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Pediatric liver disease

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https://www.readbyqxmd.com/read/29141316/-a-clinical-analysis-of-micafungin-treatment-of-pulmonary-invasive-fungal-infection-in-pediatric-patients-with-acute-leukemia-or-post-hematopoietic-stem-cells-transplantation
#1
K Huang, K Y Qiu, L L Deng, J P Fang, Y Li, H X Guo, D H Zhou
Objective: To investigate the efficacy and safety of micafungin (MCF) for pulmonary invasive fungal disease (PIFD) in pediatric patients with acute leukemia or post hematopoietic stem cells transplantation. Method: Twenty-five neutropenic PIFD children with acute leukemia or post hematopoietic stem cells transplantation in Sun Yat-sen Memorial Hospital of Sun Yat-sen University were selected from January 2012 to June 2015, including 12 males and 13 females, age range 2-15 (average 6.2±2.0) years. There were 12 cases of acute leukemia (AL) after chemotherapy, 4 cases of acute leukemia (AL) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and 9 cases of β-thalassemia major after allo-HSCT...
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29131462/outcomes-of-liver-transplantation-in-pediatric-recipients-with-cardiovascular-disease
#2
Márcio Miranda Brito, Joao Seda Neto, Eduardo A Fonseca, Renata Pugliese, Vera B Danesi, Helry L Candido, Adriana Porta, Cristian V B Borges, Gilda Porta, Paulo Chapchap, Irene Kazue Miura
LT exerts considerable stress on the heart perioperatively. Limited data exist on impact of cardiovascular diseases on LT children. This study evaluated the outcomes of children with CVD who underwent LT and compared with pretransplant findings. From 518 LT recipients, 82 (15.8%) had CVD. Sixty patients were classified as low-risk adjustment for congenital heart surgery 1 (RACHS 1 and 2). Five patients were classified as RACHS ≥3. The most common echocardiographic finding in the CVD patients (25/82) was ASD...
November 12, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29130075/association-between-cytokines-and-liver-histology-in-children-with-nonalcoholic-fatty-liver-disease
#3
Emily R Perito, Veeral Ajmera, Nathan M Bass, Philip Rosenthal, Joel E Lavine, Jeffrey B Schwimmer, Katherine P Yates, Anna Mae Diehl, Jean P Molleston, Karen F Murray, Ann Scheimann, Ryan Gill, David Glidden, Bradley Aouizerat
Background: Reliable non-invasive markers to characterize inflammation, hepatocellular ballooning, and fibrosis in nonalcoholic fatty liver disease (NAFLD) are lacking. We investigated the relationship between plasma cytokine levels and features of NAFLD histology to gain insight into cellular pathways driving NASH and to identify potential non-invasive discriminators of NAFLD severity and pattern. Methods: Cytokines were measured from plasma obtained at enrollment in pediatric participants in NASH Clinical Research Network studies with liver biopsy-proven NAFLD...
September 2017: Hepatol Commun
https://www.readbyqxmd.com/read/29128061/the-genetics-of-pediatric-nonalcoholic-fatty-liver-disease
#4
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/29122442/serum-fetuin-a-levels-in-obese-children-with-biopsy-proven-nonalcoholic-fatty-liver-disease
#5
V Pampanini, E Inzaghi, D Germani, A Alterio, A Puglianiello, A Alisi, V Nobili, S Cianfarani
BACKGROUND AND AIMS: Fetuin-A has been proposed as a marker of liver damage in adults with obesity-related NAFLD. The aim of this study was to test serum fetuin-A concentrations in obese children with NAFLD diagnosed either by ultrasonography or by liver biopsy and to determine its applicability as predictive tool in pediatric NAFLD. METHODS AND RESULTS: Metabolic parameters and fetuin-A levels were investigated in 81 obese children with NAFLD diagnosed by biopsy, 79 obese children with NAFLD defined by liver ultrasonography and 23 lean subjects...
October 3, 2017: Nutrition, Metabolism, and Cardiovascular Diseases: NMCD
https://www.readbyqxmd.com/read/29116687/erythropoietic-protoporphyria-in-an-adult-with-sequential-liver-and-hematopoietic-stem-cell-transplantation-a-case-report
#6
Annika L Windon, Rashmi Tondon, Nathan Singh, Samir Abu-Gazala, David L Porter, J Eric Russell, Colleen Cook, Elaine Lander, Georgeine Smith, Kim M Olthoff, Abraham Shaked, Maarouf Hoteit, Emma E Furth, Marina Serper
Erythropoieitic protoporphyria (EPP) is a rare inherited disorder of the heme biosynthesis pathway resulting in the accumulation of protoporphyrins in the blood, erythrocytes, and other tissues. Because of a gene mutation in the FECH gene, ferrochelatase, the enzyme involved in the final step of heme synthesis, is deficient in these patients. While the major symptom of this disorder is photosensitivity, rarely, it can cause progressive liver disease requiring liver transplantation (LT). However, LT is not curative and only bone marrow transplantation (BMT) can correct the underlying enzymatic defect...
