Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0000737 (abdominal pain)
 31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Acquired factor VIII inhibitor was found in a 69-year-old white male with insulin-dependent diabetes mellitus. He presented with left lower abdominal pain and hematoma after a fall. Preoperative hemostasis studies were normal except for prolonged aPTT. Prolonged aPTT was not corrected by 1:1 mixture with normal fresh plasma and incubation showed further prolongation with time. Factor VIII:c was 3.5%. The inhibitor titer was 7.5 Bethesda units. The possible mechanism causing antibody to factor VIII was postulated to be an autoimmune process and/or increased immunogenicity owing to glycosylation of factor VIII coagulant protein.
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PMID:Factor VIII inhibitor in insulin-dependent diabetes mellitus. 249 61

Three endothelial cysts and five hemorrhagic cysts (pseudocysts) arose in the adrenal glands of seven patients aged 23-73 years. Four patients were male and three were female. Five were symptomatic and gave abdominal pain as their chief complaint. Endothelial cysts were collapsed or filled with serous fluid, multiloculated, had an endothelial lining, and often contained adrenal cortex in their outer walls. The endothelial lining reacted only weakly for Factor VIII-related antigen (FVII-RAg), but it stained strongly for collagen type IV (C-IV). The lack of hemorrhage and the FVIII-RAg/C-IV staining pattern in endothelial cysts suggest lymphatic differentiation. Hemorrhagic cysts were spherical, firm masses containing clotted blood and hyalinized thrombus with attenuated adrenal cortex in the outer fibrous wall. Islands of intact cortical cells were present deep within the thrombi of four hemorrhagic cysts. Three of five hemorrhagic cysts stained strongly for FVIII-RAg and C-IV in irregular vascular channels of the attenuated cortex and within the cyst contents. These channels suggest that at least some hemorrhagic cysts arise when hemorrhage occurs in a preexisting blood vascular anomaly. Entrapment of cortical islands by extravasated blood in hemorrhagic cysts may be misdiagnosed as necrotic cortical neoplasm. To avoid confusion, one must recognize the normality of the entrapped cortical cells, identify an intrinsic vascular anomaly, and distinguish thrombus from necrotic tumor.
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PMID:Vascular adrenal cysts. A clinicopathologic and immunohistochemical study of endothelial and hemorrhagic (pseudocystic) variants. 250 65

A 28-year-old woman was admitted six months before her death because of abdominal pain, anemia and hepatosplenomegaly. Clinical diagnosis by biopsy examination was primary hepatic angiosarcoma. The autopsy revealed about 700 ml of bloody ascites. The spleen weighed 510 g, and was completely replaced by dark grayish-red tumors. The enlarged liver was 6,560 g and revealed multiple tumor nodules. Microscopically, the tumor cells formed irregular vascular spaces. Factor VIII-related antigens were stained in the tumor cells. Zonula adherens was observed also by electron microscopic observation.
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PMID:[An autopsy case of primary splenic hemangiosarcoma]. 407 65

Forty primary splenic angiosarcomas occurring in 21 men and 19 women, 19-84 years old (median 59 years) are reported. Patients presented with splenomegaly (35 of 38, 92%), abdominal pain (33 of 40, 83%), and systemic symptoms such as fatigue (2 of 40, 5%), fever (4 of 40, 10%), and/or weight loss (16 of 40, 40%). Five (13%) experienced splenic rupture associated with hemoperitoneum. Abnormal laboratory findings included cytopenia (31 of 34, 91%), leukocytosis (8 of 21, 38%), and thrombocytosis (1/39, 3%). Most spleens weighed 500-1,000 g (mean, 1,180 g). The cut splenic surfaces showed multiple hemorrhagic nodules that were frequently associated with infarction, although some had a diffuse pattern of involvement. Microscopically, there were a variety of histologic patterns displayed by the vasoformative component. A honeycomb or sponge-like pattern was common in some, whereas others simulated a cavernous hemangioma or normal splenic sinuses (pseudosinusoidal pattern). Papillary endothelial tufts and solid proliferations of spindled to round to epithelioid cells were also seen. Factor VIII-related antigen was detected in 19 of 23 cases, BMA-120 in 18 of 23, UEA-1 receptor in 18 of 23, and vimentin in 23 of 23 as well as CD68 antigen in 1 of 23 cases. S-100 protein and cytokeratin were not found in any of the 23 cases studied. Metastases in 22 of 32 patients (69%) were to the liver (13 patients), bone or bone marrow (7 patients), lymph nodes (1 patient), and brain (1 patient). Three patients had concomitant malignancies and one had a prior history of a mixed B-cell lymphoma 5 years previously that had been treated with chemotherapy. Follow-up in 38 patients revealed that 30 (79%) are dead at a median interval of 6 months (range 0-48 months) and 8 are alive 5-21 months after diagnosis. These findings indicate that splenic angiosarcoma is an aggressive neoplasm with a high metastatic rate and an abysmal prognosis. Recognition of the wide range of histologic patterns is of diagnostic value but no apparent prognostic significance.
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PMID:Primary angiosarcoma of the spleen. A clinicopathologic study of 40 cases. 780 32

