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hyperferritinemia

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https://www.readbyqxmd.com/read/28809726/clinical-and-laboratory-associations-with-persistent-hyperferritinemia-in-373-black-hemochromatosis-and-iron-overload-screening-study-participants
#1
James C Barton, J Clayborn Barton, Paul C Adams
BACKGROUND: 373 black participants had elevated screening and post-screening serum ferritin (SF) (> 300 μg/L men; > 200 μg/L women). MATERIAL AND METHODS: We retrospectively studied SF and post-screening age; sex; body mass index; transferrin saturation (TS); ALT; AST; GGT; elevated C-reactive protein; ß-thalassemia; neutrophils; lymphocytes; monocytes; platelets; metacarpophalangeal joint hypertrophy; hepatomegaly; splenomegaly; diabetes; HFE H63D positivity; iron/alcohol intakes; and blood/erythrocyte transfusion units...
August 8, 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28762079/elevated-serum-ferritin-is-not-specific-for-hemophagocytic-lymphohistiocytosis
#2
Zaher K Otrock, Karl G Hock, Sarah B Riley, Theo de Witte, Charles S Eby, Mitchell G Scott
Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal, syndrome of excessive and ineffective activation of the immune system. The majority of the reported data on HLH is from pediatric patients and lacks specificity. This makes HLH diagnosis challenging especially in adults where HLH is triggered by many conditions and can resemble many disease entities. Elevated ferritin is one of the diagnostic criteria for HLH. We determined the conditions associated with elevated ferritin at our medical center to assess how specific ferritin is for predicting HLH...
July 31, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28760295/diagnostic-challenges-of-hemophagocytic-lymphohistiocytosis
#3
REVIEW
Zaher K Otrock, Naval Daver, Hagop M Kantarjian, Charles S Eby
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by excessive activation of the immune system, resulting in overproduction of inflammatory cytokines. Patients usually present with high fever, cytopenias, hyperferritinemia, and hepatosplenomegaly, and their disease process ranges from mild to fatal multiorgan failure. HLH is a heterogeneous group of disorders that can be triggered by infections, neoplasms, or autoimmune diseases. The HLH diagnosis can be difficult to confidently confirm in critically ill patients while waiting for pathology or reference laboratory results to return, delaying the diagnosis with significantly worsened outcomes...
July 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28746593/ferritin-light-chain-gene-mutations-in-two-brazilian-families-with-hereditary-hyperferritinemia-cataract-syndrome
#4
Roberta Cardoso Petroni, Susana Elaine Alves da Rosa, Flavia Pereira de Carvalho, Rúbia Anita Ferraz Santana, Joyce Esteves Hyppolito, Claudia Mac Donald Bley Nascimento, Nelson Hamerschlak, Paulo Vidal Campregher
Hereditary hyperferritinemia-cataract syndrome is an autosomal dominant genetic disorder associated with mutations in the 5'UTR region of the ferritin light chain gene. These mutations cause the ferritin levels to increase even in the absence of iron overload. Patients also develop bilateral cataract early due to accumulation of ferritin in the lens, and many are misdiagnosed as having hemochromatosis and thus not properly treated. The first cases were described in 1995 and several mutations have already been identified...
July 24, 2017: Einstein
https://www.readbyqxmd.com/read/28732360/a-systematic-review-of-malignancy-associated-hemophagocytic-lymphohistiocytosis-that-needs-more-attentions
#5
REVIEW
Hongluan Wang, Lixia Xiong, Weiping Tang, Ying Zhou, Fei Li
As an infrequent but potentially life-threatening hyperinflammatory syndrome, hemophagocytic lymphohistiocytosis (HLH) is clinically characterized with prolonged fever, hepatosplenomegaly, cytopenia, hypertriglyceridemia, hyperferritinemia and hemophagocytosis in bone marrow, liver, spleen or lymph nodes. Malignancy-associated HLH (M-HLH), one type of acquired HLH, usually presents variable overlaps of symptoms with other types of HLH, thus resulting in higher incidence of misdiagnosis and mortality. In recent years, with the increasing awareness to this disease, the diagnosis and management of HLH have gained more and more attention, and improvements have been made accordingly...
