Congenital afibrinogenemia: a case report of a spontaneous hepatic hematoma
نویسندگان
چکیده
INTRODUCTION Afibrinogenemia is a rare coagulation disorder. Clinical features of spontaneous bleeding, bleeding after minor trauma, or after surgery have been described as well as thrombo-embolic complications. In this article, we presented the case of a 19-year old female with congenital afibrinogenemia who was admitted with a spontaneous intrahepatic hematoma. CONCLUSIONS Supportive treatment including transfusion and fibrinogen administration, associated with repeated packing surgeries and selective embolization, were successfully performed.
منابع مشابه
پارگی خودبهخودی کبد در بارداری بهدنبال سندرم HELLP : گزارش موردی
Background: Spontaneous hepatic rupture is a rare condition during pregnancy. Pregnant women with Hemolysis, Elevated Liver enzymes and Low Platelete count (HELLP) syndrome are more susceptible to hepatic rupture. It can occur per 40000 to 250000 pregnancies with high mortality and morbidity. There is no agreement on the best approach to this severe pregnancy complication. This is the case repo...
متن کاملCongenital Hypofibrinogenemia: A Newborn Infant with Cord Bleeding.
The genetic disorders causing decreased fibrinogen synthesis may be caused by heterozygous (hypofibrinogenemia) or homozygous (afibrinogenemia) deficiency. The consequences of the disorders are gastrointestinal bleeding, cord bleeding, eccymoses, subcutaneous hematomas and hemarthroses especially due to traumatic delivery in the neonatal period. Laboratory evaluation of the patient with hypofib...
متن کاملCongenital Hepatic Fibrosis: An Uncommon Cause of Chronic Renal Failure
Congenital Hepatic Fibrosis (CHF) is a rare disease that affects both the liver and kidneys. Congenital hepatic fibrosis (CHF) is an autosomal recessive inherited malformation defined pathologically by a variable degree of periportal fibrosis and irregularly shaped proliferating bile ducts. Affected individuals also have impaired renal function, usually caused, in children and teenagers, by an...
متن کاملCongenital Afibrinogenemia presenting as antenatal intracranial bleed: a case report
Congenital afibrinogenemia is a very rare inherited coagulation disorder, characterized by virtual absence of plasma fibrinogen (factor I). There are only about 250 cases reported in the world literature 1. We describe a case of congenital afibrinogenemia which presented as an antenatally detected intracranial bleed.
متن کاملAn Unusual Case of a Large Hematorrachis Associated with Multi-Level Osteoporotic Vertebral Compression Fractures; a Case Report
Spinal epidural haemorrhage may present as back pain associated with radicular symptoms and can be a catastrophic clinical scenario with progression to paraplegia or even sudden death. Being a rare entity, it needs a high index ofclinical suspicion to diagnose it. Fractures have been documented as a cause of hematorrachis but such hematomas only extend to one or two vertebral segments. Large ep...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
دوره 95 شماره
صفحات -
تاریخ انتشار 2016