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Liver disease in children

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https://www.readbyqxmd.com/read/28107286/liver-stiffness-measured-by-shear-wave-elastography-for-evaluating-intra-hepatic-portal-hypertension-in-children
#1
Hee Mang Yoon, So Yeon Kim, Kyung Mo Kim, Seak Hee Oh, Gi-Young Ko, Yangsoon Park, Jin Seong Lee, Ah Young Jung, Young Ah Cho
OBJECTIVES: To correlate liver stiffness (LS) and hepatic venous-pressure gradient (HVPG) and to evaluate the diagnostic performance of shear-wave elastography (SWE) for predicting clinically significant portal hypertension in children with suspected liver diseases, in consideration of the reliability criteria. METHODS: We identified 33 SWEs from 32 children who underwent HVPG measurement within two weeks between June 2012 and October 2015. The correlation between LS and HVPG was assessed...
January 20, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28107283/naspghan-clinical-practice-guideline-for-the-diagnosis-and-treatment-of-nonalcoholic-fatty-liver-disease-in-children-recommendations-from-the-expert-committee-on-nafld-econ-and-the-north-american-society-of-pediatric-gastroenterology-hepatology-and-nutrition
#2
Miriam B Vos, Stephanie H Abrams, Sarah E Barlow, Sonia Caprio, Stephen R Daniels, Rohit Kohli, Marialena Mouzaki, Pushpa Sathya, Jeffrey B Schwimmer, Shikha S Sundaram, Stavra A Xanthakos
Nonalcoholic fatty liver disease (NAFLD) is a highly prevalent chronic liver disease that occurs in the setting of insulin resistance and increased adiposity. It has rapidly evolved into the most common liver disease seen in the pediatric population and is a management challenge for general pediatric practitioners, subspecialists, and for health systems. In this guideline, the expert committee on NAFLD reviewed and summarized the available literature, formulating recommendations to guide screening and clinical care of children with NAFLD...
February 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28105511/bone-density-in-the-obese-child-clinical-considerations-and-diagnostic-challenges
#3
Jennifer C Kelley, Nicola Crabtree, Babette S Zemel
The prevalence of obesity in children has reached epidemic proportions. Concern about bone health in obese children, in part, derives from the potentially increased fracture risk associated with obesity. Additional risk factors that affect bone mineral accretion, may also contribute to obesity, such as low physical activity and nutritional factors. Consequences of obesity, such as inflammation, insulin resistance, and non-alcoholic fatty liver disease, may also affect bone mineral acquisition, especially during the adolescent years when rapid increases in bone contribute to attaining peak bone mass...
January 20, 2017: Calcified Tissue International
https://www.readbyqxmd.com/read/28101773/what-is-the-appropriate-strategy-for-diagnosing-nafld-using-ultrasonography-in-obese-children
#4
Jee Hyun Lee, Su Jin Jeong
BACKGROUND: The aim of this study is to identify obese children who are candidates for a potential diagnosis of non-alcoholic fatty liver disease (NAFLD). METHODS: We enrolled 242 obese children (122 boys and 120 girls) aged 7-16 years who were examined with abdominal ultrasonography in our pediatric obesity clinic. We compared patients in the normal group with those in the NAFLD group (mild disease, moderate to severe disease) and identified the optimal anthropometric parameters among height, weight, body mass index (BMI), waist circumference, hip circumference, waist to height ratio (WHtR), and waist to hip ratio to predict NAFLD using a receiver operating characteristic curve analysis...
January 19, 2017: World Journal of Pediatrics: WJP
https://www.readbyqxmd.com/read/28099338/liver-steatosis-in-children-with-chronic-hepatitis-b-and-c-prevalence-predictors-and-impact-on-disease-progression
#5
Maria Pokorska-Śpiewak, Barbara Kowalik-Mikołajewska, Małgorzata Aniszewska, Magdalena Pluta, Bożena Walewska-Zielecka, Magdalena Marczyńska
Only scarce data on liver steatosis in children with chronic hepatitis B and C (CHB and CHC) are available. The objective of this study was to evaluate the prevalence, predictors, and impact of hepatic steatosis on children with CHB and CHC. A total of 78 patients aged 11.5 ± 3.4 years were included: 30 (38%) had CHB, and 48 (62%) had CHC. Steatosis was scored on a 5-point scale, as follows: absent; minimal (≤5% hepatocytes affected), mild (6-33%), moderate (34-66%), and severe (>66%). Stepwise logistic regression was used to determine the factors associated with steatosis and moderate-to-severe steatosis...
