Neurofibromatosis Type 1 Associated with Dysplastic Nevus Syndrome

Summary: Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder that primarily affects the development and growth of neural cell tissues. It causes tumors to grow on nerves and produces other abnormalities such as skin changes and bone deformities. Dysplastic nevus syndrome (DNS) represen...

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Permalink: http://skupnikatalog.nsk.hr/Record/nsk.NSK01000733987/Details
Matična publikacija: Acta dermatovenerologica Croatica
17 (2009), 2 ; str. 118-122
Glavni autor: Paštar, Zrinjka (-)
Ostali autori: Lipozenčić, Jasna (-), Kovačević, Suzana, liječnica, Čanović, Samir, Didović-Torbarina, Ana, Vukasović, Anamarija
Vrsta građe: Članak
Jezik: eng
Predmet:
Online pristup: Acta Dermatovenerologica Croatica
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100 1 |a Paštar, Zrinjka 
245 1 0 |a Neurofibromatosis Type 1 Associated with Dysplastic Nevus Syndrome /  |c Zrinjka Paštar, Jasna Lipozenčić, Suzana Kovačević, Samir Ćanović, Ana Didović-Torbarina, Anamarija Vukasović. 
300 |b Ilustr. 
504 |a Bibliografija: 20 jed 
520 8 |a Summary: Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder that primarily affects the development and growth of neural cell tissues. It causes tumors to grow on nerves and produces other abnormalities such as skin changes and bone deformities. Dysplastic nevus syndrome (DNS) represents multiple atypical nevi associated with polygenetic inheritance pattern and may rarely occur together with NF-1. DNS type A is a marker of increased melanoma risk, while melanoma has been rarely reported in patients with NF-1. We describe a case of NF-1 type A with DNS presenting with multiple neural tumors, cafe-au-lait spots, hamartomas in globus pallidus and pigmented melanocytic iris hamartomas (Lisch nodules). The importance of close follow up of nevi in such patients with NF-1 and DNS for the development of melanoma as well as other NF-1 associated skin disorders and with multidisciplinary approach to other associated diseases is highlighted 
653 0 |a Neurofibromatoza tip 1  |a Autosomno dominantni poremećaj  |a Nasljedne bolesti  |a Sindrom displastičnih nevusa  |a Melanom 
700 1 |a Lipozenčić, Jasna 
700 1 |a Kovačević, Suzana,  |c liječnica 
700 1 |a Čanović, Samir 
700 1 |a Didović-Torbarina, Ana 
700 1 |a Vukasović, Anamarija 
773 0 |t Acta dermatovenerologica Croatica  |x 1330-027X  |g 17 (2009), 2 ; str. 118-122  |w nsk.(HR-ZaNSK)000009221 
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