The clinical, histologic, and immunohistochemical features of six cases of hemorrhagic adrenal pseudocysts are reported together, with a review of the English literature on this topic since 1950. The mean age at presentation was 57 years (range, 30-72 yr). There were four men and 2 women. The average cyst size was 9.2 cm (range, 6-16 cm). In four patients, the hemorrhagic adrenal pseudocysts were incidental findings. The remaining two patients presented with an abdominal mass and hypertension, respectively. The hemorrhagic pseudocysts were unilocular cystic masses surrounded by a fibrous capsule and containing abundant amorphous material, blood, and fibrin. Numerous dilated, thin-walled, vascular channels that stained strongly for Factor VIII-related antigen, collagen IV, laminin, Ulex europaeus agglutinin I lectin, and CD34 were present within the fibrous capsule, cyst contents, and surrounding residual adrenal gland. These findings support a vascular origin for these lesions, and they are thought, therefore, to be related to endothelial adrenal cysts. The literature review of 111 vascular adrenal cysts (85 hemorrhagic pseudocystic type and 26 endothelial type) showed similar clinical features. The mean age at presentation was 44.5 years (range, 5 d-95 yr), with a female predominance (62%). The most common clinical presentation was abdominal pain (35%), followed by incidental findings (32%). There were no significant clinical differences between hemorrhagic and endothelial type cysts. In some cases, the presence of intracystic islands of cortical cells can cause diagnostic confusion with adrenal cortical tumors. The presence, however, of a rich intracystic and capsular vascular network, normal-appearing islands of cortical cells, and abundant thrombotic fibrinous material, rather than necrotic tumor cells, should rule out the possibility of a degenerating adrenal cortical neoplasm.
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PMID:Vascular adrenal cysts: a clinicopathologic and immunohistochemical study of six cases and a review of the literature. 919 68

In portal vein thrombosis, various hypercoagulable conditions and inherited or acquired thrombophilias have already been described as predisposing factors. In a 33-year-old man admitted to a hospital with upper abdominal pain, a partial portal vein and upper mesenteric vein thrombosis, respectively, and a complete splenic vein thrombosis were diagnosed. Further diagnostic procedures showed no evidence for local precipitating factors or any underlying infectious, paraneoplastic or inflammatory disease. Thrombophilia screening demonstrated elevated factor VIII levels (206 %) and von Willebrand factor levels (> 440 %). An acute-phase reaction was excluded. Oral anticoagulant therapy with phenprocoumon was started. Factor VIII and von Willebrand factor were reproducibly elevated to high activity levels over a period of 12 months in absence of acute or chronic inflammatory reaction. Increased levels of factor VIII and von Willebrand factor may play a pathogenetic role in the development of portal, splenic, and mesenteric thrombosis.
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PMID:[High plasma levels of factor VIII and von Willebrand factor in a patient with portal vein thrombosis]. 1205 64

An 85-yr-old male presented with complaints of a 40-lb weight loss and a dull left upper quadrant abdominal pain. He also complained of decreased appetite, generalized weakness, generally not feeling well, and a dull left upper quadrant abdominal pain that was not relieved by food. He had a ventral and a left-sided inguinal hernia. Laboratory investigations revealed iron deficiency anemia, the cause of which was not apparent despite extensive investigation including computerized tomographic scans, esophagogastroduodenoscopy, and small-bowel follow-through examination. Surgical exploration for possible angiodysplasia, malignancy, and/or mesenteric ischemia revealed an incarcerated hernia, and the histopathological examination of the surgical specimen revealed high-grade angiosarcoma. The tumor showed strong positivity for vimentin and CD31 and a focal positivity for Factor VIII and CD34. At that time he was found to have hepatic metastases. He was started on thalidomide as an experimental measure with no change in the performance status and increasing evidence of necrosis in the metastatic lesion.
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PMID:Angiosarcoma of the small intestine: a possible role for thalidomide? 1471 38

