Rare Inherited Coagulation Disorders
Quiz by Sadeq K. Ali Al-Salait
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- Q1
Which of the following rare bleeding disorders has the strongest correlation between factor activity level and clinical bleeding severity?
Factor VII deficiency
Factor XIII deficiency
Dysfibrinogenemia
Factor XI deficiency
Factor V deficiency
30s - Q2
A newborn presents with delayed umbilical cord separation and bleeding. Which coagulation factor deficiency is most likely?
Vitamin K-dependent factor deficiency
Factor XIII deficiency
Factor XI deficiency
Factor VII deficiency
Factor X deficiency
30s - Q3
Which disorder is characterized by mild bleeding symptoms despite significantly reduced factor levels?
Dysfibrinogenemia
Prothrombin deficiency
Factor XIII deficiency
Severe Factor X deficiency
Factor XI deficiency
30s - Q4
Which bleeding symptom is most commonly associated with Factor VII deficiency?
Hemarthrosis
Hematuria
Muscle hematomas
Epistaxis and menorrhagia
Spontaneous CNS bleeding
30s - Q5
Which of the rare inherited bleeding tendency is characterized by spontaneous hemarthrosis and deep tissue bleeding?
Factor XI deficiency
Vitamin K-dependent factor deficiency
Factor X deficiency
Dysfibrinogenemia
Factor VII deficiency
30s - Q6
Which laboratory finding is characteristic of prothrombin (Factor II) deficiency?
Isolated prolonged aPTT
Normal PT and aPTT with increased bleeding time
Normal PT and prolonged thrombin time
Prolonged PT and aPTT with normal thrombin time
Isolated prolonged PT
30s - Q7
Clot solubility test is useful in the diagnosis of ...
Prothrombin deficiency
Dysfibrinogenemia
Factor VII deficiency
Hypo dysfibrinogenemia
Factor XIII deficiency
30s - Q8
Which treatment is the most effective for severe congenital Factor XIII deficiency?
Fresh frozen plasma (FFP)
Desmopressin (DDAVP)
Recombinant FXIII-A subunit concentrate
Recombinant Factor VIIa
Prothrombin complex concentrate (PCC)
30s - Q9
Which therapy is used for Factor V deficiency, given the lack of specific factor concentrates?
Cryo-supernatant
Whole blood
Cryoprecipitate
Fresh frozen plasma (FFP)
Desmopressin
30s - Q10
A patient with mild Factor XI deficiency requires dental surgery. What is the best perioperative management strategy?
Fresh frozen plasma
Recombinant Factor VIIa
Antifibrinolytics such as tranexamic acid
Prothrombin complex concentrate
Factor XI concentrate
30s - Q11
Which inherited coagulation disorder requires thrombosis risk monitoring when treated with prothrombin complex concentrate (PCC)?
Dysfibrinogenemia
Factor II deficiency
Factor VII deficiency
Factor XI deficiency
Factor V deficiency
30s - Q12
In a patient with combined Factor V and Factor VIII deficiency, which coagulation profile abnormality is most commonly seen?
Prolonged PT, normal aPTT and TT
Prolonged aPTT, normal PT and TT
Prolonged PT and aPTT with normal TT
Prolonged PT, aPTT and TT
Prolonged PT more than aPTT, prolonged TT
30s