Antley-Bixler Syndrome Phenotype [fenotyp Antley-Bixlerova syndromu]

topical
1
Terms

Antley-Bixlerův syndrom
Antley-Bixlerův syndrom typ 1
Antley-Bixlerův syndrom typ 2
Antleyho-Bixlerův syndrom - fenotyp
Antleyho-Bixlerův syndrom autozomálně dominantní
Antleyho-Bixlerův syndrom s anomáliemi genitálu a poruchou steroidogeneze
fenotyp Antley Bixlerova syndromu
POR deficience
POR-vázaný Antleyho-Bixlerův syndrom

 

Adrenal Hyperplasia, Congenital, Due To Cytochrome P450 Oxidoreductase Deficiency
Antley-Bixler Syndrome
Antley-Bixler Syndrome Type 1
Antley-Bixler Syndrome Type 2
Antley-Bixler Syndrome with Disordered Steroidogenesis
Antley-Bixler Syndrome-Like Phenotype With Disordered Steroidogenesis
Antley-Bixler Syndrome, Autosomal Dominant
Antley-Bixler Syndrome, Autosomal Recessive
Combined Partial Deficiency of 17-Hydroxylase and 21-Hydroxylase
Congenital Adrenal Hyperplasia Due To Apparent Combined P450c17 and P450c21 Deficiency
Cytochrome P450 Oxidoreductase Deficiency
Disordered Steroidogenesis Due To Cytochrome P450 Oxidoreductase Deficiency
Multisynostotic Osteodysgenesis
Multisynostotic Osteodysgenesis With Long Bone Fractures
Osteodysgenesis, Multisynostotic, With Fractures
POR Deficiency
Trapezoidocephaly-Synostosis Syndrome

Persistent link   https://www.medvik.cz/link/D054882
Definition

An inherited condition characterized by multiple malformations of CARTILAGE and bone including CRANIOSYNOSTOSIS; midface hypoplasia; radiohumeral SYNOSTOSIS; CHOANAL ATRESIA; femoral bowing; neonatal fractures; and multiple joint CONTRACTURES and, occasionally, urogenital, gastrointestinal or cardiac defects. In utero exposure to FLUCONAZOLE, as well as mutations in at least two separate genes are associated with this condition - POR (encoding P450 (cytochrome) oxidoreductase (NADPH-FERRIHEMOPROTEIN REDUCTASE)) and FGFR2 (encoding FIBROBLAST GROWTH FACTOR RECEPTOR 2).

DUI
D054882 MeSH Browser
CUI
M0508592
Previous indexing
Abnormalities, Multiple (1982-2008); Craniosynostoses (1982-2008); Syndrome (1982-2008); Synostosis (1982-2008)
History note
2009
Public note
2009

C Diseases
C05.116 Bone Diseases 792
C05.116.099.370 Dysostoses 22
C05.116.099.370.894 Synostosis 29
C05.116.099.370.894.115 Antley-Bixler Syndrome Phenotype 1
C05.116.099.370.894.232 Craniosynostoses 55
C05.116.099.370.894.819 Syndactyly 24
C05.116.099.370.894.909 Tarsal Coalition 2
C05.660.906 Synostosis 29
C05.660.906.364 Craniosynostoses 55
C05.660.906.819 Syndactyly 24
C05.660.906.909 Tarsal Coalition 2
C16.131.621.906 Synostosis 29
C16.131.621.906.364 Craniosynostoses 55
C16.131.621.906.819 Syndactyly 24
C16.131.621.906.909 Tarsal Coalition 2
C16.320.565.925.249 Adrenal Hyperplasia, Congenital 225
C16.320.565.925.400 Ichthyosis, X-Linked 9
C16.320.565.925.875 Smith-Lemli-Opitz Syndrome 30
C18.452 Metabolic Diseases 1 196
C18.452.648.925.249 Adrenal Hyperplasia, Congenital 225
C18.452.648.925.400 Ichthyosis, X-Linked 9
C18.452.648.925.875 Smith-Lemli-Opitz Syndrome 30