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Metabolic liver disease children

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https://www.readbyqxmd.com/read/29215054/in-vivo-redox-metabolic-imaging-of-mitochondria-assesses-disease-progression-in-non-alcoholic-steatohepatitis
#1
Ryosuke Nakata, Fuminori Hyodo, Masaharu Murata, Hinako Eto, Tomoko Nakaji, Takahito Kawano, Sayoko Narahara, Keiji Yasukawa, Tomohiko Akahoshi, Morimasa Tomikawa, Makoto Hashizume
Given the rising incidence of non-alcoholic fatty liver disease (NAFLD) in both adults and children, the development of a non-invasive diagnostic method for assessing disease progression to non-alcoholic steatohepatitis (NASH) has become an important research goal. Currently available non-invasive imaging technologies are only able to assess fat accumulation in the liver. Therefore, these methods are not suitable for a precise diagnosis of NASH. The standard diagnostic technique for NASH, liver biopsy, has several drawbacks, including the higher risk of complications that accompanies invasive procedures...
December 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29209135/first-case-of-cross-auxiliary-double-domino-donor-liver-transplantation
#2
Zhi-Jun Zhu, Lin Wei, Wei Qu, Li-Ying Sun, Ying Liu, Zhi-Gui Zeng, Liang Zhang, En-Hui He, Hai-Ming Zhang, Ji-Dong Jia, Zhong-Tao Zhang
We report a case of double domino liver transplantation in a 32-year-old woman who was diagnosed with familial amyloid polyneuropathy (FAP) and liver dysfunction. A two-stage surgical plan was designed, and one domino graft was implanted during each stage. During the first stage, an auxiliary domino liver transplantation was conducted using a domino graft from a 4-year-old female child with Wilson's disease. After removing the right lobe of the FAP patient's liver, the graft was rotated 90 degrees counterclockwise and placed along the right side of the inferior vena cava (IVC)...
November 28, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29187144/non-alcoholic-fatty-liver-disease-and-its-associations-among-adolescents-in-an-urban-sri-lankan-community
#3
Shaman Rajindrajith, Arunasalam Pathmeswaran, Chamilka Jayasinghe, Dulani Kottahachchi, Anuradhani Kasturiratne, Shamila T de Silva, Madunil A Niriella, Anuradha S Dassanayake, Arjuna P de Silva, H Janaka de Silva
BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is a common problem across the world. We aimed to determine the prevalence of NAFLD and its associations in Sri Lankan adolescents living in an urban Sri Lankan community. METHOD: The study population consisted of the birth cohort of the year 2000, residing in the Ragama Medical Officer of Health area. Socio-demographic and anthropometric data [anthropometric measurements, blood pressure and total body fat distribution] of these adolescents were collected by trained data collectors...
November 29, 2017: BMC Gastroenterology
https://www.readbyqxmd.com/read/29147139/insights-into-the-management-of-wilson-s-disease
#4
REVIEW
Mohmadshakil Kathawala, Gideon M Hirschfield
Wilson's disease is a rare, inherited autosomal recessive disease of copper metabolism, in which the causative gene, ATP7B, results in absent or reduced function of the ATP7B transporter important for biliary excretion of copper and incorporation of copper into caeruloplasmin. Affected patients accumulate excessive copper within the liver, brain and other tissues. A disease mainly of children, adolescents and young adults; clinical features vary from the asymptomatic state to chronic liver disease, acute liver failure, and neuropsychiatric manifestations...
November 2017: Therapeutic Advances in Gastroenterology
https://www.readbyqxmd.com/read/29134448/hyperammonemia-associated-with-distal-renal-tubular-acidosis-or-urinary-tract-infection-a-systematic-review
#5
Caterina M Clericetti, Gregorio P Milani, Sebastiano A G Lava, Mario G Bianchetti, Giacomo D Simonetti, Olivier Giannini
BACKGROUND: Hyperammonemia usually results from an inborn error of metabolism or from an advanced liver disease. Individual case reports suggest that both distal renal tubular acidosis and urinary tract infection may also result in hyperammonemia. METHODS: A systematic review of the literature on hyperammonemia secondary to distal renal tubular acidosis and urinary tract infection was conducted. RESULTS: We identified 39 reports on distal renal tubular acidosis or urinary tract infections in association with hyperammonemia published between 1980 and 2017...