January 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28094851/blood-transfusion-for-preventing-primary-and-secondary-stroke-in-people-with-sickle-cell-disease
#6
REVIEW
Lise J Estcourt, Patricia M Fortin, Sally Hopewell, Marialena Trivella, Winfred C Wang
BACKGROUND: Sickle cell disease is one of the commonest severe monogenic disorders in the world, due to the inheritance of two abnormal haemoglobin (beta globin) genes. Sickle cell disease can cause severe pain, significant end-organ damage, pulmonary complications, and premature death. Stroke affects around 10% of children with sickle cell anaemia (HbSS). Chronic blood transfusions may reduce the risk of vaso-occlusion and stroke by diluting the proportion of sickled cells in the circulation...
January 17, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28092152/issues-in-nutrition-dietary-considerations-in-select-chronic-conditions
#7
Margaret E Thompson, Mary Barth Noel
Chronic diseases that affect the gastrointestinal tract also tend to affect nutrition. The incidence of chronic liver disease is increasing. As the prevalence of obesity rises, so do the incidences of nonalcoholic fatty liver disease and nonalcoholic steatohepatitis. Patients with chronic liver disease usually have some degree of malnutrition. In the absence of encephalopathy, patients with chronic liver disease should consume more protein than that in the average diet. There is some controversy about whether diet plays a role in the development of inflammatory bowel disease...
January 2017: FP Essentials
https://www.readbyqxmd.com/read/28090653/genome-wide-study-links-pnpla3-variant-with-elevated-hepatic-transaminase-after-acute-lymphoblastic-leukemia-therapy
#8
Yiwei Liu, Christian A Fernandez, Colton Smith, Wenjian Yang, Cheng Cheng, John C Panetta, Nancy Kornegay, Chengcheng Liu, Laura B Ramsey, Seth E Karol, Laura J Janke, Eric C Larsen, Naomi Winick, William L Carroll, Mignon L Loh, Elizabeth A Raetz, Stephen P Hunger, Meenakshi Devidas, Jun J Yang, Charles G Mullighan, Jinghui Zhang, William E Evans, Sima Jeha, Ching-Hon Pui, Mary V Relling
Remission induction therapy for acute lymphoblastic leukemia (ALL) includes medications that may cause hepatotoxicity, including asparaginase. We used a genome-wide association study (GWAS) to identify loci associated with elevated alanine transaminase (ALT) levels after induction therapy in children with ALL enrolled on St. Jude Children's Research Hospital (SJCRH) protocols. Germline DNA was genotyped using arrays and exome sequencing. Adjusting for age, body mass index, ancestry, asparaginase preparation and dosage, the PNPLA3 rs738409 (C>G) I148M variant, previously associated with fatty liver disease risk, had the strongest genetic association with ALT (P = 2...
January 16, 2017: Clinical Pharmacology and Therapeutics
https://www.readbyqxmd.com/read/28087208/-child-langerhans-cell-histiocytosis
#9
Jean Donadieu, Sébastien Héritier
DEFINITION: Langerhans cell histiocytosis (LCH) is defined by the association of a clinical and radiological involvement and a biopsy of a pathological tissue. Extension: it can affect any organ or system of the body but most commonly the bone (80% of cases), the skin (33%) and the pituitary (25%). Other organs are concerned such as liver, spleen, hematopoietic system and the lungs (15% each), lymph nodes (5-10%) and central nervous system (CNS) excluding the pituitary (2-4%). Natural history: the natural history of the disease is very heterogeneous, ranging from auto-regressive lesions to a disease affecting multiple organs with fatal consequences, while some lesions may be responsible for permanent sequels...
January 10, 2017: La Presse Médicale
https://www.readbyqxmd.com/read/28073147/treating-nonalcoholic-steatohepatitis-nash-in-children-not-a-cinch-task
#10
Naim Alkhouri, Ariel E Feldstein
BACKGROUND & AIMS: No treatment for nonalcoholic fatty liver disease (NAFLD) has been approved by regulatory agencies. We performed a randomized controlled trial to determine whether 52 weeks of cysteamine bitartrate delayed release (CBDR) reduces the severity of liver disease in children with NAFLD. METHODS: We performed a double-masked trial of 169 children with NAFLD Activity Scores ≥ 4 at 10 centers. From June 2012 to January 2014, the patients were randomly assigned to receive CBDR or placebo twice daily (300 mg for ≤65 kg, 375 mg for >65-80 kg, 450 mg for >80 kg) for 52 weeks...
