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Liver disease in children

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https://www.readbyqxmd.com/read/29330965/intra-hepatic-bile-duct-primary-cilia-in-biliary-atresia
#1
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
#2
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
#3
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/29323192/baseline-il-2-and-the-aih-score-can-predict-the-response-to-standard-therapy-in-paediatric-autoimmune-hepatitis
#4
Jana Diestelhorst, Norman Junge, Danny Jonigk, Jerome Schlue, Christine S Falk, Michael P Manns, Ulrich Baumann, Elmar Jaeckel, Richard Taubert
Although autoimmune hepatitis (AIH) can be treated with corticosteroid-based first-line therapy, incomplete remission is associated with progressive liver fibrosis. So far accepted predictors of the subsequent treatment response of AIH patients are lacking. Therefore, we analysed baseline parameters, including iron homeostasis and cytokine levels, in 60 children with paediatric AIH (pAIH). In contrast to adults, elevated serum markers indicating iron overload were not commonly found in children. Therefore, ferritin was not predictive of the treatment response in pAIH...
January 11, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29322327/combined-and-sequential-liver-kidney-transplantation-in-children
#5
Ryszard Grenda, Piotr Kaliciński
Combined and sequential liver-kidney transplantation (CLKT and SLKT) is a definitive treatment in children with end-stage organ failure. There are two major indications: - terminal insufficiency of both organs, or - need for transplanting new liver as a source of lacking enzyme or specific regulator of the immune system in a patient with renal failure. A third (uncommon) option is secondary end-stage renal failure in liver transplant recipients. These three clinical settings use distinct qualification algorithms...
January 10, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29322175/association-of-psoriasis-with-comorbidity-development-in-children-with-psoriasis
#6
Megha M Tollefson, Holly K Van Houten, Dennis Asante, Xiaoxi Yao, Hilal Maradit Kremers
Importance: Children with psoriasis are at increased risk for comorbidities. Many children with psoriasis are also overweight or obese; it is unknown whether the increased risk of comorbidities in these children is independent of obesity. Objective: To determine the risk of elevated lipid levels (hyperlipidemia/hypertriglyceridemia), hypertension, metabolic syndrome, polycystic ovarian syndrome, diabetes, nonalcoholic liver disease, and elevated liver enzyme levels in children with and without psoriasis, after accounting for obesity...
January 10, 2018: JAMA Dermatology
https://www.readbyqxmd.com/read/29318365/-innovations-in-surgical-treatment-of-pediatric-solid-tumors
#7
REVIEW
A Schmidt, S W Warmann, C Urla, J Fuchs
The overall survival of children with solid tumors has shown a substantial increase in the past decades due to progress in all of the disciplines involved in the treatment. The poor prognosis for advanced stages of disease and the morbidity related to therapeutic procedures are still a challenge. Innovations in the surgical treatment of solid tumors can contribute to increase the survival rate of affected children and to decrease the treatment-related morbidity. Considering these aspects, the successful implementation of innovations is described based on four examples...
January 9, 2018: Der Chirurg; Zeitschrift Für Alle Gebiete der Operativen Medizen
https://www.readbyqxmd.com/read/29317557/prevalence-of-nonalcoholic-fatty-liver-disease-in-normal-weight-and-overweight-preadolescent-children-in-haryana-india
#8
Manoja Kumar Das, Vidyut Bhatia, Anupam Sibal, Abha Gupta, Sarath Gopalan, Raman Sardana, Reeti Sahni, Ankur Roy, Narendra K Arora
OBJECTIVE: To document the prevalence of non-alcoholic fatty liver disease (NAFLD) and metabolic parameters among normal-weight and overweight schoolchildren. STUDY DESIGN: Cross-sectional study. SETTING: Thirteen private schools in urban Faridabad, Haryana. PARTICIPANTS: 961 school children aged 5-10 years. METHODS: Ultrasound testing was done, and 215 with fatty liver on ultrasound underwent further clinical, biochemical and virological testing...
December 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/29316057/-a-promising-medium-term-follow-up-of-pediatric-sclerosing-cholangitis-mild-phenotype-or-early-diagnosis
#9
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/29315136/renal-replacement-therapy-in-the-critically-ill-child
#10
Claire A Westrope, Sarah Fleming, Melpo Kapetanstrataki, Roger C Parslow, Kevin P Morris
OBJECTIVES: Although renal replacement therapy is widely used in critically ill children, there have been few comprehensive population-based studies of its use. This article describes renal replacement therapy use, and associated outcomes, in critically ill children across the United Kingdom in the largest cohort study of this patient group. DESIGN: A retrospective observational study using prospectively collected data. SETTING: Data from the Pediatric Intensive Care Audit Network database which collects data on all children admitted to U...
