Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0043346 (xeroderma pigmentosum)
 2,924 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

There is experimental evidence that vitamin A-deficiency plays a role in tumorigenesis. Therefore, vitamin A was used in the treatment of malignant tumors. Unfortunately, it was found that the compound was too toxic and therefore its clinical usefulness was limited. Chemical modifications of the vitamin A molecule lead to what is now called the retinoids, which in general show higher efficacy and better tolerance than vitamin A itself. In this article some of the retinoids like 13-retinoic acid (Roaccutan) and etretinate (Tigason) are described with regard to their antitumor activity in animals and man. In widespread malignant disease the efficacy so far is limited to patients with basal cell carcinoma and mycosis fungoides. However, in the treatment of premalignant or in the prevention of recurrent disease retinoids have found much broader application. This is demonstrated by the successful treatment of several diseases like oral leukoplakia, actinic keratosis, xeroderma pigmentosum, bronchial metaplasia and superficial bladder carcinoma.
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PMID:[Retinoids in the prevention and treatment of cancer]. 267 75

Aromatic retinoid Ro 10-9359 1 mg/kg body weight was given to an 11-year-old girl with xeroderma pigmentosum. Therapy resulted in disappearance of actinic keratoses and basal cell carcinomas. The occurrence of new tumors could be prevented in this patient while she participated in this preventive therapy for 7 months. The reduction of the aromatic retinoid to 0.4 mg/kg body weight every other day resulted in the reappearance of four basal cell carcinomas an one keratoacanthoma within 6 weeks.
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PMID:[Tumor prevention in xeroderma pigmentosum using aromatic retinoid (Ro 10-9359)]. 714 72