July 14, 2017: Oncotarget
https://www.readbyqxmd.com/read/28725546/pulmonary-involvement-in-adult-still-s-disease-case-report-and-brief-review-of-literature
#6
Aldo Guerrieri, Giulia Angeletti, Massimiliano Mazzolini, Ilaria Bassi, Stefano Nava
BACKGROUND: Adult onset Still disease (AOSD) is a rare condition characterized by elevated fever along with arthritic symptoms, elevated polymorphonuclear neutrophil count, evanescent rash, and hyperferritinemia. Diagnosis can be made only after have ruled out more frequent conditions, and Yamagouchi or Fautrel criteria should be applied. Parenchimal lung involvement (PLI) is present in less than 5% of AOSD cases and ranges from aspecific reticular interstitial opacities to life threatening conditions, such as acute respiratory distress syndrome (ARDS)...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28679028/hepatic-iron-is-the-major-determinant-of-serum-ferritin-in-nafld-patients
#7
John D Ryan, Andrew E Armitage, Jeremy F Cobbold, Rajarshi Banerjee, Oscar Borsani, Paola Dongiovanni, Stefan Neubauer, Reza Morovat, Lai Mun Wang, Sant-Rayn Pasricha, Silvia Fargion, Jane Collier, Eleanor Barnes, Hal Drakesmith, Luca Valenti, Michael Pavlides
BACKGROUND AND AIMS: Elevated serum ferritin is common in NAFLD, and is associated with more advanced disease and increased mortality. Hyperferritinemia in NAFLD is often attributed to inflammation, while in other conditions ferritin closely reflects body iron stores. The aim of this study was to clarify the underlying cause of hyperferritinemia in NAFLD. METHODS: Ferritin levels were examined with markers of iron status, inflammation and liver injury across the clinical spectrum of NAFLD using blood, tissue and magnetic resonance (MR) imaging...
July 5, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28626188/discoid-lupus-erythematosus-complicated-with-pregnancy-induced-hemophagocytic-syndrome
#8
Hideto Takada, Naoki Kimura, Yoko Yoshihashi-Nakazato, Kimito Kawahata, Hitoshi Kohsaka
A 35-year-old woman with discoid lupus erythematosus (DLE) was admitted at 11 weeks' gestation with a persistent fever. Laboratory studies revealed pancytopenia, elevated liver enzymes, and hyperferritinemia. Bone marrow aspiration confirmed the diagnosis of hemophagocytic syndrome (HPS). She had no findings of infection or active systemic lupus erythematosus. The administration of high-dose corticosteroids resolved the clinical and laboratory findings. She delivered a healthy baby at 35 weeks' gestation. This case suggests that DLE can be a predisposing factor for pregnancy-induced HPS...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28594937/hyperferritinemia-and-hypergammaglobulinemia-predict-the-treatment-response-to-standard-therapy-in-autoimmune-hepatitis
#9
Richard Taubert, Matthias Hardtke-Wolenski, Fatih Noyan, Claudine Lalanne, Danny Jonigk, Jerome Schlue, Till Krech, Ralf Lichtinghagen, Christine S Falk, Verena Schlaphoff, Heike Bantel, Luigi Muratori, Michael P Manns, Elmar Jaeckel
Autoimmune hepatitis (AIH) is a chronic hepatitis with an increasing incidence. The majority of patients require life-long immunosuppression and incomplete treatment response is associated with a disease progression. An abnormal iron homeostasis or hyperferritinemia is associated with worse outcome in other chronic liver diseases and after liver transplantation. We assessed the capacity of baseline parameters including the iron status to predict the treatment response upon standard therapy in 109 patients with untreated AIH type 1 (AIH-1) in a retrospective single center study...
2017: PloS One
https://www.readbyqxmd.com/read/28593739/systematic-review-and-meta-analysis-to-determine-the-impact-of-iron-depletion-in-dysmetabolic-iron-overload-syndrome-and-nonalcoholic-fatty-liver-disease
#10
Arvind R Murali, Ashutosh Gupta, Kyle Brown
AIMS: Iron reduction has been proposed as treatment for dysmetabolic iron overload syndrome (DIOS) and nonalcoholic fatty liver disease (NAFLD), but results of published trials are conflicting. We aimed to perform a systematic review and meta-analysis to determine the impact of phlebotomy in DIOS and NAFLD. METHODS: We searched multiple databases systematically for studies evaluating impact of phlebotomy in DIOS and NAFLD. We calculated weighted summary estimates using inverse variance method...
