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hyperferritinemia

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https://www.readbyqxmd.com/read/28060133/secondary-hemophagocytic-lymphohistiocytosis-do-we-really-need-chemotherapeutics-for-all-patients
#1
Zeliha Haytoglu, Nalan Yazici, Ayse Erbay
Because of the acute and life-threatening course of the hemophagocytic lymphohistiocytosis (HLH) syndrome, International Histiocyte Society guidelines recommend chemoimmune therapy for the treatment of both primary and secondary HLH (sHLH). To manage children with sHLH, instead of HLH-2004 protocol we considered less immunosuppressive/cytotoxic approach. We assessed 12 children who fulfilled the diagnostic criteria for sHLH between January 2009 and March 2015. Multivariate Cox regression analysis showed that ferritin levels (hazard ratio=1...
January 5, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28052375/unexplained-isolated-hyperferritinemia-without-iron-overload
#2
Giulia Ravasi, Sara Pelucchi, Raffaella Mariani, Marco Casati, Federico Greni, Cristina Arosio, Irene Pelloni, Silvia Majore, Paolo Santambrogio, Sonia Levi, Alberto Piperno
Although hyperferritinemia may be reflective of elevated total body iron stores, there are conditions in which ferritin levels are disproportionately elevated relative to iron status. Autosomal dominant forms of hyperferritinemia due to mutations in the L-ferritin IRE or in A helix of L-ferritin gene have been described, however cases of isolated hyperferritinemia still remain unsolved. We describe 12 Italian subjects with unexplained isolated hyperferritinemia (UIH). Four probands have affected siblings, but no affected parents or offspring...
January 4, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/27976635/outcome-of-phlebotomy-for-treating-nonalcoholic-fatty-liver-disease-a-systematic-review-and-meta-analysis
#3
Veeravich Jaruvongvanich, Tanawan Riangwiwat, Anawin Sanguankeo, Sikarin Upala
BACKGROUND/AIMS: No medications have been approved for managing nonalcoholic fatty liver disease (NAFLD). Lifestyle intervention is the mainstay for its treatment. Hyperferritinemia, which appears to be associated with the severity of liver injury and insulin resistance, is frequently observed in patients with NAFLD. PATIENTS AND METHODS: We conducted a systematic review and meta-analysis of the outcomes of four interventional trials regarding the effect of phlebotomy in patients with NAFLD versus the outcomes of NAFLD patients who did not undergo phlebotomy...
November 2016: Saudi Journal of Gastroenterology: Official Journal of the Saudi Gastroenterology Association
https://www.readbyqxmd.com/read/27956374/current-review-of-iron-overload-and-related-complications-in-hematopoietic-stem-cell-transplantation
#4
Erden Atilla, Selami K Toprak, Taner Demirer
Iron overload is an adverse prognostic factor for patients undergoing hematopoietic stem cell transplantation (HSCT). In HSCT setting, pretransplant and early posttransplant ferritin and transferrin saturation were found to be highly elevated due to high transfusion requirements. In addition to that, post-HSCT iron overload had shown to be related with infections, hepatic sinusoidal obstruction syndrome (SOS), mucositis, liver dysfunction and acute GVHD. Hyperferritinemia causes decreased survival rates in both pre and post transplant setting...
December 12, 2016: Turkish Journal of Haematology: Official Journal of Turkish Society of Haematology
https://www.readbyqxmd.com/read/27922757/hemophagocytic-lymphohistiocytosis-triggered-by-gaucher-disease-in-a-preterm-neonate
#5
Simone Schüller, Andishe Attarbaschi, Angelika Berger, Caroline Hutter, Katrin Klebermass-Schrehof, Manuel Steiner
OBJECTIVE: To present the diagnostic workup in an extremely low birth weight infant patient with signs of both sepsis and hemophagocytosis. PARTICIPANTS: A preterm infant presented with clinical and laboratory signs of early-onset sepsis including hepatosplenomegaly, thrombocytopenia, direct hyperbilirubinemia, and elevated liver enzymes. METHODS: Despite extensive septic workup, no underlying infection was detected. Additional hyperferritinemia and other elevated inflammatory parameters raised the suspicion of a primary or secondary hemophagocytic lymphohistiocytosis (HLH)...
