Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0684275 (haemophilia)
 10,958 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sixty four cases of retinal and vitreous hemorrhages are reported during a 15 months prospective study in Bamako. Main diseases associated with hemorrhages are high blood pressure (56% of cases), hemoglobinopathies (33%) and diabetes mellitus (23%). In 28% of cases several aetiologies are connected. SC hemoglobin is a frequent aetiology of vitreous hemorrhage (40%). Hemoglobin AS and AC, generally asymptomatic, are also liable to hemorrhages. Terson and Eales syndromes, Werlhof disease, hemophilia and AIDS are most uncommon. In 8% of cases there is not any aetiology.
 ...
PMID:[General causes of retinal and vitreous hemorrhages in Mali]. 181 88

We have studied the conditions of in vitro binding of platelet glycoprotein IIb/IIIa (GPIIb/IIIa) to fibrinogen and applied the results to identify and measure the serum inhibitors to the binding. For the enzyme-linked immunosorbent assay, platelet extract was delivered to a fibrinogen-coated microtiter plate that was incubated for 2 hours, followed by incubation with anti-GPIIb/IIIa monoclonal antibody for another 2 hours. The plate was then incubated with peroxidase-conjugated anti-mouse IgG for color development. The binding was shown to be calcium-dependent. The binding was partially blocked by treating the coated fibrinogen with anti-fibrinogen antibody. Reduction or dissociation of GPIIb/IIIa resulted in the total loss of its ability to bind to fibrinogen. Platelet extracts of patients with hemophilia showed decreased binding (25% and 14%, compared with control platelet extract), and an extract from a patient with Glanzmann's thrombasthenia showed no binding. With the enzyme-linked immunosorbent assay we have measured serum inhibitors to GPIIb/IIIa binding to fibrinogen in 35 hemophilia A, 17 immune thrombocytopenic purpura, 22 human immunodeficiency virus-related immune thrombocytopenic purpura, and 29 systemic lupus erythematosus serum samples. In those patients with inhibition by serum, polyethylene glycol precipitation of circulating immune complexes (CICs) decreased the inhibition by the supernatants, and all the resolubilized CIC precipitates demonstrated inhibition, which indicates that CICs play a major role in the inhibition of GPIIb/IIIa binding to fibrinogen. This, then, provides evidence of CIC-mediated impaired GPIIb/IIIa binding to fibrinogen in hemophilia A, HIV-ITP, and SLE.
 ...
PMID:Inhibition of platelet GPIIb/IIIa binding to fibrinogen by serum factors: studies of circulating immune complexes and platelet antibodies in patients with hemophilia, immune thrombocytopenic purpura, human immunodeficiency virus-related immune thrombocytopenic purpura, and systemic lupus erythematosus. 200 77

Children with blood diseases do not infrequently visit the dental clinics. Therefore, it is important to recognize the actual condition of blood diseases in association with the oral treatment of patients. During the period from Sept. 1979 to June 1989, 68 patients who had blood diseases visited the Pedodontic Clinic of Niigata University Dental Hospital. This report is the investigation of the dental situation of these patients. The result were as follows: 1. The ratio of patients with blood diseases in our clinic was 0.7%. The patients with leukemia were most frequent. 2. Most of the first visit-patients with blood diseases were children aged 6, while the general patients group was younger. The ages of first visit-patients corresponded to the most frequent ages of primary diseases. 3. The majority of their chief complaints were "the treatment of decayed teeth for caries". Others were "the extraction of loose teeth for exchange" or "the diseases of oral soft tissues". 4. The patients visited our clinic from all over Niigata prefecture, some of which visited from a great distance and were introduced by Niigata University Medical Hospital. 5. As to the treatment of decayed teeth for caries, the patients with hemophilia had a large number of treated teeth as compared with the other diseases. In case of leukemia, anaphylactoid purpura and idiopathic thrombocytopenic purpura (ITP), the outpatients generally tend to be subject to more severe caries than the inpatients in Niigata University Medical Hospital. 6. The percentage of recall of patients with blood disease was lower than that of general children at the rate of about 20%.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:[An investigation into the actual condition of children with blood diseases in the pedodontic clinic of Niigata University Dental Hospital]. 215 74

