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Pediatric liver disease

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https://www.readbyqxmd.com/read/28532447/clinical-characteristics-of-disseminated-cryptococcosis-in-previously-healthy-children-in-china
#1
Li-Wei Gao, An-Xia Jiao, Xi-Rong Wu, Shun-Ying Zhao, Yun Ma, Gang Liu, Ju Yin, Bao-Ping Xu, Kun-Ling Shen
BACKGROUND: Disseminated cryptococcosis is a rare and fatal disease, and limited data exist regarding it in children. This study aimed to investigate the clinical characteristics of disseminated cryptococcosis in previously healthy children in China. METHODS: Hospitalized patients with disseminated cryptococcosis were enrolled during January 1996 to December 2015 in Beijing Children's Hospital, Capital Medical University, China. Data on clinical manifestations, laboratory tests, treatment, and prognosis were evaluated...
May 22, 2017: BMC Infectious Diseases
https://www.readbyqxmd.com/read/28531808/prenatal-diagnosis-of-biliary-atresia-a-case-series
#2
O Shen, H Y Sela, H Nagar, R Rabinowitz, E Jacobovich, D Chen, E Granot
BACKGROUND: Biliary atresia is a progressive disease presenting with jaundice, and is the most common indication for liver transplantation in the pediatric population. Prenatal series have yielded conflicting results concerning a possible association between BA and prenatal nonvisualization of the gallbladder. AIMS: This retrospective case series was performed to assess the association between biliary atresia, prenatal nonvisualization of the gallbladder and other sonographic signs...
May 19, 2017: Early Human Development
https://www.readbyqxmd.com/read/28521607/intravenous-fish-oil-and-pediatric-intestinal-failure-associated-liver-disease-changes-in-plasma-phytosterols-cytokines-and-bile-acids-and-erythrocyte-fatty-acids
#3
Kara L Calkins, Andrea DeBarber, Robert D Steiner, Martiniano J Flores, Tristan R Grogan, Susanne M Henning, Laurie Reyen, Robert S Venick
BACKGROUND: Soybean oil (SO) emulsions are associated with intestinal failure-associated liver disease (IFALD); fish oil (FO) emulsions are used to treat IFALD. SO and FO differ with respect to their fatty acid and phytosterol content. In children with IFALD whose SO was replaced with FO, we aimed to (1) quantify changes in erythrocyte fatty acids and plasma phytosterols, cytokines, and bile acids and (2) correlate these changes with direct bilirubin (DB). DESIGN: This study enrolled IFALD children who received 6 months of FO...
May 1, 2017: JPEN. Journal of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/28520981/clinical-presentation-and-outcomes-of-children-with-human-granulocytic-anaplasmosis
#4
Anna M Schotthoefer, Matthew C Hall, Satya Vittala, Raza Bajwa, Holly M Frost
Background.: Adults with the tick-borne disease human granulocytic anaplasmosis (HGA) have a spectrum of acute febrile illnesses that, if untreated, might be severe. Clinical presentation and outcomes of children with HGA have been poorly described. Methods.: A retrospective analysis was conducted to determine the frequency, presentation, and outcomes of pediatric patients with HGA between 1994 and 2015 in a region of Wisconsin in which HGA is highly endemic. Patients with related International Classification of Diseases Ninth and Tenth Revision (ICD-9 and ICD-10, respectively) codes or positive HGA laboratory test results were evaluated and classified as having had confirmed, probable, or suspected HGA on the basis of the Centers for Disease Control and Prevention (CDC) case definition...
May 16, 2017: Journal of the Pediatric Infectious Diseases Society
https://www.readbyqxmd.com/read/28515413/magnetic-susceptibility-changes-in-the-basal-ganglia-and-brain-stem-of-patients-with-wilson-s-disease-evaluation-with-quantitative-susceptibility-mapping
#5
Selim Doganay, Kazim Gumus, Gonca Koc, Ayse Kacar Bayram, Mehmet Sait Dogan, Duran Arslan, Hakan Gumus, Sureyya Burcu Gorkem, Saliha Ciraci, Halil Ibrahim Serin, Abdulhakim Coskun
OBJECTIVES: Wilson's disease (WD) is characterized with the accumulation of copper in the liver and brain. The objective of this study is to quantitatively measure the susceptibility changes of basal ganglia and brain stem of pediatric patients with neurological WD using quantitative susceptibility mapping (QSM) in comparison to healthy controls. METHODS: Eleven patients with neurological WD (mean age 15 ± 3.3 years, range 10-22 years) and 14 age-matched controls were prospectively recruited...
