Jacobsen Distal 11q Deletion Syndrome [Jacobsenův syndrom]

topical
1
Terms

Jacobsenův syndrom - distální mikrodelece 11q
mikrodelece 11q
mikrodelece distálního konce dlouhého raménka chromozómu 11
Parisův-Trousseauův syndrom
Parisův-Trousseauův typ trombocytopenie

 

11q Deletion Disorder
11q Deletion Syndrome
11q Terminal Deletion Disorder
11q- Deletion Syndrome
11q23 Deletion Disorder
Chromosome 11q Deletion Syndrome
Jacobsen Syndrome
Jacobsen Thrombocytopenia
Paris-Trousseau Syndrome
Paris-Trousseau Thrombocytopenia
Partial 11q Monosomy Syndrome
Thrombocytopenia, Paris-Trousseau Type

Persistent link   https://www.medvik.cz/link/D054868
Definition

A clinically recognized congenital malformation condition caused by a distal 11q deletion. The features of the syndrome are growth retardation, psychomotor retardation, trigonocephaly, divergent intermittent strabismus, epicanthus, telecanthus, broad nasal bridge, short nose with anteverted nostrils, carp-shaped upper lip, retrognathia, low-set dysmorphic ears, bilateral camptodactyly, and hammertoes. Platelet dysfunction is a feature in Paris-Trousseau type thrombocytopenia.

DUI
D054868 MeSH Browser
CUI
M0508419
Previous indexing
Chromosomes, Human, Pair 11 (1986-2007)
History note
2008
Public note
2008

C Diseases
C15.378.140.855 Thrombocytopenia 602
C15.378.140.855.645 Kasabach-Merritt Syndrome 3
C15.378.140.855.925 Thrombotic Microangiopathies 62
C15.378.243 Cytopenia 3
C15.378.243.937 Thrombocytopenia 602
C15.378.243.937.645 Kasabach-Merritt Syndrome 3
C15.378.243.937.925 Thrombotic Microangiopathies 62
C16.131.260 Chromosome Disorders 259
C16.131.260.019 22q11 Deletion Syndrome 2
C16.131.260.040 Angelman Syndrome 35
C16.131.260.190 Cri-du-Chat Syndrome 11
C16.131.260.210 De Lange Syndrome 4
C16.131.260.260 Down Syndrome 510
C16.131.260.380 Holoprosencephaly 8
C16.131.260.700 Prader-Willi Syndrome 92
C16.131.260.790 Rubinstein-Taybi Syndrome 12
C16.131.260.830 Sex Chromosome Disorders 11
C16.131.260.870 Silver-Russell Syndrome 3
C16.131.260.887 Smith-Magenis Syndrome 2
C16.131.260.905 Sotos Syndrome 4
C16.131.260.923 Trisomy 13 Syndrome 6
C16.131.260.932 Trisomy 18 Syndrome 5
C16.131.260.940 WAGR Syndrome 3
C16.131.260.970 Williams Syndrome 21
C16.320.180 Chromosome Disorders 259
C16.320.180.019 22q11 Deletion Syndrome 2
C16.320.180.040 Angelman Syndrome 35
C16.320.180.190 Cri-du-Chat Syndrome 11
C16.320.180.210 De Lange Syndrome 4
C16.320.180.260 Down Syndrome 510
C16.320.180.380 Holoprosencephaly 8
C16.320.180.700 Prader-Willi Syndrome 92
C16.320.180.790 Rubinstein-Taybi Syndrome 12
C16.320.180.830 Sex Chromosome Disorders 11
C16.320.180.870 Silver-Russell Syndrome 3
C16.320.180.887 Smith-Magenis Syndrome 2
C16.320.180.905 Sotos Syndrome 4
C16.320.180.923 Trisomy 13 Syndrome 6
C16.320.180.932 Trisomy 18 Syndrome 5
C16.320.180.940 WAGR Syndrome 3
C16.320.180.970 Williams Syndrome 21