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Liver disease in children

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https://www.readbyqxmd.com/read/28646278/liver-involvement-in-kidney-disease-and-vice-versa
#1
REVIEW
Karen Van Hoeve, Djalila Mekahli, Eva Morava, Elena Levtchenko, Peter Witters
The liver and kidneys are often similarly affected by a single disease. This is the case in metabolic, immunological, toxic, and infectious diseases, and in the different congenital malformation syndromes. Also, an enzymatic defect in an otherwise healthy liver or the consequences of advanced liver disease by itself can cause kidney disease as a secondary phenomenon. In this review, we describe numerous pathogenic mechanisms leading to dysfunction or malformations of the liver and kidneys in children. We encourage multidisciplinary management for optimal care...
June 23, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28644566/clinical-characteristics-and-outcome-of-liver-transplantation-for-alagille-syndrome-in-children
#2
Tao Zhou, Jianjun Zhang, Yi Luo, Yuan Liu, Sy Zhuang, Feng Xue, Longzhi Han, Qiang Xia
AIM: The aim of this study was to analyze clinical characteristics and outcome of liver transplantation for Alagille syndrome in children. METHOD: By retrospectively reviewing the medical records of 9 Alagille syndrome patients(AGS) with liver transplantation(LT) in Renji Hospital between 2006 and 2015,After operative,the height and weight Z score were compared with preoperative. RESULT: There were 9 patients included in the study; cholestasis and peculiar faces were seen in all of the patients (100%), heart defect in 8 patients (88...
June 23, 2017: Journal of Digestive Diseases
https://www.readbyqxmd.com/read/28644356/low-hepatic-tissue-copper-in-pediatric-nonalcoholic-fatty-liver-disease
#3
Michael Mendoza, Shelley Caltharp, Ming Song, Lindsay Collin, Juna V Konomi, Craig J McClain, Miriam B Vos
OBJECTIVE: Animal models and studies in adults have demonstrated that copper restriction increases severity of liver injury in nonalcoholic fatty liver disease (NAFLD). This has not been studied in children. We aimed to determine if lower tissue copper is associated with increased NAFLD severity in children. METHODS: This was a retrospective study of pediatric patients who had a liver biopsy including a hepatic copper quantitation. The primary outcome compared hepatic copper concentration in NAFLD versus non-NAFLD...
July 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28644353/extraintestinal-manifestations-of-celiac-disease-effectiveness-of-the-gluten-free-diet
#4
Hilary Jericho, Naire Sansotta, Stefano Guandalini
OBJECTIVE: The aim of the study was to evaluate the effectiveness of the gluten-free diet (GFD) on extraintestinal symptoms in pediatric and adult celiac populations at the University of Chicago. METHODS: We conducted a retrospective chart review of the University of Chicago Celiac Center clinic charts from January 2002 to October 2014. Demographics, serologic testing, intestinal biopsies, and extraintestinal symptoms at presentation, 12, 24, and >24 months were recorded...
July 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28641648/-change-of-thrombelastography-in-children-s-dic-and-analysis-of-its-sensitivity-and-specificity-for-diagnosis-of-dic
#5
Wen-Hao Yuan, Han-Chu Liu, Ling-Kong Zeng, Xiao-Yan Liu, Ling-Xia Zhao, Lu-Xia Mo
OBJECTIVE: To investigate the influence of thrombelastography index changes on its sensitivity and specificity for diagnosis of disseminated intravascular coagulation (DIC) in children. METHODS: A total of 149 children with DIC in our hospital from June 2013 to June 2016 were selected in DIC group, while 106 cases of non-DIC, including healthy children and children with diseases easily confused with DIC, were selected as non-DIC(control) group. The thrombelastography, D-dimer, coagulation functions including prothrombintime (PT), activated partial thromboplastin time (APTT), fibrinogen (FIB) and fibin degradation product (FDP), congental coagulation disorders and platelet count were detected in DIC and non-DIC groups; the statistics of data was performed and the sensitivity and specificity of thromelastraphy indexes such as R time, α angle MA value and A value were evaluated; the relationship of DIC with indexes was analyzed...
June 2017: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/28639095/knowledge-awareness-and-prevention-of-hepatitis-b-virus-infection-among-korean-american-parents
#6
Sarah Hyun, Seulgi Lee, William R Ventura, Joseph McMenamin
Hepatitis B (HB) affects 240 million people around the world, and children and young adults make up a large proportion of the infected population. Approximately 1 million people die from HB each year. Despite the seriousness of HB and its complications, many are poorly linked to clinical care. A lack of health literacy may be a critical barrier hindering access to HB care for adults as well as children in these populations. We, therefore, performed a survey to assess the level of knowledge of HB among Korean American parents...
