Facial Resurfacing in Xeroderma Pigmentosum with Monoblock Full-Thickness Skin Graft

Atabay, Kenan; Çelebi, Cemalettin; Çenetoğlu, Seyhan; Baran, Namık K.; Kiymaz, Ziya

doi:10.1097/00006534-199106000-00018

Cited by 33 publications

(21 citation statements)

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“…9 However, the most effective treatment of XP is stringent avoidance of all sources of UVB radiation from very early childhood. [10][11][12] Enzymatic therapy with liposomes containing bacteriophage T4 endonuclease V has been reported to reduce the frequency of skin cancer in addition to actinic keratoses in XP patients following topical treatment, possibly by enhancing DNA repair in the skin. 12 Complementing XPC gene, direct gene therapy has been successfully attempted in vitro and showed complete correction of repair-defected cellular phenotypes.…”

Section: Introductionmentioning

confidence: 99%

Catalase overexpression reduces UVB-induced apoptosis in a human xeroderma pigmentosum reconstructed epidermis

et al. 2008

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Xeroderma pigmentosum type C (XPC) is a rare autosomal recessive disorder that occurs due to inactivation of the XPC protein, an important DNA damage recognition protein involved in DNA nucleotide excision repair (NER). This defect, which prevents removal of a wide array of direct and indirect DNA lesions, is associated with a decrease in catalase activity. To test the hypothesis of a novel photoprotective approach, we irradiated epidermis reconstructed with XPC human keratinocytes sustainably overexpressing lentivirus-mediated catalase enzyme. Following UVB irradiation, there was a marked decrease in sunburn cell formation, caspase-3 activation and p53 accumulation in human XPC-reconstructed epidermis overexpressing catalase. Moreover, XPC-reconstructed epidermis was more resistant to UVB-induced apoptosis than normal reconstructed epidermis. While not correcting the gene defect, indirect gene therapy using antioxidant enzymes may be of help in limiting photosensitivity in XPC and probably in other monogenic/polygenic photosensitive disorders characterized by ROS accumulation.

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Section: Introductionmentioning

confidence: 99%

Catalase overexpression reduces UVB-induced apoptosis in a human xeroderma pigmentosum reconstructed epidermis

et al. 2008

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“…Some protective measures can be taken to keep patients from exposure to sunlight, 23 and skin grafts from the same patient were performed to resurface the therapeutical protocol is efficient only in the short term, complementation groups, transduced and untransduced cells were analyzed for: since skin grafts are still genetically DNA repair defective and thus cancer prone. 24 The genetic correction of XP (1 Figure 2a and genes into cells derived from XP patients. 25,26 Several b, respectively), XPCS2BASV (XP-B group, Figure 2c) retroviral constructions are efficient tools for gene delivand XP16VI and XP30VI (XP-C group, Figure 2d).…”

Section: Introductionmentioning

confidence: 99%

Retrovirus-mediated gene transfer corrects DNA repair defect of xeroderma pigmentosum cells of complementation groups A, B and C

et al. 1997

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With the aim to devise a long-term gene therapy protocol survival, unscheduled DNA synthesis and recovery of RNA for skin cancers in individuals affected by the inherited synthesis, and Western blots. The results show that the autosomal recessive xeroderma pigmentosum, we transrecombinant retroviruses are highly efficient vectors to ferred the human DNA repair XPA, XPB/ERCC3 and XPC transfer and stably express the human DNA repair genes cDNAs, by using the recombinant retroviral vector LXSN, in XP cells and correct the defect of DNA repair of group into primary and immortalized fibroblasts obtained from two A, B and C. With our previous results with XPD/ERCC2, XP-A, one XP-B (associated with Cockayne's syndrome) the present work extends further promising issues for the and two XP-C patients. After transduction, the complete gene therapy strategy for most patients suffering from this correction of DNA repair deficiency and functional cancer-prone syndrome. expression of the transgenes were monitored by UV

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“…Surgical treatment may be required in XP patients who develop suspected skin lesions. Extensive lesions need reconstruction with split-thickness skin grafts or various flaps [1,2]. In patients with no predisposing genetic factors, surgical extirpation of tumor with negative surgical margins is the appropriate standard for the removal of a skin malignancy.…”

Section: Discussionmentioning

confidence: 99%

Squamous cell carcinoma of the lower lid in a 19-month-old girl with xeroderma pigmentosum

Hadi

Tohmeh

Maalouf

2000

European Archives of Oto-Rhino-Laryngology

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Squamous cell carcinoma (SCC) of the skin usually occurs in older patients and commonly develops from actinic keratosis. Patients with xeroderma pigmentosum (XP) can acquire SCC at an early age. To our knowledge the youngest reported patient with XP and SCC was 8 years of age. We report a 19-month-old Lebanese girl with XP who presented clinically because of a rapidly growing mass of the medial part of her right lower lid that was biopsied and found to be squamous cell carcinoma. The mass was surgically excised with no evidence of recurrence after 2 years of follow-up. We describe our clinical experiences with this patient and have reviewed the available literature concerning XP and malignancy.

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Facial Resurfacing in Xeroderma Pigmentosum with Monoblock Full-Thickness Skin Graft

Cited by 33 publications

References 0 publications

Catalase overexpression reduces UVB-induced apoptosis in a human xeroderma pigmentosum reconstructed epidermis

Catalase overexpression reduces UVB-induced apoptosis in a human xeroderma pigmentosum reconstructed epidermis

Retrovirus-mediated gene transfer corrects DNA repair defect of xeroderma pigmentosum cells of complementation groups A, B and C

Squamous cell carcinoma of the lower lid in a 19-month-old girl with xeroderma pigmentosum

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