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Chronic liver disease children

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https://www.readbyqxmd.com/read/28814423/portal-hypertension-and-its-management-in-children
#1
REVIEW
Tassos Grammatikopoulos, Patrick James McKiernan, Anil Dhawan
Portal hypertension (PHT), defined as raised intravascular pressure in the portal system, is a complication of chronic liver disease or liver vascular occlusion. Advances in our ability to diagnose and monitor the condition but also predict the risk of gastrointestinal bleeding have enabled us to optimise the management of children with PHT either at a surveillance or at a postbleeding stage. A consensus among paediatric centres in the classification of varices can be beneficial in streamlining future paediatric studies...
August 16, 2017: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/28776642/diagnosis-of-monogenic-liver-diseases-in-childhood-by-next-generation-sequencing
#2
Amelie Stalke, Britta Skawran, Bernd Auber, Thomas Illig, Brigitte Schlegelberger, Norman Junge, Imeke Goldschmidt, Christoph Leiskau, Nils von Neuhoff, Ulrich Baumann, Eva-Doreen Pfister
Next-Generation Sequencing (NGS) has opened up novel diagnostic opportunities for children with unidentified, but suspected inherited diseases. We describe our single-center experience with NGS diagnostics in standard clinical scenarios in pediatric hepatology. We investigated 135 children with suspected inherited hepatopathies, where initially no causative pathogenic variant had been identified, with an amplicon-based NGS panel of 21 genes associated with acute and chronic hepatopathies. In 23 of these patients we detected pathogenic or likely pathogenic variants in ten different genes...
August 4, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28770975/paracetamol-acetaminophen-for-chronic-non-cancer-pain-in-children-and-adolescents
#3
REVIEW
Tess E Cooper, Emma Fisher, Brian Anderson, Nick Mr Wilkinson, David G Williams, Christopher Eccleston
BACKGROUND: Pain is a common feature of childhood and adolescence around the world, and for many young people, that pain is chronic. The World Health Organization guidelines for pharmacological treatments for children's persisting pain acknowledge that pain in children is a major public health concern of high significance in most parts of the world. While in the past, pain was largely dismissed and was frequently left untreated, views on children's pain have changed over time, and relief of pain is now seen as important...
August 2, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28767077/gut-liver-axis-derangement-in-non-alcoholic-fatty-liver-disease
#4
REVIEW
Marco Poeta, Luca Pierri, Pietro Vajro
Non-alcoholic fatty liver disease (NAFLD) is the most frequent type of chronic liver disease in the pediatric age group, paralleling an obesity pandemic. A "multiple-hit" hypothesis has been invoked to explain its pathogenesis. The "first hit" is liver lipid accumulation in obese children with insulin resistance. In the absence of significant lifestyle modifications leading to weight loss and increased physical activity, other factors may act as "second hits" implicated in liver damage progression leading to more severe forms of inflammation and hepatic fibrosis...
August 2, 2017: Children
https://www.readbyqxmd.com/read/28759094/conversion-of-sugar-to-fat-is-hepatic-de-novo-lipogenesis-leading-to-metabolic-syndrome-and-associated-chronic-diseases
#5
Jean-Marc Schwarz, Michael Clearfield, Kathleen Mulligan
Epidemiologic studies suggest a link between excess sugar consumption and obesity, fatty liver disease, metabolic syndrome, and type 2 diabetes mellitus. One important pathway that may link these metabolic diseases to sugar consumption is hepatic conversion of sugar to fat, a process known as de novo lipogenesis (DNL). Mechanistic studies have shown that diets high in simple sugars increase both DNL and liver fat. Importantly, removal of sugar from diets of children with obesity for only 9 days consistently reduced DNL and liver fat and improved glucose and lipid metabolism...
