نتایج جستجو برای: fanconi anemia patients

تعداد نتایج: 2119640  

Journal: :iranian biomedical journal 0
آناهیتا محسنی میبدی anahita mohseni meybodi حسین مزدرانی hossein mozdarani

background: lymphocytes of fanconi anemia (fa) show an increased sensitivity to the alkylating agents such as mitomycin c (mmc), but their responses to gamma-irradiation is controversial. the extent of dna damage in leukocytes of fa patients following irradiation and mmc treatment was studied at cellular and single chromosome level. methods: dna damage induced by gamma-rays and mmc was measured...

انصاری, شهلا, ساده‌دل, روزبه , هاشمی, مهدی, آهنچی, نوید ,

Fanconi anemia is the most prevalent form of inherited aplastic anemia which is characterized by progressive bone marrow failure, congenital anomalies and cancer susceptibility. Common anomalies are skeletal abnormalities, skin pigmentation disorder, short stature, head abnormalities, kidney and gonad disorders respectively. Complications of fanconi anemia include: leukemia due to defective DNA...

Journal: :Haematologica 2009
Fernando O Pinto Thierry Leblanc Delphine Chamousset Gwenaelle Le Roux Benoit Brethon Bruno Cassinat Jérôme Larghero Jean-Pierre de Villartay Dominique Stoppa-Lyonnet André Baruchel Gérard Socié Eliane Gluckman Jean Soulier

BACKGROUND Patients with bone marrow failure and undiagnosed underlying Fanconi anemia may experience major toxicity if given standard-dose conditioning regimens for hematopoietic stem cell transplant. Due to clinical variability and/or potential emergence of genetic reversion with hematopoietic somatic mosaicism, a straightforward Fanconi anemia diagnosis can be difficult to make, and diagnost...

Journal: :Haematologica 2010
Hannah Tamary Daniella Nishri Joanne Yacobovich Rama Zilber Orly Dgany Tanya Krasnov Shraga Aviner Polina Stepensky Shoshana Ravel-Vilk Menachem Bitan Chaim Kaplinsky Ayelet Ben Barak Ronit Elhasid Joseph Kapelusnik Ariel Koren Carina Levin Dina Attias Ruth Laor Isaac Yaniv Philip S Rosenberg Blanche P Alter

BACKGROUND Inherited bone marrow failure syndromes are rare genetic disorders characterized by bone marrow failure, congenital anomalies, and cancer predisposition. Available single disease registries provide reliable information regarding natural history, efficacy and side effects of treatments, and contribute to the discovery of the causative genes. However, these registries could not shed li...

الهه کیهانی, , حسین نجم آبادی, , رکسانا کریمی‌نژاد, , زهره علومی, , ساغر قاسمی فیروز آبادی, , فرحناز امینی, , فرخنده بهجتی, , فریده موسوی, , یوسف شفقتی, ,

Background: Fanconi anemia (FA) is a rare autosomal recessive disorder characterized by short stature, skeletal anomalies, increased incidence of solid tumors and leukemia, and bone marrow failure (aplastic anemia). FA has been reported in all races and ethnic groups and affects men and women in an equal proportion. The frequency of FA has been estimated at approximately 1 per 360,000 live birt...

Journal: :The Laryngoscope 2017
Emmy Verheij Karin P Q Oomen Stephanie E Smetsers Gijsbert A van Zanten Lucienne Speleman

OBJECTIVES/HYPOTHESIS Fanconi anemia is a hereditary chromosomal instability disorder. Hearing loss and ear abnormalities are among the many manifestations reported in this disorder. In addition, Fanconi anemia patients often complain about hearing difficulties in situations with background noise (speech perception in noise difficulties). Our study aimed to describe the prevalence of hearing lo...

Journal: :Klinicka onkologie : casopis Ceske a Slovenske onkologicke spolecnosti 2016
A Puchmajerová K Švojgr D Novotná E Macháčková D Sumerauer P Smíšek R Kodet M Kynčl A Křepelová L Foretová

Fanconi anemia is a rare autosomal recessive disorder, clinically and genetically heterogeneous, characterized by typical clinical features, such as short stature, microcephaly, skeletal abnormalities, abnormal skin pigmentations, developmental delay and congenital heart, kidney anomalies etc. Pancytopenia leading to bone marrow failure occurs in the first decade. Patients with Fanconi anemia h...

Journal: :Journal of the National Cancer Institute 2003
David I Kutler Volkert B Wreesmann Andy Goberdhan Leah Ben-Porat Jaya Satagopan Ivan Ngai Andrew G Huvos Philip Giampietro Orna Levran Kanan Pujara Rafaella Diotti Diane Carlson Laryssa A Huryn Arleen D Auerbach Bhuvanesh Singh

Fanconi anemia is an autosomal recessive disorder characterized by congenital malformations, bone marrow failure, and the development of squamous cell carcinomas (SCCs) and other cancers. Recent clinicopathologic evidence has raised the possibility that an environmental factor such as human papillomavirus (HPV) may be involved in the pathogenesis of SCCs in Fanconi anemia patients. Given the hi...

Journal: :Clinical cancer research : an official journal of the American Association for Cancer Research 2015
Anne J Lombardi Elizabeth E Hoskins Grant D Foglesong Kathryn A Wikenheiser-Brokamp Lisa Wiesmüller Helmut Hanenberg Paul R Andreassen Allison J Jacobs Susan B Olson Winifred W Keeble Laura E Hays Susanne I Wells

PURPOSE Fanconi anemia is an inherited disorder associated with a constitutional defect in the Fanconi anemia DNA repair machinery that is essential for resolution of DNA interstrand crosslinks. Individuals with Fanconi anemia are predisposed to formation of head and neck squamous cell carcinomas (HNSCC) at a young age. Prognosis is poor, partly due to patient intolerance of chemotherapy and ra...

2012
Shi-Long Lu

DNA repair defect is one of the hallmarks of tumorigenesis, and is intimately linked to various human cancers, both inherited and sporadic (1). The two best examples are perhaps the DNA mismatch repair pathway in colorectal cancer, and the Fanconi Anemia/Brca (Fanc/Brca) pathway in head and neck squamous cell carcinomas (HNSCCs) (1). In this book chapter, I will review the updated knowledge of ...

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