November 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29132978/intensive-care-in-severe-malaria-report-from-the-task-force-on-tropical-diseases-by-the-world-federation-of-societies-of-intensive-and-critical-care-medicine
#6
REVIEW
Dilip R Karnad, Mohd Basri Mat Nor, Guy A Richards, Tim Baker, Pravin Amin
Severe malaria is common in tropical countries in Africa, Asia, Oceania and South and Central America. It may also occur in travelers returning from endemic areas. Plasmodium falciparum accounts for most cases, although P vivax is increasingly found to cause severe malaria in Asia. Cerebral malaria is common in children in Africa, manifests as coma and seizures, and has a high morbidity and mortality. In other regions, adults may also develop cerebral malaria but neurological sequelae in survivors are rare...
November 8, 2017: Journal of Critical Care
https://www.readbyqxmd.com/read/29122442/serum-fetuin-a-levels-in-obese-children-with-biopsy-proven-nonalcoholic-fatty-liver-disease
#7
V Pampanini, E Inzaghi, D Germani, A Alterio, A Puglianiello, A Alisi, V Nobili, S Cianfarani
BACKGROUND AND AIMS: Fetuin-A has been proposed as a marker of liver damage in adults with obesity-related NAFLD. The aim of this study was to test serum fetuin-A concentrations in obese children with NAFLD diagnosed either by ultrasonography or by liver biopsy and to determine its applicability as predictive tool in pediatric NAFLD. METHODS AND RESULTS: Metabolic parameters and fetuin-A levels were investigated in 81 obese children with NAFLD diagnosed by biopsy, 79 obese children with NAFLD defined by liver ultrasonography and 23 lean subjects...
October 3, 2017: Nutrition, Metabolism, and Cardiovascular Diseases: NMCD
https://www.readbyqxmd.com/read/29118811/obese-children-with-metabolic-syndrome-have-3-times-higher-risk-to-have-nonalcoholic-fatty-liver-disease-compared-with-those-without-metabolic-syndrome
#8
Dimitrios Papandreou, Mirey Karavetian, Zacharoula Karabouta, Eleni Andreou
Background: The aim of this study was to investigate the relationship between metabolic syndrome (MS) and nonalcoholic fatty liver disease (NAFLD) in obese children. One hundred and twenty-five subjects aged 11-12 years old participated in the study. Methods: Anthropometric and biochemical indices were measured, including lipid and liver profile, blood glucose, serum insulin, and liver ultrasound. Results: Forty-four children (58.6%) were found to have MS...
2017: International Journal of Endocrinology
https://www.readbyqxmd.com/read/29108191/-an-interpretation-of-the-aasld-practice-guideline-on-the-diagnosis-and-management-of-nonalcoholic-fatty-liver-disease-in-2017
#9
Y M Nan, N Fu, W C Li, L L Kong, X W Yuan, S Y Zhang, L D Liu, Y Lu, L Y Cui
The American Association for the Study of Liver Diseases (AASLD) updated and published the Practice Guidance for the Diagnosis and Management of Nonalcoholic Fatty Liver Disease (NAFLD) in July 2017, which provides recommendations for the accurate diagnosis, treatment, and effective prevention of NAFLD. Related metabolic diseases should be considered during the initial evaluation of patients suspected of NAFLD. Noninvasive diagnostic techniques including transient elastography, magnetic resonance elastography, and serum biochemical models should be used to evaluate the development and progression of liver fibrosis in patients with NAFLD...
