-4 (IL-4) variable number of tandem repeats (VNTRs) and IL-4-590 promoter polymorphisms and susceptibility to autoimmune hepatitis type 1 in children. SUBJECTS AND METHODS: The study was performed on 101 children diagnosed with AIH and 104 apparently healthy, age and sex-matched control children, diagnosis of AIH was based on the simplified score for the diagnosis of AIH...
January 26, 2018: Cytokine
https://www.readbyqxmd.com/read/29391313/post-transplant-malignancies-in-pediatric-liver-transplant-recipients-experience-of-two-centers-in-turkey
#7
Miray Karakoyun, Şebnem Önen, Maşallah Baran, Murat Çakır, Çiğdem Ömür Ecevit, Murat Kılıç, Mehmet Kantar, Serap Aksoylar, Funda Özgenç, Sema Aydoğdu
BACKGROUND/AIMS: A liver transplant is the preferred treatment for patients with end-stage liver disease, as it usually results in longterm survival. However, due to the use of chronic immunosuppressive therapy, which is necessary to prevent rejection, de novo cancer is a major risk after transplantation. The aim of this study was to assess the incidence of post-transplant malignancies in children after liver transplantations. MATERIALS AND METHODS: The study group consisted of 206 liver transplant recipients, with no history of cancer, including hepatocellular carcinoma, in two liver transplantation centers in Turkey between 1997 and 2015...
January 2018: Turkish Journal of Gastroenterology: the Official Journal of Turkish Society of Gastroenterology
https://www.readbyqxmd.com/read/29380318/assessment-of-fatty-liver-in-models-of-disease-programming
#8
Kimberley D Bruce, Karen R Jonscher
Nonalcoholic fatty liver disease (NAFLD) is currently the most common cause of chronic liver disease worldwide and is present in a third of the general population and the majority of individuals with obesity and type 2 diabetes. Importantly, NAFLD can progress to severe nonalcoholic steatohepatitis (NASH), associated with liver failure and hepatocellular carcinoma. Recent research efforts have extensively focused on identifying factors contributing to the additional "hit" required to promote NALFD disease progression...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29379536/bifidobacteria-and-lactobacilli-in-the-gut-microbiome-of-children-with-non-alcoholic-fatty-liver-disease-which-strains-act-as-health-players
#9
Valerio Nobili, Lorenza Putignani, Antonella Mosca, Federica Del Chierico, Pamela Vernocchi, Anna Alisi, Laura Stronati, Salvatore Cucchiara, Marco Toscano, Lorenzo Drago
Introduction: Non-alcoholic fatty liver disease (NAFLD), considered the leading cause of chronic liver disease in children, can often progress from non-alcoholic fatty liver (NAFL) to non-alcoholic steatohepatitis (NASH). It is clear that obesity is one of the main risk factors involved in NAFLD pathogenesis, even if specific mechanisms have yet to be elucidated. We investigated the distribution of intestinal bifidobacteria and lactobacilli in the stools of four groups of children: obese, obese with NAFL, obese with NASH, and healthy, age-matched controls (CTRLs)...
January 2018: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/29343274/coexistent-sickle-cell-anemia-and-autoimmune-disease-in-eight-children-pitfalls-and-challenges
#10
Valerie Li-Thiao-Te, Florence Uettwiller, Pierre Quartier, Florence Lacaille, Brigitte Bader-Meunier, Valentine Brousse, Mariane de Montalembert
BACKGROUND: Patients with sickle cell disease (SCD) present a defective activation of the alternate complement pathway that increases the risk of infection and is thought to predispose to autoimmune disease (AID). However, coexisting AID and SCD is rarely reported, suggesting possible underdiagnosis due to an overlapping of the symptoms. STUDY DESIGN: Among 603 patients with SCD followed between 1999 and June 2016, we retrospectively searched for patients with coexisting SCD and AID...
