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Liver disease in children

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https://www.readbyqxmd.com/read/28340217/establishment-of-reference-interval-for-alkaline-phosphatase-in-healthy-children-of-various-ethnicities-aged-0-12-years
#1
Gu Wanjian, Hu Jie, Ge Liang, Wang Cheng, Xu Tian, Shen Jianjiang, Zhang Chunni
Background: To establish a reference interval of alkaline phosphatase for healthy children of various ethnicities, aged 0 years to 12 years in China. Methods: According to the Clinical and Laboratory Standards Institute guideline C28-A3, blood specimens were collected in the morning after overnight fasting from 502 healthy children to determine the alkaline phosphatase (ALP) level, using the Beckman AU5800 automatic biochemical analyzer. Children were grouped by sex and age...
March 18, 2017: Laboratory Medicine
https://www.readbyqxmd.com/read/28333768/management-of-pediatric-non-alcoholic-fatty-liver-disease-by-academic-hepatologists-in-canada-a-nationwide-survey
#2
Marialena Mouzaki, Simon C Ling, Richard A Schreiber, Binita M Kamath
BACKGROUND: The literature on the optimal clinical management of pediatric patients with non-alcoholic fatty liver disease (NAFLD) is limited. The objective of this study was to identify discrepancies in the care provided to patients with NAFLD by hepatologists practicing in academic centers across Canada. METHODS: A nationwide survey was distributed electronically to all pediatric hepatologists practicing in university-affiliated hospitals using the infrastructure of the Canadian Pediatric Hepatology Research Group...
March 22, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28332779/intrafamilial-phenotypic-variability-in-a-polish-family-with-sensenbrenner-syndrome-and-biallelic-wdr35-mutations
#3
Joanna Walczak-Sztulpa, Anna Wawrocka, Agata Sobierajewicz, Lukasz Kuszel, Jan Zawadzki, Ryszard Grenda, Anna Swiader-Lesniak, Beata Kocyla-Karczmarewicz, Anna Wnuk, Anna Latos-Bielenska, Krystyna H Chrzanowska
Sensenbrenner syndrome (cranioectodermal dysplasia, CED) is a very rare autosomal recessive ciliopathy. Cranioectodermal dysplasia is characterized by craniofacial, skeletal, and ectodermal abnormalities. About 50 patients have been described to date. Sensenbrenner syndrome belongs to a group of ciliary chondrodysplasias and is a genetically heterogeneous disorder. Mutations in five genes: IFT122, WDR35, IFT43, WDR19, and IFT52 have been associated with CED. All known genes encode proteins that are part of the intraflagellar transport complex, which plays an important role in the assembly and maintenance of cilia...
March 23, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28332028/infection-profile-in-chronic-granulomatous-disease-a-23-year-experience-from-a-tertiary-care-center-in-north-india
#4
Amit Rawat, Pandiarajan Vignesh, Avinash Sharma, Jitendra K Shandilya, Madhubala Sharma, Deepti Suri, Anju Gupta, Vikas Gautam, Pallab Ray, Shivaprakash M Rudramurthy, Arunaloke Chakrabarti, Kohsuke Imai, Shigeaki Nonoyama, Osamu Ohara, Yu L Lau, Surjit Singh
PURPOSE: Chronic granulomatous disease (CGD) is an inherited phagocytic disorder characterized by recurrent infections with usually catalase-positive organisms. Infections in CGD from developing countries are expected to be different from those in the Western countries. We report the profile of infections in children diagnosed with CGD from a tertiary care center in North India. METHODOLOGY: Case records of children diagnosed with CGD at Pediatric Immunodeficiency Clinic, Advanced Pediatrics Centre, Postgraduate Institute of Medical Education and Research, Chandigarh, India, from August 1993 to April 2016 (23 years) were analyzed...