January 10, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28065514/review-of-childhood-obesity-from-epidemiology-etiology-and-comorbidities-to-clinical-assessment-and-treatment
#11
REVIEW
Seema Kumar, Aaron S Kelly
Childhood obesity has emerged as an important public health problem in the United States and other countries in the world. Currently 1 in 3 children in the United States is afflicted with overweight or obesity. The increasing prevalence of childhood obesity is associated with emergence of comorbidities previously considered to be "adult" diseases including type 2 diabetes mellitus, hypertension, nonalcoholic fatty liver disease, obstructive sleep apnea, and dyslipidemia. The most common cause of obesity in children is a positive energy balance due to caloric intake in excess of caloric expenditure combined with a genetic predisposition for weight gain...
January 5, 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/28063601/bone-marrow-fat-content-is-correlated-with-hepatic-fat-content-in-paediatric-non-alcoholic-fatty-liver-disease
#12
N Y Yu, T Wolfson, M S Middleton, G Hamilton, A Gamst, J E Angeles, J B Schwimmer, C B Sirlin
AIM: To investigate the relationship between bone marrow fat content and hepatic fat content in children with known or suspected non-alcoholic fatty liver disease (NAFLD). MATERIALS AND METHODS: This was an institutional review board-approved, Health Insurance Portability and Accountability Act (HIPAA)-compliant, cross-sectional, prospective analysis of data collected between October 2010 to March 2013 in 125 children with known or suspected NAFLD. Written informed consent was obtained for same-day research magnetic resonance imaging (MRI) of the lumbar spine, liver, and abdominal adiposity...
January 4, 2017: Clinical Radiology
https://www.readbyqxmd.com/read/28057514/systemic-effects-of-subtilase-cytotoxin-produced-by-escherichia-coli-o113-h21
#13
E Abril Seyahian, Gisela Oltra, Federico Ochoa, Santiago Melendi, Ricardo Hermes, James C Paton, Adrienne W Paton, Nestor Lago, Mauricio Castro Parodi, Alicia Damiano, Cristina Ibarra, Elsa Zotta
: Subtilase cytotoxin (SubAB) is a member of the AB5 cytotoxin family and is produced by certain strains of Shiga toxigenic Escherichia coli. The toxin is known to be lethal to mice, but the pathological mechanisms that contribute to Uremic Hemolytic Syndrome (HUS) are poorly understood. In this study we show that intraperitoneal injection of a sublethal dose of SubAB in rats triggers a systemic response, with ascitic fluid accumulation, heart hypertrophy and damage to the liver, colon and kidney...
January 3, 2017: Toxicon: Official Journal of the International Society on Toxinology
https://www.readbyqxmd.com/read/28052602/optn-srtr-2015-annual-data-report-intestine
#14
J M Smith, M A Skeans, S P Horslen, E B Edwards, A M Harper, J J Snyder, A K Israni, B L Kasiske
Intestine and intestine-liver transplant remains important in the treatment of intestinal failure, despite decreased morbidity associated with parenteral nutrition. In 2015, 196 new patients were added to the intestine transplant waiting list, with equal numbers waiting for intestine and intestine-liver transplant. Among prevalent patients on the list at the end of 2015, 63.3% were waiting for an intestine transplant and 36.7% were waiting for an intestine-liver transplant. The pretransplant mortality rate decreased dramatically over time for all age groups...
January 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28052307/ultrashort-bowel-syndrome-outcome-in-children-treated-in-a-multidisciplinary-intestinal-rehabilitation-unit
#15
Mariela Dore, Paloma Triana Junco, Ane Andres Moreno, Vanesa Nuñez Cerezo, Martha Romo Muñoz, Alba Sánchez Galán, Alejandra Vilanova Sánchez, Gerardo Prieto, Esther Ramos, Francisco Hernandez, Leopoldo Martínez Martínez, Manuel Lopez Santamaria
Aim Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in the pediatric population. Our aim was to review long-term outcome of ultrashort bowel syndrome (USBS) in an Intestinal Rehabilitation Unit (IRU). Patients and Methods Retrospective study of patients with USBS (defined as < 10 cm of remnant small bowel) treated between 2000 and 2015. Demographic data, clinical, and treatment variables including parenteral nutrition (PN), surgical techniques, and intestinal transplantation (IT) were analyzed...
