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Prospects for nutritional interventions in the clinical management of Fanconi anemia

Authors
  • Pagano, Giovanni1
  • Korkina, Ludmila G.2
  • 1 Istituto Nazionale Tumori, Fondazione “G. Pascale”, v. M. Semmola, Naples, I-80131, Italy , Naples
  • 2 Russian State Medical University, Moscow, 117513, Russia , Moscow
Type
Published Article
Journal
Cancer Causes & Control
Publisher
Springer-Verlag
Publication Date
Dec 01, 2000
Volume
11
Issue
10
Pages
881–889
Identifiers
DOI: 10.1023/A:1026503020755
Source
Springer Nature
Keywords
License
Yellow

Abstract

The evidence associating Fanconi anemia (FA) phenotype to in-vitro and ex-vivo oxidative stress is reviewed. A cancer-prone genetic disease, FA is characterized by delayed bone marrow failure with a progression to aplastic anemia. It is diagnosed by excess chromosomal instability induced by two clastogens, either diepoxybutane (DEB) or mitomycin C (MMC). Clinical symptoms vary in a broad range including a life-threatening hematological impairment, and an extended set of developmental abnormalities, growth retardation and skin pigmentation. Cancer-proneness in FA results in excess incidence of non-lymphoblastic leukemias, and of some defined solid tumors. The relationships of oxidative stress with FA phenotype rely on a consistent body of evidence that includes: (1) excess formation of DNA oxidative damage (both in vitro and in vivo); (2) cellular protection by hypoxia, low molecular-weight antioxidants, antioxidant enzymes, and thioredoxin overexpression; (3) impaired expression and/or activity of antioxidant enzymes, and (4) the redox-dependent action mechanisms of MMC and DEB. This evidence points to a re-appraisal of FA phenotype, suggesting a causative role for oxidative stress in disease progression towards malignancies and/or bone marrow depletion. A well-established literature reporting epidemiological and experimental data provides the nutritional bases for cancer control. Thus, the present state-of-the-art in the related fields of oxidative stress, nutrition, cancer-proneness and FA phenotype, altogether implies the need to undertake the most appropriate efforts to counteract oxidative stress in the clinical management of FA patients.

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