Hemorrhage - Hemophilia

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What is the most common inherited vascular bleeding disorder?

Cutis Hyperelastica

Hereditary Hemorrhagic Telangiectasia (correct)

Ehlers-Danlos Syndrome

Osler-Weber-Rendu Disease

What is the primary characteristic of Ehlers-Danlos Syndrome?

Recurrent chronic bruising in multiple locations

Localized dilation of capillary walls

Hyperextensible joints and hyperelastic skin (correct)

Vomiting of blood

Which of the following is a sign of a possible hemostatic defect?

Menorrhagia with appropriate bleeding

Epistaxis lasting less than 10 minutes

Bleeding that is delayed or recurrent (correct)

Petechiae larger than 1cm in diameter

What is the inheritance pattern of Hereditary Hemorrhagic Telangiectasia?

Autosomal dominant Signup and view all the answers

Which of the following is NOT a generalized bleeding sign?

Epistaxis lasting less than 5 minutes Signup and view all the answers

What condition includes unintentional injury and accounts for most fatal hemorrhage?

Acute Coagulopathy of Trauma-Shock (ACOTS) Signup and view all the answers

In massive transfusion, when is plasma transfusion used?

In patients with preexisting single coagulation factor deficiency Signup and view all the answers

What is the primary criterion for RBC transfusion in massive transfusion management?

$1.5 \times$ the mean PT value Signup and view all the answers

Which condition requires the administration of more than 3 RBC units within 1 hour or 8-10 units within 24 hours under massive transfusion management?

ACOTS Signup and view all the answers

When is Activated Prothrombin complex concentrate (PCC) ordered?

When there is a Factor VIII inhibitor Signup and view all the answers

Which of the following triggers Acute Coagulopathy of Trauma-Shock (ACOTS)?

$Hypothermia$ Signup and view all the answers

Which of the following is NOT a cause of decreased platelet production?

Heparin-Induced Thrombocytopenia Signup and view all the answers

Which of the following is a type of autonomous thrombocytosis (primary thrombocytosis)?

Essential Thrombocythemia Signup and view all the answers

What is the primary cause of Thrombotic Thrombocytopenic Purpura (TTP)?

Deficiency of ADAMTS13 enzyme Signup and view all the answers

Which of the following is a cause of reactive thrombocytosis (secondary thrombocytosis)?

Major surgery Signup and view all the answers

Which of the following is a genetic disorder associated with MYH9 gene mutations?

Fechtner syndrome Signup and view all the answers

What is the main characteristic of autonomous thrombocytosis (primary thrombocytosis)?

Marked increase in platelet count Signup and view all the answers

Which of the following conditions is characterized by skeletal defects such as long extremities and spider fingers?

Marfan's Syndrome Signup and view all the answers

In which condition are bruised areas commonly seen on the forearms of the elderly due to collagen degeneration?

Senile Purpura Signup and view all the answers

Which condition is most commonly seen in children and is characterized by gastrointestinal hemorrhage and joint swelling?

Henoch-Schonlein Purpura Signup and view all the answers

Which condition involves the adrenal glands and is characterized by severe capillary damage following meningococcal septicemia?

Waterhouse-Friedrichsen Syndrome Signup and view all the answers

Which condition is associated with tumors composed of vessels that commonly swell and bleed at the surface, leading to a localized consumptive coagulopathy and risk for disseminated intravascular coagulation (DIC)?

Kasabach-Merritt Syndrome Signup and view all the answers

Which condition is characterized by defects in the synthesis of collagen and hyaluronic acid due to a deficiency of ascorbic acid (vitamin C)?

Scurvy Signup and view all the answers

Which of the following is a characteristic of an acquired bleeding disorder?

Bleeding episodes begin after childhood Signup and view all the answers

What type of hemorrhage is characterized by systemic bleeding that appears as petechiae, purpura, or ecchymosis?

Mucocutaneous hemorrhage Signup and view all the answers

Which of the following is an indication of congenital hemorrhagic disorders?

Hemorrhage into joints, CNS, soft tissues & peritoneum Signup and view all the answers

Which type of hemorrhage is seen in acquired or congenital defects in secondary hemostasis or plasma coagulopathies?

Soft tissue hemorrhage Signup and view all the answers

What is the prevalence of congenital bleeding disorders?

Occurring in fewer than 1/100 people Signup and view all the answers

Which of the following is a potential cause of the bleeding disorders described in the text?

Single/multiple coagulation factor deficiencies Signup and view all the answers

Hemorrhage - Hemophilia - VWF

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