Abstrakt: |
Introduction: F13 deficiency is an inherited coagulation disorder with an estimated frequency of 1-3:1000000. F13, the last factor in the coagulation cascade, plays a vital role in clot stabilization by cross-linking fibrin dimers. It also has a great importance in wound healing and pregnancy by stimulating angiogenesis. Symptoms of F13 deficiency include umbilical cord bleeding, deep tissue bleeding, impaired wound healing and recurrent miscarriages. As PT, aPTT, TT remain unchanged, clot solubility test, F13 activity test and F13 antigen test can be used in the diagnosis. FFP and cryoprecipitate are used in treatment. Case: A 46-year-old woman with history of F13 deficiency, epilepsy and two intracranial and three abdominal hemorrhages presented to the ER with non-traumatic headache, nausea and vomiting for 3 days. Upon the CT findings of 3x3.5 cm intracranial hemorrhage forming a 7 mm shift in the right frontal lobe, the patient was transferred to the ICU for follow-up and treatment. The patient was conscious, coherent and oriented, pupils were NIC, IR +/+, and there was no sensory-motor deficit. Vitals were stable. Coagulation parameters and platelet count was within the normal range. FFP treatment was initiated at 15 cc/kg. Furosemide, mannitol and dexamethasone used for anti-edema therapy. The patient did not develop any symptoms during the follow-up period. The patient received antiedema and TDP treatment for a total of 7 days. Brain CT scan on the 8th day of admission showed significant regression in the bleeding. On the 10th day of follow-up, the patient was transferred to the neurology inpatient clinic with normal neurological status and vitals. Discussion: F13 deficiency is a rare disorder that can lead to serious complications. It should be kept in mind in patients with bleeding findings and symptoms such as delayed wound healing and recurrent miscarriage without a history of trauma or abnormal coagulation tests. [ABSTRACT FROM AUTHOR] |