August 1, 2017: Journal of the American Osteopathic Association
https://www.readbyqxmd.com/read/28752463/semiquantitation-of-monomer-and-polymer-alpha-1-antitrypsin-by-centrifugal-separation-and-assay-by-western-blot-of-soluble-and-insoluble-components
#6
Keith S Blomenkamp, Jeffrey H Teckman
Alpha-1 antitrypsin (a1AT) deficiency, in its classical form, is an autosomal recessive disease associated with an increased risk of liver disease in adults and children, and with lung disease in adults. The vast majority of liver disease is associated with homozygosity for the Z mutant allele, also called PiZZ. This homozygous allele synthesizes large quantities of a1AT mutant Z protein in the liver, but the mutant protein also folds improperly during biogenesis. As a result, approximately 85% of the molecules are retained within the hepatocytes instead of being appropriately secreted...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28752441/pathophysiology-of-alpha-1-antitrypsin-deficiency-liver-disease
#7
Jeffrey H Teckman, Keith S Blomenkamp
Classical alpha-1 antitrypsin (a1AT) deficiency is an autosomal recessive disease associated with an increased risk of liver disease in adults and children, and with lung disease in adults (Teckman and Jain, Curr Gastroenterol Rep 16(1):367, 2014). The vast majority of the liver disease is associated with homozygosity for the Z mutant allele, the so-called PIZZ. These homozygous individuals synthesize large quantities of a1AT mutant Z protein in the liver, but the mutant protein folds improperly during biogenesis and approximately 85% of the molecules are retained within the hepatocytes rather than appropriately secreted...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28731917/the-impact-of-iron-overload-in-acute-leukemia-chronic-inflammation-but-not-the-presence-of-nontransferrin-bound-iron-is-a-determinant-of-oxidative-stress
#8
Lale Olcay, Mustafa Serteser, Murat Kolay, Havva F Balci, Ülkü M Yildirim, Sibel A Tekgündüz, Tuncay Hazirolan, Yunus K Terzi
In the literature, studies on the oxidant effects of nontransferrin bound iron [NTBI (eLPI assay)] during chemotherapy of acute lymphoblastic leukemia and acute myeloblastic leukemia are lacking. We established NTBI and oxidative stress determinants (OSD), iron parameters, high-sensitive C-reactive protein (hs-CRP) levels, liver tests, cumulative chemotherapeutic doses, and transfused blood in 36 children with acute leukemia throughout chemotherapy. These parameters were determined at the beginning and end of chemotherapy blocks (11 time points) and in 20 healthy children using enzyme-linked immunosorbent assay, and colorimetric and fluorometric enzymatic methods...
August 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28715116/delayed-transplantation-may-affect-intellectual-ability-in-children
#9
Jiwon M Lee, Yeon Kyung Jung, Jeong-Hoon Bae, Sun Ah Yoon, Ju Hee Kim, YoungRok Choi, Hyeyoung Kim, Kwang-Woong Lee, Hye Young Ahn, Jae Won Kim, Min-Sup Shin, Kyung-Suk Suh, Il-Soo Ha, Hae Il Cheong, Hee Gyung Kang, Nam-Joon Yi
BACKGROUND: Decline in neurocognitive function is a reported complication in children with chronic illness. Concerns have been increasing that exposure to a major surgery or trauma may negatively impact cognitive performance in children. This study has evaluated cognitive function in 43 Korean children who received organ transplantation, and sought to identify associated clinical factors. METHODS: Pediatric recipients of kidney (KT) or liver transplantation (LT) from years 1999 to 2011 were recruited for cognitive tests...
July 17, 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28704229/treatment-of-hyponatremic-encephalopathy-in-the-critically-ill
#10
Steven G Achinger, Juan Carlos Ayus
OBJECTIVES: Hyponatremic encephalopathy, symptomatic cerebral edema due to a low osmolar state, is a medical emergency and often encountered in the ICU setting. This article provides a critical appraisal and review of the literature on identification of high-risk patients and the treatment of this life-threatening disorder. DATA SOURCES, STUDY SELECTION, AND DATA EXTRACTION: Online search of the PubMed database and manual review of articles involving risk factors for hyponatremic encephalopathy and treatment of hyponatremic encephalopathy in critical illness...
