The naevoid basal cell carcinoma syndrome. Clinical, biochemical and radiological aspects. 1982

H Lindeberg, and H Halaburt, and P O Larsen

Eight living patients with the nevoid basal cell carcinoma syndrome (NBS) underwent a clinical and radiological examination, and data on a deceased patient was included. Cerebral CT-scannings were performed on eight patients, and calcification in the falx and tentorium cerebelli was found in all patients but one. Congenital hydrocephalus or agenesis of the corpus callosum could not be demonstrated. Laboratory results, including serum levels of alpha 1-antitrypsin and alpha 2-macroglobulin, haptoglobin, inorganic phosphorus, alkaline phosphatase and calcium, were normal in all patients but two. It is postulated that elevated serum alkaline phosphatase in patients with NBS is correlated to the presence of growing odontogenic keratocysts.

UI MeSH Term Description Entries
D007570 Jaw Cysts Saccular lesions lined with epithelium and contained within pathologically formed cavities in the jaw; also nonepithelial cysts (pseudocysts) as they apply to the jaw, e.g., traumatic or solitary cyst, static bone cavity, and aneurysmal bone cyst. True jaw cysts are classified as odontogenic or nonodontogenic. Cyst, Jaw,Cysts, Jaw,Jaw Cyst
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009807 Odontogenic Cysts Cysts found in the jaws and arising from epithelium involved in tooth formation. They include follicular cysts (e.g., primordial cyst, dentigerous cyst, multilocular cyst), lateral periodontal cysts, and radicular cysts. They may become keratinized (odontogenic keratocysts). Follicular cysts may give rise to ameloblastomas and, in rare cases, undergo malignant transformation. Keratocysts,Cyst, Odontogenic,Cysts, Odontogenic,Keratocyst,Odontogenic Cyst
D002280 Carcinoma, Basal Cell A malignant skin neoplasm that seldom metastasizes but has potentialities for local invasion and destruction. Clinically it is divided into types: nodular, cicatricial, morphaic, and erythematoid (pagetoid). They develop on hair-bearing skin, most commonly on sun-exposed areas. Approximately 85% are found on the head and neck area and the remaining 15% on the trunk and limbs. (From DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1471) Carcinoma, Basal Cell, Pigmented,Epithelioma, Basal Cell,Rodent Ulcer,Ulcer, Rodent,Basal Cell Carcinoma,Basal Cell Carcinomas,Basal Cell Epithelioma,Basal Cell Epitheliomas,Carcinomas, Basal Cell,Epitheliomas, Basal Cell,Rodent Ulcers,Ulcers, Rodent
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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