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)

A group of values were prospectively analyzed in 24 infants under 3 months, of age, who showed over 3% fragmented RBC's with no history of blood transfusions. Results were compared with those obtained in group of 26 infants of the same age and less than 1% fragmented RBC's. These infants with over 3% fragmented cells were found to have a significant association with: sepsis, jaundice, crenated RBC's, low levels of hemoglobin, increased reticulocyte count, and low vitamin E levels. No relationship was found with weight at birth, feeding history and disseminated intravascular coagulation. No cases of hemangioma or cardiac diseases were found. These findings are commented.
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PMID:[Jaundice caused by microangiopathic hemolysis associated to septicemia in the newborn]. 739 25

Five cases of hepatic haemangioma are described, and a sixth (previously reported) is reviewed. Clinical features, investigation, and management are described to show the great variability of the complications and prognosis. Five children presented in the first 10 weeks of life with hepatomegaly; 4 developed congestive cardiac failure; 3 had cutaneous haemangiomata. One child presented at age 4 years with hepatomegaly and anaemia, and on investigation had features of chronic disseminated intravascular coagulation. Focal decrease or patchiness in hepatic uptake of technetium-99m colloid, and abnormal intrahepatic circulation was shown in all cases. In 3 children liver biopsy was performed to exclude malignant disease. In one patient there was spontaneous regression of the tumour by age 3 years. In 3 cases hepatic artery ligation was necessary to control congestive cardiac failure which had persisted despite treatment with digoxin, diuretics, and oral corticosteroids, a procedure which was without complications after up to 8 years. One infant with intractable portal hypertension, hepatic vein obstruction, and severe cholestasis died with persisting alimentary haemorrhage and intra-abdominal sepsis. One child aged 4 years showed no immediate response to hepatic artery ligation but the size of her tumour got smaller and the clinical features diminished after irradiation. These tumours cause considerable morbidity and have a high reported mortality. If congestive cardiac failure is not rapidly controlled, hepatic artery ligation should be performed.
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PMID:Hepatic haemangiomata: diagnosis and management. 746 56

A 20 year-old female patient with Kasabach-Merrit syndrome, suffered from chronic consumption coagulopathy due to localized intravascular coagulation in the tumors. She had been diagnosed as Kasabach-Merrit syndrome immediately after birth and below knee amputation of her right lower leg was performed at the age of 2 years because of her giant hemangioma on the right foot and lower leg. After the operation, she had often complained of severe pain and enlargement of the residual tumors due to continuous thrombus formation within the tumors. She was admitted to the third Department of Tohoku University Hospital in order to initiate oral anticoagulant therapy with Warfarin at the age of 12 years. After the administration of 2.5 mg/day Warfarin, she has maintained good clinical condition until now, despite the occasional occurrence of coagulation abnormalities. We believe that the results of this case indicate the efficacy of oral anticoagulant therapy in the treatment of chronic consumption coagulopathies complicated with other diseases.
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PMID:[A case of Kasabach-Merrit syndrome complicated with DIC treated effectively by long term oral administration of warfarin]. 778 22

We describe the case of a 42-year-old woman with giant cavernous hemangioma and Kasabach-Merritt syndrome. The patient presented with consumption coagulopathy due to intravascular, intratumoral coagulation as revealed by low platelet levels, fibrinogenopenia and an increase in fibrinolysis with high levels of fibrinogen degradation products. She was scheduled to receive an orthotopic liver transplant because of three factors: respiratory distress caused by compression of the diaphragm by the giant tumor; the risk of bleeding caused by spontaneous rupture or trauma; and the presence of Kasabach-Merritt syndrome due to consumption coagulopathy. Before surgery fibrinogen deficit was corrected with 4 units of cryoprecipitates and low platelet level was treated with 10 units of platelets. Coagulopathy during surgery was corrected with fresh plasma (17 units), cryoprecipitates (6 U), aprotinin (1 x 10(6) U/kg) and antithrombin 3 (2000 U). Blood loss was compensated for with 9 units of packed red blood cells. This report describes the procedures used for anesthesia, for prevention of accidental bleeding during surgery, hemodynamic control and preoperative coagulation testing.
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PMID:[Orthotopic liver transplant for giant cavernous hemangioma and Kasabach-Merritt syndrome]. 789 56

Kasabach-Merritt syndrome (thrombocytopenia, consumption coagulopathy and occasional hemolysis) is an infrequent but often fatal complication of rapidly growing hemangiomas in infants. We describe a 1-month-old infant with a huge hemangioma involving the left submandibular region associated with a severe consumptive coagulopathy, who was successfully treated with transfusion of blood products, prednisone and radiation therapy. It is stressed that pediatric otorhinolaryngologists should always be aware of the lethal status of this condition in infants.
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PMID:Submandibular hemangioma as the initial manifestation of Kasabach-Merritt syndrome. 843 76