July 11, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28699612/relationship-between-immune-parameters-and-non-alcoholic-fatty-liver-disease-in-obese-children
#11
Jun-Qi Shi, Wen-Xia Shen, Xiang-Zhi Wang, Ke Huang, Chao-Chun Zou
OBJECTIVE: To investigate the relationship between immune parameters and non-alcoholic fatty liver disease (NAFLD) in obese children. DESIGN: Case-control study. SETTING: Zhejiang Province, China on July to September 2015. PARTICIPANTS: A total of 117 obese children and 209 healthy non-obese children were studied as the obese and control groups. Depending on the severity of NAFLD, the obese group was divided into subgroups 1 (without NAFLD), 2 (with simple fatty liver) and 3 (with steatohepatitis)...
July 11, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/28690716/wilson-s-disease-and-diagnostic-conundrum-in-a-low-income-country
#12
Pratap Kumar Patra
Wilson's disease is a well-known leading cause of chronic liver disease in children. However it may remain undiagnosed in a resource limited setting for a long period. We describe a six year male child diagnosed Wilson's disease with extreme elevation of liver enzymes which is not reported earlier. The diagnosis was also baffling because of inconsistency of other laboratory parameters.
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28686220/the-association-between-non-alcoholic-fatty-liver-disease-and-cardiovascular-risk-in-children
#13
REVIEW
Anna Di Sessa, Giuseppina Rosaria Umano, Emanuele Miraglia Del Giudice
The rising prevalence of childhood obesity in the past decades has made Non-Alcoholic Fatty Liver Disease (NAFLD) the most common cause of pediatric chronic liver disease worldwide. Currently, a growing body of evidence links NAFLD with cardiovascular disease (CVD) even at an early age. Data on the pediatric population have shown that NAFLD could represent an independent risk factor not only for cardiovascular events but also for early subclinical abnormalities in myocardial structure and function. Briefly, we review the current knowledge regarding the relationship between pediatric NAFLD and cardiovascular risk in an attempt to clarify our understanding of NAFLD as a possible cardiovascular risk factor in childhood...
July 7, 2017: Children
https://www.readbyqxmd.com/read/28675359/the-romanian-national-program-for-liver-transplantation-852-procedures-in-815-patients-over-17-years-2000-2017-a-continuous-evolution-to-success
#14
Irinel Popescu, Mihnea Ionescu, Vladislav Braşoveanu, Doina Hrehoreţ, Narcis Copca, Cristian Lupaşcu, Florin Botea, Bogdan Dorobanţu, Sorin Alexandrescu, Mihai Grigorie, Emil Matei, Radu Zamfir, Vasile Lungu, Dana Tomescu, Gabriela Droc, Daniela Ungureanu, Ruxandra Fota, Graţiela Manga, Mihai Popescu, Laura Popa, Liana Gheorghe, Speranţa Iacob, Corina Pietrăreanu, Mariana Mihailă, Laurenţiu Mic, Sanda Constantinescu, Cristian Gheorghe, Bogdan Cotruta, Ioana Lupescu, Mugur Grasu, Mirela Boroş, Radu Dumitru, Mihai Toma, Liliana Paslaru, Laura Vlad, Ileana Constantinescu, Ileana Dima, Vlad Herlea, Gabriel Becheanu, Cătălin Pecheanu, Daniela Sasalovici
Background: Liver transplantation (LT) has become an established treatment for end-stage liver disease, with more than 20.000 procedures yearly worldwide. The aim of this study was to analyze the results of Romanian National Program of LT. Methods: Between April 2000 and April 2017, 817 pts received 852 LTs in Romania. Male/female ratio was 487/330, while adult/pediatric ratio was 753/64, with a mean age of 46 years (median 50 yrs; range 7 months - 68 yrs). Main LT indications were HBV cirrhosis (230 pts; 28...
May 2017: Chirurgia
https://www.readbyqxmd.com/read/28663066/successful-management-of-acute-liver-failure-in-italian-children-a-16-year-experience-at-a-referral-centre-for-paediatric-liver-transplantation
#15
A Di Giorgio, A Sonzogni, A Piccichè, G Alessio, E Bonanomi, M Colledan, L D'Antiga
BACKGROUND: Identifying the causes of acute liver failure (ALF) and predictors of death or liver transplantation (LTX) is crucial to decide its management. We aimed to describe features and outcome of ALF in Italian children. METHODS: Retrospective review of cases presenting between 1996-2012. ALF was defined by high transaminases, INR ≥2.0 regardless of hepatic encephalopathy (HE), no evidence of underlying chronic liver disease. RESULTS: 55 children (median age 2...
