hemofilie B [Hemophilia B]

tematický
214
Termíny

Christmasova choroba
Christmasova nemoc

 

Christmas Disease
Deficiency, Factor IX
F9 Deficiency
Factor IX Deficiency
Haemophilia B
Hemophilia B Leyden
Hemophilia B(M)
Plasma Thromboplastin Component Deficiency

Perzistentní odkaz   https://www.medvik.cz/link/D002836
Definice

Hemofilie způsobená nedostatkem koagulačního faktoru IX (antihemofilického faktoru). Je méně častá než h. A, je rovněž recesivně vázaná na X chromozom. (cit. Velký lékařský slovník online, 2017 http://lekarske.slovniky.cz)

A deficiency of blood coagulation factor IX inherited as an X-linked disorder. (Also known as Christmas Disease, after the first patient studied in detail, not the holy day.) Historical and clinical features resemble those in classic hemophilia (HEMOPHILIA A), but patients present with fewer symptoms. Severity of bleeding is usually similar in members of a single family. Many patients are asymptomatic until the hemostatic system is stressed by surgery or trauma. Treatment is similar to that for hemophilia A. (From Cecil Textbook of Medicine, 19th ed, p1008)

DUI
D002836 MeSH Prohlížeč
CUI
M0004363
Historická pozn.
1999(1966)
Veřejná pozn.
1999; see CHRISTMAS DISEASE 1966-1998; for HEMOPHILIA B see CHRISTMAS DISEASE 1974-1998; for Hemophilia B Leyden see FACTOR IX 1990-2012

C Nemoci
C15.378 krevní nemoci 973
C15.378.100 koagulopatie 599
C15.378.100.100 dědičné koagulopatie 61
C15.378.100.100.056 afibrinogenemie 39
C15.378.100.100.075 nedostatek antitrombinu III 24
C15.378.100.100.080 Bernardův-Soulierův syndrom 9
C15.378.100.100.300 nedostatek faktoru V 7
C15.378.100.100.310 nedostatek faktoru VII 6
C15.378.100.100.320 nedostatek faktoru X 2
C15.378.100.100.325 nedostatek faktoru XI 12
C15.378.100.100.330 nedostatek faktoru XII 8
C15.378.100.100.335 nedostatek faktoru XIII 4
C15.378.100.100.500 hemofilie A 688
C15.378.100.100.510 hemofilie B 214
C15.378.100.100.550 hypoprotrombinemie 4
C15.378.100.100.690 nedostatek proteinu C 31
C15.378.100.100.820 trombastenie 7
C15.378.100.100.900 von Willebrandova nemoc 103
C15.378.100.100.970 Wiskottův-Aldrichův syndrom 20
C15.378.100.141.072 afibrinogenemie 39
C15.378.100.141.300 nedostatek faktoru V 7
C15.378.100.141.310 nedostatek faktoru VII 6
C15.378.100.141.320 nedostatek faktoru X 2
C15.378.100.141.325 nedostatek faktoru XI 12
C15.378.100.141.330 nedostatek faktoru XII 8
C15.378.100.141.335 nedostatek faktoru XIII 4
C15.378.100.141.500 hemofilie A 688
C15.378.100.141.510 hemofilie B 214
C15.378.100.141.550 hypoprotrombinemie 4
C15.378.100.141.900 von Willebrandova nemoc 103
C15.378.463 hemoragické poruchy 95
C15.378.463.067 afibrinogenemie 39
C15.378.463.300 nedostatek faktoru V 7
C15.378.463.310 nedostatek faktoru VII 6
C15.378.463.320 nedostatek faktoru X 2
C15.378.463.325 nedostatek faktoru XI 12
C15.378.463.330 nedostatek faktoru XII 8
C15.378.463.335 nedostatek faktoru XIII 4
C15.378.463.500 hemofilie A 688
C15.378.463.510 hemofilie B 214
C15.378.463.515 poruchy hemostázy 49
C15.378.463.550 hypoprotrombinemie 4
C15.378.463.810 trombastenie 7
C15.378.463.825 esenciální trombocytemie 93
C15.378.463.841 nedostatek vitaminu K 42
C15.378.463.920 von Willebrandova nemoc 103
C16.320.099.056 afibrinogenemie 39
C16.320.099.300 nedostatek faktoru V 7
C16.320.099.310 nedostatek faktoru VII 6
C16.320.099.320 nedostatek faktoru X 2
C16.320.099.325 nedostatek faktoru XI 12
C16.320.099.330 nedostatek faktoru XII 8
C16.320.099.335 nedostatek faktoru XIII 4
C16.320.099.500 hemofilie A 688
C16.320.099.510 hemofilie B 214
C16.320.099.550 hypoprotrombinemie 4
C16.320.099.690 nedostatek proteinu C 31
C16.320.099.820 trombastenie 7
C16.320.099.920 von Willebrandova nemoc 103
C16.320.322.030 Aicardiho syndrom
C16.320.322.068 Barthův syndrom 6
C16.320.322.092 choroideremie 3
C16.320.322.100 Dentova choroba 1
C16.320.322.108 dyskeratosis congenita 9
C16.320.322.124 Fabryho nemoc 187
C16.320.322.201 glykogenóza typu IIb 11
C16.320.322.217 glykogenóza typu VIII
C16.320.322.235 hemofilie B 214