November 8, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/29114140/analysis-of-hospital-records-of-children-with-hydatid-cyst-in-south-of-iran
#7
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/29112089/assessment-of-liver-disease-progression-in-cystic-fibrosis-using-transient-elastography
#8
Anne-Laure Gominon, Eric Frison, Jean-Baptiste Hiriart, Julien Vergniol, Haude Clouzeau, Raphael Enaud, Stephanie Bui, Michael Fayon, Victor de Ledinghen, Thierry Lamireau
OBJECTIVES: Cystic fibrosis related liver disease (CFLD) can develop silently in early life and approximately 10% of children with cystic fibrosis (CF) become cirrhotic before adulthood. Clinical, biological and ultrasound criteria used to define CFLD often reveal liver involvement at an advanced stage. The aim of this retrospective study was to assess the progression of liver stiffness measurement (LSM) in pediatric CF patients. METHODS: The change of LSM, expressed as kPa/year and %/year, was measured using transient elastography (TE, Fibroscan) in 82 CF children (median age: 6...
November 3, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29110830/long-term-outcomes-of-pediatric-intestinal-failure
#9
Brenna S Fullerton, Charles R Hong, Tom Jaksic
Management of pediatric intestinal failure has evolved in recent decades, with improved survival since the advent of specialized multidisciplinary intestinal failure centers. Though sepsis and intestinal failure associated liver disease still contribute to mortality, we now have growing data on the long-term outcomes for this population. While intestinal adaptation and parenteral nutrition weaning is most rapid during the first year on parenteral support, achievement of enteral autonomy is possible even after many years as energy and protein requirements decline dramatically with age...
October 2017: Seminars in Pediatric Surgery
https://www.readbyqxmd.com/read/29108345/mri-based-evaluation-of-multiorgan-iron-overload-is-a-predictor-of-adverse-outcomes-in-pediatric-patients-undergoing-allogeneic-hematopoietic-stem-cell-transplantation
#10
Natalia Maximova, Massimo Gregori, Giulia Boz, Roberto Simeone, Davide Zanon, Giulia Schillani, Floriana Zennaro
The medical records of 44 pediatric patients who underwent allogeneic transplantation from 2011 to 2015 were retrospectively reviewed. Magnetic resonance imaging was used to measure iron concentrations in the liver, spleen, pancreas and bone. These patients were divided into two groups, 18 with non-elevated (< 100 μmol/g; Group 1) liver iron concentration before transplantation and 26 with elevated (> 100 μmol/g; Group 2) concentration . We compared transplant-related outcomes in the two groups. Iron overload was a negative prognostic risk factor for sinusoidal obstruction syndrome (OR = 17), osteoporosis (OR = 6...
October 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/29104077/assessment-of-atp8b1-deficiency-in-pediatric-patients-with-cholestasis-using-peripheral-blood-monocyte-derived-macrophages
#11
Hisamitsu Hayashi, Sotaro Naoi, Takao Togawa, Yu Hirose, Hiroki Kondou, Yasuhiro Hasegawa, Daiki Abukawa, Mika Sasaki, Koji Muroya, Satoshi Watanabe, Satoshi Nakano, Kei Minowa, Ayano Inui, Akinari Fukuda, Mureo Kasahara, Hironori Nagasaka, Kazuhiko Bessho, Mitsuyoshi Suzuki, Hiroyuki Kusuhara
Progressive familial intrahepatic cholestasis type 1 (PFIC1), a rare inherited recessive disease resulting from a genetic deficiency in ATP8B1, progresses to liver failure. Because of the difficulty of discriminating PFIC1 from other subtypes of PFIC based on its clinical and histological features and genome sequencing, an alternative method for diagnosing PFIC1 is desirable. Herein, we analyzed human peripheral blood monocyte-derived macrophages (HMDM) and found predominant expression of ATP8B1 in interleukin-10 (IL-10)-induced M2c, a subset of alternatively activated macrophages...
October 7, 2017: EBioMedicine
https://www.readbyqxmd.com/read/29099463/associations-of-insulin-resistance-and-glycemia-with-liver-enzymes-in-hispanic-latino-youths-results-from-the-hispanic-community-children-s-health-study-study-of-latino-youth-sol-youth
#12
Christina M Parrinello, Bryan J Rudolph, Mariana Lazo, Linda C Gallo, Bharat Thyagarajan, Scott J Cotler, Qibin Qi, Tossaporn Seeherunvong, Denise C Vidot, Howard D Strickler, Robert C Kaplan, Carmen R Isasi
BACKGROUND: Associations of insulin resistance and hyperglycemia with a panel of liver enzymes have not been well studied in a young, heterogenous Hispanic/Latino population. We aimed to assess the associations of insulin resistance and glycemia with nonalcoholic fatty liver disease (NAFLD), as measured by liver enzymes and the pediatric NAFLD fibrosis index (PNFI), and whether these associations are modified by body mass index and mediated by inflammation or endothelial dysfunction. MATERIALS AND METHODS: We conducted a cross-sectional study of 1317 boys and girls aged 8 to 16 years from the Hispanic Community Children's Health Study/Study of Latino Youth...
