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https://www.readbyqxmd.com/read/29770484/inter-method-reproducibility-of-biexponential-r-2-mr-relaxometry-for-estimation-of-liver-iron-concentration
#1
Ali Pirasteh, Qing Yuan, Diego Hernando, Scott B Reeder, Ivan Pedrosa, Takeshi Yokoo
PURPOSE: To assess the reproducibility of biexponential R2 -relaxometry MRI for estimation of liver iron concentration (LIC) between proprietary and nonproprietary analysis methods. METHODS: This single-center retrospective study, approved by investigational review board and compliant with the Health Insurance Portability and Accountability Act, included 40 liver MRI exams in 38 subjects with suspected or known iron overload. From spin-echo images of the liver, acquired at 5 different echo times (TE = 6-18 ms), biexponential R2 maps were calculated using 1 proprietary (FerriScan, Resonance Health Ltd...
May 16, 2018: Magnetic Resonance in Medicine: Official Journal of the Society of Magnetic Resonance in Medicine
https://www.readbyqxmd.com/read/29769501/thyroid-status-in-children-with-transfusion-dependent-hb-e-%C3%AE-thalassaemia
#2
T Sharmin, A H Mollah, A A Morshed, M K Chowdhury
Despite improved haematological care, multi-endocrine dysfunction is a common complication in thalassemia. Iron overload is thought to be the most likely mechanism in thyroid dysfunction in these patients. Moreover, chronic tissue hypoxia might havedirect toxic effect on thyroid gland resulting in hypothyroidism. This study was designed to evaluate the thyroid status of children with Hb-E β-thalassemia. This cross sectional analytic study was conducted among thepatients with Hb-E β-thalassemia attending both in-patient & out-patient department of Paediatrics, Dhaka Medical College & Hospital, Dhaka, Bangladesh from April 2012 to March 2013...
April 2018: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/29769185/unusual-case-of-iron-overload-with-cancer-mimicking-abdominal-splenosis
#3
Giacomo Marchi, Giacomo Avesani, Alberto Zamò, Domenico Girelli
A 48-year-old man, former alcohol abuser and drug addicted, was referred to our tertiary referral centre for iron disorders because of marked hyperferritinaemia. His clinical history revealed chronic hepatitis C, ß-thalassaemia trait and post-traumatic splenectomy at age of 22. MRI-estimated liver iron content was markedly elevated, while first-line genetic test for haemochromatosis was negative. Alpha-fetoprotein was increased but liver ultrasonography did not reveal focal liver lesions. Multiphasic contrast-enhanced CT confirmed this result but showed two abdominal masses (diameter of 9 cm and 7 cm, respectively) among bowel loops, strongly suspicious for cancer...
May 16, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29768045/mfe-hi-adipose-tissue-macrophages-compensate-for-tissue-iron-pertubations-in-mice
#4
Merla J Hubler, Keith M Erikson, Arion J Kennedy, Alyssa H Hasty
Resident adipose tissue macrophages (ATMs) play multiple roles to maintain tissue homeostasis, such as removing excess FFAs and regulation of extracellular matrix. The phagocytic nature and oxidative resiliency of macrophages not only allows them to function as innate immune cells but also to respond to specific tissue needs, such as iron homeostasis. MFehi ATMs are a subtype of resident ATMs that we recently identified to have twice the intracellular iron content as other ATMs and elevated expression of iron handling genes...
May 16, 2018: American Journal of Physiology. Cell Physiology
https://www.readbyqxmd.com/read/29766365/iron-overload-prevents-oxidative-damage-to-rat-brain-after-chlorpromazine-administration
#5
Natacha E Piloni, Andres A Caro, Susana Puntarulo
The hypothesis tested is that Fe administration leads to a response in rat brain modulating the effects of later oxidative challenges such as chlorpromazine (CPZ) administration. Either a single dose (acute Fe overload) or 6 doses every second day (sub-chronic Fe overload) of 500 or 50 mg Fe-dextran/kg, respectively, were injected intraperitoneally (ip) to rats. A single dose of 10 mg CPZ/kg was injected ip 8 h after Fe treatment. DNA integrity was evaluated by quantitative PCR, lipid radical (LR· ) generation rate by electron paramagnetic resonance (EPR), and catalase (CAT) activity by UV spectrophotometry in isolated brains...