March 22, 2017: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28326205/shigella-sonnei-bacteremia-presenting-with-profound-hepatic-dysfunction
#5
Oluwaseun Shogbesan, Andrew Rettew, Bilal Shaikh, Abdullateef Abdulkareem, Anthony Donato
Worldwide, Shigellosis is a significant public health issue, associated with nearly one million deaths annually. About half a million cases of Shigella infection are reported annually in the United States. Shigella bacteremia is uncommon and generally seen in children and immunocompromised adults. We present a case of a Shigella sonnei bacteremia with marked hepatic derangement in a 27-year-old previously healthy homosexual male with history of Roux-en-Y gastric bypass, who presented to the emergency room with a 4-day history of loose watery stool, abdominal cramps, nausea and vomiting, and yellow skin of 2-day duration...
2017: Case Reports in Gastrointestinal Medicine
https://www.readbyqxmd.com/read/28325055/-experience-with-hepatoblastoma-treatment-in-small-children-the-use-of-preoperative-3d-virtual-analysis-mevis-for-liver-resections
#6
J Janek, P Bician, P Kenderessy, P Kothaj, K Novotná, J Koňarčík, Ľ Laca, J Ulianko, I Dedinská
INTRODUCTION: Hepatoblastoma is an uncommon but most frequent liver cancer in infants and children. The therapy is complex, including neoadjuvant, surgical, and adjuvant therapy. For surgery, the presence of a specialized surgeon and an anaesthesiologist is required. For planning the surgery, the 3D analysis MeVis is currently available. The objective of the work is the assessment of a group of children operated for a liver hepatoblastoma and the assessment of contribution of a pre-surgery virtual 3D analysis MeVis for a surgeon...
2017: Rozhledy V Chirurgii: Měsíčník Československé Chirurgické Společnosti
https://www.readbyqxmd.com/read/28324110/effects-of-metreleptin-in-pediatric-patients-with-lipodystrophy
#7
Rebecca J Brown, Cristina Adelia Meehan, Elaine Cochran, Kristina I Rother, David E Kleiner, Mary Walter, Phillip Gorden
Context: Lipodystrophy syndromes are rare disorders of deficient adipose tissue. Metreleptin, a human analog of leptin, improved metabolic abnormalities in mixed cohorts of leptin-deficient children and adults with lipodystrophy. Objective: Determine effects of metreleptin on diabetes, hyperlipidemia, non-alcoholic fatty liver disease (NAFLD), growth, and puberty in leptin-deficient pediatric patients with lipodystrophy. Design: Prospective, single-arm, open-label studies with continuous enrollment since 2000...
January 23, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28321802/sepsis-risk-factors-in-infants-with-congenital-diaphragmatic-hernia
#8
Michaël Levy, Nolwenn Le Sache, Mostafa Mokhtari, Guy Fagherazzi, Gaelle Cuzon, Benjamin Bueno, Virginie Fouquet, Alexandra Benachi, Sergio Eleni Dit Trolli, Pierre Tissieres
BACKGROUND: Congenital diaphragmatic hernia (CDH) is a rare congenital anomaly and remains among the most challenging ICU-managed disease. Beside severe pulmonary hypertension, lung hypoplasia and major abdominal surgery, infective complications remain major determinants of outcome. However, the specific incidence of sepsis as well as associated risk factors is unknown. METHODS: This prospective, 4-year observational study took place in the pediatric intensive care and neonatal medicine department of the Paris South University Hospitals (Le Kremlin-Bicêtre, France), CDH national referral center and involved 62 neonates with CDH...
December 2017: Annals of Intensive Care
https://www.readbyqxmd.com/read/28321162/mortality-associated-with-gastrointestinal-bleeding-in-children-a-retrospective-cohort-study
#9
Thomas M Attard, Mikaela Miller, Chaitanya Pant, Ashwath Kumar, Mike Thomson
AIM: To determine the clinical characteristics of children with gastrointestinal bleeding (GIB) who died during the course of their admission. METHODS: We interrogated the Pediatric Hospital Information System database, including International Classification of Diseases, Current Procedural Terminology and Clinical Transaction Classification coding from 47 pediatric tertiary centers extracting the population of patients (1-21 years of age) admitted (inpatient or observation) with acute, upper or indeterminate GIB (1/2007-9/2015)...
