Fanconi Anemia (Fanconi's Anemia)

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711 relevant articles (13 outcomes, 36 trials/studies) found for this Disease

Description: Congenital disorder affecting all bone marrow elements, resulting in ANEMIA; LEUKOPENIA; and THROMBOPENIA, and associated with cardiac, renal, and limb malformations as well as dermal pigmentary changes. Spontaneous CHROMOSOME BREAKAGE is a feature of this disease along with predisposition to LEUKEMIA. There are at least 7 complementation groups in Fanconi anemia: FANCA, FANCB, FANCC, FANCD1, FANCD2, FANCE, FANCF, FANCG, and FANCL. (from Online Mendelian Inheritance in Man, http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=227650, August 20, 2004)

Also Known As:

Fanconi's Anemia; Anemia, Fanconi's; Anemias, Fanconi; Fanconi Anemias; Anemia, Fanconi

Relationship Network

Disease Context: Research Results

Hemic and Lymphatic Diseases

Hematologic Diseases: 1420

Anemia: 15158

Aplastic Anemia: 3024

Congenital Hypoplastic Anemia: 46

Fanconi Anemia: 711

Bone Marrow Diseases: 60

Aplastic Anemia: 3024

Congenital Hypoplastic Anemia: 46

Fanconi Anemia: 711

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 421

Inborn Genetic Diseases: 549

Congenital Hypoplastic Anemia: 46

Fanconi Anemia: 711

Nutritional and Metabolic Diseases

Metabolic Diseases: 1267

DNA Repair-Deficiency Disorders: 67

Fanconi Anemia: 711

Related Diseases

1.	Pancytopenia
2.	DNA Damage
3.	Hepatocellular Carcinoma (Hepatoma)
4.	Adenoma (Adenomas)
5.	Neoplasms (Cancer)

Experts

1.	Ayas, M: 6 articles (08/2008 - 01/2001)
2.	D'Andrea, Alan D: 6 articles (05/2008 - 05/2003)
3.	Gluckman, Eliane: 6 articles (09/2007 - 01/2004)
4.	Taniguchi, Toshiyasu: 6 articles (08/2007 - 05/2003)
5.	Auerbach, Arleen D: 6 articles (06/2007 - 07/2003)
6.	Joenje, Hans: 6 articles (09/2005 - 05/2003)
7.	Wagner, John E: 5 articles (10/2008 - 02/2004)
8.	Harris, Richard E: 5 articles (10/2008 - 11/2003)
9.	Pasquini, Ricardo: 5 articles (10/2008 - 08/2004)
10.	Al-Mahr, M: 5 articles (08/2008 - 01/2001)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Fanconi Anemia:

