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Chronic liver disease children

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https://www.readbyqxmd.com/read/29209118/chronic-liver-disease-is-universal-in-children-with-biliary-atresia-living-with-native-liver
#1
Way Seah Lee, Sik Yong Ong, Hee Wei Foo, Shin Yee Wong, Chen Xi Kong, Ru Bin Seah, Ruey Terng Ng
AIM: To examine the medical status of children with biliary atresia (BA) surviving with native livers. METHODS: In this cross-sectional review, data collected included complications of chronic liver disease (CLD) (cholangitis in the preceding 12 mo, portal hypertension, variceal bleeding, fractures, hepatopulmonary syndrome, portopulmonary hypertension) and laboratory indices (white cell and platelet counts, total bilirubin, albumin, international normalized ratio, alanine aminotransferase, aspartate aminotransferase, γ-glutamyl transpeptidase)...
November 21, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/29201174/effects-of-activated-carbon-n-acetylcysteine-sustained-release-microcapsule-on-dipeptidyl-peptidase-iv-expression-in-young-rats-with-non-alcoholic-fatty-liver-disease
#2
Hongping Zhou, Tingting Shi, Jun Yan, Xiaojin Chen, Li Liao, Shiyong Zhao, Hongying Fang, Rangxiao Zhuang
Non-alcoholic fatty liver disease (NAFLD) in children has become the most common liver disease influencing adolescent health and one of the most influencing chronic liver diseases among children in Chinese wealthy families, particularly in coastal regions. However, the medicine available for the treatment of NAFLD is deficient. In order to solve this problem, our team studied the activated carbon N-acetylcysteine (NAC) sustained-release microcapsule, which improves the oxidation resistance, bioavailability and drug stability of acetylcysteine and reduces toxic and side effects...
November 2017: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29177681/clinical-strategy-of-diagnosing-and-following-patients-with-nonalcoholic-fatty-liver-disease-based-on-invasive-and-noninvasive-methods
#3
REVIEW
Masato Yoneda, Kento Imajo, Hirokazu Takahashi, Yuji Ogawa, Yuichiro Eguchi, Yoshio Sumida, Masashi Yoneda, Miwa Kawanaka, Satoru Saito, Katsutoshi Tokushige, Atsushi Nakajima
Nonalcoholic fatty liver disease (NAFLD) is an important cause of chronic liver injury in many countries. The incidence of NAFLD is rising rapidly in both adults and children, because of the currently ongoing epidemics of obesity and type 2 diabetes. Notably, histological liver fibrosis is recognized as the main predictive factor for the overall long-term outcome of NAFLD, including cardiovascular disease and liver-related mortality. Thus, staging of liver fibrosis is essential in determining the prognosis and optimal treatment for patients with NAFLD and in guiding surveillance for the development of hepatocellular carcinoma (HCC)...
November 24, 2017: Journal of 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/29146214/-treatments-of-steroid-dependent-nephrotic-syndrome-in-children
#5
A Couderc, E Bérard, V Guigonis, I Vrillon, J Hogan, V Audard, V Baudouin, C Dossier, O Boyer
Primary nephrotic syndrome (NS) is the most common glomerular disease in children. It is characterized by massive proteinuria and hypoalbuminemia. It typically has a sudden onset and more than 70% of patients will experience at least one relapse. An immunological origin has long been postulated, although the precise molecular mechanisms underlying the disease remain debated. Steroids are the first-line therapy with cumulative dose and duration of initial treatment varying among countries. Steroid-sparing agents may be indicated in case of steroid-dependency or frequent relapses...
November 13, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29128761/inborn-errors-of-immunity-underlying-fungal-diseases-in-otherwise-healthy-individuals
#6
REVIEW
Juan Li, Donald C Vinh, Jean-Laurent Casanova, Anne Puel
It has been estimated that there are at least 1.5 million fungal species, mostly present in the environment, but only a few of these fungi cause human disease. Most fungal diseases are self-healing and benign, but some are chronic or life-threatening. Acquired and inherited defects of immunity, including breaches of mucocutaneous barriers and circulating leukocyte deficiencies, account for most severe modern-day mycoses. Other types of infection typically accompany these fungal infections. More rarely, severe fungal diseases can strike otherwise healthy individuals...
