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Titolo:
Early diagnosis of nevoid basal cell carcinoma syndrome
Autore:
Lo Muzio, L; Nocini, P; Bucci, P; Pannone, G; Consolo, U; Procaccini, M;
Indirizzi:
Univ Ancona, Fac Med, Div Oral Pathol & Med, Ancona, Italy Univ Ancona Ancona Italy Fac Med, Div Oral Pathol & Med, Ancona, Italy Univ Naples Federico II, Fac Med, Div Oral Pathol & Med, Naples, Italy Univ Naples Federico II Naples Italy v Oral Pathol & Med, Naples, Italy Univ Verona, Fac Med, Div Oral & Maxillofacial Surg, I-37100 Verona, ItalyUniv Verona Verona Italy I-37100 xillofacial Surg, I-37100 Verona, Italy Univ Modena, Fac Med, Div Oral & Maxillofacial Surg, I-41100 Modena, ItalyUniv Modena Modena Italy I-41100 xillofacial Surg, I-41100 Modena, Italy Univ Ancona, Fac Med, Inst Dent, Ancona, Italy Univ Ancona Ancona ItalyUniv Ancona, Fac Med, Inst Dent, Ancona, Italy
Titolo Testata:
JOURNAL OF THE AMERICAN DENTAL ASSOCIATION
fascicolo: 5, volume: 130, anno: 1999,
pagine: 669 - 674
SICI:
0002-8177(199905)130:5<669:EDONBC>2.0.ZU;2-S
Fonte:
ISI
Lingua:
ENG
Soggetto:
NEVUS SYNDROME; GORLIN SYNDROME; GENE; FAMILIES;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
27
Recensione:
Indirizzi per estratti:
Indirizzo: Lo Muzio, L Via Carelli 28, I-71100 Foggia, Italy Via Carelli 28 Foggia Italy I-71100 8, I-71100 Foggia, Italy
Citazione:
L. Lo Muzio et al., "Early diagnosis of nevoid basal cell carcinoma syndrome", J AM DENT A, 130(5), 1999, pp. 669-674

Abstract

Background. Nevoid basal cell carcinoma syndrome,or NBCCS, is a hereditarycondition characterized by basal cell carcinomas, or BCCs; odontogenic keratocysts, or OKCs; and skeletal abnormalities. The authors conducted this study to determine the early signs of NBCCS. Methods. The authors reviewed files from two Italian dental schools from January 1980 to January 1995 to determine the early signs of NBCCS and the age at which patients were first examined. They re-examined all of the patients, using the diagnostic criteria for NBCCS. Results. The authors found 14 patients who fulfilled the criteria for diagnosis of NBCCS in five families. All of the patients were 16 years bf age or younger. In 11 cases (78 percent), the first sign of NBCCS in the patients was an OKC. The OKCs diagnosed in patients older than 13 years of age were large and characterized by widespread bone resorption. One Ii-year-old patient had six large OKCs. The authors also found a case of multiple OKCs inan 8-year-old patient. Only one patient showed BCCs. Conclusions. OKCs are often the first signs of NBCCS and can be detected in patients younger than 10 years of age. Our data suggest that OKCs arise earlier in patients who have NBCCS than in patients who do not have NBCCS. The patients' young ages explain the low incidence of BCCs in this study. Clinical Implications. The presence of multiple OKCs in a child or onset of BCC in a patient younger than 20 years of age should alert dentists to the possibility of the patient's having NBCCS.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 06/07/20 alle ore 08:16:50