The clinicopathological characteristics are presented of 19 extragenital cystic lesions of female patients located at the retroperitoneum (9 cases), the mesentery (6 cases) and the peritoneum (4 cases). Median age was 42.3 years and the main symptom was abdominal pain. The cysts measured 4-27 cm in diameter and were classified as: epithelial (7/19), mesothelial (5/19), vascular (2/19), parasitic (1/19) and developmental in origin (4/19). Of the cases 18/19 were benign lesions and one case was a borderline mucinous cystic tumor. Immunohistochemistry by a streptavidin-biotin method was performed to investigate CEA transcripts (MoAb, Novosan), CA125 (MoAb, CIS Diagnostics), vimentin (MoAb Ve6, Novocastra), secretory component (a polyclonal antibody, DAKO), Factor VIII (MoAb, DAKO), CD34 (MoAb, Scytec), calretinin (a polyclonal antibody, Zymed, San Francisco, CA). Cytokeratins were of low and high molecular weight (Immunon, AE1-MoAb). The results are helpful in the correct classification of various tumors. The treatment of choice is complete surgical resection of the tumors.
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PMID:Extragenital cystic lesions of peritoneum, mesentery and retroperitoneum of the female. Clinicopathological characteristics of 19 cases. 1599 37

Brodifacoum, also known as a superwarfarin, is a four-hydroxycoumarin derivative. It exerts an anticoagulant effect by inhibiting the reduction of vitamin K-2,3 epoxide, thereby decreasing the production of vitamin K-dependent clotting factors. It is a readily available rodenticide that has been associated with accidental ingestions in children. We report the case of a 21-year-old male who was admitted to the hospital with spontaneous bruising, hematuria and abdominal pain secondary to a perinephric hematoma. The patient was found to have a markedly prolonged prothrombin time and activated partial thromboplastin time that corrected with mixing of normal plasma. He had a normal factor V level; however, factors VII and X were less than 1% and factors II and IX were between 2% and 4% of normal. Ingestion of an anticoagulant was suspected, although the patient denied intentional or accidental ingestion. He was treated with FEIBA (Factor VIII Inhibitor Bypass Activity), fresh frozen plasma and oral vitamin K. The patient was stabilized and discharged from the hospital on oral vitamin K 50 mg twice daily. A serum brodifacoum level was later found to be markedly elevated at 320 ng/ml. We followed the brodifacoum level, which decreased to 31 ng/ml approximately six weeks after initial presentation. The exact length of treatment required to prevent recurrence of the coagulopathy was not determined because the patient did not return for follow-up. Superwarfarin ingestion must be suspected and quickly identified in patients with depletion of vitamin K-dependent clotting factors resulting in potentially catastrophic bleeding.
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PMID:Acquired deficiency of vitamin K-dependent clotting factors due to brodifacoum ingestion. 1847 86

Epithelioid hemangioendothelioma (EHAE) is a malignant vascular tumor derived from endothelial cell often misdiagnosed as Hepatic carcinoma on the basis of radiological features. Till now etiology of this rare curiosity is unknown but it is related with use of oral contraceptives pills (OCP), liver trauma, exposure to vinyl chloride and hepatitis. We herein report on a case which failed to be diagnosed by cytopathology, computed tomography (CT) and magnetic resonance imaging (MRI). Patient was a 46 yr old man presented with abdominal distension for a month. Initial liver function test (LFT) was increased whereas renal function test (RFT) and alpha-fetoprotein (AFP) were normal. His abdominal ultrasound revealed multiple hypoechoic nodules and multiple liver calcifications. Subsequently laparoscopic omental biopsy and Ultrasound guided liver biopsy was done showing the neoplastic cells scattered in fibrous stroma. The immunohistochemistry for endothelial tumor cells stained positive for Vimentin (+++), CD10 (+++), CD34 (++), CD31 (+), Factor VIII antigen (focal) (+) and low proliferative activity for ki-67. Our case is very interesting in which patient admitted with nonspecific symptoms of abdominal pain and diagnosed to be a Malignant Hepatic EHAE metastasized to the peritoneum, omentum and mesentery. The patient was on thalidomide 50 mg/day and increased to 100 mg/day. 5-Flurouracil (FU) intraperitoneal chemotherapy and other symptomatic and supportive treatment was given to the patient. Our case highlights on the importance of immunohistopathological diagnosis, compare the radiological findings of this disease and discuss the treatment strategy with review of available literature.
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PMID:Hepatic epithelioid hemangioendothelioma metastasized to the peritoneum, omentum and mesentery: a case report. 2619 13


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