September 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29080393/lipid-profile-pattern-in-pediatric-overweight-population-with-or-without-nafld-in-relation-to-idf-criteria-for-metabolic-syndrome-a-preliminary-study
#10
Anca Bălănescu, Paul Bălănescu, Valentina Comanici, Iustina Stan, Beata Acs, Laura Prisăcariu, Florin Brezan, Tatiana Ciomârtan, Ioan Gherghina
BACKGROUND AND AIMS: The aim of this study is to assess the lipid profile pattern of pediatric overweight and/or obese patients with Non-Alcoholic Fatty Liver Disease (NAFLD) in relation to IDF Consensus Criteria for Metabolic Syndrome (MetS). MATERIAL AND METHODS: We conducted a cross-sectional preliminary study on 45 consecutive pediatric patients. Overweight or obese children aged from 3 to 18 years were included. Standardized measurement of blood pressure and anthropometric parameters were performed...
October 26, 2017: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
https://www.readbyqxmd.com/read/29080241/next-generation-sequencing-in-pediatric-hepatology-and-liver-transplantation
#11
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). Since nearly half of children with chronic liver disorders have a genetic cause and approximately 20% of pediatric liver transplants 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 work up in unclear clinical scenarios...
October 28, 2017: Liver Transplantation
https://www.readbyqxmd.com/read/29059700/favorable-response-to-immunosuppressive-combination-therapy-with-mizoribine-and-azathioprine-in-children-with-primary-sclerosing-cholangitis
#12
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...
October 23, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29046229/fetal-overnutrition-and-adolescent-hepatic-fat-fraction-the-exploring-perinatal-outcomes-in-children-study
#13
Anna Bellatorre, Ann Scherzinger, Elizabeth Stamm, Mercedes Martinez, Brandy Ringham, Dana Dabelea
OBJECTIVE: To determine if fetal overnutrition resulting from maternal obesity or gestational diabetes mellitus (GDM) is associated with increased liver fat during adolescence, adjusting for past and current metabolic risk factors. STUDY DESIGN: Data come from a historical prospective cohort study (Exploring Perinatal Outcomes in Children) of 254 mother-child pairs in Colorado who participated in 2 research visits at T1 (mean age 10.4, SD = 1.5 years) and at T2 (mean age 16...
October 15, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/29046209/-paroxysmal-muscle-weakness-liver-enlargement-and-hypoglycemia-in-a-boy
#14
Ya-Jie Cui, Chun-Lan Song, Yi-Bing Cheng
A boy aged 11 years was admitted due to intermittent weakness and difficulty in walking for 6 years, and hepatomegaly, glycopenia and unconsciousness for 4 years. The laboratory examinations showed severe metabolic acidosis, hypoglycemia, and abnormal liver function. CT scan showed marked liver enlargement with fat density shadow. The boy was given fluid infusion, correction of acidosis, intravenous injection of glucose, L-carnitine, compound vitamin B, and coenzyme Q10, but he was in a persistent coma and it was difficult to correct refractory metabolic acidosis and hypoglycemia...
October 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/29039873/tillf%C3%A3-rlitliga-referensintervall-kr%C3%A3-vs-f%C3%A3-r-v%C3%A3-rdering-av-p-alp-nya-pediatriska-referensintervall-f%C3%A3-r-alkaliskt-fosfatas-har-klinisk-betydelse-f%C3%A3-r-att-hitta-r%C3%A3-tt-till-diagnosen
#15
Per Magnusson
Age- and gender-specific reference intervals are pivotal to ensure appropriate interpretation of plasma alkaline phosphatase activities in the lower range Hypophosphatasia (HPP) is an inborn error of metabolism caused by loss-of-function mutations of the ALPL gene that mainly express alkaline phosphatase (ALP) in bone and liver. The clinical expression of HPP is highly variable and is classified into six different forms mainly affecting bone and tooth mineralization. The prognosis for each of these HPP forms depends upon the severity of the skeletal disease which reflects the age at presentation...