January 17, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29330965/intra-hepatic-bile-duct-primary-cilia-in-biliary-atresia
#11
Roberta Frassetto, Filippo Parolini, Salvatore Marceddu, Giulia Satta, Valeria Papacciuoli, Maria Antonia Pinna, Alessandra Mela, Giannina Secchi, Grazia Galleri, Roberto Manetti, Luisa Bercich, Vincenzo Villanacci, Antonio Dessanti, Roberto Antonucci, Francesco Tanda, Daniele Alberti, Kathleen B Schwarz, Maria Grazia Clemente
AIM: The etiopathogenesis of non-syndromic biliary atresia (BA) is obscure. The primary aim was to investigate intra-hepatic bile duct cilia (IHBC) in BA at the diagnosis and correlation with clinical outcome. The secondary aim was to analyze IHBC in routine paraffin-embedded liver biopsies using conventional scanning electron microscopy (SEM). METHODS: Surgical liver biopsies taken at the diagnosis from 22 BA infants (age range: 39 - 116 days) and from 8 children with non-BA chronic cholestasis (age range: 162 days -16,8 years) were evaluated for IHBC, both by immunofluorescence (IF) and by SEM...
January 13, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29325287/-the-diagnostic-value-and-limits-of-diagnostic-parameters-for-wilson-s-disease
#12
X Yang
Wilson disease (WD) is a rare and treatable genetic disorder. This paper describes the new advances and author's long-term experiences in the diagnosis of WD. The characteristics in clinical and routine tests are: the age of presentation can be quite broad, the WD could not be excluded based on age only; the patients usually have mild digestive symptoms but obvious chronic liver disease signs; liver function tests may reveal normal or a mild elevation in bilirubin, ALT and AST, but quite abnormal in serum albumin and prothrombin time in most patients; Coombs-negative hemolytic anemia, normal or markedly subnormal serum alkaline phosphatase (typically < 40 IU/L) are useful for the diagnosis of fulminant WD...
December 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29324774/activated-carbon-n-acetylcysteine-microcapsule-protects-against-nonalcoholic-fatty-liver-disease-in-young-rats-via-activating-telomerase-and-inhibiting-apoptosis
#13
Tingting Shi, Xingxin Yang, Hongping Zhou, Jianjun Xi, Jingjing Sun, Yunling Ke, Jiankang Zhang, Yidan Shao, Xiaojie Jiang, Xuwang Pan, Shourong Liu, Rangxiao Zhuang
Non-alcoholic fatty liver disease (NAFLD) is becoming one of the world's most common chronic liver diseases in childhood, yet no therapy is available that has been approved by the food and drug administration (FDA). Previous studies have reported that telomere and telomerase are involved the development and progression of NAFLD. This study was designed to investigate the potential beneficial effects of activated carbon N-acetylcysteine (ACNAC) microcapsules on the development of NAFLD in young rats as well as the underlying mechanism(s) involved...
2018: PloS One
https://www.readbyqxmd.com/read/29316057/-a-promising-medium-term-follow-up-of-pediatric-sclerosing-cholangitis-mild-phenotype-or-early-diagnosis
#14
F Ferrari, G Ranucci, M Aloi, L Della Volpe, F Viola, E Miele, S Cucchiara, R Iorio
BACKGROUND AND AIMS: Sclerosing cholangitis (SC) is a chronic cholestatic liver disease whose diagnosis is being increasingly recognized in childhood. Long-term course and prognosis of pediatric SC is 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...
January 9, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29303884/the-first-episode-of-spontaneous-bacterial-peritonitis-is-a-threat-event-in-children-with-end-stage-liver-disease
#15
Sandra M G Vieira, Fernando P Schwengber, Melina Melere, Marília R Ceza, Melina Souza, Carlos O Kieling
OBJECTIVE: Studies on native liver survival (NLS) after the first episode of spontaneous bacterial peritonitis (SBP) are rare. Our objective was to evaluate NLS in children up to 1 year after SBP. METHODS: A historical cohort study of 18 children followed after the first episode of SBP was conducted. NLS, in-hospital mortality, causes of death, and rate of multidrug-resistant organisms were reported. RESULTS: Biliary atresia was the most prevalent diagnosis (72...
