Cases reported "Syndrome"

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1/289. argon laser photocoagulation in ocular histoplasmosis syndrome.

    argon laser photocoagulation was performed on 30 patients with ocular histoplasmosis syndrome involving the macula. Selection of patients for photocoagulation was dependent upon locating the sub-retinal neovascularization (SRNV) at least one vein-width removed from the capillary-free zone of the fovea on fluorescein angiography. Of the 30 treated patients, 27 maintained or improved visual acuity an average of 1 1/2 years following photocoagulation.
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ranking = 1
keywords = coagulation
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2/289. Anaemia, thrombocytopenia and coagulopathy due to occult diffuse infantile haemangiomatosis of spleen and pancreas.

    Diffuse infantile haemangiomatosis of the spleen is a very rare lesion. Large haemangiomas may cause trapping of platelets and coagulation disorders known as Kasabach-Merrit syndrome. We here report the case of an infant with splenic and pancreatic haemangiomatosis presenting with life-threatening thrombocytopenia, anaemia and intravascular coagulation. diagnosis was hampered by reactive erythroblastosis and non-conclusive radiological findings. While treatment with corticosteroids was ineffective, administration of antithrombin iii improved coagulation parameters. After splenectomy the child recovered promptly and has remained free of disease for 3 years to date. CONCLUSION: Occult visceral haemangiomatosis without visible cutaneous haemangiomas should be included in the differential diagnosis of thrombocytopenia, anaemia and consumption coagulopathy. antithrombin iii treatment may be considered to overcome bleeding problems in patients with Kasabach-Merrit syndrome.
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ranking = 75.187176179165
keywords = consumption coagulopathy, intravascular coagulation, coagulopathy, consumption, coagulation, intravascular
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3/289. Trousseau's syndrome with brachiocephalic vein thrombosis in a patient with uterine carcinosarcoma. A case report.

    The authors treated a patient with the previously unreported occurrence of brachiocephalic vein and superior vena cava thrombosis in association with a distantly located cancer. A 71-year-old woman presented with swelling over the right side of the neck and abdominal distension. physical examination revealed a huge mass, and computed tomography demonstrated thrombosis of the brachiocephalic vein and superior vena cava accompanied by jugular vein dilatation. No coagulation disorder was demonstrable. After anticoagulation and thrombolysis, hysterectomy was performed; microscopic examination of the specimen revealed uterine carcinosarcoma. Even though local tumor obstruction is a much more common cause of neck vein thrombosis, a distant occult cancer can present as this form of Trousseau's syndrome. In patients with otherwise unexplained neck vein thrombosis, examination not only of the head and neck but also of the abdomen and pelvis should be pursued.
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ranking = 0.28571428571429
keywords = coagulation
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4/289. Paget-Schroetter syndrome associated with FV:Q506 and prothrombin 20210A--a case report.

    Effort thrombosis of the axillary-subclavian vein (Paget-Schroetter syndrome) develops usually secondary to heavy arm exertion. An underlying chronic venous compressive anomaly at the thoracic outlet or intimal damage of the axillary vein following forceful hyperabduction, external rotation of the shoulder joint has been proposed to explain the pathophysiology of this thrombosis. This condition is usually not attributed to an underlying hypercoagulability such as deficiency of natural coagulation inhibitors. Here, the authors present a case with thrombosis of the axillary-subclavian vein following an effort, with factor V Leiden and prothrombin 20210A mutations. Both factor V Leiden and the genetic variant in the prothrombin gene have been shown to confer an increased risk for venous thrombosis. Although rare, effort thrombosis may develop in a patient with hereditary thrombophilia, so laboratory evaluation should include the common causes of thrombosis.
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ranking = 0.14285714285714
keywords = coagulation
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5/289. A case report: pregnancy complicated by blue rubber-bleb nevus syndrome.

    Blue rubber-bleb nevus (BRBN) syndrome, first reported in 1958 by Bean, manifests with multiple hemangiomas located in the skin and gastarointestinal tract. Characteristic laboratory data include chronic anemia with iron deficiency and consumption coagulopathy. We describe herein a pregnancy complicated by BRBN syndrome resulting in the delivery of a male infant by cesarean section.
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ranking = 71.431600129857
keywords = consumption coagulopathy, coagulopathy, consumption
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6/289. may-thurner syndrome in an adolescent: persistence despite operative management.