1.	DNA (Deoxyribonucleic Acid)IBA 03/01/2003 - "That alphaSpIISigma* is essential for this re-localization is demonstrated by the finding that in cells from patients with Fanconi anemia complementation group A (FA-A), which have decreased ability to repair DNA interstrand cross-links and decreased levels of alphaSpIISigma, there is a significant reduction in formation of damage-induced XPF as well as alphaSpIISigma nuclear foci, even though levels of XPF are normal in these cells" 12/01/1997 - "Conventional subtraction library techniques or DNA-transfection studies are standard techniques applied for identification and isolation of genes relevant in monogenetic diseases like Fanconi anemia (FA)" 10/01/1982 - "In order to develop the usefulness of Fanconi's anemia (FA) lymphoblast lines for biochemical and genetic studies, we have determined their sensitivity to a variety of DNA-damaging chemicals" 11/01/1977 - "Study of ultraviolet repair & spontaneous DNA strand breaks in Fanconi's anemia cells." 08/28/2008 - "The removal of DNA interstrand cross-links (ICLs) has proven to be notoriously complicated due to the involvement of multiple pathways of DNA repair, which include the Fanconi anemia/BRCA pathway, homologous recombination and components of the nucleotide excision and mismatch repair pathways" Order ALL the reference details at left...
2.	Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF)IBA 07/01/1995 - "In the Fanconi's anemia trial, 6 of 7 patients showed some improvement when treated with GM-CSF" 12/01/2005 - "Overexpression of NPM suppresses stress-induced apoptosis in the granulocyte-macrophage colony-stimulating factor (GM-CSF)-dependent myeloid cell line MO7e and the lymphoblast HSC536 cells derived from a Fanconi anemia (FA) patient" 01/01/1994 - "We studied the toxicity and efficacy of daily subcutaneous administration of recombinant human granulocyte-macrophage colony-stimulating factor (GM-CSF) in patients with Fanconi anemia and pancytopenia" 07/01/1995 - "Two clinical trials were undertaken to evaluate the effect of human recombinant granulocyte-macrophage colony stimulating factor (GM-CSF) in pancytopenic pediatric patients with aplastic anemia and Fanconi's anemia" 07/01/1995 - "GM-CSF clinical trials: pediatric aplastic anemia and Fanconi's anemia." Order ALL the reference details at left...
3.	Alkylating AgentsIBA 12/01/1992 - "Fanconi anemia cells in culture are highly sensitive to oxidative stress and alkylating agents; Fanconi anemia may provide a model for a genetic disorder potentially predisposing to environmental insults." 05/23/1979 - "Effects of alkylating agents on lymphocytes from controls and from patients with Fanconi's anemia" 10/01/1975 - "Induction by alkylating agents of sister chromatid exchanges and chromatid breaks in Fanconi's anemia." 12/01/2007 - "Cells from Fanconi anemia (FA) patients are hypersensitive to alkylating agents and radiation traditionally used as conditioning regimens for marrow cell transplantation, and patients experience serious toxicities" 01/01/2004 - "BACKGROUND AND OBJECTIVES: Fanconi anemia (FA) cells are characteristically hypersensitive to bifunctional alkylating agents, notably mitomycin C (MMC), causing increased programmed cell death (PCD)" Order ALL the reference details at left...
4.	Reactive Oxygen Species (Oxygen Radicals)IBA 08/15/2001 - "Several lines of evidence point to an abnormality in the response of Fanconi anemia cells to reactive oxygen species" 07/14/2000 - "Fanconi's anemia (FA) is a clinically and genetically heterogeneous disease which has been hypothesized to be defective in the detoxification of reactive oxygen species" 02/25/2000 - "Fanconi's anemia (FA) is a very rare genetically heterogeneous disease which has been hypothesized to be defective in the detoxification of reactive oxygen species" 09/01/1992 - "The release of oxygen radicals by blood and bone marrow leukocytes of patients with Fanconi anemia (FA) has been studied" 09/01/1992 - "Release of active oxygen radicals by leukocytes of Fanconi anemia patients." Order ALL the reference details at left...
5.	TransferasesIBA 01/01/2003 - "The cellular control enzyme polyADP ribosyl transferase is eliminated in cultured Fanconi anemia fibroblasts at confluency." 01/01/1989 - "The basal and the mutagen-induced levels of ADP-ribosyl transferase activity are not modified in Fanconi's anemia cells." 01/01/1989 - "The activity of ADP-ribosyl transferase, an enzyme thought to be involved in several basic functions of the chromatin and in DNA repair, has been investigated in normal and Fanconi's anemia (FA) cells" 07/01/1993 - "The molecular spectra of mutations photoinduced (405 nm) by 4,5',8-trimethylpsoralen monoadducts (MA), at an endogenous locus, hypoxanthine-guanine phosphoribosyl-transferase (HPRT) in normal and in a Fanconi anemia (FA) lymphoblast cell line, complementation group D, are presented" Order ALL the reference details at left...
6.	HormonesIBA 11/01/2008 - "Improved growth velocity during thyroid hormone therapy in children with Fanconi anemia and borderline thyroid function." 07/01/2008 - "Correlation of thyroid and growth hormones to chromosomal instability in Egyptian Fanconi anemia patients." Order ALL the reference details at left...
7.	Cytochrome P-450 CYP2E1 (CYP2E1)IBA 01/01/2002 - "The FANCG Fanconi anemia protein interacts with CYP2E1: possible role in protection against oxidative DNA damage." Order ALL the reference details at left...
8.	Proliferating Cell Nuclear Antigen (PCNA)IBA 10/01/1995 - "Proliferating cell nuclear antigen (PCNA) labeling index (LI) of the adenoma arising in patients with Fanconi's anemia was significantly greater than the PCNA-LI of adenoma in the other children (mean 4.1% versus 0.9% of nuclei), approaching the lower end of the spectrum for reported hepatocellular carcinoma cases" Order ALL the reference details at left...
9.	Rutin (Rutoside)IBA 09/01/1992 - "It was proposed that elevated reactivity of the oxygen radicals released by FA leukocytes may be a major factor in the development of Fanconi anemia; this proposal is supported by the first positive results of treatment of FA patients with rutin (a nontoxic natural free radical scavenger and chelator)." Order ALL the reference details at left...
10.	erythritol anhydride (butanedione)IBA 05/01/1983 - "Fanconi's anemia: a cytogenetic study on lymphocyte and bone marrow cultures utilizing 1,2:3,4-diepoxybutane." 07/01/2003 - "Diagnosis of fanconi anemia by diepoxybutane analysis." 01/01/1998 - "Variable response to the diepoxybutane test in two dizygotic twins with Fanconi's anemia and flow cytometry for diagnosis confirmation." 04/15/1995 - "Comparative evaluation of diepoxybutane sensitivity and cell cycle blockage in the diagnosis of Fanconi anemia." 06/01/1993 - "Fanconi anemia diagnosis and the diepoxybutane (DEB) test." Order ALL the reference details at left...

