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Xeroderma Pigmentosum: General Aspects and Management.
Piccione, Monica; Belloni Fortina, Anna; Ferri, Giulia; Andolina, Gloria; Beretta, Lorenzo; Cividini, Andrea; De Marni, Emanuele; Caroppo, Francesca; Citernesi, Ugo; Di Liddo, Rosa.
Afiliación
  • Piccione M; Department of Pharmaceutical and Pharmacological Sciences, University of Padova, 35131 Padova, Italy.
  • Belloni Fortina A; Pediatric Dermatology Unit, Department of Medicine DIMED, University of Padova, 35128 Padova, Italy.
  • Ferri G; I.R.A. Istituto Ricerche Applicate S.p.A., 20865 Usmate Velate, Italy.
  • Andolina G; I.R.A. Istituto Ricerche Applicate S.p.A., 20865 Usmate Velate, Italy.
  • Beretta L; I.R.A. Istituto Ricerche Applicate S.p.A., 20865 Usmate Velate, Italy.
  • Cividini A; I.R.A. Istituto Ricerche Applicate S.p.A., 20865 Usmate Velate, Italy.
  • De Marni E; I.R.A. Istituto Ricerche Applicate S.p.A., 20865 Usmate Velate, Italy.
  • Caroppo F; Pediatric Dermatology Unit, Department of Medicine DIMED, University of Padova, 35128 Padova, Italy.
  • Citernesi U; I.R.A. Istituto Ricerche Applicate S.p.A., 20865 Usmate Velate, Italy.
  • Di Liddo R; Department of Pharmaceutical and Pharmacological Sciences, University of Padova, 35131 Padova, Italy.
J Pers Med ; 11(11)2021 Nov 04.
Article en En | MEDLINE | ID: mdl-34834498
Xeroderma Pigmentosum (XP) is a rare genetic syndrome with a defective DNA nucleotide excision repair. It is characterized by (i) an extreme sensitivity to ultraviolet (UV)-induced damages in the skin and eyes; (ii) high risk to develop multiple skin tumours; and (iii) neurologic alterations in the most severe form. To date, the management of XP patients consists of (i) early diagnosis; (ii) a long-life protection from ultraviolet radiation, including avoidance of unnecessary UV exposure, wearing UV blocking clothing, and use of topical sunscreens; and (iii) surgical resections of skin cancers. No curative treatment is available at present. Thus, in the last decade, in order to prevent or delay the progression of the clinical signs of XP, numerous strategies have been proposed and tested, in some cases, with adverse effects. The present review provides an overview of the molecular mechanisms featuring the development of XP and highlights both advantages and disadvantages of the clinical approaches developed throughout the years. The intention of the authors is to sensitize scientists to the crucial aspects of the pathology that could be differently targeted. In this context, the exploration of the process underlining the conception of liposomal nanocarriers is reported to focus the attention on the potentialities of liposomal technology to optimize the administration of chemoprotective agents in XP patients.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Tipo de estudio: Screening_studies Idioma: En Revista: J Pers Med Año: 2021 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Suiza

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Tipo de estudio: Screening_studies Idioma: En Revista: J Pers Med Año: 2021 Tipo del documento: Article País de afiliación: Italia Pais de publicación: Suiza