June 7, 2017: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/28591160/the-association-of-ferritin-with-cardiovascular-and-all-cause-mortality-in-community-dwellers-the-english-longitudinal-study-of-ageing
#11
Nikolaos P E Kadoglou, Jane P Biddulph, Snorri B Rafnsson, Marialena Trivella, Petros Nihoyannopoulos, Panayotes Demakakos
BACKGROUND: Ferritin constitutes a sensitive iron-storage index and multi-functional protein. Evidence on its association with mortality in general population is scarce and conflicting. We investigated the sex-specific associations of ferritin levels with all-cause and cardiovascular mortality in a population-based cohort. METHODS: Data came from the English Longitudinal Study of Ageing and the national mortality registry. The sample comprised 5,471 participants aged ≥52 years...
2017: PloS One
https://www.readbyqxmd.com/read/28585071/impact-of-non-transferrin-bound-iron-ntbi-in-comparison-to-serum-ferritin-on-outcome-after-allogeneic-stem-cell-transplantation-asct
#12
COMPARATIVE STUDY
Annekathrin Hilken, Claudia Langebrake, Christine Wolschke, Jan Felix Kersten, Holger Rohde, Peter Nielsen, Nicolaus Kröger
The optimal parameters and time points for the measurement of iron overload (IO) in allogeneic stem cell transplantation (ASCT) patients are still under discussion. Hyperferritinemia and IO are poor prognostic factors in ASCT. We hypothesize that non-transferrin-bound iron (NBTI) is possibly a better marker to predict the effect of IO on the outcome than serum ferritin (SF), which however is not specific for IO. The aim of this prospective observational trial was to evaluate the influence of NBTI in comparison to SF on the outcome of ASCT patients [overall survival, bloodstream infections (BSIs), and invasive fungal infections (IFIs)]...
August 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28562511/kawasaki-disease-complicated-with-macrophage-activation-syndrome-a-systematic-review
#13
Susana García-Pavón, Marco A Yamazaki-Nakashimada, Milton Báez, Karla L Borjas-Aguilar, Chiharu Murata
Macrophage activation syndrome (MAS), also known as secondary hemophagocytic lymphohistiocytosis, is a rare and potentially fatal complication of Kawasaki disease (KD). We report 2 cases, performed a literature search, and analyze the characteristics of MAS associated with KD. A total of 69 patients were evaluated, 34 reported the date of the diagnosis of MAS and KD, 6% had a diagnosis of MAS before KD, 21% had a simultaneous presentation, and 73% had the diagnosis of MAS after KD. Different treatment approaches were observed with corticosteroids administered in 87%, cyclosporine in 49%, etoposide (VP-16) in 39%, and monoclonal anti-TNF in 6% of cases...
August 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28541437/hyperferritinemia-and-inflammation
#14
Kate Kernan, Joseph A Carcillo
Understanding of ferritin biology has traditionally centered on its role in iron storage and homeostasis, with low ferritin levels indicative of deficiency and high levels indicative of primary or secondary hemochromatosis. However, further work has shown that iron, redox biology and inflammation are inexorably linked. During infection, increased ferritin levels represent an important host defense mechanism that deprives bacterial growth of iron and protect immune cell function. It may also be protective, limiting the production of free radicals and mediating immunomodulation...
May 25, 2017: International Immunology
https://www.readbyqxmd.com/read/28489771/chronic-active-epstein-barr-virus-infection-associated-with-hemophagocytic-syndrome-and-extra-nodal-natural-killer-t-cell-lymphoma-in-an-18-year-old-girl-a-case-report
#15
Yawei Xing, Junwen Yang, Guanghui Lian, Shuijiao Chen, Linlin Chen, Fujun Li
RATIONALE: Chronic active Epstein-Barr virus infection (CAEBV) associated with hemophagocytic syndrome (HPS) and extra-nodal natural killer (NK)/T-cell lymphoma (ENKL) is a rare life-threatening disorder. This disease is easily misdiagnosed because of its varied presentations. PATIENT CONCERNS: An 18-year-old girl was admitted to our hospital with a history of edema in the lower limbs and intermittent fever lasting for more than 1 month. At admission, she had severe liver injury of unknown etiology...