Isolated thrombocytopenia occurs frequently in patients infected with HIV. Studies of mechanisms of thrombocytopenia and clinical response to therapy suggest that the thrombocytopenia is often antibody mediated (ITP). The best approach to treatment of these patients is uncertain in that the routine modalities (steroids, splenectomy, vinca alkaloids) that are used to increase the platelet count in patients with classic ITP are known to be immunosuppressive. We report here the results of intravenous gammaglobulin (IVGG) treatment of 22 patients with HIV-related acute and chronic ITP who had severe thrombocytopenia and bleeding symptoms. Only one patient had an opportunistic infection at the time of treatment. Eight patients were homosexual, eight had hemophilia, three were i.v. drug abusers, two children had congenital acquisition of HIV, and one was the wife of an HIV + i.v. drug abuser. The average pretreatment platelet count was 22,000/microliter (hemophiliacs were treated at higher platelet counts than were the other patients), and the mean peak platelet count measured on days 5 to 8 was 182,000/microliter. Nineteen of 22 patients had peak platelet counts greater than 50,000/microliter following IVGG and 17/22 had peak counts greater than 100,000/microliter. After the initial infusions, all but three refractory patients could maintain adequate platelet counts with IVGG alone infused no more often than once every 2 weeks. The outcomes for the 22 patients after multiple maintenance IVGG infusions were remission, 5; stable without therapy, 1; maintenance, 13; and refractory, 3. The eight hemophiliacs with ITP responded better than did the eight homosexual ITP patients; their mean peak platelet count was 227,000/microliter versus 142,000/microliter in the homosexuals. In summary, patients with HIV-related ITP without opportunistic infections responded well to IVGG, with peak platelet counts comparable to those of ITP patients not infected with HIV. IVGG may be a useful therapy of ITP in HIV+ patients, since it appears to be less immunosuppressive than are conventional therapies, and none of the 22 HIV+ patients developed an opportunistic infection while receiving IVGG alone.
 ...
PMID:Isolated thrombocytopenia in patients infected with HIV: treatment with intravenous gammaglobulin. 245 12

Five patients with severe hemophilia A receiving long-term treatment with commercial factor VIII concentrates developed severe immune thrombocytopenia (ITP, platelet counts less than 20 X 10(9)/l). Concomitantly, they presented with a marked elevation of serum IgG concentrations (mean, 2364 mg/dl;range, 1712-2954 mg/dl). In four patients, the T helper to suppressor cell ratio was below 1. Treatment with high-dose intravenous immunoglobulin (IgG, 7s) at doses of 0.2g (n = 2) or 0.4g (n = 3) per kg of body weight on 5 consecutive days was effective immediately. The bleeding tendency ceased and platelet counts rose transiently. In three cases, treatment was repeatedly effective and patients underwent uneventful splenectomies. Thus, high-dose IgG therapy may serve as a life-saving agent in patients with severe hemophilia complicated by ITP.
 ...
PMID:Immune thrombocytopenia in severe hemophilia A treated with high-dose intravenous immunoglobulin. 308 Aug 31

Intramural duodenal hematoma in children is one of the least common injuries encountered. Because of the rarity of this problem, there has been little conformity of opinion as to the relative merits of operative vs nonoperative management. We reviewed 182 cases of this condition, of which 168 were taken from the English literature and 14 from our hospital. Pancreatitis was the most common associated intra-abdominal lesion (21%), while central nervous system, skeletal, and thoracic injuries (5%) accounted for the major extra-abdominal injuries. Hemophilia, von Willebrand's disease, and idiopathic thrombocytopenic purpura occurred in 7 cases and played a major role in the prognosis. Of these cases, 121 patients were treated surgically and 61 conservatively. The average hospitalization was 14 days for the surgical group and 11 days for the conservative group. There were 18 complications in the surgical group. It appears that most patients with intramural duodenal hematoma would respond well to conservative management. Surgery should be reserved for those cases that remain obstructed over seven to ten days or have evidence of perforation.
 ...
PMID:Intramural hematoma of the duodenum. 325 85

