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Biomedical subjects

P Saidi

Publications and source records attributed to P Saidi.

At least 37 records · Page 2Linked to original sources

Immune thrombocytopenia in hemophiliacs infected with human immunodeficiency virus and their response to splenectomy.

We studied five patients with hemophilia A in the age range of 18 to 64 years who were infected with human immunodeficiency virus and who developed immune thrombocytopenia. The clinical course of immune thrombocytopenia in relation to human immunodeficiency virus infection and the patients' responses to splenectomy and immune variables were determined. All five patients developed antibody to human immunodeficiency virus 6 to 60 months (median, 24 months) before the onset of thrombocytopenia, and two patients became human immunodeficiency virus antigenemic (one patient at the onset of immune thrombocytopenia and the other 60 months after the onset of immune thrombocytopenia [24 months after splenectomy]). All five patients had a strong platelet-associated immunoglobulin G and three patients also had a weak platelet-associated immunoglobulin M on their platelets. In four of five patients danazol therapy failed, and three patients required moderate doses of prednisone. Because of the progression of immune thrombocytopenia, four of the five patients underwent splenectomy with preoperative high-dose intravenous immune globulin. All four had an excellent immediate response to splenectomy, with a rise in platelet count to more than 300 x 10(9)/L and sustained remission during postsplenectomy follow-up of 6 to 45 months. There was no significant drop in CD4 and CD8 counts after splenectomy, and all four patients remained clinically well.

Adult↗

Patients with prolonged bleeding time of undefined etiology, and their response to desmopressin.

Between 1981 and 1986, we evaluated 59 patients who presented with isolated prolongation of bleeding time with normal platelet counts, platelet aggregation and coagulation parameters (including von Willebrand's factor), and without evidence of liver or kidney disease, or exposure to anti-platelet agents. These patients, termed as vascular fragility syndrome (VFS), were analyzed to further characterize their bleeding patterns. The pattern of bleeding manifestations was similar to that of patients with platelet dysfunction, such as mucocutaneous bleeding or excessive post-operative bleeding. In 16 patients, desmopressin (1-desamino-8-d-arginine vasopressin, DDAVP) was infused to control active bleeding or as a part of pre-surgical evaluation. Bleeding time improved (pre-DDAVP bleeding time 15.3 +/- 3.4 min, mean +/- S.D.; post-DDAVP bleeding time 10.7 +/- 3.9 min; p less than 0.01) within 30 minutes following the DDAVP infusion with either satisfactory arrest of acute bleeding or good control of subsequent hemostasis with surgery. Side effects with DDAVP were transient and minor, i.e. facial flushing, or conjunctival erythema. These findings indicate that VFS with isolated prolongation of bleeding time is a frequently encountered bleeding disorder and that DDAVP is effective in achieving hemostasis for the management of acute bleeding and may be useful prior to surgical procedures.

Bleeding Time↗

Adverse reactions to trimethoprim-sulfamethoxazole in hemophiliacs.

Adverse reactions to trimethoprim-sulfamethoxazole are very prevalent in patients with acquired immunodeficiency syndrome (AIDS). Recently we have observed severe toxicities associated with trimethoprim-sulfamethoxazole in three hemophiliacs, a group known to be at risk for developing AIDS. At the time of these reactions to the antibiotic, none of the patients had yet manifested any stigmata of AIDS per se. We advise caution in the use of trimethoprim-sulfamethoxazole in hemophiliacs and other patients at high risk for the development of AIDS.

Acquired Immunodeficiency Syndrome↗

Human immunodeficiency virus infection in sexually active wives of infected hemophilic men.

PURPOSE: Because of past multiple exposures to contaminated coagulation factor concentrates, the prevalence of human immunodeficiency virus (HIV) infection among adult hemophilic men in the United States is reported to range from 75 to 90 percent. The risk of HIV transmission through a long-term monogamous heterosexual contact can be estimated by studying the spouses of hemophilic subjects since these couples generally do not abuse intravenous drugs, usually maintain stable monogamous relationships, and are usually free of other risk factors. Our purpose was to gather data on the risk of heterosexual transmission of HIV infection in the context of long-term monogamous relations according to the duration of HIV antibody seropositivity and of HIV antigenemia in HIV-infected hemophilic men, and their sexual habits. SUBJECTS AND METHODS: Infection with HIV was studied in 14 sexually active spouses of infected hemophilic men who had been HIV antibody reactive for a mean of 46 +/- 23 (SD) months. One half of the hemophilic men studied had overt HIV antigenemia for a mean duration of 27 +/- 23 (SD) months; six of the men studied fulfilled clinical criteria for the diagnosis of acquired immunodeficiency syndrome (AIDS). All 14 couples were sexually active in a strictly monogamous fashion, in marriages of 13.5 +/- 10.5 (SD) years with an average reported frequency of four sexual encounters per month (range: one to 12). Plasma samples of the hemophilic husbands were retrospectively analyzed for HIV and hepatitis B virus markers. Blood samples were obtained from female spouses on at least two occasions, six months apart. Comprehensive questionnaires regarding sexual habits and other risk factors were filled out by each couple; during this interview, the couple was counseled about safe sexual practices. None of the couples studied used condoms prior to January 1986. RESULTS: Antibodies to HIV developed in only one of the 14 wives. At the time when this seroconversion was detected, her husband, in whom AIDS developed, had been reactive for HIV antibody for 49 months, and showed positive findings for HIV antigen for 26 months. No additional risk factors were identified for this couple. The infected female spouse, however, has a 14-year history of multiple sclerosis, and had been treated with immunosuppressant intermittently. Despite a significantly reduced number of CD4 lymphocytes, she has remained clinically asymptomatic for 17 months since seroconversion. HIV antibodies did not develop in any of the 13 remaining wives, despite the frequent practice of oral sex by six couples and reports of occasional anal intercourse by another couple.(ABSTRACT TRUNCATED AT 400 WORDS)

Acquired Immunodeficiency Syndrome↗

Reconstructive plastic surgery in hemophiliacs.