    We describe a patient with may-thurner syndrome who underwent operative transection and transposition of the right common iliac artery without direct venous repair, because preoperative and intraoperative intravascular ultrasound scans were negative for "spurs" in the left common iliac vein. When symptoms and signs persisted, a postoperative magnetic resonance venogram (MRV) showed severe stenosis in the left common iliac vein. Progressive, but incomplete, clinical improvement occurred with conservative management.
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ranking = 0.097336847166257
keywords = intravascular
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7/289. Isolated diffuse hemangiomatosis of the spleen with Kasabach-Merritt-like syndrome.

    AIMS: Diffuse haemangiomatosis of the spleen is a rare benign vascular condition occurring as a manifestation of systemic angiomatosis or, less commonly, confined to the spleen. It is sometimes accompanied by severe disturbance of blood coagulation. The goal of this study was to characterize an additional case of isolated diffuse haemangiomatosis of the spleen and to determine the histogenesis of this lesion which remains obscure. methods AND RESULTS: We describe a case of isolated diffuse haemangiomatosis of the spleen in which histological and immunohistological findings suggested the possibility of a malformative tumour-like lesion. The pathological cavernous vessels were distributed randomly through the red pulp, without continuity with sinuses. The endothelial cells expressed vimentin, factor viii related antigen and CD34, but not CD8. Some cells lining the sinus lumen expressed CD68, lysozyme and myeloperoxidase. In addition, trabecular veins presented with intimal thickening. These results allow making a diagnosis between diffuse haemangiomatosis and other tumours/tumour-like lesions of the spleen, especially littoral cell angioma, splenoma and peliosis. CONCLUSION: If diffuse haemangiomatosis is usually classified as a benign proliferation of endothelial cells, we suggest that diffuse haemangiomatosis, when confined to the spleen, could be a tumour-like vascular lesion. In this hypothesis, the aetiology may be hamartomatous or malformative as is suspected in arterio-venous haemangioma of the lower extremities. The histogenesis is still questionable and no definitive proof in favour of one or the other hypothesis has been reported.
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ranking = 0.14285714285714
keywords = coagulation
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8/289. Successful transcutaneous arterial embolization of a giant hemangioma associated with high-output cardiac failure and kasabach-merritt syndrome in a neonate: a case report.

    We describe the case of a patient with a neonatal giant cutaneous hemangioma with high-output cardiac failure and kasabach-merritt syndrome and successfully treated with transcutaneous arterial embolization aimed at controlling severe congestive heart failure and consumption coagulopathy. A patient was admitted to the neonatal care unit on the first day of age because of a large hemangioma on his right lateral chest wall and respiratory distress, associated with cardiac failure resulting from arteriovenous shunting. On the second day of age the platelet count decreased to 5.7 x 10(4)/microliter and fibrinogen level was 85 mg/dl. The values of prothrombin time and activated partial thromboplastin time were prolonged. Intravenous predonisone therapy was started immediately, but bleeding tendency was getting worse and the evidence of congestive heart failure persisted. On the third day the patient then underwent embolization of feeding arteries with microcoils. The cardiac failure and thrombocytopenic coagulopathy had improved significantly without complications. We conclude that transcutaneous arterial embolization is an effective and safe treatment in this neonate and should be considered for the treatment of control high-output cardiac failure and coagulopathy in infants with hemangioma and kasabach-merritt syndrome.
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ranking = 72.691754942591
keywords = consumption coagulopathy, coagulopathy, consumption
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9/289. Capillary hemangioma of the liver with kasabach-merritt syndrome in a neonate: report of a case.

    Hepatic hemangiomas are benign tumors, and therefore minimally invasive treatment such as irradiation or steroid therapy is often recommended. However, in patients who have a hemangioma complicated by kasabach-merritt syndrome, surgical intervention should also be considered because of its confirmative therapeutic effect. We present herein the case of a 32-day-old male infant in whom a huge hepatic hemangioma associated with kasabach-merritt syndrome was treated by surgical intervention together with strong antidisseminated intravascular coagulation therapy.
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ranking = 0.94955213812658
keywords = intravascular coagulation, coagulation, intravascular
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10/289. kasabach-merritt syndrome with terminal gram negative infection.

    A case of giant haemangioma with disseminated intravascular coagulation (Kasabach-Merritt) syndrome) is presented. death occurred despite therapy and postmortem evidence of clinically unrecognised gram negative bacteraemia was seen.
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ranking = 0.94955213812658
keywords = intravascular coagulation, coagulation, intravascular
(Clic here for more details about this article)
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