Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0012739 (disseminated intravascular coagulation)
 8,673 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Kasabach-Merritt syndrome comprises consumption coagulation associated with giant haemangioma. A case is reported of disseminated intravascular coagulation complicating dental extraction in a 36-year-old male with this syndrome. The pre-operative management with anticoagulant therapy is discussed and the relevant literature reviewed.
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PMID:The management of patients with the Kasabach-Merritt syndrome. 108 7

Three patients with cutaneous and/or synovial hemangiomas demonstrated roentgenographic alterations in the knees, simulating hemophilic arthropathy. In 2 cases, unilateral synovial hemangiomas appeared to be present; in the third patient, who had bilateral abnormalities, the Kasabach-Merritt syndrome was evident, resulting in a consumption coagulopathy and hemorrhagic tendency. The pathogenesis of this unusual arthropathy is discussed.
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PMID:Hemophilia-like arthropathy of the knee associated with cutaneous and synovial hemangiomas. Report of 3 cases and review of the literature. 111 Sep 96

A case of giant hemangiolymphangioma of abdominal cavity is reported. It showed severe bleeding from consumption coagulopathy and secondary fibrinolysis. (Kasabach-Merritt syndrome). A review is made of the production mechanism of coagulation abnormalities that may accompany vascular neoplasias, the usefulness of anticoagulants and the selective treatment in this type of disease.
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PMID:[Hemangiolymphangioma of the abdominal cavity complicated with consumption coagulopathy]. 116 52

An infant with a large occipital hemangioendothelioma with thrombocytopenia, anemia, and hypofibrinogenemia--Kasabach-Merritt syndrome--was reported. The case, a male neonate is unique, for this is the first report with this syndrome in whom the large hemangioma was noted at birth on the midocciput simulating the occipital encephalomeningocele. With the development of thrombocytopenia of 84,000 per mm3, hypofibrinogenemia of 92 mg/dl, anemia (erythrocyte 193 X 10(4) per mm3, hemoglobin 5.9 g/dl, hematocrit 16 vol%), hepato-splenomegalia, enlargement and bluish discoloration of the tumor noted on the 21/2 months of life, total excision was intended prior to the expected occurence of the systemic purpura. The patient received fresh whole blood transfusion immediately prior to surgery, and the total excision was successfully performed. Excessive bleeding was not encountered. Abrupt rise in the platelet count, red blood cell count, hemoglobin and hematocrit to normal range was noted at the first postoperative day; he was discharged on the 17th day after surgery. Nineteen months' follow-up showed normal hematologic findings with good somatic and mental development. The specimen weighing 250 g. revealed benigh hemangioendothelioma. Silver impregnation demonstrated lobular aggregates of small vascular channels. Papillary projection of interstitial cells into the lumen, reaction of the endothelium of the vessels, newly formed thrombus, ishemic necrosis and hemorrhage, hyaline degeneration of interstitial tissue were noted. These findings suggested the disseminated intravascular coagulation within the tumor followed by fibrinolysis accounts for loss of blood corpuscles, platelet, fibrinogen and clotting factors, which leads ultimately to the consumption coagulopathy and diffuse bleeding.
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PMID:[Giant occipital hemangioendothelioma with thrombocytopenia, anemia and hypofibrinogenemia treated by total excision (author's transl)]. 123 5

The Kasabach-Merritt syndrome consists of hemangioma in association with thrombocytopenia and localized consumption coagulopathy. It is primarily a disease of childhood, but may be seen in adult patients too. When untreated, it involves considerable risk of mortality. Two adult patients are described. The disease can be cured by surgery. We discuss different options for treatment when surgery is not feasible.
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PMID:[Kasabach-Merritt syndrome. A differential diagnosis in thrombocytopenia]. 141 62

Kasabach-Merritt syndrome is a combination of thrombocytopenia, intravascular coagulation, and a rapid increase in the size of an angioma. Anemia and disseminated intravascular coagulation may develop. This infrequent syndrome is severe and may be life-threatening. Pathophysiologic mechanisms underlying the condition are incompletely understood and, consequently, many different treatments are used, including systemic corticosteroids, compression, embolization, antifibrinolytic agents, platelet aggregation inhibitors, irradiation, and others. From findings in eight personal cases, the authors review clinical and biological features, pathophysiologic hypotheses and therapeutic strategies.
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PMID:[Kasabach-Merritt syndrome in children]. 141 65

A 6 week old infant presented with a giant angiomatous naevus of the leg complicated by chronic disseminated intravascular coagulation. The bleeding and laboratory abnormalities responded to intermittent pneumatic compression of the affected limb. This innocuous treatment should be considered in the Kasabach-Merritt syndrome.
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PMID:The Kasabach-Merritt syndrome: treatment with intermittent pneumatic compression. 238 88

A 6-week-old girl with a hemangioma with thrombocytopenia, consumption coagulopathy, extensive bleeding, disappearance of the right pubic bone, and destruction of part of the right iliac bone was successfully treated with prednisone. Treatment lasted 30 weeks. Bleeding ceased within a few days; bone rebuilding was first observed at 30 days and was extensive at the end of treatment. When the child was 3 years old, pelvic films were normal. The occurrence of Kasabach-Merritt syndrome associated with complete osteolysis as in our patient is rare and bone reconstruction is unusual.
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PMID:Giant hemangioma with thrombocytopenia and osteolysis successfully treated with prednisone. 273 30

Kasabach-Merritt syndrome (thrombocytopenia, consumption coagulopathy, and, occasionally, microangiopathic hemolysis) is an infrequent but often lethal complication of giant capillary hemangiomas in the infant. We report the clinical course of an infant who came to us with this syndrome shortly after birth. She was successfully treated with transfusions of blood products, high-dose oral prednisone, and a fibrinolytic inhibitor, tranexamic acid. No complications of the treatment occurred. The hematological abnormalities resolved completely by 2 years of age. Although prednisone may have been necessary for stabilization and involution of the hemangioma, the addition of tranexamic acid allowed earlier tapering of the steroid therapy and fewer transfusions. Tranexamic acid was extremely well tolerated in this newborn.
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PMID:Tranexamic acid in the treatment of Kasabach-Merritt syndrome in infants. 275 Oct 74

We reported a female with vascular dementia due to chronic disseminated intravascular coagulation (DIC). The patient underwent operation for skin hemangiomas three times at age 18, 27, and 38. At age 43, she presented a slowly progressive dementia. She had bluish, compressive hemangiomas of skin and upper gastrointestinal tract, and she was suspected to have hemangiomas of brain, pleura, mediastinum, liver and pancreas. The hemangiomas were diagnosed blue-rubber-bleb-nevus syndrome (BRBNS). Laboratory data revealed persistent increase of serum FDP, FDP-D, FDP-E, and D-dimer. This condition was considered chronic DIC, therefore this case was the first case of combination of BRBNS and Kasabach-Merritt syndrome (chronic DIC). Brain MRI demonstrated bilateral lesions of paraventricular deep white matter and hemangioma of left temporal lobe. Because cerebral CT showed no contrast enhancement in paraventricular lesions, the lesions were considered multiple cerebral infarctions. Central nervous system (CNS)involvement in BRBNS is rare, and most cases of CNS involvement were associated with CNS angiomata. In this case it was suggested that brain was the target organ of DIC because thrombomodulin is absent or few in the human brain, therefore vascular dementia (multiple cerebral infarctions) developed due to chronic DIC.
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PMID:[Blue-rubber-bleb-nevus syndrome presented vascular dementia and chronic DIC--a case report]. 275 49


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