October 2016: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/27912127/hyperferritinemia-is-a-potential-marker-of-chronic-chikungunya-a-retrospective-study-on-the-island-of-cura%C3%A3-ao-during-the-2014-2015-outbreak
#6
Fatih Anfasa, Lisette Provacia, Corine GeurtsvanKessel, Robert Wever, Izzy Gerstenbluth, Albert D M E Osterhaus, Byron E E Martina
BACKGROUND: Recently Chikungunya virus (CHIKV) outbreaks have been reported in the Carribean. There is no data regarding the outbreak in Curaçao. In addition, to date there is no biomarker that could be used to predict chronic infection. OBJECTIVES: To characterize the first CHIKV outbreak in Curaçao and to identify potential biomarkers for chronic infection. STUDY DESIGN: A serological test and quantitative polymerase chain reaction (qPCR) were used on samples collected in Curaçao to confirm infection...
January 2017: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
https://www.readbyqxmd.com/read/27893685/acute-liver-failure-caused-by-hemophagocytic-lymphohistiocytosis-in-adults-a-case-report-and-review-of-the-literature
#7
Shide Lin, Ying Li, Jun Long, Qichuan Liu, Fangwan Yang, Yihuai He
BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare condition that can be caused by a primary or acquired disorder of uncontrolled immune response. Liver injury is a common complication of HLH; however, HLH presenting as acute liver failure (ALF) has rarely been reported in adults. CASE SUMMARY: A 34-year-old man was admitted to our hospital with nausea and fatigue persisting for 2 weeks and jaundice for 1 week. He had hyperthermia at the onset of disease...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27883370/epstein-barr-virus-associated-hemophagocytic-syndrome-after-scrub-typhus-infection
#8
Jeong Woo Hong, Hyun Seon You, Tae Won Lee, Won Yong Jo, Bo Ra Kim, Young Sun Suh, In Gyu Bae, Oh Hyun Cho
There have been a small number of cases of scrub typhus-associated hemophagocytic syndrome (HPS), most of which were treated successfully using adequate antibiotics. Here, we report a case of Epstein-Barr virus (EBV)-associated HPS after scrub typhus infection that was not improved using antirickettsial treatment. A 73-year-old male who had been diagnosed with scrub typhus according to an eschar and a positive serology was transferred to our institution because of a persistent fever despite 7-day doxycycline therapy...
December 2016: Infection & Chemotherapy
https://www.readbyqxmd.com/read/27879547/hyperferritinemia-in-pediatric-acute-lymphoblastic-leukemia-what-does-it-mean
#9
Charlotte K Brierley, Raquel Revuelta Iniesta, Neill Storrar, Angela E Thomas
No abstract text is available yet for this article.
November 22, 2016: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/27818826/macrophage-activation-syndrome-associated-with-adult-onset-still-s-disease-successfully-treated-with-anakinra
#10
Aswini Kumar, Hiroshi Kato
Macrophage activation syndrome (MAS) is a potentially fatal complication of Adult-Onset Still's disease (Still's disease). Whereas an increasing body of evidence supports interleukin-1 (IL-1) blockade as a promising treatment for Still's disease, whether it is therapeutic for MAS associated with Still's disease remains unclear. We report a 34-year-old Caucasian man with one-decade history of TNF-blockade-responsive seronegative arthritis who presented with abrupt onset of fever, serositis, bicytopenia, splenomegaly, hepatitis, and disseminated intravascular coagulation...