January 8, 2018: Pediatric Critical Care Medicine
https://www.readbyqxmd.com/read/29314568/genetic-determinants-of-hepatic-steatosis-and-serum-cytokeratin-18-fragment-levels-in-taiwanese-children
#11
Yu-Cheng Lin, Pi-Feng Chang, Mei-Hwei Chang, Yen-Hsuan Ni
BACKGROUND/AIMS: There are substantial genetic components contributing to the susceptibility of nonalcoholic NAFLD disease (NAFLD). It has recently been reported that the rs641738 C>T variant in the membrane-bound O-acyltransferase domain-containing protein 7 (MBOAT7) gene increased severity of NAFLD in adults of European descent. We aimed to test the hypothesis that MBOAT7 rs641738 variant would increase hepatic steatosis and hepatocellular injury in obese children. METHODS: A total of 831 obese children aged 7-15 years were recruited...
January 4, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29305416/involvement-of-hepcidin-in-iron-metabolism-dysregulation-in-gaucher-disease
#12
Thibaud Lefebvre, Niloofar Reihani, Raed Daher, Thierry Billette de Villemeur, Nadia Belmatoug, Christian Rose, Yves Colin-Aronovicz, Hervé Puy, Caroline Le Van Kim, Mélanie Franco, Zoubida Karim
Gaucher disease is an inherited deficiency of glucocerebrosidase leading to accumulation of glucosylceramide in tissues such as the spleen, liver, and bone marrow. The resulting lipid-laden macrophages lead to the appearance of Gaucher cells. Anemia associated with an unexplained hyperferritinemia is a frequent finding in Gaucher disease, but whether this pathogenesis is related to an iron metabolism disorder has remained unclear. To investigate this issue, we explored the iron status of a large cohort of 90 type I Gaucher disease patients, including 66 patients treated with enzyme replacement therapy...
January 5, 2018: Haematologica
https://www.readbyqxmd.com/read/29303884/the-first-episode-of-spontaneous-bacterial-peritonitis-is-a-threat-event-in-children-with-end-stage-liver-disease
#13
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
#14
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/29302509/a-novel-homozygous-lipa-mutation-in-a-korean-child-with-lysosomal-acid-lipase-deficiency
#15
Kwang Yeon Kim, Ju Whi Kim, Kyung Jae Lee, Eunhyang Park, Gyeong Hoon Kang, Young Hun Choi, Woo Sun Kim, Jung Min Ko, Jin Soo Moon, Jae Sung Ko
Patients with lysosomal acid lipase (LAL) deficiency and glycogen storage disease (GSD) demonstrated hepatomegaly and dyslipidemia. In our case, a 6-year-old boy presented with hepatosplenomegaly. At 3 years of age, GSD had been diagnosed by liver biopsy at another hospital. He showed elevated serum liver enzymes and dyslipidemia. Liver biopsy revealed diffuse microvesicular fatty changes in hepatocytes, septal fibrosis and foamy macrophages. Ultrastructural examination demonstrated numerous lysosomes that contained lipid material and intracytoplasmic cholesterol clefts...
December 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/29302503/celiac-disease-in-south-jordan
#16
Eyad Altamimi
Purpose: Celiac disease, an autoimmune enteropathy triggered by exposure to gluten, is not uncommon in South Jordan. However, its prevalence is underestimated due to lack of physician awareness of the diversity of disease presentation. The clinical spectrum includes classic gastrointestinal manifestations, as well as rickets, iron-deficiency anemia, short stature, elevated liver enzymes, and edema. Our goal was to evaluate celiac disease presentation in clinically diagnosed children. Methods: Retrospective study included all children diagnosed with celiac disease between September 2009 and September 2015...
December 2017: Pediatric Gastroenterology, Hepatology & Nutrition
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/29296904/soluble-interleukin-2-receptor-is-a-sensitive-diagnostic-test-in-adult-hlh
#19
Anna Hayden, Molly Lin, Sujin Park, Morris Pudek, Marion Schneider, Michael B Jordan, Andre Mattman, Luke Y C Chen
Serum soluble interleukin-2 receptor (sIL-2r) is an important disease marker in hemophagocytic lymphohistiocytosis (HLH), but there are no published data on its diagnostic value in adults. We conducted a single-center retrospective study of 78 consecutive adults who had sIL-2r measured for suspected HLH. Serum sIL-2r levels were measured by enzyme-linked immunosorbent assay (adult reference range, 241-846 U/mL). There were 38 patients with HLH and 40 with a non-HLH diagnosis (such as sepsis, liver disease, histiocyte disorders, autoimmune disease, leukemia, or lymphoma)...
December 12, 2017: Blood Advances
https://www.readbyqxmd.com/read/29296723/atg-vs-thiotepa-with-busulfan-and-cyclophosphamide-in-matched-related-bone-marrow-transplantation-for-thalassemia
#20
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
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