May 18, 2017: Magnetic Resonance in Medical Sciences: MRMS
https://www.readbyqxmd.com/read/28514463/pediatric-psoriasis-comorbidity-screening-guidelines
#6
Emily Osier, Audrey S Wang, Megha M Tollefson, Kelly M Cordoro, Stephen R Daniels, Andrew Eichenfield, Joel M Gelfand, Alice B Gottlieb, Alexa B Kimball, Mark Lebwohl, Nehal N Mehta, Amy S Paller, Jeffrey B Schwimmer, Dennis M Styne, Abby S Van Voorhees, Wynnis L Tom, Lawrence F Eichenfield
Importance: Psoriasis is a complex inflammatory skin condition associated with serious medical comorbidities in adults, including obesity, hypertension, dyslipidemia, type 2 diabetes mellitus, psoriatic arthritis, nonalcoholic fatty liver disease, depression, anxiety, and decreased quality of life. Because psoriasis begins in childhood in almost one-third of patients, early identification of risk may be critical to minimizing effects on future health. Objective: To develop the first set of guidelines for comorbidity screening for patients with pediatric psoriasis based on current evidence...
May 17, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28506363/contemporary-evaluation-of-the-pediatric-liver-biopsy
#7
REVIEW
Deborah A Schady, Milton J Finegold
Liver disease in the neonate, infant, child, and adolescent may manifest differently depending on the type of disorder. These disorders show marked overlap clinically and on light microscopy. Histology and ultrastructural examination are used in tandem for the diagnosis of most disorders. A final diagnosis or interpretation of the pediatric liver biopsy depends on appropriate and adequate clinical history, laboratory test results, biochemical assays, and molecular analyses, as indicated by the light microscopic and ultrastructural examination...
June 2017: Gastroenterology Clinics of North America
https://www.readbyqxmd.com/read/28506362/current-concepts-in-pediatric-nonalcoholic-fatty-liver-disease
#8
REVIEW
Sarah E Fleet, Jay H Lefkowitch, Joel E Lavine
Nonalcoholic fatty liver disease (NAFLD) represents a spectrum of disease. Its increasing prevalence is a direct result of historically high rates of obesity. Hepatocyte lipid accumulation is the first step in a cascade of metabolic and inflammatory events thought to precipitate NAFLD. Histologic findings provide insight into these events. Lifestyle modification remains the primary therapy in children. Current recommendations include vitamin E treatment in those with biopsy-proven NASH. Trials of novel drugs are ongoing in adults...
June 2017: Gastroenterology Clinics of North America
https://www.readbyqxmd.com/read/28506338/-clinical-efficacy-of-compound-glycyrrhizin-tablets-in-the-treatment-of-children-with-nonalcoholic-fatty-liver-disease
#9
Yu-Wen Li, Yu-Hua Hu, Tian-Tian Zhu, An-Zhen Chu, Chuan-Long Zhu
Department of Pediatrics, First Affiliated Hospital of Nanjing Medical University, Nanjing 210029, China. zhuchuanlong@jsph.org.cn.
May 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28504496/echinococcosis-in-children-experience-in-a-tertiary-care-hospital-outside-the-endemic-area
#10
Griselda Berberian, Teresa Rosanova, Laura Inda, Claudia Sarkis, Horacio Questa, Patricia Paulin, Marina Costa, Moira Taicz
Echinococcosis is a zoonotic disease that is widely spread across Argentina and worldwide. It is acquired during childhood but it is more common during adulthood. The pediatric population accounts for 10-20% of all cases. This study included 47 children diagnosed with echinococcosis over a 20-year period. Their median age was 8 years old (range: 3-17); most patients had some epidemiological history, such as having lived or living in an endemic or rural area and/ or having dogs that are fed with viscera. Findings included 85% of single cysts in only one parenchyma...
June 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28502445/pediatric-liver-transplantation
#11
REVIEW
Nidhi Rawal, Nada Yazigi
Excellent outcomes over the last 3 decades have made liver transplantation the treatment of choice for many advanced liver disorders. This success also opened liver transplantation to new indications such as liver tumors and metabolic disorders. The emergence of such new indications for liver transplantation is bringing a new stream of patients along with disease-specific challenges. The cumulative number of liver transplant recipients is peaking, requiring novel systems of health care delivery that meet the needs of this special patient population...
