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The level of evidence and grade of recommendation for each therapeutic option is assigned according to “Oxford center of evidence-based medicine (EBM) guidelines.” Therefore, early diagnosis and timely symptom management are very pivotal for the long-term survival of patients with XP.ĭifferent databases including Pubmed were accessed with different keywords such as “xeroderma pigmentosum,” “therapeutic strategies,” “skin cancer,” “DNA repair,” and “gene therapy.” Among the articles found in the databases, the articles focused on the treatment and management of xeroderma pigmentosum rather than only focusing on the molecular mechanism of XP and DNA repair mechanism were selected for this study.
#Xeroderma pigmentosum pdf skin
In accordance with Hong et al., the patients of XP have a 100% risk of skin cancer, and approximately two-thirds of them die of cancer before the age of 20. XP patients experience idiopathic neurodegenerative complications reducing the quality of life (QOL). Now XP is considered as one of the most serious photosensitive disorders in which patients are developing multiple skin cancers if not prevented by avoiding UV. Cleaver (1968) first reported that it is due to genetic abnormalities of the DNA repair process and XP cells were unable to repair DNA damage caused by ultraviolet radiation (UV). Though research in rare genetic diseases such as XP is considered as a lesser priority compared to the common ones, the rare disease may be considered as human models for better understanding the physiology and mechanistic of the human body as like the role of rare genetic disorders tuberous sclerosis complex (TSC) in the understanding of the mammalian target of rapamycin complex 1 (mTORC1).
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Another survey of Western Europe suggested that approximately 2.3 per million are affected by this disease. According to an estimate made in the 1970s, in the USA 1 in 250,000 and in Japan 1 in 20,000 are affected with XP. It affects people of all continents and all racial groups with autosomal recessive inheritance similarly affecting both males and females. Xeroderma pigmentosum (XP), a rare genetic photosensitive disorder, exhibits increased susceptibility to skin cancers on the sun-exposed body sites of the patients. Current Therapeutic Strategies of Xeroderma Pigmentosum.
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How to cite this URL: Hossain M, Hasan A, Khan Shawan MM, Banik S, Jahan I. How to cite this article: Hossain M, Hasan A, Khan Shawan MM, Banik S, Jahan I. Keywords: DNA repair, gene therapy, skin cancer, therapeutic strategies, xeroderma pigmentosum In the near future, these approaches may be effective tools to manage XP. Besides these, gene therapy, including the introduction of missing genes and genome edition, may be a promising approach to combat this disease, which is also not well established now. In addition, some approaches including oral vismodegib, immunotherapy, nicotinamide, acetohexamide, glimepiride-restricted diet are found to be effective to minimize the complications secondary to defects in the nucleotide excision repair (NER) system and also enhance the NER, which are under experimental level yet. Furthermore, surgical removal of cancerous lesions and use of antioxidants are considered to be effective against this disease however, efficacies of these are not experimentally determined.
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However, some approaches including laser and photodynamic therapy, and the use of retinoids are effective against skin cancer having severe side effects. Among these options, sun avoidance, use of 5-fluorouracil and imiquimod, and interferon therapy are effective.
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Different approaches including sun avoidance surgical removal of cancerous lesions laser and photodynamic therapy use of retinoid, 5-fluorouracil, imiquimod, photolyase, and antioxidant interferon therapy and gene therapy are chosen by doctors and patients to lessen the adverse effects of this disease. This review article takes an attempt to explore the current therapeutic advancements in XP. As it is an incurable disease, therapeutic strategies of this disease are critical. Xeroderma pigmentosum (XP) is an autosomal recessive genetic disease caused by a defect in the DNA repair system, exhibiting skin cancer on sun exposure.
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