Basal Cell Nevus Syndrome [syndrom bazocelulárního névu]

topical
32
Terms

Gorlinův syndrom
Gorlinův-Goltzův syndrom
syndrom névoidních bazaliomů

 

Fifth Phacomatosis
Gorlin Syndrome
Gorlin-Goltz Syndrome
Multiple Basal Cell Nevi, Odontogenic Keratocysts, and Skeletal Anomalies
NBCCS
Nevoid Basal Cell Carcinoma Syndrome
Nevus Syndrome, Basal Cell

Persistent link   https://www.medvik.cz/link/D001478
Definition

Hereditary disorder consisting of multiple basal cell carcinomas, odontogenic keratocysts, and multiple skeletal defects, e.g., frontal and temporoparietal bossing, bifurcated and splayed ribs, kyphoscoliosis, fusion of vertebrae, and cervicothoracic spina bifida. Genetic transmission is autosomal dominant.

Annotation
coordinate IM with precoordinated organ/neoplasm (IM or NIM) only if pertinent; do not confuse entry term GORLIN-GOLTZ SYNDROME with GOLTZ- GORLIN SYNDROME see FOCAL DERMAL HYPOPLASIA
DUI
D001478 MeSH Browser
CUI
M0002195
Previous indexing
Bone Cyst (1966-1979); Carcinoma, Basal Cell (1966-1979); Jaw Diseases (1967-1979); Jaw Neoplasms (1967-1979); Skin Neoplasms (1967-1979); specific jaw disease (1967-1979); specific jaw neoplasm (1967-1979); Syndrome (1972-1979)
History note
91(80); was see under CARCINOMA, BASAL CELL 1980-90
Public note
91; was see under CARCINOMA, BASAL CELL 1980-90

Allowable subheadings

BL
blood 0
CF
cerebrospinal fluid 0
CI
chemically induced 0
CL
classification 0
CO
complications 5
DI
diagnosis 18
DG
diagnostic imaging 1
DH
diet therapy 0
DT
drug therapy 2
EC
economics 0
EM
embryology 0
EN
enzymology 0
EP
epidemiology 2
EH
ethnology 0
ET
etiology 4
GE
genetics 14
HI
history 1
IM
immunology 0
ME
metabolism 1
MI
microbiology 0
MO
mortality 1
NU
nursing 0
PS
parasitology 0
PA
pathology 8
PP
physiopathology 2
PC
prevention & control 1
PX
psychology 0
RT
radiotherapy 0
RH
rehabilitation 0
SU
surgery 2
TH
therapy 9
UR
urine 0
VE
veterinary 0
VI
virology 0