June 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/28497365/clinical-utility-of-soluble-interleukin-2-receptor-in-hemophagocytic-syndromes-a-systematic-scoping-review
#12
REVIEW
Molly Lin, Sujin Park, Anna Hayden, Dean Giustini, Martina Trinkaus, Morris Pudek, Andre Mattman, Marion Schneider, Luke Y C Chen
The serum-soluble interleukin-2 receptor (sIL-2r) level is considered an important diagnostic test and disease marker in hemophagocytic syndromes/hemophagocytic lymphohistiocytosis (HPS/HLH). However, this cytokine receptor is rarely measured in clinical practice and has been excluded from recent diagnostic/classification criteria such as the HScore and macrophage activation syndrome (MAS) 16. We performed a systematic scoping review of 64 articles (1975-2016) examining the clinical utility of sIL-2r in HPS/HLH...
May 12, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28494529/nash-therapy-omega-3-supplementation-vitamin-e-insulin-sensitizers-and-statin-drugs
#13
Stephen Caldwell
Non-alcoholic steatohepatitis (NASH) is the more aggressive form of non-alcoholic fatty liver disease (NAFLD). NASH can progress to hepatic fibrosis, cirrhosis, portal hypertension and primary liver cancer. Therapy is evolving with a substantial number of trials of promising new agents now in progress. In this article however, we will examine data for several older forms of therapy which have been fairly extensively studied over the years: Polyunsaturated Fatty Acid (PUFA) supplements, vitamin E, insulin sensitizing agents with a focus on pioglitazone and statin agents...
May 10, 2017: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/28493388/magnetic-resonance-elastography-measured-shear-stiffness-as-a-biomarker-of-fibrosis-in-pediatric-nonalcoholic-fatty-liver-disease
#14
Jeffrey B Schwimmer, Cynthia Behling, Jorge Eduardo Angeles, Melissa Paiz, Janis Durelle, Jonathan Africa, Kimberly P Newton, Elizabeth M Brunt, Joel E Lavine, Stephanie H Abrams, Prakash Masand, Rajesh Krishnamurthy, Kelvin Wong, Richard L Ehman, Meng Yin, Kevin J Glaser, Bogdan Dzyubak, Tanya Wolfson, Anthony C Gamst, Jonathan Hooker, William Haufe, Alexandra Schlein, Gavin Hamilton, Michael S Middleton, Claude B Sirlin
Magnetic Resonance Elastography (MRE) is promising for non-invasive assessment of fibrosis, a major determinant of outcome in nonalcoholic fatty liver disease (NAFLD). However, data in children are limited. Study aims were to determine accuracy of MRE for detection of fibrosis and advanced fibrosis in children with NAFLD, and to assess agreement between manual and novel automated reading methods. We performed a prospective, multi-center study of 2D-MRE in children with NAFLD. MR-elastograms were analyzed manually at 2 reading centers and using a new automated technique...
May 11, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28492501/urinary-metabolomics-in-pediatric-obesity-and-nafld-identifies-metabolic-pathways-metabolites-related-to-dietary-habits-and-gut-liver-axis-perturbations
#15
Jacopo Troisi, Luca Pierri, Annamaria Landolfi, Francesca Marciano, Antonella Bisogno, Federica Belmonte, Carmen Palladino, Salvatore Guercio Nuzio, Pietro Campiglia, Pietro Vajro
To get insight into still elusive pathomechanisms of pediatric obesity and non-alcoholic fatty liver disease (NAFLD) we explored the interplay among GC-MS studied urinary metabolomic signature, gut liver axis (GLA) abnormalities, and food preferences (Kid-Med). Intestinal permeability (IP), small intestinal bacterial overgrowth (SIBO), and homeostatic model assessment-insulin resistance were investigated in forty children (mean age 9.8 years) categorized as normal weight (NW) or obese (body mass index <85th or >95th percentile, respectively) ± ultrasonographic bright liver and hypertransaminasemia (NAFLD)...