November 9, 2017: Current Opinion in Microbiology
https://www.readbyqxmd.com/read/29128061/the-genetics-of-pediatric-nonalcoholic-fatty-liver-disease
#7
REVIEW
Nidhi P Goyal, Jeffrey B Schwimmer
Nonalcoholic fatty liver disease (NAFLD) is the leading cause of chronic liver disease in children. Severe fibrosis and cirrhosis are potential consequences of pediatric NAFLD and can occur within a few years of diagnosis. Observations suggest that genetics may be a strong modifying factor in the presentation, severity, and natural history of the disease. There is increasing interest in determining at-risk populations based on genetics in the hope of finding genotypes that correlate to NAFLD phenotype. Ultimately, the hope is to be able to tailor therapeutics to genetic predispositions and decrease disease morbidity in children with NAFLD...
February 2018: Clinics in Liver Disease
https://www.readbyqxmd.com/read/29114140/analysis-of-hospital-records-of-children-with-hydatid-cyst-in-south-of-iran
#8
Anahita Sanaei Dashti, Mohammad Rahim Kadivar, Abdolvahab Alborzi, Esmaeel Sadeghi, Gholam Reza Pouladfar, Neda Bagherian, Naser Honar, Masoomeh Khalifeh
The clinical manifestations of hydatidosis are various and related to anatomic location. Defining frequent symptoms and signs of the disease is imperative for early management of it. The aim of this report was to analyse the clinical features of infected children with hydatid cysts located in different organs. In this study, medical charts of 57 children between 3 and 16 years of age with hydatid cyst admitted to Pediatric Wards of Nemazee Hospital were evaluated over a 12 year period (from 2003 to 2014, prospectively)...
December 2017: Journal of Parasitic Diseases: Official Organ of the Indian Society for Parasitology
https://www.readbyqxmd.com/read/29080241/next-generation-sequencing-in-pediatric-hepatology-and-liver-transplantation
#9
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/29072244/oral-and-dental-health-in-children-with-chronic-liver-disease-in-the-turkey-northeast
#10
O Baygin, M Cakır, N Ucuncu
BACKGROUND: It is important to be aware of oral and dental problems in the early period in children with chronic liver disease (CLD) to prevent late complications. Therefore, we aimed to analyze the oral and dental health status in children with CLD. METHODS: The three groups of children (3-18 years old); Group 1 (disease group, n = 31) patients with CLD, Group 2 (disease control group, n = 17) patients with chronic renal failure, and Group 3 healthy children (control group, n = 35)...
September 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/29062906/elevated-cystatin-c-is-it-a-reflection-for-kidney-or-liver-impairment-in-hepatic-children
#11
Behairy El-Sayed, Hanaa El-Araby, Nermin Adawy, Mona Hassona, Naglaa El-Nady, Haidy Zakaria, Mohammed Khedr
AIM OF THE STUDY: To assess if elevated serum cystatin C (Cyst-C) is an indicator for renal or hepatic dysfunction in presence of liver fibrosis. MATERIAL AND METHODS: Data of 50 children with chronic liver diseases (CLDs), out of which 25 were without renal impairment, and 25 with renal impairment were analyzed. Twenty healthy children served as a healthy control group. Routine investigations, creatinine clearance, hepatitis viral markers, abdominal ultrasonography, and liver biopsy were performed for patients with CLDs...
September 2017: Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29035331/nutritional-needs-and-support-for-children-with-chronic-liver-disease
#12
REVIEW
Christine H Yang, Brandon J Perumpail, Eric R Yoo, Aijaz Ahmed, John A Kerner
Malnutrition has become a dangerously common problem in children with chronic liver disease, negatively impacting neurocognitive development and growth. Furthermore, many children with chronic liver disease will eventually require liver transplantation. Thus, this association between malnourishment and chronic liver disease in children becomes increasingly alarming as malnutrition is a predictor of poorer outcomes in liver transplantation and is often associated with increased morbidity and mortality. Malnutrition requires aggressive and appropriate management to correct nutritional deficiencies...
October 16, 2017: Nutrients
https://www.readbyqxmd.com/read/29031874/an-update-on-the-physiopathology-and-therapeutic-management-of-cholestatic-pruritus-in-children
#13
A Thébaut, D Debray, E Gonzales
Pruritus is a disabling symptom accompanying chronic cholestasis. In extreme cases, the refractory nature of pruritus can result in a need for invasive therapies including liver transplantation. The pathogenesis of pruritus in cholestatic disease is poorly understood. It may involve a specific neural pathway (similar to that associated with pain) regulated by several pruritogenic substances such as bile acids, opioids, serotonin, and the more recently identified lysophosphatidic acid. While the therapeutic management of cholestatic pruritus is well established in adults, there is no consensus in children, in light of the difficulty of conducting controlled clinical studies...