May 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28489136/hyperferritinemia-in-patients-with-nonalcoholic-fatty-liver-disease
#16
Raffaelle K Barros, Helma Pinchemel Cotrim, Carla H Daltro, Yanaihara A Oliveira
Objective: In liver diseases, hyperferritinemia (HYF) is related to injured cells in acquired and genetic conditions with or without iron overload. It is frequent in patients with nonalcoholic fatty liver disease (NAFLD), in which it is necessary to define the mean of HYF to establish the better approach for them. The present study evaluated the significance of elevated ferritin in patients with NAFLD and steatohepatitis (NASH). Method: The review was performed using search instruments of indexed scientific material, including MEDLINE (by PubMed), Web of Science, IBECS and LILACS, to identify articles published in Portuguese, English and Spanish, from 2005 to May, 2016...
March 2017: Revista da Associação Médica Brasileira
https://www.readbyqxmd.com/read/28409239/macrophage-activation-syndrome-in-systemic-lupus-erythematosus-a-multicenter-case-control-study-in-china
#17
Ai-Chun Liu, Yue Yang, Meng-Tao Li, Yuan Jia, Sheng Chen, Shuang Ye, Xiang-Zong Zeng, Zhao Wang, Jin-Xia Zhao, Xiang-Yuan Liu, Jian Zhu, Yan Zhao, Xiao-Feng Zeng, Zhan-Guo Li
The objective of this study was to describe the clinical and laboratory characteristics, precipitating factors, treatment, and outcome of macrophage activation syndrome (MAS) complicating systemic lupus erythematosus (SLE). A multicenter case-control study was performed across six tertiary hospitals from 1997 to 2014. A total of 32 patients with SLE-associated MAS were enrolled. Sixty-four age- and sex-matched SLE patients diagnosed in the same period without MAS episodes were selected as controls. The most frequent clinical feature was fever, followed by splenomegaly...
April 13, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28376538/-hepatic-manifestation-of-a-macrophage-activation-syndrome-mas
#18
Michael Nagel, Andreas Schwarting, Beate K Straub, Peter R Galle, Tim Zimmermann
Background Elevated liver values are the most common pathological laboratory result in Germany. Frequent findings, especially in younger patients, are nutritive- or medicamentous- toxic reasons, viral or autoimmune hepatitis. A macrophage activation syndrome (MAS) may manifest like a viral infectious disease with fever, hepatosplenomegaly and pancytopenia and is associated with a high mortality. It is based on an enhanced activation of macrophages with increased cytokine release, leading to organ damage and multi-organ failure...
May 2017: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/28348452/hyperferritinemia-as-a-diagnostic-marker-for-severe-fever-with-thrombocytopenia-syndrome
#19
Uh Jin Kim, Tae Hoon Oh, Bansuk Kim, Seong Eun Kim, Seung-Ji Kang, Kyung-Hwa Park, Sook-In Jung, Hee-Chang Jang
Severe fever with thrombocytopenia syndrome (SFTS) is an emerging viral disease in East Asia with high mortality. Few studies have examined markers that suggest SFTS in febrile patients. To determine useful biochemical markers for SFTS, patients aged 18 years or older with SFTS or microbiologically confirmed community-onset bacteremia with thrombocytopenia (BT) at presentation between June 2013 and December 2015 were included from two tertiary university hospitals in Republic of Korea retrospectively. Eleven patients with SFTS and 62 patients with bacteremia and thrombocytopenia were identified in the study period...
2017: Disease Markers
https://www.readbyqxmd.com/read/28342280/clinical-and-histopathological-features-of-cutaneous-manifestations-of-adult-onset-still-disease
#20
Erin Santa, Jeanne M McFalls, Joya Sahu, Jason B Lee
Adult-onset Still disease (AOSD) is a rare autoinflammatory syndrome characterized by recurring fevers, arthralgia, and consistent laboratory abnormalities that include leukocytosis and hyperferritinemia. Skin findings accompany the disease in nearly 90% of the cases. Early reports described evanescent, pruritic, salmon-pink or urticarial lesions, referred to as the typical eruption of AOSD. Histopathologic findings consist of superficial perivascular dermatitis with varying number of interstitial neutrophils...
June 2017: Journal of Cutaneous Pathology
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