Xeroderma pigmentosum: Twelve cases at the National Hospital of Niamey, Niger.

Autor: Laouali, Salissou, Adam, Nouhou Diori, Issaka, Hamani, Maïmouna, Oueodraogo Mamadou, Sani, Laouali Idi Mamane, Doulla, Moussa, Hassan, Nouhou
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Zdroj: Our Dermatology Online / Nasza Dermatologia Online; Jan2023, Vol. 14 Issue 1, p56-59, 4p
Abstrakt: Background: Xeroderma pigmentosum (XP) is a usually autosomal recessive disorder linked to a deficiency of the enzyme systems of DNA repair. The pathological sensitivity to the sun exposes the patient to develop multiple cancers. Materials and Methods: We conducted a retrospective study over a period from January 2006 to December 2014. A total of twelve patients were enrolled. Results: These were nine male cases and three female, with a sex ratio of 3/1. The average age was 7.9 years, ranging from 1 to 40 years. Consanguinity between the parents was found in ten cases (83.3%). The first non-tumor cutaneous manifestations appeared in eight patients before the age of six months. During follow-up, seven patients, including three (43%) at the age of eight years, died from metastasis. Conclusion: XP is complicated in the development of cancers, even in children, and is linked to the intensity of solar radiation in Niger. [ABSTRACT FROM AUTHOR]
Databáze: Complementary Index