October 11, 2017: Clinics and Research in Hepatology and Gastroenterology
https://www.readbyqxmd.com/read/29024698/pediatric-clif-sofa-score-is-the-best-predictor-of-28-day-mortality-in-children-with-decompensated-chronic-liver-disease
#14
Rishi Bolia, Anshu Srivastava, Surender Kumar Yachha, Ujjal Poddar
BACKGROUND & AIMS: Early identification of children with decompensated chronic liver disease (DCLD) at risk of short-term mortality helps improve outcome. We aimed to evaluate the predictors of outcome and role of Child-Pugh, pediatric end-stage liver disease (PELD) and pediatric chronic liver failure sequential organ failure assessment (pCLIF-SOFA) score for prognosticating 28-day mortality in children with DCLD. METHODS: DCLD children were prospectively evaluated with a clinico-laboratory proforma and followed for 28 days to determine outcome...
October 10, 2017: Journal of Hepatology
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
#15
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
#16
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/28981167/serum-complement-factor-5a-levels-are-associated-with-non-alcoholic-fatty-liver-il-6-disease-in-obese-children
#17
Wei Hu, Min Wang, Chunyan Yin, Shuangshaung Li, Yuesheng Liu, Yanfeng Xiao
AIM: Nonalcoholic fatty liver disease (NAFLD) is a leading cause of progressive and chronic liver injury. Complement factor 5a (C5a) may be involved in many inflammation disorders. This study investigated levels of systemic C5a in patients with and without NAFLD and lean controls METHODS: A cross-sectional study was conducted from July 2012 to June 2013 among 96 Chinese children, aged 6-17 years, recruited from the Pediatric Department of the Second Affiliated Hospital of Xi'an Jiao Tong University: 40 obese children with NAFLD, 31 obese children without NAFLD and 25 lean controls...
October 5, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28977113/primary-sclerosing-cholangitis-in-children-and-adolescents
#18
Eleonora Druve Tavares Fagundes, Alexandre Rodrigues Ferreira, Caroline Caldeira Hosken, Thaís Costa Nascentes Queiroz
BACKGROUND: Primary sclerosing cholangitis is a rare disease, but its prevalence has been underestimated in children and adolescents due to broad variation in clinical presentation as well as diagnostic challenges in this life period. OBJECTIVE: To evaluate children and adolescents with primary sclerosing cholangitis and to describe their clinical, laboratorial, histopathological, and cholangiography conditions. METHODS: This is an observational descriptive research that took place from 2005 to 2016 and included all the patients seen in the Outpatient Unit for Pediatric Hepatology of Hospital das Clinicas of UFMG who had been diagnosed with primary sclerosing cholangitis before the age of 18...
October 2, 2017: Arquivos de Gastroenterologia
https://www.readbyqxmd.com/read/28970719/autoimmune-hepatitis-standard-treatment-and-systematic-review-of-alternative-treatments
#19
REVIEW
Benedetta Terziroli Beretta-Piccoli, Giorgina Mieli-Vergani, Diego Vergani
Autoimmune hepatitis is a rare chronic inflammatory liver disease, affecting all ages, characterised by elevated transaminase and immunoglobulin G levels, positive autoantibodies, interface hepatitis at liver histology and good response to immunosuppressive treatment. If untreated, it has a poor prognosis. The aim of this review is to summarize the evidence for standard treatment and to provide a systematic review on alternative treatments for adults and children. Standard treatment is based on steroids and azathioprine, and leads to disease remission in 80%-90% of patients...
September 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28948665/performance-of-fibrosis-prediction-scores-in-paediatric-non-alcoholic-fatty-liver-disease
#20
Jasmine A Jackson, Juna V Konomi, Michael V Mendoza, Alyssa Krasinskas, Ran Jin, Shelley Caltharp, Marialena Mouzaki, Miriam B Vos
AIM: Non-alcoholic fatty liver disease (NAFLD) is the leading cause of chronic liver disease in children. The phenotype of NAFLD varies widely, and non-invasive predictors of disease severity are scarce and are needed to tailor clinical management. METHODS: We compared liver fibrosis by histology with proposed non-invasive predictors of fibrosis, including alanine transaminase (ALT), aspartate transaminase (AST), AST/ALT ratio, AST to platelet ratio index, fibrosis-4, paediatric NAFLD fibrosis index and paediatric NAFLD fibrosis score...
September 25, 2017: Journal of Paediatrics and Child Health
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