Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020538 (hypertension)
 170,190 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Classical sex-linked hemophilia (Hemophilia A) has been described as due to deficiency in the synthesis of Factor VIII procoagulant activity (VIII:C). The availability of immunological techniques provided the means of identifying Factor VIII-Related Antigen(VI-IIR:Ag) detectable by rabbit antibodies to F VIII, which is distinct from VIII:C detected by human anti-F VIII available from multitransfused patients. Hemophilia A is lacking in VIII:C but not VIIIR:Ag. Recently, a third function of the F VIII "complex" was discovered with the help of ristocetin (von Willebrand's Factor, VIIIR: RCo). This activity is reduced in von Willebrand's syndrome. Estimation of the titers of VIII:C and VIIIR:Ag provides a method for more accurate detection of hemophilic carriers. Newly available chromogenic substrates perhaps will give rise to more simplified assays of VIII:C. The development of cryoprecipitates and stable lyophilized concentrates of F VIII has greatly simplified and intensified maintenance therapy, and has opened a new era in treatment. Prophylactic therapy has been shown to be very helpful in certain "high risk" cases. The impact and benefits of home care and self-administration has been tremendous. However, the varying quality of cryoprecipitates and the high cost of more purified concentrates are still stumbling blocks in treatment regimes. Other problems exist. Spontaneous bleeding, especially central nervous system bleeding, account for the majority deaths by haemorrhage. Inhibitor kinetics have been well characterized. It is clear that there exists "low" and "high" responders. For the "high" responders, plasmapheresis, immunosuppressives and the infusion of Factor IX concentrates have been utilized with varying success. The prevention of hemophilic arthropathy and its progression by maintenance therapy seems to be still inadequate. The results of trials with more vigorous regimes are awaited. The complications of therapy still remain to be solved. Apart from the well-known complications wuch as hepatitis, haemolytic disease and F VIII inhibitors, the existence of previously unnoticed complications as splenomegaly, hypertension, renal disease and paradoxal bleeding have been recently realized. The role of altered fibrinogen, fibrin degradation products (FDP) and unclassified fibrinogen derivatives (UFD) present in cryoprecipitates and F VIII concentrates in the above complications needs to be further clarified. In conclusion, tremendous progress in various aspects of hemophilia has been achieved in developed countries. Comprehensive care can now be carried out in various centers. On the other hand, developing countries still face a number of basic problems. The concept that hemophilia is a "manageable" disease and that chronic crippling and death from exsanguination can be prevented, should be disseminated widely by various means...
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PMID:Recent advances in hemophilia. 52 46

Factor VIII complex was studied in patients presenting arterial hypertension. Visceral involvement was quantified using a clinical index calculated from ocular fundus, renal function and left ventricular hypertrophy data. A significant correlation was found between the mean arterial pressure, the visceral involvement and the level of complex VIII. Nevertheless, other data obtained in different patients (Conn's disease) suggest that the visceral involvement (and not the mean arterial pressure) is the main determining factor in the increase of factor VIII complex.
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PMID:Increased level of factor VIII complex in severe arterial hypertension. 72 Sep 55

Recently, we have reported two cases with endothelin (ET)-secreting tumor presenting with hypertension and elevated plasma endothelin-1 (ET-1) levels. The present study examines the histopathology of the ET-secreting tumor in one of these cases. The neoplasm was located in the skin and microscopically characterized as a malignant hemangioendothelioma by remarkable intravascular proliferations of atypical endothelium with the expression of Factor VIII-related antigen in the tumor cells. The tumor enlarged rapidly with rising ET-1 and blood pressure levels. The ET-1 content and its messenger RNA expression in the tumor extract were higher than those from normal parts of skin. ET-1 may play a pathophysiological role in patients with ET-secreting malignant hemangioendothelioma.
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PMID:Endothelin-secreting tumor. 172 90

During pregnancy there is an elevation of factor VIII. In hypertensive pregnancy this increase may be smaller. On the other hand factor VIII associated antigen-factor VIII-ratio is said to be increased in these women compared with normotensive ones. There is a discussions about using these change for early detection of PIH. Estimations of factor VIII have been made chemically. Estimations of factor VIII associated antigen have been carried out immunologically. Factor VIII associated antigen-factor VIII-ratio was higher in pregnant women with hypertension than in normotensive ones.
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PMID:[Factor VIII-associated antigen/factor VIII ratio in pregnant patients with and without hypertension]. 211 22

Primary tumor of the aorta is extremely rare. An instance of aortic intimal sarcoma, namely fibromyxosarcoma, which extended from the beginning of the descending aorta to 7 cm above the abdominal bifurcation, with clinical evidence of acutely occurring hypertension, arterial embolism of the lower extremities, renal infarction, and aortic occlusion in a 50-year-old male is reported. The tumor was limited to the intima and composed of spindle-shaped tumor cells with abundant myxoid extracellular matrices. The tumor cells were negative for Factor VIII, Desmin, or Myoglobin, but were positive for Vimentin or Factor XIIIa in immunoperoxidase studies. An electron microscopic examination revealed a large amount of rough endoplasmic reticulum in the cytoplasm. Parenchymal metastases were observed in both the lungs and thoracic vertebrae. A review of literature on the clinical and pathological aspects of the tumor was made.
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PMID:A case of aortic intimal sarcoma manifested with acutely occurring hypertension and aortic occlusion. 258 79

