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Hemachromatosis

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https://www.readbyqxmd.com/read/25916291/-mri-monitoring-in-diagnosis-and-follow-up-of-iron-overload
#1
Qian Zhang, Bo Hou, Lu Wang, Yali Du, Bing Han, Feng Feng
OBJECTIVE: To use the technique of magnetic resonance imaging (MRI) T2* mapping to diagnose and follow-up of patients with iron overload. METHODS: 107 patients who were suspected to have iron overload between 2011.7-2014.3 in Peking Union Medical Colleague Hospital were analyzed retrospectively. Patients had the document of MRI T2* value of liver, heart and pancreas, serum ferritin (SF), transferrin saturation (TS), transfusion amount and other related laboratory tests...
April 2015: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/25248753/a-phase-i-and-pharmacokinetic-study-of-ganetespib-sta-9090-in-advanced-hepatocellular-carcinoma
#2
Lipika Goyal, Raymond C Wadlow, Lawrence S Blaszkowsky, Brian M Wolpin, Thomas A Abrams, Nadine Jackson McCleary, Susan Sheehan, Eamala Sundaram, Michael D Karol, John Chen, Andrew X Zhu
BACKGROUND: Ganetespib (STA-9090) is an Hsp90 inhibitor that downregulates VEGFR, c-MET, HER2, IGF-IR, EGFR, and other Hsp90 client proteins involved in hepatocarcinogenesis, thereby making it an attractive therapy for HCC. This Phase I study was performed to establish the safety, tolerability, recommended Phase 2 dose (RP2D), and preliminary clinical activity of ganetespib in previously treated patients with advanced HCC. METHODS: Patients with advanced HCC, Child-Pugh A cirrhosis, progression on or intolerance to sorafenib, and ECOG PS ≤ 1 were enrolled in a standard 3x3 dose escalation study at doses of 100 mg/m(2), 150 mg/m(2), and 200 mg/m(2) IV given on days 1, 8, and 15 of each 28-day cycle...
February 2015: Investigational New Drugs
https://www.readbyqxmd.com/read/24521890/-significance-of-magnetic-resonance-imaging-in-the-detection-of-iron-overload
#3
Qian Zhang, Bo Hou, Lu Wang, Xiao-ying Wang, Feng Feng, Bin Jiang, Hong-xia Shi, Yi-gai Ma, Hui Liu, Bing Han, Yong-qiang Zhao
OBJECTIVE: To evaluate the significance of magnetic resonance imaging (MRI) T2(*) value analysis in patients with iron overload and compare it with other clinical parameters. METHODS: A total of 53 patients with suspected iron overload were recruited from four Beijing hospitals from December 2010 to December 2012. Their liver and heart T2(*) values were calculated and their serum ferritin (SF), transferrin saturation, blood transfusion volume and other clinical parameters were recorded and analyzed...
November 26, 2013: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/21283470/articular-manifestations-of-systemic-diseases
#4
W G Bensen
Many systemic diseases present with articular manifestations. An understanding of the clinical, laboratory and radiological features of these diseases can lead to early diagnosis and appropriate therapy. This article describes the articular presentation and management of four generalized disorders: idiopathic hemachromatosis; sarcoidosis; hepatitis-B virus-induced arthritis, and polymyositis-dermatomyositis induced arthritis.
November 1983: Canadian Family Physician Médecin de Famille Canadien
https://www.readbyqxmd.com/read/20955973/inherited-hepatocellular-carcinoma
#5
REVIEW
Augusto Villanueva, Pippa Newell, Yujin Hoshida
Inherited liver disorders that cause chronic inflammation, fibrosis, and cirrhosis can lead to the development of liver cancer. Because of the rarity and diversity of some of these syndromes, the relative risk of developing HCC in these patients and the age at which tumours typically arise cannot be accurately estimated. Among patients with hereditary hemachromatosis (HH), the annual incidence of HCC is 4% once cirrhosis has been established. Fibrosis and portal hypertension associated with HH can be partially reversed with therapeutic phlebotomy, but it is unclear whether this treatment alters the incidence of HCC in these patients...
October 2010: Best Practice & Research. Clinical Gastroenterology
https://www.readbyqxmd.com/read/18634918/sudden-cardiac-death-in-infiltrative-cardiomyopathies-sarcoidosis-scleroderma-amyloidosis-hemachromatosis
#6
REVIEW
Steven A Lubitz, Seth H Goldbarg, Davendra Mehta
Sarcoidosis, scleroderma, amyloidosis, and hemachromatosis are systemic infiltrative disorders that commonly affect the heart. Owing to their potential for diffuse organ involvement, these diseases may present with myriad clinical manifestations. Conduction system abnormalities are common, and each of these disorders has been associated with sudden cardiac death. In this review, we summarize the epidemiology, clinical features, diagnosis, and treatment of each of these entities. We place special emphasis on existing literature as it pertains to risk stratification and therapy aimed at the prevention of sudden cardiac death in these infiltrative cardiomyopathies...