C Diseases
C04 Neoplasms 12 762
C04.182 Cysts 722
C04.182.089 Bone Cysts 78
C04.182.089.530 Jaw Cysts 41
C04.182.089.530.690 Odontogenic Cysts 49
C04.182.089.530.690.150 Basal Cell Nevus Syndrome 32
C04.182.089.530.690.310 Dentigerous Cyst 4
C04.182.089.530.690.605 Odontogenic Cyst, Calcifying 2
C04.182.089.530.690.790 Periodontal Cyst 8
C04.557 Neoplasms by Histologic Type 270
C04.557.470 Neoplasms, Glandular and Epithelial 75
C04.557.470.200 Carcinoma 4 827
C04.557.470.200.165 Carcinoma, Basal Cell 346
C04.557.470.200.165.150 Basal Cell Nevus Syndrome 32
C04.557.470.565 Neoplasms, Basal Cell 17
C04.557.470.565.165 Carcinoma, Basal Cell 346
C04.557.470.565.165.150 Basal Cell Nevus Syndrome 32
C04.700 Neoplastic Syndromes, Hereditary 143
C04.700.100 Adenomatous Polyposis Coli 92
C04.700.175 Basal Cell Nevus Syndrome 32
C04.700.212 Birt-Hogg-Dube Syndrome 8
C04.700.250 Colorectal Neoplasms, Hereditary Nonpolyposis 114
C04.700.305 Dysplastic Nevus Syndrome 17
C04.700.330 Exostoses, Multiple Hereditary 12
C04.700.435 Hamartoma Syndrome, Multiple 25
C04.700.517 Hereditary Breast and Ovarian Cancer Syndrome 53
C04.700.600 Li-Fraumeni Syndrome 39
C04.700.630 Multiple Endocrine Neoplasia 32
C04.700.631 Neurofibromatoses 28
C04.700.633 Peutz-Jeghers Syndrome 37
C04.700.700 Tuberous Sclerosis 141
C04.700.900 Wilms Tumor 112
C05 Musculoskeletal Diseases 1 119
C05.116 Bone Diseases 792
C05.116.099 Bone Diseases, Developmental 291
C05.116.099.052 Acro-Osteolysis 3
C05.116.099.105 Basal Cell Nevus Syndrome 32
C05.116.099.343 Dwarfism 97
C05.116.099.370 Dysostoses 22
C05.116.099.386 Funnel Chest 38
C05.116.099.492 Gigantism 14
C05.116.099.655 Leg Length Inequality 118
C05.116.099.674 Marfan Syndrome 105
C05.116.099.708 Osteochondrodysplasias 123
C05.116.099.736 Osteolysis, Essential 8
C05.116.099.739 Pectus Carinatum 1
C05.116.099.742 Platybasia 12
C05.116.099.750 Proteus Syndrome 8
C05.500 Jaw Diseases 134
C05.500.470 Jaw Cysts 41
C05.500.470.690 Odontogenic Cysts 49
C05.500.470.690.150 Basal Cell Nevus Syndrome 32
C05.500.470.690.310 Dentigerous Cyst 4
C05.500.470.690.605 Odontogenic Cyst, Calcifying 2
C05.500.470.690.790 Periodontal Cyst 8
C07 Stomatognathic Diseases 117
C07.320 Jaw Diseases 134
C07.320.450 Jaw Cysts 41
C07.320.450.670 Odontogenic Cysts 49
C07.320.450.670.130 Basal Cell Nevus Syndrome 32
C07.320.450.670.275 Dentigerous Cyst 4
C07.320.450.670.495 Odontogenic Cyst, Calcifying 2
C07.320.450.670.513 Periodontal Cyst 8
C16 Congenital, Hereditary, and Neonatal Diseases and Abnormalities 439
C16.131 Congenital Abnormalities 1 740
C16.131.077 Abnormalities, Multiple 330
C16.131.077.019 22q11 Deletion Syndrome 2
C16.131.077.065 Alagille Syndrome 19
C16.131.077.095 Angelman Syndrome 35
C16.131.077.121 Barth Syndrome 6
C16.131.077.130 Basal Cell Nevus Syndrome 32
C16.131.077.133 Beckwith-Wiedemann Syndrome 17
C16.131.077.137 Bloom Syndrome 11
C16.131.077.208 Branchio-Oto-Renal Syndrome 5
C16.131.077.229 Carney Complex 8
C16.131.077.245 Ciliopathies 9
C16.131.077.250 Cockayne Syndrome 4
C16.131.077.256 Costello Syndrome 3
C16.131.077.262 Cri-du-Chat Syndrome 11
C16.131.077.272 De Lange Syndrome 4
C16.131.077.299 Deaf-Blind Disorders 65
C16.131.077.313 Donohue Syndrome 1
C16.131.077.327 Down Syndrome 510
C16.131.077.350 Ectodermal Dysplasia 33
C16.131.077.371 Fraser Syndrome 2
C16.131.077.393 Gardner Syndrome 20
C16.131.077.401 Heterotaxy Syndrome 7
C16.131.077.410 Holoprosencephaly 8
C16.131.077.445 Incontinentia Pigmenti 19
C16.131.077.477 Isolated Noncompaction of the Ventricular Myocardium 11
C16.131.077.509 Laurence-Moon Syndrome 6
C16.131.077.525 LEOPARD Syndrome 11
C16.131.077.537 Loeys-Dietz Syndrome 7
C16.131.077.550 Marfan Syndrome 105
C16.131.077.578 Mobius Syndrome 10
C16.131.077.592 Monilethrix 1
C16.131.077.606 Nail-Patella Syndrome 8
C16.131.077.619 Netherton Syndrome 2
C16.131.077.633 Nevus, Sebaceous of Jadassohn 7
C16.131.077.662 Oculocerebrorenal Syndrome 9
C16.131.077.676 Orofaciodigital Syndromes 7
C16.131.077.690 Pallister-Hall Syndrome
C16.131.077.696 Pentalogy of Cantrell 1
C16.131.077.703 POEMS Syndrome 34
C16.131.077.717 Polycystic Kidney Diseases 82
C16.131.077.730 Prader-Willi Syndrome 91
C16.131.077.735 Prolidase Deficiency
C16.131.077.740 Proteus Syndrome 8
C16.131.077.745 Prune Belly Syndrome 3
C16.131.077.790 Rubella Syndrome, Congenital 20
C16.131.077.804 Rubinstein-Taybi Syndrome 12
C16.131.077.850 Short Rib-Polydactyly Syndrome 3
C16.131.077.855 Silver-Russell Syndrome 3
C16.131.077.860 Smith-Lemli-Opitz Syndrome 30
C16.131.077.879 Smith-Magenis Syndrome 2
C16.131.077.889 Sotos Syndrome 4
C16.131.077.899 Trichothiodystrophy Syndromes 1
C16.131.077.919 Trisomy 13 Syndrome 6
C16.131.077.929 Trisomy 18 Syndrome 5
C16.131.077.938 Waardenburg Syndrome 10
C16.131.077.941 Weill-Marchesani Syndrome 1
C16.131.077.944 Wolf-Hirschhorn Syndrome
C16.131.077.970 Zellweger Syndrome 10
C16.320 Genetic Diseases, Inborn 1 213
C16.320.700 Neoplastic Syndromes, Hereditary 143
C16.320.700.100 Adenomatous Polyposis Coli 92
C16.320.700.175 Basal Cell Nevus Syndrome 32
C16.320.700.212 Birt-Hogg-Dube Syndrome 8
C16.320.700.250 Colorectal Neoplasms, Hereditary Nonpolyposis 114
C16.320.700.305 Dysplastic Nevus Syndrome 17
C16.320.700.330 Exostoses, Multiple Hereditary 12
C16.320.700.435 Hamartoma Syndrome, Multiple 25
C16.320.700.517 Hereditary Breast and Ovarian Cancer Syndrome 53
C16.320.700.600 Li-Fraumeni Syndrome 39
C16.320.700.630 Multiple Endocrine Neoplasia 32
C16.320.700.633 Neurofibromatoses 28
C16.320.700.667 Peutz-Jeghers Syndrome 37
C16.320.700.700 Tuberous Sclerosis 141
C16.320.700.900 Wilms Tumor 112

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