Therapies and Procedures

1.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 03/01/2007 - "Bone marrow transplantation (BMT) is the only known cure for the hematologic manifestations of Fanconi anemia (FA)" 02/01/1995 - "Bone marrow transplantation for patients with Fanconi anemia: a study of 24 cases from a single institution." 04/15/2007 - "The second, from Veitia and colleagues, attempts to draw the same conclusions as Eggan et al. from a single clinical case report of a female Fanconi anemia patient who conceived a genetically-related daughter after allogeneic bone marrow transplantation" 03/01/2007 - "Unrelated donor bone marrow transplantation for the treatment of Fanconi anemia." 04/01/2006 - "Seventeen patients with Fanconi anemia (FA) underwent allogeneic bone marrow transplantation (BMT) from matched related donors (MRD) between January 1999 and June 2003" Order ALL the reference details at left...
2.	Transplantation (Transplant Recipients) 03/01/2008 - "Gluckman and her colleagues performed - successfully - the first familial CB transplantation and cured a 5 years old child suffering from Fanconi's anemia" 09/01/2008 - "Previous studies showed that Fanconi anemia (FA) murine stem cells have defective reconstitution after bone marrow (BM) transplantation" 01/01/1991 - "This paper reviews our recent laboratory and clinical studies demonstrating the efficacious use of human umbilical cord blood for HLA-matched allogeneic sibling stem/progenitor cell transplantation in cases of Fanconi's anemia" 05/01/2008 - "Favourable long-term outcome after matched sibling transplantation for Fanconi-anemia (FA) and in vivo T-cell depletion." 09/01/2007 - "We retrospectively analyzed results of unrelated cord blood transplantation (UCBT) in 93 Fanconi anemia (FA) patients" Order ALL the reference details at left...
3.	Hematopoietic Stem Cell Transplantation 02/01/2004 - "The only proven cure for Fanconi anemia (FA)-associated bone marrow failure is successful allogeneic hematopoietic stem cell transplantation (HSCT)" 11/01/2008 - "niveus in a 21-year-old woman after allogeneic hematopoietic stem cell transplantation for Fanconi anemia." 10/01/2008 - "HLA-matched sibling hematopoietic stem cell transplantation for fanconi anemia: comparison of irradiation and nonirradiation containing conditioning regimens." 05/01/2008 - "Hematopoietic stem cell transplantation is the only permanent treatment for the hematological manifestations in Fanconi anemia (FA)" 10/01/2007 - "BACKGROUND AND OBJECTIVES: Hematopoietic stem cell transplantation (HSCT) still represents the only treatment potentially able to prevent/rescue the development of marrow failure and myeloid malignancies in patients with Fanconi anemia (FA)" Order ALL the reference details at left...
4.	Transplants (Transplant) 09/01/2005 - "Secular trends in outcomes for Fanconi anemia patients who receive transplants: implications for future studies." 01/01/2004 - "We report on the effectiveness of molecular studies regarding Fanconi anemia (FA) for a better selection of bone marrow graft donors and for post-transplant follow up" 01/01/2004 - "Fanconi anemia: contribution of molecular analyses to the identification of bone marrow graft donors and the study of chimerism in grafted patients." 11/01/2008 - "Comparison of patients and transplant characteristics between our and Seattle group's cohorts, however, revealed significant differences in more children, in more cord blood HSCT and in HSCT for Fanconi anemia in St Louis" 09/01/2007 - "Results of unrelated cord blood transplant in fanconi anemia patients: risk factor analysis for engraftment and survival." Order ALL the reference details at left...
5.	Stem Cell Transplantation 08/01/2008 - "Matched-related allogeneic stem cell transplantation in Saudi patients with Fanconi anemia: 10 year's experience." 05/01/2008 - "Hematopoetic stem cell transplantation, even from an HLA 6/6 identical family member is associated with an increased frequency of complication in fanconi anemia (FA)" 05/01/2008 - "CD-34 selected hematopoetic stem cell transplantation from HLA identical family members for fanconi anemia." 03/01/2005 - "In all, 22 patients with confirmed Fanconi anemia (FA) underwent stem cell transplantation (SCT) from HLA-matched, related donors at KFSHRC" 01/15/2000 - "Allogeneic stem cell transplantation is the only treatment that can restore a normal hematopoiesis in Fanconi anemia (FA)" Order ALL the reference details at left...

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