March 7, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28320214/progression-of-liver-disease-in-children-and-adults-with-lysosomal-acid-lipase-deficiency
#10
Barbara K Burton, Nancy Silliman, Sachin Marulkar
BACKGROUND AND OBJECTIVE: Manifestations of the autosomal recessive disorder lysosomal acid lipase deficiency (LAL-D) include hepatomegaly, elevated serum liver enzymes, and progressive liver disease. We report an analysis of time to progression from first clinical manifestation to first documentation of hepatic fibrosis, cirrhosis, or liver transplantation from an observational study of pediatric and adult patients with LAL-D (clinical trial registration: NCT01528917). METHODS: Data were analyzed from 31 patients with available biopsy data and 1 patient without biopsy data who had undergone liver transplantation...
March 21, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28319602/low-hepatic-tissue-copper-in-pediatric-non-alcoholic-fatty-liver-disease
#11
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 non-alcoholic 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...
March 17, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28318371/p-selectin-endocan-and-some-adhesion-molecules-in-obese-children-and-adolescents-with-non-alcoholic-fatty-liver-disease
#12
Ala Ustyol, Esra Aycan Ustyol, Figen Gurdol, Funda Kokali, Seldag Bekpınar
There is increasing evidence for a direct relationship between the vascular system and non-alcoholic fatty liver disease (NAFLD). The aim of this study was to investigate endocan and adhesion molecules such as P-selectin derived from the endothelium and platelets in obese children and adolescents with NAFLD. One hundred obese patients and 40 lean controls were enrolled. The obese subjects were divided into two subgroups based on the presence or absence of fatty liver. Blood samples were assayed for endocan, P-selectin, platelet-derived growth factor (PDGF), intercellular cell adhesion molecule (ICAM)-1, and vascular cell adhesion molecule (VCAM)-1...
May 2017: Scandinavian Journal of Clinical and Laboratory Investigation
https://www.readbyqxmd.com/read/28318185/neonatal-acute-liver-failure-a-diagnosis-challenge
#13
Mirta Ciocca, Fernando Álvarez
Neonatal acute liver failure is a rare, very severe disease with a high rate of mortality. It is clinically and etiologically different from acute liver failure seen in older children and adults. Coagulopathy with an international normalized ratio ≥ 3 is the critical parameter that defines it. The most common causes are fetal alloimmune hepatitis, previously called neonatal hemochromatosis, viral infections, metabolic disorders, and hemophagocytic lymphohistiocytosis. There is a group of treatable diseases that require a very early diagnosis for the prescription of an adequate treatment...
April 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28314323/bevacizumab-combined-with-chemotherapy-in-children-affected-by-hepatocellular-carcinoma-a-single-center-experience
#14
Maria Debora DE Pasquale, Jean de Ville de Goyet, Lidia Monti, Chiara Grimaldi, Alessandro Crocoli, Aurora Castellano
BACKGROUND: Hepatocellular carcinoma (HCC) is very rare in children and is traditionally associated with a poor prognosis because many patients are not amenable to conventional resection, even in the absence of metastatic disease. For patients with locally advanced or metastatic tumors, conventional chemotherapy appears to offer limited survival benefits. PATIENTS AND METHODS: We report a case series of five consecutive pediatric patients with HCC who were treated with bevacizumab along with conventional platinum-based chemotherapy...
March 2017: Anticancer Research
https://www.readbyqxmd.com/read/28306597/diagnosis-of-viral-hepatitis
#15
Philippa J Easterbrook, Teri Roberts, Anita Sands, Rosanna Peeling
PURPOSE OF REVIEW: Chronic hepatitis B virus (HBV) and hepatitis C virus (HCV) infections and HIV-HBV and HCV coinfection are major causes of chronic liver disease worldwide. Testing and diagnosis is the gateway for access to both treatment and prevention services, but there remains a large burden of undiagnosed infection globally. We review the global epidemiology, key challenges in the current hepatitis testing response, new tools to support the hepatitis global response (2016-2020 Global Hepatitis Health Sector strategy, and 2017 WHO guidelines on hepatitis testing and their key recommendations) and future directions and innovations in hepatitis diagnostics...