A 4-month-old boy with benign hemangioma of the porta hepatis is described. Obstructive jaundice and consumption coagulopathy developed, which were treated by percutaneous transhepatic drainage (PTHD), without resection of the tumor or bypass surgery. Because of tumor regression, the patient has remained free of symptoms even after the PTHD tube was removed. Because juvenile hemangioma is a benign tumor and occasional spontaneous regression is known to occur (as in our case and other reports), it is suggested that complete resection or bypass surgery is not necessary for juvenile hemangioendothelioma, even with obstructive jaundice, if bile drainage is adequately maintained.
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PMID:Successful management of infantile hepatic hilar hemangioendothelioma with obstructive jaundice and consumption coagulopathy. 878 96

Kasabach-Merritt syndrome is characterized by thrombocytopenia and bleeding tendency leading to disseminated intravascular coagulation with giant hemangiomas. We present a very low birth weight infant with this syndrome who underwent four operations. A male baby (1179 g, 37 cm) was born at a gestational age of 28 weeks and 6 days by caesarean section. A large hemangioma, 7 x 8 cm in size, was recognized on the left thigh. As associated consumption coagulopathy (Kasabach-Merritt syndrome) was diagnosed with platelet count 5.1 +/- 10(4) mm-3 and fibrinogen 49 mg.dl-1. Despite treatment with liniac X-ray radiation, systemic steroid and component transfusion, coagulopathy became worse with extremely low platelet count of 1.1 x 10(4) mm-3. Infusion of dopamine and dobutamine was necessary for high output cardiac failure. On day 9, PDA ligation was performed. Cerebro-ventricular drainage, ventricuro-peritoneal shunt and shunt revision were required on day 15, 49 and 88, respectively, for hydrocephalus due to intraventricular hemorrhage. Main anesthetics used were fentanyl and sevoflurane. Major problems encountered by anesthetists were: bleeding tendency, water and electrolyte management, body temperature control, and immaturity and fragility of premature infant. Coagulopathy in Kasabach-Merritt syndrome must be a risk factor for intraventricular hemorrhage, which is a characteristic complication of a very low birth weight infant.
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PMID:[Anesthetic problems in a very low birth weight infant with Kasabach-Merritt syndrome]. 881 2

We describe a 40-yr-old woman with a giant hepatic hemangioma and consumption coagulopathy who underwent transcatheter arterial embolization (TAE) prior to liver resection. Post-contrast computed tomography showed a large mass in the right hepatic lobe with a peripheral, nodular enhancement pattern that enlarged on delayed-phase images. There was a low fibrinogen concentration and decreased platelet count. The patient received i.v. nafamostat mesilate and underwent selective embolization of the arteries feeding the hemangioma; consequently, the plasma fibrinogen concentration increased to 1.6-fold before surgery. Right hepatic lobectomy with partial resection of the caudate lobe was performed. The intraoperative blood loss was only 1380 g. This patient illustrates the usefulness of preoperative TAE for hepatic hemangioma with consumption coagulopathy; TAE appears to improve coagulopathy and increase the safety of surgery.
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PMID:Preoperative transcatheter arterial embolization for giant cavernous hemangioma of the liver with consumption coagulopathy. 912 26

A case of a giant cavernous hemangioma of the liver is presented. Selective angiograms revealed secondary portal hypertension, probably caused by a temporal obstruction of the extra-hepatic portal vein due to the physical pressure of the tumor, and a marked dislocation of the hepatic artery. The patient also had consumption coagulopathy. Both the portal obstruction and hematological disorders improved after excision of the tumor. Since surgery the patient has been in good health with no complaints or recurrence.
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PMID:A giant hepatic hemangioma with secondary portal hypertension: a case report of successful surgical treatment. 926 28

We report an unusual case of Kasabach-Merritt syndrome in a 62 year-old woman with bladder tumor who suffered from acute consumption coagulopathy that increased the fibrinolytic activity due to the presence of a huge hemangioma in the upper and lower extremities. Two days after the transurethral resection of the solitary bladder tumor, serious hematuria and life-threatening disseminated intravascular coagulation developed. Following treatments with heparin and subsequent steroid treatment, hemorrhage and abnormal hemostatic values improved. It is suggested that extensive investigation of the coagulation system should be done in patients with giant hemangioma, particularly before a surgical procedure.
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PMID:Acute onset of coagulopathy in a patient with Kasabach-Merritt syndrome following transurethral resection of bladder tumor. 987 52


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