November 3, 2017: Journal of Clinical Gastroenterology
https://www.readbyqxmd.com/read/29098318/-wilson-s-disease-what-has-been-confirmed-in-diagnostic-and-therapy
#13
REVIEW
E-D Pfister
Wilson's disease (WD) is a rare autosomal recessive disorder characterized by abnormal copper accumulation. Presenting a broad variety of phenotypes and, thus, being a chameleon within the group of metabolic diseases, the manifold clinical symptoms of WD can include hepatologic, neurologic, and psychiatric manifestations. Early onset presentations in infancy and late-onset manifestations in adults older than 70 years of age have been described. If the typical laboratory blood test values are missing, the diagnosis of WD may be difficult and often involves a combination of different parameters...
November 2, 2017: Der Internist
https://www.readbyqxmd.com/read/29095346/pediatric-non-alcoholic-fatty-liver-disease-current-thinking
#14
Valerio Nobili, Piotr Socha
Non-alcoholic fatty liver disease (NAFLD), an increasingly prevalent paediatric disorder is diagnosed and managed by both paediatric gastroenterologists / hepatologists but also frequently by the general paediatrician. This paper updates recent advances in diagnostic and therapeutic approach which may be applied to everyday practice. Diagnosis of NAFLD takes into account the risk factor profile and is a diagnosis of exclusion. Techniques such as transient elastography and specific biomarkers aimed at improving diagnosis and monitoring of NAFLD need further validation in the paediatric population...
October 31, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29080393/lipid-profile-pattern-in-pediatric-overweight-population-with-or-without-nafld-in-relation-to-idf-criteria-for-metabolic-syndrome-a-preliminary-study
#15
Anca Bălănescu, Paul Bălănescu, Valentina Comanici, Iustina Stan, Beata Acs, Laura Prisăcariu, Florin Brezan, Tatiana Ciomârtan, Ioan Gherghina
BACKGROUND AND AIMS: The aim of this study is to assess the lipid profile pattern of pediatric overweight and/or obese patients with Non-Alcoholic Fatty Liver Disease (NAFLD) in relation to IDF Consensus Criteria for Metabolic Syndrome (MetS). MATERIAL AND METHODS: We conducted a cross-sectional preliminary study on 45 consecutive pediatric patients. Overweight or obese children aged from 3 to 18 years were included. Standardized measurement of blood pressure and anthropometric parameters were performed...
October 26, 2017: Romanian Journal of Internal Medicine, Revue Roumaine de Médecine Interne
https://www.readbyqxmd.com/read/29080241/next-generation-sequencing-in-pediatric-hepatology-and-liver-transplantation
#16
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/29076289/orthotopic-liver-transplantation-for-sensenbrenner-syndrome
#17
Ruth Linda Ackah, Dor Yoeli, Michael Kueht, Nhu Thao Nguyen Galván, Ronald Timothy Cotton, Abbas Rana, Christine Ann O'Mahony, John Alan Goss
Sensenbrenner syndrome, or cranioectodermal dysplasia, is a rare heterogeneic autosomal recessive disorder, affecting ~1 of 1 000 000 live births. The syndrome usually manifests within the first year of life and can present with progressive liver and renal involvement. For all Sensenbrenner patients, renal and liver diseases are the main contributors of morbidity and mortality. In this report, we present the case of a 7-year-old boy with congenital liver disease progressing to liver failure secondary to Sensenbrenner syndrome...
October 26, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29072244/oral-and-dental-health-in-children-with-chronic-liver-disease-in-the-turkey-northeast
#18
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/29071779/risk-factors-and-clinical-outcomes-of-pediatric-liver-transplant-recipients-with-post-transplant-lymphoproliferative-disease-in-a-multi-ethnic-asian-cohort
#19
James Guoxian Huang, Mervin Ye Qing Tan, Seng-Hock Quak, Marion Margaret Aw
BACKGROUND: We aimed to evaluate clinical characteristics, risk factors, and disease outcomes for liver transplant recipients (LTR) with post-transplant lymphoproliferative disease (PTLD) at our center. METHODS: Retrospective review of data of all pediatric LTR (1991-2015) was conducted. RESULTS: The overall incidence of PTLD was 16.4% (18/110), the majority (13/18) were early lesions, while 3/18 were polymorphic/monomorphic PTLD. The risk factors significant on univariate analysis were as follows: mean age (years) at transplant (1...
October 26, 2017: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/29064931/transaminases-and-pediatric-nonalcoholic-fatty-liver-disease-diagnosis
#20
Pietro Vajro
No abstract text is available yet for this article.
November 2017: Journal of Pediatric Gastroenterology and Nutrition
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