May 15, 2018: Biometals: An International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine
https://www.readbyqxmd.com/read/29765443/management-of-cardiac-hemochromatosis
#6
Wilbert S Aronow
Iron-overload syndromes may be hereditary or acquired. Patients may be asymptomatic early in the disease. Once heart failure develops, there is rapid deterioration. Cardiac hemochromatosis is characterized by a dilated cardiomyopathy with dilated ventricles, reduced ejection fraction, and reduced fractional shortening. Deposition of iron may occur in the entire cardiac conduction system, especially the atrioventricular node. Cardiac hemochromatosis should be considered in any patient with unexplained heart failure...
April 2018: Archives of Medical Science: AMS
https://www.readbyqxmd.com/read/29764842/role-of-the-hepcidin-ferroportin-axis-in-pathogen-mediated-intracellular-iron-sequestration-in-human-phagocytic-cells
#7
Rodrigo Abreu, Frederick Quinn, Pramod K Giri
Upon infection, pathogen and host compete for the same iron pool, because this trace metal is a crucial micronutrient for all living cells. Iron dysregulation in the host is strongly associated with poor outcomes in several infectious diseases, including tuberculosis, AIDS, and malaria, and inefficient iron scavenging by pathogens severely affects their virulence. Hepcidin is the master regulator of iron homeostasis in vertebrates, responsible for diminishing iron export from macrophages during iron overload or infection...
May 22, 2018: Blood Advances
https://www.readbyqxmd.com/read/29763925/the-influence-of-hyperlipidemia-on-endothelial-function-of-fpn1-tek-cre-mice-and-the-intervention-effect-of-tetramethylpyrazine
#8
Ming-Yue Sun, Miao Zhang, Shui-Ling Chen, Shu-Ping Zhang, Chun-Yu Guo, Jing-Shang Wang, Xin Liu, Yang Miao, Hui-Jun Yin
BACKGROUND/AIMS: Systemic iron homeostasis is strictly governed in mammals; however, disordered iron metabolism (such as excess iron burden) is recognized as a risk factor for various types of diseases including AS (Atherosclerosis). The hepcidin-ferroportin axis plays the key role in regulation of iron homeostasis and modulation of this signaling could be a potential therapeutic strategy in the treatment of these diseases. TMP (Tetramethylpyrazine) has been reported to have therapeutical effect on AS...
May 9, 2018: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/29762515/iron-status-and-gestational-diabetes-a-meta-analysis
#9
REVIEW
Yachana Kataria, Yanxin Wu, Peter de Hemmer Horskjær, Thomas Mandrup-Poulsen, Christina Ellervik
A meta-analysis of the association of iron overload with gestational diabetes mellitus (GDM) may inform the health debate. We performed a meta-analysis investigating the association of iron biomarkers and dietary iron exposure with GDM. We identified 33 eligible studies ( N = 44,110) published in 2001⁻2017. The standardized mean differences (SMD) in women who had GDM compared to pregnant women without were 0.25 µg/dL (95% CI: 0.001⁻0.50) for iron, 1.54 ng/mL (0.56⁻2.53) for ferritin, 1.05% (0.02 to 2...
May 15, 2018: Nutrients
https://www.readbyqxmd.com/read/29761371/tolerability-and-efficacy-of-deferasirox-in-patients-with-transfusional-iron-overload-results-from-a-german-2-year-non-interventional-study
#10
Florian Nolte, Holger Nückel, Burkhard Schmidt, Thomas Geer, Oleg Rubanov, Holger Hebart, Andrea Jarisch, Stefan Albrecht, Christiane Johr, Christiane Schumann, Wolf-Karsten Hofmann
BACKGROUND: Iron overload (IOL) due to repetitive transfusions of packed red blood cells (pRBC) has a major impact on morbidity and mortality in patients with inherited bone marrow failure syndromes and hemoglobinopathies such as thalassemia and sickle cell disease. However, whether IOL influences the outcome of elderly patients with myeloid malignancies is not yet clear. Moreover, clinical trials have reported high drop-out rates during treatment with the oral iron chelator deferasirox (DFX)...