July 2008: Progress in Cardiovascular Diseases
https://www.readbyqxmd.com/read/18214364/-the-fibroscan-a-new-non-invasive-method-of-liver-fibrosis-evaluation
#7
REVIEW
A Lamproye, J Belaiche, J Delwaide
The FibroScan is a device allowing a non invasive diagnosis and quantification of liver fibrosis. The procedure is based on transient elastography and allows to record liver stiffness by measuring the velocity of shear wave across liver parenchyma. The elasticity is directly correlated to velocity of the wave. In chronic hepatitis C, there is a good correlation between liver elasticity and stage of fibrosis. The FibroScan has also been studied in other chronic liver diseases, such as hepatitis B, primary biliary cirrhosis, sclerosing cholangitis, auto-immune hepatitis, alcohol, steatosis, hemachromatosis with reproductible results...
2007: Revue Médicale de Liège
https://www.readbyqxmd.com/read/17784037/the-etiology-of-hemachromatosis
#8
(no author information available yet)
No abstract text is available yet for this article.
February 13, 1925: Science
https://www.readbyqxmd.com/read/17053826/-hereditary-hemachromatosis-clinical-case-report-and-literature-review
#9
Ricardo Prochazka, Martín Tagle
Hemachromatosis is a hereditary condition, producing progressive iron overload as a result of the mutation in proteins that regulate intestinal iron absorption. It is a systemic disease with several manifestations including cirrhosis, diabetes mellitus, cardiomyopathy, joint disease and a proportion of asymptomatic patients. When it is diagnosed and treatment with phlebotomies is initiated before any organ damage is developed, the prognosis is very good, with normal survival free of manifestations. This condition is common in European populations...
July 2006: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
https://www.readbyqxmd.com/read/16136860/early-diagnosis-of-hemochromatosis-related-cardiomyopathy-with-magnetic-resonance-imaging
#10
Leon M Ptaszek, Erik T Price, Mary Y Hu, Phillip C Yang
The hallmark of hemochromatosis is the deposition of iron in multiple tissue types, most notably the skin, liver, pancreas, thyroid, and heart. Definitive diagnosis of iron deposition generally requires invasive methods, such as direct tissue biopsy. We describe a 40 year-old woman with end-stage liver disease secondary to hereditary hemochromatosis and alcohol abuse, who was referred to the cardiology service as part of an evaluation for orthotopic liver transplant. The patient had no cardiac history but a dobutamine stress echocardiogram, performed as a portion of the pre-operative cardiac evaluation, could not be completed due to intermittent, supraventricular tachycardia...
2005: Journal of Cardiovascular Magnetic Resonance
https://www.readbyqxmd.com/read/15812589/yersinia-enterocolitica-infection-in-a-patient-with-hemachromatosis-masquerading-as-proximal-colon-cancer-with-liver-metastases-report-of-a-case
#11
James Crosbie, Jag Varma, John Mansfield
A patient with genetic hemachromatosis presented with a clinical picture suggesting malignancy and CT evidence of lesions in the right colon and liver. Colonoscopy failed to confirm the suspected diagnosis. Blood and stool cultures were positive for yersinia enterocolittica infection. This case illustrates the need to confirm clinically and radiologically suspected malignancy. It also serves as a model of how localized gastrointestinal pathology can result from the interaction of host genetic factors and specific microbial species...
February 2005: Diseases of the Colon and Rectum
https://www.readbyqxmd.com/read/14471779/copper-metabolism-in-wilson-s-disease-laennec-s-cirrhosis-and-hemachromatosis-studies-with-radiocopper-cu64
#12
W J MAYTUM, N P GOLDSTEIN, W F McGUCKIN, C A OWEN
No abstract text is available yet for this article.
December 6, 1961: Proceedings of the Staff Meetings
https://www.readbyqxmd.com/read/13686641/-radioisotopes-in-the-study-of-pernicious-anemia-hemolytic-diseases-and-ferrokinetics-of-hemachromatosis
#13
A M BAPTISTA
No abstract text is available yet for this article.
1960: Anales de la Real Academia Nacional de Medicina
https://www.readbyqxmd.com/read/13581503/idiopathic-hemachromatosis-treated-by-massive-venesection-report-of-a-case
#14
H P MACCUBBIN
No abstract text is available yet for this article.