Ageing does not bring with it any major changes in the coagulation or fibrinolytic proteins or platelets. It does bring a greater burden of disease, with less reserves, and so when haemorrhage occurs in the elderly it has more serious consequences. The cause of a bleeding diathesis can usually be determined after a careful history, and examination of the patient followed by simple tests--the platelet count, blood film, bleeding time, prothrombin time, partial thromboplastin time, thrombin time, fibrin degradation products and the euglobulin clot lysis time. Other confirmatory tests, assays and inhibitor titres, will seal the diagnosis. Treatment is mainly directed at removing the underlying cause, if possible, and remedying the defect, with platelet transfusion, fresh frozen plasma or factor concentrates. These treatments will not be effective where there is an inhibitor or antibody present; steroids, splenectomy (for ITP), plasma exchange or immunosuppression are needed. Two major advances have occurred in the early 1980s. One has been the introduction of high-dose intravenous immunoglobulin in the management of ITP, although worries remain about thrombotic events in elderly patients. The other is the spreading use of DDAVP, originally introduced for von Willebrand's disease and mild haemophilia, and now finding a role in uraemia and with cardiopulmonary bypass. Drugs are a significant and potentially preventable cause of bleeding in the elderly. The most frequent problems arise with anticoagulants. The risk of interactions increase with the number of other medications which are prescribed.
 ...
PMID:Bleeding and coagulation disorders in the elderly. 332 49

This report describes a technique for the performance of a sonographically guided percutaneous umbilical blood sampling and its potential uses in the management of diagnostic problems in the second and third trimester of pregnancy. The method has been employed in the prenatal assessment of 13 fetuses at risk for hemostatic failure (hemophilia and idiopathic thrombocytopenic purpura), chromosomal disorders, isoimmunization, and fetal hypoxia. This simple and rapid procedure offers access to the fetal circulation for diagnostic and therapeutic purposes.
 ...
PMID:Percutaneous umbilical blood sampling. 392 26

Immunologic abnormalities resembling those seen in patients with the acquired immunodeficiency syndrome (AIDS) are frequently observed in multitransfused but otherwise healthy individuals with hemophilia. To determine whether there was clinical or laboratory evidence to suggest an abnormality of immunoregulation in persons with hemophilia before the recognition of AIDS, we examined data collected by the Hemophilia Study Group from 1975 to 1979 on 1,551 patients with factor VIII deficiency. The prevalence of lymphocytopenia and thrombocytopenia in patients over 5 years of age on entry was found to be 9.3% (94/1,013) and 5.0% (26/518), respectively. These rates were significantly different from a normal population (P less than .00001 and less than .0003). No cases meeting the definition of AIDS were noted during the study. However, on follow-up in 1984 of a cohort of 79 patients with thrombocytopenia or lymphocytopenia on two or more occasions during the study, eight patients (10%) with AIDS-related abnormalities, including idiopathic thrombocytopenic purpura, non-Hodgkin's lymphoma, generalized lymphadenopathy, and oral moniliasis without obvious cause were identified. Of the 79 patients, liver disease accounted for five of the ten deaths (12.6% mortality) observed during a minimum follow-up of five years after detection of cytopenia. Only one death was attributed to bleeding in the absence of liver disease. We conclude that (a) the frequency of lymphocytopenia and thrombocytopenia was increased in multitransfused factor VIII-deficient hemophiliacs before the advent of AIDS, and (b) persistent lymphocytopenia and thrombocytopenia appear to be strongly associated with liver disease, which was the leading cause of death in a cohort of hemophiliacs followed five or more years.
 ...
PMID:Long-term follow-up of hemophiliacs with lymphocytopenia or thrombocytopenia. 406 24

Controlling hemorrhage from dental treatment in bleeding disorder patients is one of the most serious procedures encountered by the dentist. In the Dental Division, Ramathibodi hospital, dentists use local hemostatic technics combined with replacement therapy, local hemostatic agents and antifibrinolytics in the management of bleeding disorders in dental patients, such as leukemia, ITP, hemophilia. Celluloid splints as an adjunct therapy is very beneficial in controlling hemorrhage in dental procedures as shown by 5 years experience with 278 patients. The advantages are: less expensive, lesser days hospitalized, better outcomes. Presently it is used as a routine technic in dental treatment.
 ...
PMID:Local hemostatic technic using a celluloid splint in bleeding disorders. 788 64


1 2 3 Next >>