March 16, 2017: Current Opinion in HIV and AIDS
https://www.readbyqxmd.com/read/28302003/liver-transplant-for-nonhepatocellular-carcinoma-malignancy
#16
Nihan Haberal Reyhan
Liver transplant is now an acceptable and effective treatment for specific nonhepatocellular malignancies. Worldwide, hilar cholangiocarcinoma accounts for 3% of all primary gastrointestinal malignancies and for 10% of primary hepatobiliary malignancies. For patients who have early-stage, unresectable cholangiocarcinoma, liver transplant preceded by neoadjuvant radiotherapy can result in tumor-free margins, accomplish a radical resection, and treat the underlying primary sclerosing cholangitis when present...
March 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28301910/soluble-fms-like-tyrosine-kinase-1-as-a-link-between-angiogenesis-and-endothelial-dysfunction-in-pediatric-patients-with-%C3%AE-thalassemia-intermedia
#17
Azza Abdel Gawad Tantawy, Amira Abdel Moneam Adly, Eman Abdel Rahman Ismail, Omneya Ibrahim Youssef, Mohamed ElSayed Ali
Endothelial damage has been implicated in the pathogenesis of vascular complications in β-thalassemia intermedia (β-TI). Soluble fms-like tyrosine kinase 1 (sFLT-1) is a member of the vascular endothelial growth factor receptor (VEGFR) family. Soluble fms-like tyrosine kinase 1 is an antiangiogenic protein that induces endothelial dysfunction by adhering to and inhibiting VEGF and placenta growth factor. The aim of this study was to assess the level of sFLT-1 in 35 children and adolescents with β-TI, correlating it with markers of hemolysis and iron overload as well as cardiopulmonary complications...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28298942/liver-acoustic-radiation-force-impulse-arfi-in-childhood-obesity-comparison-and-correlation-with-biochemical-markers
#18
COMPARATIVE STUDY
Rajeev Kamble, Kushaljit S Sodhi, Babu R Thapa, Akshay K Saxena, Anmol Bhatia, Devi Dayal, Niranjan Khandelwal
PURPOSE: To compare and correlate the diagnostic efficiency of acoustic radiation force impulse (ARFI) elastography with biochemical markers for assessing hepatic changes in overweight and obese children. METHODS: This prospective study was approved by the institutional ethics committee. It included 54 overweight and obese children and 50 normal children (as a control group) in the age range 5-18 years. For all children, we performed grayscale ultrasonography to diagnose fatty liver, ARFI elastography to measure liver stiffness, and biochemical evaluation for aspartate aminotransferase (AST), alanine aminotransferase (ALT), and serum triglyceride (TG) levels...
March 2017: Journal of Ultrasound
https://www.readbyqxmd.com/read/28288778/videolaparoscopic-radical-nephrectomy-after-chemotherapy-in-the-treatment-of-wilms-tumor-long-term-results-of-a-pioneer-group
#19
Ricardo Jordão Duarte, Lilian Maria Cristofani, Vicente Odone Filho, Miguel Srougi, Francisco Tibor Dénes
INTRODUCTION: A high cure rate for Wilms' tumor has been achieved using a multidisciplinary approach. The natural step forward is to offer the benefits of a minimally invasive technique for surgery, which is an obligatory part of treatment. Nevertheless, some authors resist using videolaparoscopic radical nephrectomy (VRN) because of concerns about reducing the cure index. METHODS: The present study included children with unilateral Wilms' tumor treated from December 2003 to December 2015 with neoadjuvant chemotherapy followed by VRN...
February 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28288234/-benign-transient-hyperphosphatasaemia-in-infants-clinical-series
#20
Luisa Schonhaut B, Ana Rocha R
Alkaline Phosphatase (ALP) is a group of 4 isoenzymes produced in different tissues. Elevated levels of ALP can be developed under physiological conditions, and can indicate the presence of bone or hepatobiliary diseases. In children, one of its most common harmless causes is benign transient hyperphosphatasaemia (BTH), a little known condition. The objective is to report BTH cases and propose a monitoring plan. CASE REPORTS: The cases of 5 children aged between 11 and 50 months are presented, 4 of them female, with the incidental finding of a sudden and severe ALP elevation (> 1,000 U/l), in tests ordered due to either abnormal growth and development, or intercurrent infections...
February 2017: Revista Chilena de Pediatría
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