1/11. Recurrent spontaneous intracerebral hemorrhage in a congenitally afibrinogenemic patient: diagnostic pitfalls and therapeutic options.BACKGROUND: Coagulation disorders can cause intracerebral bleeding that may be difficult to detect since subsequent aberrant clot formation may mask early detection. This is an important pitfall because, when diagnosed early, bleeding in these patients is treatable. CASE DESCRIPTION: A patient with congenital afibrinogenemia presented with recurrent hemiparesis. Spontaneous intracerebral hemorrhage was diagnosed, despite an initial negative CT scan. diagnosis, therapy, and complications of therapy are discussed. CONCLUSIONS: Intracerebral hemorrhage must be strongly suspected in any patient with a coagulation disorder presenting with matching clinical symptoms. Therapy must be installed immediately, before additional investigations, and should be continued even when initial neuroimaging is negative.- - - - - - - - - - ranking = 1keywords = intracerebral, cerebral (Clic here for more details about this article) |
2/11. leg ulcer presenting in a patient with congenital afibrinogenaemia.Congenital afibrinogenaemia is a rare hemorrhagic disorder characterized by the absence of fibrinogen. We report a case of congenital afibrinogenaemia presented with leg ulcer. A 30-year-old man presented with a history of prolonged bleeding from birth. His parents are cousins. He repeatedly showed haematoma after traumas on his leg. He was diagnosed as having congenital afibrinogenaemia because of plasma fibrinogen deficiency. Because his leg ulcer gradually increased in size, he was admitted to our department for treatment. Laboratory examinations revealed prolonged bleeding time, prolonged coagulation time, prolonged prothrombin time, prolonged activated partial thromboplastin time and plasma fibrinogen was not measurable. Histological examination revealed hyperkeratosis, acanthosis and severe fibrotic change in the whole dermis. Severe hemosiderin deposit was found in the middle dermis. His leg ulcer cured 2 months after the beginning of fresh frozen plasma administration (FFP), but recurrence of the leg ulcer after FFP treatment was found. This is the second reported case of congenital afibrinogenaemia presented with leg ulcer.- - - - - - - - - - ranking = 0.060453118211239keywords = haematoma (Clic here for more details about this article) |
3/11. Unusually quick resorption of an intracerebral hemorrhage in congenital afibrinogenemia.neuroimaging diagnostics of cerebral hemorrhage in congenitally afibrinogenic patients may be compromised by different pitfalls. We describe the case of a 28-year-old patient with the diagnosis of congenital afibrinogenemia who suffered a large intracerebral hemorrhage. Initial cerebral computed tomography showed typical bleeding, but follow-up cerebral CT scans 1 and 2 weeks later revealed an unusually quick and subtotal resorption of hemorrhage with only a small hypodense lesion within the former bleeding area left. Imaging findings and differential diagnosis are discussed with respect to previous reports about intracerebral hemorrhage in afibrinogenemia.- - - - - - - - - - ranking = 1.021674690834keywords = intracerebral, cerebral (Clic here for more details about this article) |
4/11. Middle cerebral arterial thrombosis in a patient with hypofibrinogenemia, 5 days after rFVIIa and FFP infusion.A 13-year-old female patient is presented who had hypofibrinogenemia diagnosed as von Willebrand disease at 5 years of age at another hospital. She was admitted to the department of pediatric hematology with a severe headache, vomiting, and progressive right flaccid hemiplegia and lethargy. Contrast-enhanced computed tomography scan showed subdural hematoma in posterior parietal region of the brain and impending cerebellar herniation. She was given fresh-frozen plasma (FFP) and then activated factor vii (rFVIIa), 80 microg/kg was infused for replacement of von willebrand factor. The subdural hematoma was emergently drained. The results of coagulation tests before infusion of FFP and rFVIIa revealed hypofibrinogenemia, and FFP was given every 48 hours. The patient recovered dramatically in a few days. Five days after rFVIIa infusion, a magnetic resonance angiography-proven right middle cerebral arterial thrombosis developed. It is an interesting point of discussion whether the middle cerebral arterial thrombosis was provoked as a consequence of rFVIIa and FFP infusion.- - - - - - - - - - ranking = 0.065024072502091keywords = cerebral (Clic here for more details about this article) |
5/11. Congenital afibrinogenaemia.A case of congenital afibrinogenaemia in a young female child is described. She had haemorrhagic tendency since birth in the form of markedly prolonged umbilical bleeding and easy bruising afterwards. Two of her brothers had bleeding tendencies, one died shortly after birth due to uncontrollable umbilical bleeding and other died at the age of 12 years from internal haemorrhage. The family study indicates the mode of inheritance to be probably autosomal recessive. The principal laboratory findings are complete non-coagulability of blood, grossly abnormal coagulation tests, zero ESR value, failure to detect fibrinogen by heat coagulation or chemical precipitation tests and biuret reaction and correction of thrombin time after fibrinogen infusion.- - - - - - - - - - ranking = 0.90559272336714keywords = haemorrhage (Clic here for more details about this article) |
