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trombomodulinegerelateerde stollingsstoornis (aandoening)
trombomodulinegerelateerde stollingsstoornis
THBD-gerelateerde coagulopathie
Thrombomodulin-related bleeding disorder
THBD (thrombomodulin) related bleeding disorder
Thrombomodulin-related coagulopathy
A rare genetic coagulation disorder characterized by marked bleeding tendency and posttraumatic bleeding with easy bruising, soft tissue and muscle bleeding, hemarthroses and menorrhagia. Caused by an increase of soluble thrombomodulin in plasma with subsequent protein C activation and reduction of thrombin generation within a potential thrombus. Abnormal laboratory findings include markedly elevated plasma thrombomodulin, reduced prothrombin consumption and decreased thrombin generation.
Id1197595004
StatusPrimitive
Has interpretationafwijkend
Interpretshemostase
SNOMED CT to Orphanet simple map436169
SNOMED CT to ICD-10 extended map
TargetD68.3
RuleTRUE
AdviceALWAYS D68.3 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified