A 15-year-old female presented to the emergency depament with history of recurrent epistaxis, hematuria and hematochezia. There was a history of profuse bleeding from the umbilical stump at bih. Previous investigations revealed normal prothrombin time, activated paial thromboplastin time, thrombin time and fibrinogen levels. Her platelet counts as well as platelet function tests were normal but urea clot lysis test was positive. Which one of the following clotting factor is most likely to be deficient?

## **Core Concept**
The patient's symptoms suggest a bleeding disorder characterized by mucocutaneous bleeding (epistaxis, hematuria, hematochezia) and a history of bleeding from the umbilical stump. The normal prothrombin time (PT), activated partial thromboplastin time (aPTT), thrombin time, and fibrinogen levels, along with normal platelet counts and function, point towards a specific coagulation factor deficiency.

## **Why the Correct Answer is Right**
The key to this question lies in the patient's clinical presentation and the laboratory findings. The positive urea clot lysis test indicates a problem with fibrinolysis or clot stability, rather than coagulation per se. The normal PT, aPTT, and thrombin time suggest that the common coagulation pathway is intact. Given the symptoms and the positive urea clot lysis test, the most likely deficiency is in **Factor XIII**, also known as fibrin stabilizing factor. Factor XIII deficiency leads to the formation of unstable clots that are prone to fibrinolysis, resulting in recurrent bleeding episodes. The urea clot lysis test is particularly useful in diagnosing Factor XIII deficiency because it directly assesses clot stability.

## **Why Each Wrong Option is Incorrect**
- **Option A (Factor I - Fibrinogen):** Deficiency in fibrinogen would lead to abnormalities in PT, aPTT, and thrombin time, which are all normal in this patient.
- **Option B (Factor II - Prothrombin):** A deficiency in prothrombin would affect PT and aPTT, which are normal here.
- **Option C (Factor XII):** While Factor XII deficiency can prolong aPTT, it is not typically associated with a bleeding phenotype and would not explain the positive urea clot lysis test.

## **Clinical Pearl / High-Yield Fact**
A critical point to remember is that **Factor XIII deficiency** often presents with a history of umbilical stump bleeding at birth and later with mucocutaneous bleeding, and it is diagnosed with a urea clot lysis test or specific Factor XIII assays. This condition is a rare cause of bleeding disorders but is crucial to identify due to its distinct management.

## **Correct Answer:** D. Factor XIII.

✓ Correct Answer: D. Factor Xlll