2016: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/27816428/ferritinemia-and-serum-inflammatory-cytokines-in-swedish-adults-with-gaucher-disease-type-1
#11
Fryderyk Lorenz, Ewa Pawłowicz, Monika Klimkowska, Soheir Beshara, Agnes Bulanda Brustad, Aleksander B Skotnicki, Anders Wahlin, Maciej Machaczka
BACKGROUND: The storage of glucosylceramide in macrophages produces an inflammatory response in Gaucher disease type 1 (GD1) resulting in iron metabolism dysregulation and cytokine release. PATIENTS AND METHODS: The study included 16 adults with GD1 aged 20-86years. All but one patient carried at least one allele with the c.1226A>G (N370S) mutation in the GBA1 gene. Ferritinemia, iron metabolism profiles including hepcidin, and inflammatory cytokine concentrations were assessed in GD1 patients in Sweden...
October 20, 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/27733745/unusual-association-of-hemophagocytic-lymphohistiocytosis-in-systemic-lupus-erythematosus-cases-reported-at-tertiary-care-center
#12
Devika Gupta, Supreet Mohanty, Deepshi Thakral, Arvind Bagga, Naveet Wig, Dipendra Kumar Mitra
BACKGROUND Hemophagocytic lymphohistiocytosis (HLH) in the background of systemic lupus erythematosus (SLE) is rare. Inability to discriminate between these two entities may be fatal for the patient. Here we report two cases of SLE with secondary HLH, one of which manifested HLH as the initial presentation, and the significance of HLH's timely diagnosis. CASE REPORT We describe two cases of SLE secondarily affected by HLH, which were diagnosed by various laboratory parameters and detection of profoundly reduced NK cell activity by using flow cytometry...
October 13, 2016: American Journal of Case Reports
https://www.readbyqxmd.com/read/27708893/an-unusual-manifestation-in-a-patient-with-adult-onset-still-s-disease-minimal-glomerular-lesion
#13
Imane El Mezouar, Fatima Zahra Abourazzak, Najoua Ghani, Taoufik Harzy
Adult-onset Still's disease (AOSD) is a multisystem inflammatory disease of unknown etiology. It is characterized by arthritis, hectic fever, transient rash and visceral lesions such as pleuropericarditis, lymphadenopathy and hepato splenomegaly. Although kidney involvement may appear in some cases of AOSD, minimal glomerular lesion (MGL) has not been described. We describe a female patient, who presented with multisystemic manifestations, including high spiking fever, arthralgias, striking hyperferritinemia, and proteinuria...
September 2014: European Journal of Rheumatology
https://www.readbyqxmd.com/read/27683412/congenital-hyperferritinemia-diagnosed-in-a-2-month-old-a-case-report-from-india
#14
Moushumi Lodh, Joshi Anand Kerketta
BACKGROUND: In clinical medicine, ferritin is predominantly utilized as a serum marker of total body iron stores. In cases of iron deficiency and overload, serum ferritin serves a critical role in both diagnosis and management. Elevated serum and tissue ferritin are linked to coronary artery disease, malignancy, and poor outcomes following stem cell transplantation. Ferritin is directly implicated in less common but potentially devastating human diseases including sideroblastic anemias, neurodegenerative disorders, and hemophagocytic syndrome...
July 2012: EJIFCC
https://www.readbyqxmd.com/read/27683338/determination-of-serum-ferritin-glycosylation-in-hyperferritinemia-associated-to-iron-overload-and-inflammation
#15
Bethina Isasi Gasser
BACKGROUND: Serum ferritin is a commonly used clinical biochemical parameter and hyperferritinemia is used as a surrogate marker for iron overload, acute or chronic inflammation, malignancy or cell death. The aim of the present study was to develop purification strategies of ferritin from sera to determine if micro-heterogeneity of serum ferritin can be used to differentiate the underlying cause of the hyperferritinemia. PATIENTS MATERIALS AND METHODS: Sera from patients with hemochromatosis, rheumatologic diseases, aceruloplasminemia, ferroportin disease or iron loading anemia have been collected and stored and ferritin purified by negative affinity followed by ion exchange and size exclusion chromatography...