June 12, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28658434/language-assessment-of-children-with-severe-liver-disease-in-a-public-service-in-brazil
#16
Erica Macêdo de-Paula, Gilda Porta, Ana Cristina Aoun Tannuri, Uenis Tannuri, Debora Maria Befi-Lopes
OBJECTIVE: The aim of this research was to compare language development (expressive and receptive skills) in children awaiting liver transplantation with that of children who have already undergone the surgical procedure. METHODS: An observational, descriptive, cross-sectional study was conducted with 76 children divided into groups, as follows: 31 children who were candidates for liver transplantation (Group 1; G1), 45 children who had already undergone liver transplantation (Group 2; G2), and a control group (CG) of 60 healthy, normally developing children...
June 2017: Clinics
https://www.readbyqxmd.com/read/28638943/elevated-serum-heat-shock-protein-70-and-liver-stiffness-reflect-hepatic-dysfunction-and-severity-in-postoperative-biliary-atresia
#17
Sittisak Honsawek, Wanvisa Udomsinprasert, Napaphat Jirathanathornnukul, Voranush Chongsrisawat, Yong Poovorawan
BACKGROUND: Biliary atresia (BA) is a severe chronic liver disease characterized by progressive obstructive cholangiopathy of biliary tract. Heat shock protein 70 (HSP70) is involved in protecting cells against a wide variety of stress and plays a protective role in tissue damage. The purpose of this study was to investigate serum HSP70 and liver stiffness in BA and determine the association of serum HSP70, liver stiffness, and outcome parameters in post-Kasai BA patients. METHODS: One hundred post-Kasai BA patients and 40 controls were enrolled...
August 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28635067/maternal-coping-with-the-prospect-of-liver-transplant-among-their-school-age-children
#18
Katsuhiro Hiratsuka, Nobue Nakamura, Naho Sato
The purpose of the current study was to describe the following: maternal coping with the prospect of becoming the living-donor liver transplant for their child; the daily lives of school-age children surviving biliary atresia with their native liver; and to explore the relationship between these individuals. Semistructured interviews were conducted with 6 school-age children surviving biliary atresia with their native liver and their mothers. The interviews were conducted from June to August 2014, and a qualitative content analysis was used...
June 2017: International Journal of Nursing Practice
https://www.readbyqxmd.com/read/28625038/chronic-liver-disease-is-a-risk-factor-for-malnutrition-and-growth-retardation-in-children
#19
Ariani Dewi Widodo, Eva J Soelaeman, Novitria Dwinanda, Prajnya P Narendraswari, Budi Purnomo
BACKGROUND: Despite distinct advancements in nutritional therapy, malnutrition and growth retardation remain inevitable consequences of chronic liver disease. The global prevalence of chronic liver disease in children is about 3%, with a quarter undernourished. Malnutrition itself is a negative prognostic indicator of survival. Further research is necessary for delivering adequate nutritional support to reduce morbidity and mortality. OBJECTIVE: To evaluate the nutritional status and growth of children with chronic liver disease and its contributing factors...
June 2017: Asia Pacific Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/28608806/targeting-overconsumption-of-sugar-sweetened-beverages-vs-overall-poor-diet-quality-for-cardiometabolic-diseases-risk-prevention-place-your-bets
#20
REVIEW
Benoit J Arsenault, Benoît Lamarche, Jean-Pierre Després
Chronic overconsumption of sugar-sweetened beverages (SSBs) is amongst the dietary factors most consistently found to be associated with obesity, type 2 diabetes (T2D) and cardiovascular disease (CVD) risk in large epidemiological studies. Intervention studies have shown that SSB overconsumption increases intra-abdominal obesity and ectopic lipid deposition in the liver, and also exacerbates cardiometabolic risk. Similar to the prevalence of obesity and T2D, national surveys of food consumption have shown that chronic overconsumption of SSBs is skyrocketing in many parts of the world, yet with marked heterogeneity across countries...
June 13, 2017: Nutrients
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