October 16, 2017: Läkartidningen
https://www.readbyqxmd.com/read/28991837/serum-osteocalcin-levels-in-children-with-nonalcoholic-fatty-liver-disease
#16
Saleh Amin, Doaa El Amrousy, Shaymaa Elrifaey, Rasha Gamal, Hossam Hodeib
OBJECTIVES: to investigate the relationship between osteocalcin and non alcoholic fatty liver disease (NAFLD) in obese children. METHOD: 60 obese children with NAFLD were taken as patient group and 60 non - NAFLD children with matches age, sex and BMI were taken as control group. Anthropometric measurements, abdominal ultrasonography for diagnosis and grading of NAFLD, and laboratory investigations in the form of liver function tests, lipid profile, fasting serum glucose and insulin, and serum osteocalcin levels were done for all children...
October 6, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28988228/the-relationship-between-non-alcoholic-fatty-liver-disease-and-small-intestinal-bacterial-overgrowth-among-overweight-and-obese-children-and-adolescents
#17
Oana Belei, Laura Olariu, Andreea Dobrescu, Tamara Marcovici, Otilia Marginean
BACKGROUND: The increasing rate of obesity and overweight among children has highlighted nonalcoholic fatty liver disease (NAFLD) as the most common cause of chronic pediatric liver diseases. There are many publications supporting the idea that gut microbiota is altered in NAFLD. The aim of this study was to evaluate the prevalence of NAFLD among overweight and obese children with and without small intestinal bacterial overgrowth (SIBO) compared to a control group and to assess if intestinal dysbiosis represents a risk factor for NAFLD...
October 26, 2017: Journal of Pediatric Endocrinology & Metabolism: JPEM
https://www.readbyqxmd.com/read/28986475/pediatric-cytochrome-p450-activity-alterations-in-nonalcoholic-steatohepatitis
#18
Hui Li, Canet J Mark, John D Clarke, Dean Billheimer, Stavra A Xanthakos, Joel E Lavine, Robert P Erickson, Nathan J Cherrington
Variable drug responses(VDRs) are dependent upon individual variation in the activity of drug-metabolizing enzymes including cytochrome P450s(CYPs). As the most common chronic liver disease in children and adults, nonalcoholic steatohepatitis(NASH) has been identified as a source of significant inter-individual variation in hepatic drug metabolism. Compared to adults, children present age-related differences in pharmacokinetics and pharmacodynamics. The purpose of this study was to determine the impact of fatty liver disease severity on the activity of a variety of CYPs in children and adolescents...
October 6, 2017: Drug Metabolism and Disposition: the Biological Fate of Chemicals
https://www.readbyqxmd.com/read/28979846/the-transcription-factor-atf7-mediates-in-vitro-fertilization-induced-gene-expression-changes-in-mouse-liver
#19
Yang Liu, Toshio Maekawa, Keisuke Yoshida, Hideki Kaneda, Bruno Chatton, Shigeharu Wakana, Shunsuke Ishii
Assisted reproductive technologies, including in vitro fertilization (IVF), are now frequently used, and increasing evidence indicates that IVF causes gene expression changes in children and adolescents that increase the risk of metabolic diseases. Although such gene expression changes are thought to be due to IVF-induced epigenetic changes, the mechanism remains elusive. We tested whether the transcription factor ATF7-which mediates stress-induced changes in histone H3K9 tri- and dimethylation, typical marks of epigenetic silencing-is involved in the IVF-induced gene expression changes...
October 2017: FEBS Open Bio
https://www.readbyqxmd.com/read/28953935/association-of-mttp-gene-variants-with-pediatric-nafld-a-candidate-gene-based-analysis-of-single-nucleotide-variations-in-obese-children
#20
Dongling Dai, Feiqiu Wen, Shaoming Zhou, Zhe Su, Guosheng Liu, Mingbang Wang, Jianli Zhou, Fusheng He
OBJECTIVE: We used targeted next-generation sequencing to investigate whether genetic variants of lipid metabolism-related genes are associated with increased susceptibility to nonalcoholic fatty liver disease (NAFLD) in obese children. METHODS: A cohort of 100 obese children aged 6 to 18 years were divided into NAFLD and non-NAFLD groups and subjected to hepatic ultrasound, anthropometric, and biochemical analyses. We evaluated the association of genetic variants with NAFLD susceptibility by investigating the single nucleotide polymorphisms in each of 36 lipid-metabolism-related genes...
2017: PloS One
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