June 21, 2017: Journal of Immigrant and Minority Health
https://www.readbyqxmd.com/read/28638943/elevated-serum-heat-shock-protein-70-and-liver-stiffness-reflect-hepatic-dysfunction-and-severity-in-postoperative-biliary-atresia
#7
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...
June 21, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28635067/maternal-coping-with-the-prospect-of-liver-transplant-among-their-school-age-children
#8
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/28634626/hepatic-shear-wave-elastography-in-children-under-free-breathing-and-breath-hold-conditions
#9
Caroline Jung, Michael Groth, Kay Uwe Petersen, Anna Hammel, Florian Brinkert, Enke Grabhorn, Sören Alexander Weidemann, Jasmin Busch, Gerhard Adam, Jochen Herrmann
OBJECTIVES: To compare hepatic 2D shear wave elastography (2D SWE) in children between free-breathing and breath-hold conditions, in terms of measurement agreement and time expenditure. METHODS: A cohort of 57 children (12.7±4.3 years) who underwent standardized 2D SWE between May and October 2015 were retrospectively evaluated. Liver elastograms were obtained under free-breathing and breath-hold conditions and time expenditure was measured. Median stiffness, interquartile range (IQR), and IQR/median ratio were calculated based on 12, six, and three elastograms...
June 20, 2017: European Radiology
https://www.readbyqxmd.com/read/28632826/-pyogenic-liver-abscess-in-children-clinical-experience-in-a-pediatric-reference-center
#10
Carolina Carballo, Claudia Cazes, Mariana Matsuda, M Laura Praino, Noemí Rivas, Eduardo López
INTRODUCTION: Liver abscess is a serious and an uncommon pediatric disease. OBJECTIVE AND METHOD: Description of 20 pediatric patients with liver abscess admitted in "Ricardo Gutierrez" Children's Hospital, a Tertiary Reference Center of Buenos Aires, between 2009 and 2015. RESULTS: The hospitalization rate was 35/100.000 admissions. Median age was 5 years old, male/female ratio 4/1. Five patients (25%) had predisposing factors. Median days at diagnosis were 12...
April 2017: Revista Chilena de Infectología: órgano Oficial de la Sociedad Chilena de Infectología
https://www.readbyqxmd.com/read/28630222/acute-pneumatosis-cystoides-intestinalis-with-atrophic-desmosis-of-the-colon-in-a-child
#11
Blanca Schuster, Johannes Mayr
Acute pneumatosis cystoides intestinalis (PCI) has been described after bone marrow transplantation (BMT). Several case series have demonstrated successful conservative treatment of PCI in children. We present a child with Fanconi anaemia, who developed severe graft versus host disease of the gastrointestinal tract, skin and liver after BMT and an acute, severe form of PCI. Our case report illustrates the complexity of diagnostic and therapeutic procedures in PCI in immunocompromised children.
June 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28629372/novel-nbas-mutations-and-fever-related-recurrent-acute-liver-failure-in-chinese-children-a-retrospective-study
#12
Jia-Qi Li, Yi-Ling Qiu, Jing-Yu Gong, Li-Min Dou, Yi Lu, A S Knisely, Mei-Hong Zhang, Wei-Sha Luan, Jian-She Wang
BACKGROUND: Underlying causes in Chinese children with recurrent acute liver failure (RALF), including liver crises less than full acute liver failure, are incompletely understood. We sought to address this by searching for genes mutated in such children. METHODS: Five unrelated Chinese boys presenting between 2012 and 2015 with RALF of unexplained etiology were studied. Results of whole exome sequencing were screened for mutations in candidate genes. Mutations were verified in patients and their family members by Sanger sequencing...
June 19, 2017: BMC Gastroenterology
https://www.readbyqxmd.com/read/28627213/nonalcoholic-fatty-liver-disease-nafld-pathophysiology-in-obese-children-and-adolescents-update
#13
Silvana Neves Ferraz de Assunção, Ney Christian Boa Sorte, Crésio Dantas Alves, Patricia S Almeida Mendes, Carlos Roberto Brites Alves, Luciana Rodrigues Silva
OBJECTIVE: Although the nonalcoholic fatty liver disease was first identified in 1980, it presents multifactorial and unclear pathophysiology. In this review, we intend to update the pathophysiological mechanisms of a high morbidity and mortality associated disease that is affecting obese children worldwide. DATA SOURCES: The PubMed and the Cochrane Library databases were used in the search strategy for articles related to nonalcoholic fatty liver disease and published in the last three decades...