January 4, 2018: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29303286/-cystic-fibrosis-in-adults
#16
Libor Fila
Cystic fibrosis (CF) is an inherited disease caused by mutations in the transmembrane conductance regulator (CFTR) gene. The disease leads to dysfunction of the exocrine glands with high concentration of chloride in the sweat and formation of abnormally viscous mucus in the respiratory, digestive and reproductive tract. Chronic sinopulmonary disease, exocrine pancreatic insufficiency, liver disease, intestinal obstruction, impaired nutritional status, salt loss syndrome and male infertility dominates in the clinical presentation...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29297972/prognostic-scoring-systems-and-outcome-of-endovascular-radiological-intervention-of-chronic-budd-chiari-syndrome-in-children
#17
Sumit Kumar Singh, Moinak Sen Sarma, Rajanikant Yadav, Sheo Kumar, Raghunandan Prasad, Surender Kumar Yachha, Anshu Srivastava, Ujjal Poddar
BACKGROUND AND AIM: Prognostic scoring systems (PSS) have not been validated in children with chronic Budd-Chiari syndrome (BCS). We aimed to analyse the long-term outcome of radiological intervention (RI) and validate the PSS in children. METHODS: Chronic BCS children were analysed in four subgroups: SI: successful intervention (primary or secondary stent patency) b) PO: poor outcome (refractory stent block or requirement of liver transplantation), c) NU: naïve unintervened (awaiting RI) and d) DBI: died before intervention...
January 3, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29297940/oral-mucosa-lesions-and-gingival-bleeding-can-indicate-the-progression-of-liver-disease-in-children-and-adolescents-aged-two-to-18-years
#18
Dorota Olczak-Kowalczyk, Ewa Krasuska-Sławińska, Dariusz Gozdowski, Wojciech Kowalczyk, Joanna Pawłowska
AIM: This study assessed correlations between systemic disturbances of paediatric chronic liver diseases (CLD) and oral symptoms in subjects aged 2-18 years. METHODS: It was carried out during outpatient appointments at the Children's Memorial Health Institute, Warsaw, Poland, from 2010-2015 and comprised 52 CLD patients with a mean age of 12.3 ±4.6. We also recruited 54 generally healthy controls with a mean age of 12.0 ±3.7 from the Department of Paediatric Dentistry at the Medical University of Warsaw...
January 3, 2018: Acta Paediatrica
https://www.readbyqxmd.com/read/29296723/atg-vs-thiotepa-with-busulfan-and-cyclophosphamide-in-matched-related-bone-marrow-transplantation-for-thalassemia
#19
Lawrence Faulkner, Cornelio Uderzo, Sadaf Khalid, Priya Marwah, Rajpreet Soni, Naila Yaqub, Samina Amanat, Itrat Fatima, Sarah Khan Gilani, Tatheer Zahra, Stalin Ramprakash, Lallindra Gooneratne, Ruwangi Dissanayake, Senani Williams, Wasantha Rathnayake, Reshma Srinivas, Amit Sedai, Ankita Kumari, Lailith Parmar, Rakesh Dhanya, Rajat Kumar Agarwal
Matched-related bone marrow transplantation (BMT) may cure >80% of low-risk children with severe thalassemia (ST). Very long-term follow-up studies have shown how the standard busulfan-cyclophosphamide (BuCy) regimen may be associated with normalization of health-related quality of life, no second malignancies in the absence of chronic graft-versus-host disease, and fertility preservation in many patients. However, because BuCy may be associated with high rejection rates, some centers incorporate thiotepa (Tt) in busulfan- or treosulfan-based regimens, a combination that may increase the risk of permanent infertility...
May 23, 2017: Blood Advances
https://www.readbyqxmd.com/read/29287004/non-invasive-markers-of-portal-hypertension-appraisal-of-adult-experience-and-potential-utilisation-in-children
#20
Harry Sutton, Anil Dhawan, Tassos Grammatikopoulos
Portal Hypertension (PHT) is a significant cause of morbidity and mortality in children with chronic liver disease (CLD) and portal vein obstruction. Increased portal pressure results in variceal formation along the gastrointestinal (GI) tract resulting in major bleeding. Identifying children with significant PHT who are more likely to suffer GI bleeding has been challenging and the role of surveillance upper GI endoscopy has been debated. This review analyses research done on serum biomarkers and imaging techniques as possible predictors of significant PHT...
December 28, 2017: Journal of Pediatric Gastroenterology and Nutrition
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