September 1958: Virginia Medical Monthly
https://www.readbyqxmd.com/read/13060154/-concordant-incidence-of-liver-cirrhosis-and-diabetes-mellitus-hemachromatosis-in-unioval-twins
#15
K LOHR, H REINWEIN
No abstract text is available yet for this article.
1952: Deutsches Archiv Für Klinische Medizin
https://www.readbyqxmd.com/read/12234269/long-term-survival-after-liver-transplantation-in-children-with-metabolic-disorders
#16
Liise K Kayler, Robert M Merion, Samuel Lee, Randall S Sung, Jeffrey D Punch, Steven M Rudich, Jeremiah G Turcotte, Darrell A Campbell, Ronald Holmes, John C Magee
BACKGROUND: Liver transplantation for inherited metabolic disorders aims to save the patient's life when the disorder is expected to progress to organ failure, and to cure the underlying metabolic defect. METHODS: We retrospectively analyzed 146 pediatric liver transplants (28 metabolic; 118 non-metabolic) performed between 1986 and 2000. RESULTS: Twenty-eight transplants were performed in 24 children with metabolic disease (8 females; 16 males; age range 3 months to 17 yr)...
August 2002: Pediatric Transplantation
https://www.readbyqxmd.com/read/12149232/regulation-of-iron-absorption-in-hfe-mutant-mice
#17
Richard S Ajioka, Joanne E Levy, Nancy C Andrews, James P Kushner
Hereditary hemochromatosis is most commonly caused by homozygosity for a point mutation (C282Y) in the human hemochromatosis gene (HFE). The mechanism by which HFE regulates iron absorption is not known, but the C282Y mutation results in loss of cell surface expression of the human hemachromatosis protein (HFE) and hyperabsorption of iron by the duodenal enterocyte. Mice homozygous for a deletion in the mouse hemochromatosis gene (Hfe) or a mutation equivalent to that seen in human hereditary hemochromatosis (C282Y) were compared with wild-type animals for their ability to regulate iron absorption...
August 15, 2002: Blood
https://www.readbyqxmd.com/read/11410162/hmgn4-a-newly-discovered-nucleosome-binding-protein-encoded-by-an-intronless-gene
#18
Y Birger, Y Ito, K L West, D Landsman, M Bustin
We describe a newly discovered nuclear protein, HMGN4, that is closely related to the canonical HMGN2 nucleosome-binding protein. The protein is encoded by an intronless gene, which, in humans, is located in the hereditary hemochromatosis [correction of hemachromatosis] region at position 6p21.3. A single approximately 2-kb HMGN4 mRNA was found to be expressed, in variable amounts, in all human tissues tested; however, the HMGN4 transcript was significantly less abundant than that of HMGN2. The HMGN4 protein could be detected in HeLa cells by Western analysis with an antibody elicited against a unique region of the protein...
May 2001: DNA and Cell Biology
https://www.readbyqxmd.com/read/8920884/defective-iron-homeostasis-in-beta-2-microglobulin-knockout-mice-recapitulates-hereditary-hemochromatosis-in-man
#19
M Santos, M W Schilham, L H Rademakers, J J Marx, M de Sousa, H Clevers
Previously, hepatic iron overload resembling that in hereditary hemachromatosis (HH) has been found in beta 2-microglobulin knockout (beta 2m-/-) mice. We have now characterized iron metabolism in beta 2m-/- mice. The mutant mice fail to limit the transfer of iron from mucosal cells into the plasma. Transferrin saturation is abnormally high. Pathologic iron depositions occur predominantly in liver parenchymal cells. Reconstitution with normal hematopoietic cells redistributes the iron from parenchymal to Kupffer cells, but does not correct the mucosal defect...
November 1, 1996: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/8114297/-liver-cirrhosis-in-metabolic-disorders
#20
Y Tazawa
The most early cirrhosis is observed in newborns with neonatal hemachromatosis. Early cirrhosis occurs in hereditary tyrosinemia type I, peroxisomal diseases and glycogen storage disease (type IV). In Wilson's disease, a case complicated with cirrhosis was reported in a 4-year-old patient. Slowly progressive cirrhosis is seen in patients with familial progressive intrahepatic cholestasis. Focal biliary cirrhosis is found in cystic fibrosis of the pancreas. Moreover, many other metabolic disorders, except for urea cycle disorders, are occasionally or rarely complicated with cirrhosis...
January 1994: Nihon Rinsho. Japanese Journal of Clinical Medicine
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