June 5, 2017: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/28625218/the-response-of-male-and-female-rats-to-a-high-fructose-diet-during-adolescence-following-early-administration-of-hibiscus-sabdariffa-aqueous-calyx-extracts
#14
K G Ibrahim, E Chivandi, F B O Mojiminiyi, K H Erlwanger
Metabolic syndrome is linked to the consumption of fructose-rich diets. Nutritional and pharmacological interventions perinatally can cause epigenetic changes that programme an individual to predispose or protect them from the development of metabolic diseases later. Hibiscus sabdariffa (HS) reportedly has anti-obesity and hypocholesterolaemic properties in adults. We investigated the impact of neonatal intake of HS on the programming of metabolism by fructose. A total of 85 4-day-old Sprague Dawley rats were divided randomly into three groups...
June 19, 2017: Journal of Developmental Origins of Health and Disease
https://www.readbyqxmd.com/read/28625038/chronic-liver-disease-is-a-risk-factor-for-malnutrition-and-growth-retardation-in-children
#15
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/28611971/diagnosis-and-management-of-hepatobiliary-complications-in-autosomal-recessive-polycystic-kidney-disease
#16
REVIEW
Andrew Wehrman, Alyssa Kriegermeier, Jessica Wen
Autosomal recessive polycystic kidney disease (ARPKD) is a congenital hepatorenal fibrocystic disease. The hepatic manifestations of ARPKD can range from asymptomatic to portal hypertension and massively dilated biliary system that results in liver transplantation. Hepatic complications of ARPKD typically present with signs of portal hypertension (splenomegaly and thrombocytopenia) or cholangitis. Liver disease in ARPKD does not always correlate with severity of renal disease. Management of ARPKD-related liver disease is largely treating specific symptoms, such as antibiotics for cholangitis or endoscopic treatment for variceal bleeding...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28611345/neuroblastoma-amplified-sequence-gene-mutation-a-rare-cause-of-recurrent-liver-failure-in-children
#17
Mohammed Y Hasosah, Alaa I Iskandarani, Ayman I Shawli, Ashraf F Alsahafi, Ghassan A Sukkar, Mansour A Qurashi
Neuroblastoma amplified sequence (NBAS) gene mutation or infantile liver failure syndrome type 2 (ILFS type 2) is an extremely rare disease characterized by episodic liver failure precipitated by intercurrent febrile illness, and liver function recovering completely. Here, we report a 4-year-old girl with recurrent hepatitis. A diagnosis of ILFS type 2 was made based on NBAS mutation gene found by whole-exome sequencing. Our case provides a new insight toward considering NBAS mutation as a part of the differential diagnoses of any infant presenting with recurrent liver failure or hepatitis...
May 2017: Saudi Journal of Gastroenterology: Official Journal of the Saudi Gastroenterology Association
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
#18
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
https://www.readbyqxmd.com/read/28608518/liver-transplantation-may-prevent-neurodevelopmental-deterioration-in-high-risk-patients-with-urea-cycle-disorders
#19
Jun Kido, Shirou Matsumoto, Ken Momosaki, Rieko Sakamoto, Hiroshi Mitsubuchi, Fumio Endo, Kimitoshi Nakamura
UCDs are among the most common inherited metabolic diseases in Japan. We investigated the clinical manifestations, treatment, and prognoses of 177 patients with UCDs who were evaluated and treated from January 1999 to March 2009 in Japan, using a questionnaire survey. Among these 177 patients, 42 (seven with carbamoyl phosphate synthetase 1 deficiency, 27 with ornithine transcarbamylase deficiency, seven with argininosuccinate synthetase deficiency, and one with arginase 1 deficiency) underwent living-donor LT...
June 12, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28606575/good-adherence-to-the-mediterranean-diet-reduces-the-risk-for-nash-and-diabetes-in-pediatric-patients-with-obesity-the-results-of-an-italian-study
#20
Claudia Della Corte, Antonella Mosca, Andrea Vania, Arianna Alterio, Salvatore Iasevoli, Valerio Nobili
OBJECTIVE: In the last decade, it was demonstrated that the Mediterranean diet (MD) represents an ideal diet for all age groups and has an important role in the prevention of metabolic and cardiovascular diseases, as well as nonalcoholic fatty liver disease (NAFLD). The aim of the present study was to analyze the association between adherence to the MD and NAFLD, with laboratory and histologic evaluation, in a group of children and adolescents with obesity. METHODS: We enrolled 243 patients with obesity referred to our department from March 2014 to November 2015...
July 2017: Nutrition
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