May 14, 2018: Journal of Cancer Research and Clinical Oncology
https://www.readbyqxmd.com/read/29760547/comparison-of-the-effects-of-deferasirox-deferoxamine-and-combination-of-deferasirox-and-deferoxamine-on-an-aplastic-anemia-mouse-model-complicated-with-iron-overload
#11
Dijiong Wu, Xiaowen Wen, Wenbin Liu, Huijin Hu, Baodong Ye, Yuhong Zhou
Background and aim: Iron overload is commonly observed during the course of aplastic anemia (AA), which is believed to aggravate hematopoiesis, cause multiple organ dysfunction, lead to disease progression, and impair quality of life. Deferasirox (DFX) and deferoxamine (DFO) are among the most common iron chelation agents available in the clinical setting. The aim of this study was to investigate if the combination therapy with DFX and DFO is superior in hematopoietic recovery and iron chelation...
2018: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/29755708/growth-and-endocrine-function-in-tunisian-thalassemia-major-patients
#12
Naouel Guirat Dhouib, Monia Ben Khaled, Monia Ouederni, Habib Besbes, Ridha Kouki, Fethi Mellouli, Mohamed Bejaoui
β-thalassemia major (β-TM) is among the most common hereditary disorders imposing high expenses on health-care system worldwide. The patient's survival is dependent on lifetime blood transfusion which leads to iron overload and its toxicity in various organs including endocrine glands. This article provides an overview of endocrine disorders in beta-TM patients. This single center investigation enrolled 28 β-TM patients (16 males, 12 females) regularly transfused with packed red cell since early years of life...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29754517/diagnosis-and-treatment-of-cardiac-iron-overload-in-transfusion-dependent-thalassemia-patients
#13
Natthaphat Siri-Angkul, Siriporn C Chattipakorn, Nipon Chattipakorn
Thalassemia is among the most common genetic diseases. Patients with severe forms of the disease are transfusion-dependent, leading to iron overload. A condition which can eventually develop in the iron-loaded heart is iron overload cardiomyopathy, a debilitating disease that accounts for the majority of deaths in thalassemia patients. Areas covered: This review article provides a comprehensive summary of the diagnosis and treatment of cardiac iron overload in transfusion-dependent thalassemia patients, with discussion covering current weak points and potential improvements of the relevant diagnostic and therapeutic strategies...
May 12, 2018: Expert Review of Hematology
https://www.readbyqxmd.com/read/29751366/synthesis-characterization-and-electrochemistry-studies-of-iron-iii-complex-with-curcumin-ligand
#14
Gülüzar Özbolat, Arash Alizadeh Yegani, Abdullah Tuli
Iron overload is a serious clinical condition for humans and is a key target in drug development. The aim of this study was to investigate the coordination of iron(III) ions with curcumin ligand that may be used in the treatment of iron overload. Iron(III) complex of curcumin was synthesized and structurally characterized in its solid and solution state by FT-IR, UV-Vis, elemental analysis, and magnetic susceptibility. Electrochemical behavior of the ligand and the complexes were examined using cyclic voltammetry...
May 11, 2018: Clinical and Experimental Pharmacology & Physiology
https://www.readbyqxmd.com/read/29749247/iron-chelating-ligand-for-iron-overload-diseases
#15
G Ozbolat, A Tuli
Iron overloads are a serious clinical condition in the health of humans and are therefore a key target in drug development. In this study, iron(III) complex of 8-hydroxyquinoline-5 sulphonic acid was synthesized and structurally characterized in its solid state and solution state by FT-IR, UV-Vis, elemental analysis, magnetic susceptibility and 1H-NMR. The catalase activities of complex were investigated. It was showed that the complex has the catalase activity. It is suggested that this type of complex may constitute a new and interesting basis for the future search for new and more potential drugs...
2018: Bratislavské Lekárske Listy
https://www.readbyqxmd.com/read/29748130/effects-of-the-iron-chelator-deferiprone-and-the-t-type-calcium-channel-blocker-efonidipine-on-cardiac-function-and-ca-2-regulation-in-iron-overloaded-thalassemic-mice
#16
Juthamas Khamseekaew, Sirinart Kumfu, Siripong Palee, Suwakon Wongjaikam, Somdet Srichairatanakool, Suthat Fucharoen, Siriporn C Chattipakorn, Nipon Chattipakorn
Although disturbance of cardiac Ca2+ regulation is involved in the pathophysiology of iron-overload cardiomyopathy, the obvious mechanisms involved in the dysregulation of iron-induced cardiac Ca2+ are unclear. Moreover, the roles of the iron chelator deferiprone and the T-type calcium channel blocker efonidipine on cardiac intracellular Ca2+ transients and Ca2+ regulatory proteins in thalassemic mice are still unknown. We tested the hypothesis that treatment with either deferiprone or efonidipine attenuated cardiac Ca2+ dysregulation and led to improved left ventricular (LV) function in iron-overloaded thalassemic mice...