August 2009: EJIFCC
https://www.readbyqxmd.com/read/27678349/exploring-the-metabolic-syndrome-nonalcoholic-fatty-pancreas-disease
#16
REVIEW
Roberto Catanzaro, Biagio Cuffari, Angelo Italia, Francesco Marotta
After the first description of fatty pancreas in 1933, the effects of pancreatic steatosis have been poorly investigated, compared with that of the liver. However, the interest of research is increasing. Fat accumulation, associated with obesity and the metabolic syndrome (MetS), has been defined as "fatty infiltration" or "nonalcoholic fatty pancreas disease" (NAFPD). The term "fatty replacement" describes a distinct phenomenon characterized by death of acinar cells and replacement by adipose tissue. Risk factors for developing NAFPD include obesity, increasing age, male sex, hypertension, dyslipidemia, alcohol and hyperferritinemia...
September 14, 2016: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/27664384/prognostic-factors-of-macrophage-activation-syndrome-at-the-time-of-diagnosis-in-adult-patients-affected-by-autoimmune-disease-analysis-of-41-cases-collected-in-2-rheumatologic-centers
#17
MULTICENTER STUDY
Piero Ruscitti, Paola Cipriani, Francesco Ciccia, Francesco Masedu, Vasiliki Liakouli, Francesco Carubbi, Onorina Berardicurti, Giuliana Guggino, Paola Di Benedetto, Salvatore Di Bartolomeo, Marco Valenti, Giovanni Triolo, Roberto Giacomelli
Macrophage activation syndrome (MAS) is a rare, life-threatening disease in which early diagnosis and aggressive therapeutic strategy may improve the outcome. Due to its rarity, epidemiologic data are still lacking. Hyperferritinemia is frequently associated with MAS and might modulate the cytokine storm, which is involved in the development of multiple organ failure. In this paper, we investigated clinical data, treatments, and outcome of a homogeneous cohort of 41 adult MAS patients, complicating autoimmune rheumatic diseases...
January 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27659401/hyperferritinemia-increases-the-risk-of-hyperuricemia-in-hfe-hereditary-hemochromatosis
#18
Jérémy Flais, Edouard Bardou-Jacquet, Yves Deugnier, Guillaume Coiffier, Aleth Perdriger, Gérard Chalès, Martine Ropert, Olivier Loréal, Pascal Guggenbuhl
OBJECTIVES: Hyperuricemia is becoming increasingly frequent in the population, and is known to be sometimes the cause of gout. The impact of uric acid is still not clearly understood, however. The iron metabolism may interact with the uric acid metabolism. The aim of this study was to examine the relationship between the serum uric acid and serum ferritin levels in a cohort of hemochromatosis patients who were homozygous for the HFE p.Cys282Tyr mutation. METHODS: 738 patients with the HFE gene mutation Cys282Tyr in the homozygous state were included in the study...
September 19, 2016: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/27658824/hemophagocytic-lymphohistiocytosis-responding-to-withdrawal-of-gluten-a-case-report
#19
Nicholas J Fordham, Richa Ajitsaria, Leena Karnik, Subarna Chakravorty
BACKGROUND: This is the first documented case of a patient with hemophagocytic lymphohistiocytosis in association with coeliac disease. There was complete clinical and biochemical remission of hemophagocytic lymphohistiocytosis following the introduction of a gluten-free diet. CASE PRESENTATION: A 7-year-old white girl presented with fevers and maculopapular rash with a recent history of tonsillitis. Blood tests revealed thrombocytopenia (64×10(9)/L), anemia (80 g/L), hypofibrinogenemia (1 g/L), and hyperferritinemia (71,378 μg/L)...
2016: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/27636724/extreme-hyperferritinemia-in-dizygotic-twins-with-human-parechovirus-3-infection
#20
Dídac Casas-Alba, Antonio Martínez-Monseny, Laura Monfort, Carmen Muñoz-Almagro, María Cabrerizo, Àngela Deyà, Cristian Launes
Human parechovirus-3 has been associated with severe clinical manifestations in infants, such as sepsis-like illness and meningoencephalitis. Nevertheless, the vast majority of patients have a favorable outcome. We report the occurrence of this infection in dizygotic infants with extreme hyperferritinemia and a transient impairment of natural-killer cell cytotoxicity.
September 15, 2016: Pediatric Infectious Disease Journal
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