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 9-year-old black girl underwent right hepatectomy because of a large primary tumor in the right lobe of the liver. Histopathologic examination revealed the diagnosis of mesenchymal hamartoma, a rare benign tumor occurring mostly in children below the age of 2 years. Preoperatively, the patient had laboratory evidence of mild disseminated intravascular coagulation (DIC) but had no bleeding manifestations. In preparation for the surgery, the patient received whole blood exchange transfusion and platelet transfusion which re-resulted in marked improvement in hemostatic parameters. Despite what appeared to be adequate replacement of blood loss during the surgical procedure, the patient developed sudden cardiac arrest near the end of the procedure and died. The probable cause of death was hypovolemia. This case of mesenchymal hepatic hamartoma illustrates two unusual features: age of the patient and the association with DIC. The latter, to our knowledge, has not been reported before.
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PMID:Mesenchymal hamartoma of the liver in an older child: association with disseminated intravascular coagulation. 670 May 45

A spontaneous retroperitoneal haematoma is an uncommon cause of haemorrhagic shock. We report a case of spontaneous rupture of a renal angiomyolipoma resulting in haemorrhagic shock in a 52-year-old woman. The renal tumor was recognized by sonography and diagnosed by CT-scan. Renal angiography was performed, but embolization was not successful. During the surgical procedure, nephrectomy was required because of persistent bleeding, related to disseminated intravascular coagulation. Outcome was uneventful. Diagnosis and treatment of renal angiomyolipoma are discussed. The Lenk's triad, consisting of acute lumbar pain, symptoms of internal bleeding and lumbar tumefaction, is the usual clinical picture of retroperitoneal haemorrhage. The kidney is the most frequent cause and renal angiomyolipoma is the most frequent benign tumor. Renal angiomyolipoma is either isolated or associated with tuberous sclerosis in up to 20 per cent of patients. Diagnosis is suggested by sonography and confirmed by CT-scan. Renal angiography, performed in haemodynamically stable patients, shows the origin of bleeding and allows embolization. Considering the frequent bilaterality of angiomyolipoma, surgery should be as conservative as possible in order to preserve renal function.
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PMID:[Spontaneous retroperitoneal hematoma: a rare cause of hemorrhagic shock]. 766 26

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

Hemangioma is the most common benign tumor of the liver and it is often asymptomatic. Spontaneous or traumatic rupture, intratumoral bleeding, consumption coagulopathy, and rapid growth are mandatory surgical indications. We report a case of giant hemangioma of hepatic segments II and III, which presented as hemoperitoneum, and were treated successfully with preoperative transcatheter arterial embolization (TAE) and hepatic bisegmentectomy. A PubMed Medline search has identified up to now 32 cases of spontaneous rupture of hepatic hemangioma in adults (age >14 years) without a history of trauma, including the present case. Twenty-seven out of these were reviewed. Sixteen (84.2%) of 19 tumors of known size were giant hemangiomas (mean diameter 14.8 cm; range 6-25). Twenty-two (95.7%) patients underwent surgery. Thirteen patients (59.1%) had a resection, 5 (22.8%) were sutured, and 4 (18.1%) underwent tamponade. Three (23%) out of the 13 resected patients died. Four patients (30.8%) underwent TAE prior to elective hepatic resection without any operative mortality. Among the 5 sutured patients, 2 (40%) died as well as 3 (75%) out of 4 patients who underwent tamponade. The mortality rate of all surgery patients was 36.4% (8/22).
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PMID:Hemoperitoneum from a spontaneous rupture of a giant hemangioma of the liver: report of a case. 1276 74

Tumor-induced osteomalacia (TIO) is a rare acquired form of hypophosphatemia commonly associated with phosphaturic mesenchymal tumors (PMTs) located in the bone or soft tissue. Resection of the tumor can cure osteomalacia. Fibroblast growth factor 23 has been identified as a major pathophysiological factor responsible for phosphaturia. The majority of PMTs are benign, and malignant PMTs are uncommon. Even in rare cases, the malignant transformation of PMTs is extremely uncommon. The current study presents two cases in which the patients succumbed to malignant PMTs that developed in the pelvis. The first patient was a 35-year-old female with a malignant PMT occurring as a synchronous double cancer associated with papillary thyroid carcinoma. Diagnosis was difficult, as the multiple uptake on positron emission tomography with 18F-fluorodeoxyglucose presented as pseudofractures mimicking the metastases of thyroid carcinoma. The patient succumbed to rapidly progressive lung metastases. The second patient presented with a pelvic tumor that had developed over 26 years. The patient was diagnosed with a benign PMT by open biopsy and a complete resection was performed. However, two years later, the tumor recurred and lung metastases were observed. The patient ultimately succumbed to respiratory failure due to relapsing lung metastases and disseminated intravascular coagulation. These two cases demonstrate the potential lethality of malignant PMTs and the malignant transformation of benign PMTs. Therefore, TIO patients must be followed up even if diagnosed with a benign tumor. Although TIO is an extremely rare disease, the possibility of malignant PMTs must be recognized.
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PMID:Malignant phosphaturic mesenchymal tumor of the pelvis: A report of two cases. 2495 20