Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.4.21.6 (thromboplastin)
 13,278 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acquired factor X deficiency has been described in patients with amyloidosis but acquired factor V deficiency is quite rare. We report here a case of life-threatening bleeding and acquired factor V deficiency associated with primary amyloidosis. A 50-year-old man who had no previous hemorrhagic diathesis was referred to our hospital because of recurrent epistaxis, gingival bleeding and hemospermia. The laboratory examination revealed that both the prothrombin time (PT) and the activated partial thromboplastin time (aPTT) were significantly prolonged, and factor V activities were markedly decreased to 14-39% of the normal value. Other coagulation factors such as fibrinogen, prothrombin, factor VII, factor VIII, factor IX and factor X were subnormal and normal. Transaminases were slightly elevated but serological tests of hepatitis B and hepatitis C were negative. Mild hepatosplenomegaly was noted without sign of liver cirrhosis. The PT and aPTT obtained 8 years ago when he received a cholecystectomy due to cholecystitis were both normal. Specific assays for the detection of factor V inhibitor were repeatedly performed but no factor V inhibitor was found. Furthermore, a significant recovery of the infused factor V was noted shortly after an intravenous administration of 5-10 U fresh frozen plasma, but it did not last more than 6 h. Melena, bleedings into the left shoulder and buttock, and finally mortal retroperitoneal hemorrhage developed despite repeated infusions of large amounts of fresh frozen plasma. Acquired factor V deficiency associated with primary amyloidosis was suspected but histological diagnosis was not obtained because of the severe bleeding tendency. Autopsy revealed hepatosplenomegaly and massive deposits of AL amyloid in the liver, spleen, heart and other parenchymal organs. Perivascular amyloid deposition and factor V deficiency are both thought to be the cause of the severe hemorrhagic tendency seen in this patient.
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PMID:Life-threatening bleeding and acquired factor V deficiency associated with primary systemic amyloidosis. 1219 8

Percutaneous ultrasound-guided cholecystocentesis was performed on 13 healthy beagle dogs to determine whether percutaneous ultrasound-guided cholecystocentesis in the dog was a feasible and safe procedure. Clinical, laboratory and ultrasonographic examinations were done at 0 and 10 minutes, in the 2nd and 16th hour, and on the 7th day. They included a detailed physical examination of the mucous membranes, cardiorespiratory system and abdominal organs. Laboratory examinations of the blood consisted of a complete blood count, determination of packed cell volume (PCV), haemoglobin (Hb), total plasma protein (TPP), parameters of haemostasis including prothrombin time (PT), activated partial thromboplastin time (APTT), and enzyme activities reflecting hepatobiliary function, i.e. aspartate aminotransferase (AST), alanine aminotransferase (ALT), and gamma-glutamyltransferase (GGT). Ultrasonographic findings of the gallbladder (size, shape, wall, content) and appearance of the biliary tract and the surrounding cranial intraabdominal organs were also evaluated. Percutaneous ultrasound-guided cholecystocentesis was performed easily during the study, and dogs tolerated well the procedure performed without anaesthesia. All laboratory parameters of the blood remained within normal limits throughout the study. However, some follow-up values, i.e. PCV, TPP, APTT and ALT, demonstrated statistically significant differences when compared to baseline measurements, which might reflect the effect of 24-hour fasting before the experiment, as well as day-to-day metabolic fluctuations due to feeding and water supply during the study. There were no visible signs of bleeding from the liver, bile leakage from the gallbladder or accumulation of free peritoneal fluid during repeated ultrasonographic examinations. Percutaneous ultrasound-guided cholecystocentesis seems to be an important diagnostic procedure in canine gallbladder diseases and can be used safely and easily to gain gallbladder bile for diagnosis of bacterial cholecystitis or for investigating hepatobiliary function in the dog.
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PMID:Percutaneous ultrasound-guided cholecystocentesis in dogs. 1249 44

Hemorrhage in the gallbladder (GB) is usually associated with cholecystitis, GB neoplasm, trauma, hemobilia, and cystic artery aneurysm. Our patient had not experienced any previous abdominal trauma, and GB hemorrhage was unlikely to result from cholecystitis or bleeding diathesis. A 55-year-old male was admitted because of right upper quadrant pain. Both prothrombin time and partial thromboplastin time were normal. Abdominal computed tomography, endoscopic ultrasound and magnetic resonance cholangiopancreatography were performed. Image studies revealed GB wall thickening and an intraluminal mass. Laparoscopic cholecystectomy was performed. Upon opening the GB postoperatively, a large amount of fresh blood and old blood clot was noted. The incidence of GB hematoma is very rare. GB hematoma should always be considered in the differential diagnosis of GB tumor. In such a situation, surgical intervention is needed for further patient evaluation and management. We present a rare case of intramural GB hematoma, of which we were unable to make a definitive diagnosis preoperatively.
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PMID:Intramural gallbladder hematoma mimicking gallbladder neoplasm in a 55-year-old male patient. 2206 24