A family in which there were two certain and three possible cases of thrombotic microangiopathy in two generations is presented. All afflicted members studied presented with acute renal failure, and accelerated hypertension. No abnormal platelet-aggregating activity could be identified in the plasma of asymptomatic family members or in the surviving patient in remission. Although an increase in the Factor VIII: von Willebrand's factor level was found in the sole surviving patient, the multimer pattern was normal. Platelet-associated IgG level was also normal in this individual. Thus, individuals predisposed to this familial form of thrombotic microangiopathy do not have a demonstrable marker which can be implicated in the development of the disorder. Long-term study of this kindred is required to determine the factors which are important in the pathogenesis of thrombotic microangiopathy in the affected individuals.
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PMID:Familial thrombotic microangiopathy. 408 Sep 59

An example of multicentric, skeletal, myxoid angioblastomas in a Japanese woman is reported. The disease was symptomatic at age 12 years and was characterized by slowly progressive, multiple, lytic bone defects. In addition the patient had juvenile hypertension, and, at age 20 years, had focal brain infarction. The primitive vascular nature of the process was supported by the following observations: occasional erythrocytes within cytoplasmic lumina and capillary-like cellular tubes; Weibel-Palade bodies, numerous pinocytotic vesicles, prominent microvilli, elaborate intercellular contacts, desmosomes, and numerous arrays of fine intracytoplasmic filaments by electron microscopy; and, in addition, Factor VIII positivity. The clinical findings in this case are more consistent with a multicentric, rather than a metastatic process. The name myxoid angioblastomatosis of bones is appropriate.
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PMID:Myxoid angioblastomatosis of bones. A case report of a rare, multifocal entity with light, ultramicroscopic, and immunopathologic correlation. 409 Nov 81

1. The epidemiological characteristics of plasma renin activity (PRA) were established in 1999 members of occupational groups in North and West London. 2. The main finding was that PRA was inversely associated with systolic blood pressure in men, the percentage fall in PRA (on a log scale) being 8.4% for each increase of one standard deviation in systolic blood pressure. There was a less obvious inverse relationship in women. 3. However, blood pressure accounted for less than 1% of the variance in PRA. 4. Mean PRA in both smokers and ex-smokers was about 20% higher than in non-smokers. 5. PRA fell with increasing age, was lower in women than in men and considerably lower in blacks (of either sex) than whites. 6. PRA was lower in the first quarter of the menstrual cycle than in the rest of the cycle. 7. Haemoglobin, blood cholesterol, leucocyte count and Factor VIII were positively correlated with PRA. 8. PRA was lower in men with diagnosed hypertension than in those without; there was no significant difference in the women. 9. PRA was lower in those who had had myocardial infarcts in the past than in those who had not. 10. The data as a whole suggest that it may be low, not high, levels of PRA which are associated with increased risks of cardiovascular disease in which hypertension is a predisposing factor. 11. The explanation may be a homoeostatic fall in PRA in response to a rise in blood pressure rather than a major causal role for PRA in the pathogenesis of essential hypertension and target-organ damage.
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PMID:The epidemiology of plasma renin. 633 13

We report an incidence of thrombosis of 17.6% in 159 patients treated with a five-drug chemotherapy regimen (cyclophosphamide, methotrexate, 5-fluorouracil, vincristine, and prednisone) for Stage IV breast carcinoma. Chi-squared analysis of risk factors for thrombosis (ambulatory status, obesity, family history, smoking, diabetes mellitus, hypertension, liver dysfunction, thrombocytosis, and previous endocrine therapy) showed no difference between the patients who had a thromboembolic event and those who did not. Statistical analysis revealed that a significantly higher incidence of thrombosis occurred during the chemotherapy regimen than when off this regimen (P less than 0.05). Detailed coagulation studies done prospectively on 10 patients receiving the five-drug chemotherapy regimen compared with 10 control patients showed a significantly elevated Factor VIII antigen:activity ratio in the group receiving the chemotherapy regimen compared with the control group and normals. These results implicate the chemotherapeutic regimen in the pathogenesis of the increased incidence of thrombosis. The pathophysiology of thrombosis in settings such as this awaits better in vitro tests defining the "hypercoagulable state."
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PMID:Increased incidence of thromboembolism in stage IV breast cancer patients treated with a five-drug chemotherapy regimen. A study of 159 patients. 654 74

Hypertension produces myocyte hypertrophy and increases the extracellular matrix. In order to determine the composition of the extracellular matrix we studied the hearts from 14 hypertensive patients by immunohistochemistry using antibodies against collagen I, III, IV and V, fibronectin, myoglobin, muscular specific actin, Factor VIII, CD 34 and vimentin. The myocardium showed a focal increase in fibronectin, collagen I and III and diffuse deposition of laminin, collagen IV and V. Cells positive for vimentin, Factor VIII and CD 34 were also increased, but with considerable variation from case to case. We found no relation between matrix variation and the degree of hypertension or the time elapsed from the beginning of the disease. We conclude that the main role of the matrix in hypertension may be remodelling of the heart so that it entraps muscle fibres and increases their contractility.
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PMID:Alterations in the extracellular matrix of the myocardium in essential hypertension. 828 55


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