May 11, 2017: Nutrients
https://www.readbyqxmd.com/read/28483736/child-with-pancreatic-hydatid-cyst-presenting-with-cholestasis
#16
Nagehan Aslan, Tuğba Koca, Aykut Recep Aktaş, Füsun Zeynep Akçam, Mustafa Akçam
Although hydatid cysts are often seen in the liver and lungs, they may be present in many organs. Even in countries where hydatid cyst disease is endemic, the occurrence of pancreatic hydatid cysts is rare. Pancreatic hydatid cysts are important for the differential diagnosis of patients with pancreatic pseudocysts and cystic carcinomas. We could not find cystic echinococcosis cases which are kept together pancreas and liver in PubMed. In this article, we highlight the fact that pancreatic cystic echinococcosis may play a role in the etiology of cholestasis and that cysts may not be isolated in the pancreas in a pediatric population...
March 2017: Türkiye Parazitolojii Dergisi
https://www.readbyqxmd.com/read/28473302/glassy-droplet-inclusions-within-the-cytoplasm-of-kupffer-cells-a-novel-ultrastructural-feature-for-the-diagnosis-of-pediatric-autoimmune-hepatitis
#17
Joanna Maria Lotowska, Maria Elzbieta Sobaniec-Lotowska, Urszula Daniluk, Dariusz Marek Lebensztejn
Since Kupffer cells/macrophages (KCs/MPs) may be involved in the pathogenesis of autoimmune hepatitis (AIH), this pioneer study was undertaken to evaluate KCs/MPs in pediatric AIH in transmission-electron microscope. METHODS: Ultrastructural analyses were performed using liver biopsies from 14 children with clinicopathologically diagnosed AIH. RESULTS: In all AIH children, ultrastructural findings revealed changes in the cells lining sinusoidal vessels, especially KCs/MPs and endothelial cells...
April 12, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/28471905/predictive-variables-for-abnormal-comprehensive-metabolic-panel-testing-and-potential-cost-savings-in-children-receiving-pediatric-emergency-department-care
#18
Matthew David Huckaby, Shayla Freeman, Christopher Thurmond, Matthew Cooper, Joseph D Losek
OBJECTIVE: The aim of this study was to determine variables predictive of abnormal comprehensive metabolic panel (CMP) results in pediatric emergency department (PED) patients and the potential cost savings of a basic metabolic panel (BMP) versus a CMP. METHODS: This is a retrospective cross-sectional descriptive study of children (<18 y) at an urban academic PED (annual census, 22,000). Clinical data included 12 clinical variables: right upper quadrant pain, overdose, emesis, liver disorder, malignancy, heart disease, bleeding disorder, jaundice, right upper quadrant tenderness, hepatomegaly, ascites/peripheral edema and shock, and the liver function test (LFT) results not in a BMP (alanine aminotransferase, aspartate aminotransferase, alkaline phosphatase, total bilirubin, total protein, and albumin)...
May 2017: Pediatric Emergency Care
https://www.readbyqxmd.com/read/28467377/natural-history-of-nafld-diagnosed-in-childhood-a-single-center-study
#19
Catherine E Cioffi, Jean A Welsh, Rebecca L Cleeton, Shelley A Caltharp, Rene Romero, Mark L Wulkan, Juna V Konomi, Jennifer K Frediani, Miriam B Vos
Little is known regarding the subsequent course of non-alcoholic fatty liver disease (NAFLD) diagnosed in childhood. The objectives of this single-center study were to gather data on long-term health outcomes and to assess the feasibility of contacting former pediatric patients. In a large pediatric medical center, electronic records were searched to initially identify 162 former patients who had a liver biopsy between 2000 and 2010. Of these, 44 subjects met the criteria for age at follow-up (≥18 year) and biopsy-proven NAFLD, and were recruited via postal and electronic mail...
May 3, 2017: Children
https://www.readbyqxmd.com/read/28461829/celiac-disease-phenotype-in-clinically-diagnosed-romanian-adults-and-children
#20
Balaban Vasile, Balaban Vasile, Popp Alina, Vasilescu Florina, Ene Adina, Jinga Mariana
BACKGROUND: Once considered a disease of childhood, celiac disease (CD) is now seen quite frequently in adults also, but with different and various clinical presentation. Little data is currently available about pediatric and adult CD features in Romanian patients. METHODS: 38 newly-diagnosed CD patients (17 adults and 21 children) were recruited for this study. The two groups (adult and pediatric) were compared regarding demographic, clinical, serologic and histological data...
June 2016: Mædica
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