Fatih AZIK, Aydın YAĞMURLU, Meral BARLAS, Uğur BEZİRGAN

Faktör XIII eksikliği

Faktör 13 eksikliği olan 15 yaşında erkek çocuk, iki gündür devam eden karın, sol omuz ağrısı ve kusma şikayetleri ile başvurdu.Herhangi bir travma öyküsü yoktu. Kan basıncı 65/25 mmHg, kalp tepe atımı:140/dk idi. Karında belirgin distansiyon ve hassasiyet vardı. Hemoglobin:6.3 g/dL, hematokrit:%21.8 idi. Hastanın Ultrasonografisinde dalakta 4x2 cm boyutlarında subkapsüler hematom ve karın içinde yaygın serbest sıvı bulundu. Hastaya 15 mL/ Kg eritrosit süspansiyonu, 20 mL/ Kg taze donmuş plazma tedavisi başlandı.Yaşamsal bulgular, idrar çıkışı ve hematokrit stabil olduğunda yapılan abdominal tomografide dalakta 7x6 cm boyutlarında hematom tespit edildi. Hastanın takibinde sorunla karşılaşılmadı. Solid organ yaralanması saptanan ve akut karın bulgularıyla gelen hastalarda nadir görülen bir kanama diyatezi olan Faktör XIII eksikliğinin de daima akılda tutulması gerekliliği nedeniyle olgunun sunulması uygun bulunmuştur.

Factor XIII deficiency

A fifteen yearold boy with Factor XIII deficiency had admitted to hospital for abdominal, left shoulder pain and vomiting for two days. There was no trauma history. His blood pressure was 65/25 mmHg and heart rate:140 beat/minute. He had abdominal distention and ten-dency.Hemoglobin and hematocrit levels were 6.3 g/dL and 21.8% respectively. Ultrasonography revealed a 4x2 cm subcapsular splenic hematoma and massive intraperi-toneal fluid collection.15 ml/day erythrocyte suspension and 20 ml/day fresh frozen plasma replacemant therapy was started. After stabilization of vital signs, urine output and hematocrit measures he had an abdominal CT scan.lt revealed a 7x6 cm splenic hematoma.The follow-up period was uneventful. Factor XIII deficiency is a rare coagulation defect as a cause of nontraumatic solid organ injury and acute abdomen.For to remind and emphasize this uncommon pathology as a source of acute abdomen and solid organ injury,the present case here in was reported.

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