January 4, 2017: European Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28044976/-kidsetransplant-a-serious-game-for-liver-transplanted-children
#16
Ana-Maria Calinescu, Valérie McLin, Stéphane Spahni, Thomas Boggini, Mirana Randriambelonoro, Roxane Jaquier-Grant, Michèle Steiner, Antoine Geissbuhler, Barbara E Wildhaber
Serious games are increasingly used in pediatric care, especially to educate young patients with chronic diseases, to improve their understanding of the disease and develop independence in disease management, as it can ultimately improve clinical results. This is also true for liver transplanted children and adolescents, who are not only challenged by their therapeutic burden, but who need to face transition to adult care, often without remembering their transplantation, which was performed, most of the time, very early in their life...
December 2016: Médecine Sciences: M/S
https://www.readbyqxmd.com/read/28040394/long-term-hematological-visceral-and-growth-outcomes-in-children-with-gaucher-disease-type-3-treated-with-imiglucerase-in-the-international-collaborative-gaucher-group-gaucher-registry
#17
Amal El-Beshlawy, Anna Tylki-Szymanska, Ashok Vellodi, Nadia Belmatoug, Gregory A Grabowski, Edwin H Kolodny, Julie L Batista, Gerald F Cox, Pramod K Mistry
In Gaucher disease (GD), deficiency of lysosomal acid β-glucosidase results in a broad phenotypic spectrum that is classified into three types based on the absence (type 1 [GD1]) or presence and severity of primary central nervous system involvement (type 2 [GD2], the fulminant neuronopathic form, and type 3 [GD3], the milder chronic neuronopathic form). Enzyme replacement therapy (ERT) with imiglucerase ameliorates and prevents hematological and visceral manifestations in GD1, but data in GD3 are limited to small, single-center series...
December 6, 2016: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28040302/omega-3-fatty-acids-as-a-treatment-for-non-alcoholic-fatty-liver-disease-in-children-a-systematic-review-and-meta-analysis-of-randomized-controlled-trials
#18
Lian-Hui Chen, Yong-Fen Wang, Qing-Hong Xu, Shan-Shan Chen
BACKGROUND: The most typical chronic liver disease in children and adolescents is non-alcoholic fatty liver disease (NAFLD). The dietary addition of ω-3 polyunsaturated fatty acids (PUFAs) provides a promising therapy for children with NAFLD due to its convenience and safety; however, several studies suggested contradictory results for PUFA supplementation in children. Hence, we performed a systematic review and meta-analysis to evaluate the effectiveness of PUFA supplementation in children with NAFLD...
December 23, 2016: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28039895/genetic-profiling-of-children-with-advanced-cholestatic-liver-disease
#19
Mohammad Shagrani, Jessica Burkholder, Dieter Broering, Mohamed Abouelhoda, Tariq Faquih, Mohamed El-Kalioby, Shazia N Subhani, Ewa Goljan, Renad Albar, Dorota Monies, Nejat Mazhar, Basma S AlAbdulaziz, Khalid Al Abdelrahman, Nada Altassan, Fowzan S Alkuraya
Advanced cholestatic liver disease is a leading referral to pediatric liver transplant centers. Recent advances in the genetic classification of this group of disorders promise a highly personalized management although the genetic heterogeneity also poses a diagnostic challenge. Using a next-generation sequencing-based multi-gene panel, we performed retrospective analysis of 98 pediatric patients who presented with advanced cholestatic liver disease. A likely causal mutation was identified in the majority (61%), spanning many genes including ones that have only rarely been reported to cause cholestatic liver disease e...
December 30, 2016: Clinical Genetics
https://www.readbyqxmd.com/read/28039349/primary-embryonal-rhabdomyosarcoma-of-the-liver
#20
Aditi Arora, Ritika Jaiswal, Nidhi Anand, Nuzhat Husain
Rhabdomyosarcomas are malignant neoplasms with striated muscle differentiation. This is the most common type of soft-tissue sarcoma in children, but occurs rarely in adults. Its occurrence in liver is infrequent. We report a case of primary hepatic embryonal rhabdomyosarcoma in a 67-year-old man. The tumour was occupying the left lobe of the liver with large component of lesion seen bulging in left subhepatic space indenting over the stomach, compressing the pancreas and gall bladder. A percutaneous biopsy was performed which revealed embryonal rhabdomyosarcoma...
December 30, 2016: BMJ Case Reports
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