February 6, 2018: Cell Calcium
https://www.readbyqxmd.com/read/29743790/safety-and-efficacy-of-sofosbuvir-and-daclatasvir-for-hepatitis-c-virus-infection-in-patients-with-%C3%AE-thalassemia-major
#17
Rajiv Mehta, Mayank Kabrawala, Subhash Nandwani, Pankaj Desai, Vishwa Bhayani, Sanjay Patel, Viral Parekh
Background and Aims: β-thalassemia major patients are susceptible to Hepatitis C Virus (HCV) infection owing to life-long dependency for blood-transfusion. Moreover, this patient population is at risk of progression of liver fibrosis or development of cirrhosis as a consequence of both iron overload and HCV infection. Hence, this study was carried out to evaluate efficacy and safety of the combination regimen of sofosbuvir and daclatasvir for HCV infection in β-thalassemia major patients...
March 2018: Journal of Clinical and Experimental Hepatology
https://www.readbyqxmd.com/read/29743442/fluoride-induced-iron-overload-contributes-to-hepatic-oxidative-damage-in-mouse-and-the-protective-role-of-grape-seed-proanthocyanidin-extract
#18
Qiang Niu, Ping He, Shangzhi Xu, Ruling Ma, Yusong Ding, Lati Mu, Shugang Li
Emerging evidence has demonstrated that iron overload plays an important role in oxidative stress in the liver. This study aimed to explore whether fluoride-induced hepatic oxidative stress is associated with iron overload and whether grape seed proanthocyanidin extract (GSPE) alleviates oxidative stress by reducing iron overload. Forty Kunming male mice were randomly divided into 4 groups and treated for 5 weeks with distilled water (control), sodium fluoride (NaF) (100 mg/L), GSPE (400 mg/kg bw), or NaF (100 mg/L) + GSPE (400 mg/kg bw)...
2018: Journal of Toxicological Sciences
https://www.readbyqxmd.com/read/29743399/-successful-treatment-of-x-linked-sideroblastic-anemia-with-alas2-r452h-mutation-using-vitamin-b-6
#19
Toru Kawakami, Hideyuki Nakazawa, Fumihiro Kawakami, Shuji Matsuzawa, Yuriko Sudo, Hitoshi Sakai, Sayaka Nishina, Noriko Senoo, Yasushi Senoo, Michiharu Komatsu, Takeji Umemura, Tomomi Yamaguchi, Tomoki Kosho, Tohru Fujiwara, Hideo Harigae, Fumihiro Ishida
A 45-year-old man presented with fatigue and pain in the finger joints. Despite having a history of suspected sideroblastic anemia since the age of 18 years, he had not been followed up for years. Upon presentation, laboratory data revealed microcytic anemia and elevated serum ferritin levels. In addition, ringed sideroblasts were increased in the bone marrow. A liver biopsy revealed hemochromatosis and cirrhosis. Furthermore, genetic analysis revealed that he harbored the ALAS2 R452H mutation, leading to the diagnosis of X-linked sideroblastic anemia (XLSA)...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29743178/phenotypic-analysis-of-hemochromatosis-subtypes-reveals-variations-in-severity-of-iron-overload-and-clinical-disease
#20
Kam Sandhu, Kaledas Flintoff, Mark D Chatfield, Jeannette L Dixon, Louise E Ramm, Grant A Ramm, Lawrie W Powell, V Nathan Subramaniam, Daniel F Wallace
The clinical progression of HFE-related hereditary hemochromatosis (HH) and its phenotypic variability has been well studied. Less is known about the natural history of non-HFE HH caused by mutations in the HJV , HAMP or TFR2 genes. The purpose of this study was to compare the phenotypic and clinical presentations of hepcidin-deficient forms of HH. A literature review of all published cases of genetically confirmed HJV, HAMP and TFR2 HH was performed. Phenotypic and clinical data from a total of 156 subjects with non-HFE HH was extracted from 53 publications and compared with data from 984 subjects with HFE -p...
May 9, 2018: Blood
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