6/11. Fibrinogen Barcelona I. Congenital dysfibrinogenemia characterized by defective release of fibrinopeptide a and fibrinogen degradation products.A congenital dysfibrinogenemia, fibrinogen Barcelona I, was detected in a 28 year-old woman with no prior history of bleeding. The thrombin induced clotting of plasma and purified fibrinogen was much prolonged. Fibrin monomer aggregation was impaired. The abnormal fibrinogen polymerized in the presence of calcium and can be further cross-linked by factor xiiia. The turbidity of fibrin gels obtained from fibrinogen Barcelona was much lower than normal fibrinogen. The kinetic constant Km for fibrinogen Barcelona plus normal fibrinogen gelation was similar to normal fibrinogen gelation. The release rate of fibrinopeptide a by thrombin was slower than that of normal fibrinogen. However, two mol of fibrinopeptide a was released per mol of fibrinogen in 30 min. SDS-PAGE of abnormal and normal fibrinogens and of reduced fibrinogens showed identical patterns. Sialic acid content was markedly decreased in fibrinogen Barcelona. Plasmin digestion of two fibrinogens showed identical patterns in SDS-PAGE as regards X fragment formation. The kinetics of fibrinogen degradation showed a decrease in the formation rate of D and E fragments. The fact that the patient was in threat of abortion and developing a haemorrhagic syndrome may indicate that the defect in the fibrinogen was important in the pathogenesis of haemorrhage in this patient.- - - - - - - - - - ranking = 0.90559272336714keywords = haemorrhage (Clic here for more details about this article) |
7/11. Haemorrhagic diathesis as a possible early sign of hereditary fructose intolerance.An infant girl three weeks of age with the leading symptom of skin haemorrhages is presented. On further investigation, the signs of severe hepatic damage with hypofibrinogenaemia and prothrombin complex impairment, and renal tubular dysfunction were disclosed. All these pathological symptoms, which were reversed on fructose free diet, were caused by hereditary fructose intolerance.- - - - - - - - - - ranking = 0.90559272336714keywords = haemorrhage (Clic here for more details about this article) |
8/11. thrombocytopenia and hypofibrinogenemia in association with inflammatory bowel disease.There are many extra-intestinal manifestations of inflammatory bowel disease (IBD), some which are very uncommon in the pediatric age group. Coagulation abnormalities occur infrequently. Both hypercoagulability and hypocoagulability have been reported in adults (1). The hypercoagulable state is reflected in a general increase in concentration of clotting factors. This is observed most frequently in patients with poorly controlled intestinal disease and can lead to thromboembolic phenomena, including pulmonary and cerebral embolism. Conversely, patients with hypocoagulability may present with bleeding manifestations. We report a young patient with an abnormal coagulation profile as well as with thrombocytopenia in association with IBD.- - - - - - - - - - ranking = 0.010837345417015keywords = cerebral (Clic here for more details about this article) |
9/11. Chronic consumption coagulopathy due to an occult splenic haemangioma: kasabach-merritt syndrome.We report an 11-year-old girl with a 2-year history of bruising associated with thrombocytopenia and dysfibrinogenaemia. On admission she presented with a large subcutaneous haematoma and splenomegaly and was severely anaemic. Laboratory investigations revealed signs of consumption coagulopathy. Radiological examination showed splenic, retroperitoneal and intra-ossal haemangiomas. After splenectomy, platelet count and coagulation parameters returned to normal. CONCLUSION: Contrary to widely held views, occult visceral haemangioma can lead to kasabach-merritt syndrome beyond infancy and is not necessarily associated with visible cutaneous haemangioma. It should be included in the differential diagnosis of chronic thrombocytopenia at any age. Early determination of fibrinogen degradation product levels is advised in order to detect an underlying chronic consumption coagulopathy prompted by an extensive search for multifocal haemangioma.- - - - - - - - - - ranking = 0.060453118211239keywords = haematoma (Clic here for more details about this article) |
10/11. afibrinogenemia with intracerebral hematoma. Report of a successfully treated case.A patient with congenital afibrinogenemia underwent removal of an intracerebral hematoma following specific therapy with fibrinogen-containing concentrates. We believe the successful neurosurgical intervention in a life-threatening complication of this unusual coagulation disorder to be unique.- - - - - - - - - - ranking = 0.82430221215249keywords = intracerebral, cerebral (Clic here for more details about this article) |
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