Major surgery can be safely performed in hemophiliacs when adequate factor activity is provided, i.e., 100 percent activity perioperatively and 50 percent activity thereafter. These patients require complete hemostatic evaluation in order to properly titrate perioperative needs. The extreme cost of surgery and hospitalization mandates that, when possible, patients needing multiple procedures have them performed at the same time. A case is presented in which a paraplegic hemophiliac with extensive pressure sores underwent quadruple flaps following these tenets.

Adult↗

Natural history of acquired immunodeficiency syndrome in hemophilic patients.

During the 5-year period from 1981 to 1985, we have observed 8 cases of acquired immunodeficiency syndrome (AIDS) among our 85 patients with hemophilia A. Thus, the prevalence of AIDS with hemophilia A is 9.4% in our patient population. By utilizing stored serum or plasma samples dating back to 1978, antibody against HTLV-III was detected in all 8 cases with AIDS. Based on the time interval from the appearance of antibody to HTLV-III to the diagnosis of AIDS in these patients, the incubation period ranged from 27 months to 60 months, with a median of 36 months. Before the diagnosis of full-blown AIDS, all patients exhibited a variety of prodromal manifestations of non-specific nature, including weight loss, oral candidiasis, unexplained non-productive chronic cough, generalized lymphadenopathy, and thrombocytopenia lasting several months to several years. Serial T-lymphocyte subset studies were available in some patients during the HTLV-III seropositive period and showed progressive lymphopenia, depletion of T4 cells with an average absolute count of 94 +/- 128 per mm3 (mean +/- 1 S.D.), and a markedly reversed T4/T8 ratio of 0.26 +/- 0.19 (mean +/- 1 S.D.). These findings suggest that the incubation period of AIDS is considerably long and that prospective study of serial immunologic markers and HTLV-III markers may be warranted in hemophilic patients at risk.

Acquired Immunodeficiency Syndrome↗

Long term follow-up of arthroscopic synovectomy for chronic hemophilic synovitis.

Recurrent hemarthrosis of the knee in hemophiliac patients leads to chronic synovitis, predisposing the joint to further hemarthroses and degeneration. Open synovectomy controls bleeding, however, significant loss of motion frequently results. We are reporting on seven patients who underwent arthroscopic synovectomy and had decreased bleeding episodes while maintaining range of motion. The seven patients had frequent recurrent hemarthroses despite medical management. All had had signs of degenerative arthritis preoperatively. Five of the seven had loss of motion. Length of follow-up averaged 4 years. Six of the seven had reduced bleeding episodes with an average of 0.22 hemarthroses per week. The seventh patient required significantly less Factor to control bleeding. No patient lost more than 10 degrees of motion. Three patients had increases in motion; two were unchanged. Radiographic progression of degenerative changes was noted in five patients, the other two were unchanged. We recommend early arthroscopic synovectomy in the treatment of recurrent hemarthrosis in hemophiliac patients.

Adolescent↗

Clinical trial of a new disposable bleeding-time device.

A new disposable bleeding time device (Hemalet) was tested in 20 normal individuals and 11 patients with various bleeding disorders. The results were compared with those of Simplate II. The mean bleeding time for normal individuals was 5.4 +/- 1.5 (mean +/- 1 SD) minutes by Hemalet and 5.8 +/- 1.4 (mean +/- 1 SD) minutes by Simplate II, with good correlation between the results by the two devices (r = 0.81). The bleeding time in patients with various bleeding disorders were also comparably prolonged between the two devices. The new disposable bleeding time device with a disposable blade has quick release (penetration) into skin and retraction, and offers an alternative means of a bleeding time test.

Bleeding Time↗

Effect of danazol on clotting factor levels, bleeding incidence, factor infusion requirements, and immune parameters in hemophilia.

Several recent studies have reported conflicting results on the effectiveness of danazol, an attenuated androgen, in raising plasma levels of clotting factors VIII and IX in patients with hemophilia. We undertook a randomized, double-blind cross-over trial using 8 weeks' administration of danazol (D), 600 mg/d, and 8 weeks' administration of placebo (P) separated by 2 weeks of rest in 12 patients with hemophilia A and four patients with hemophilia B. Plasma factor VIII and IX levels, frequency and type of bleeding episodes, amount of factor concentrate infused, fibrinogen, fibrinolysis assays, antithrombin III, liver function, and immune parameters were followed. During the danazol phase a minimal increase was noted in the average clotting factor levels, an increase that, although statistically significant, was of hemostatically marginal magnitude. Significant increases in protein C and plasminogen levels, however, were observed during the danazol period, suggestive of danazol-mediated enhanced fibrinolysis. Clinically, bleeding frequency was significantly increased, and more clotting factor was consumed during the danazol period. Furthermore, eight episodes of hematuria and oral mucosal bleeding was reported during the danazol phase in contrast to only one episode of hematuria during the placebo phase, consistent with an enhancement of fibrinolytic activity with danazol. We conclude that danazol does not have a hemostatically significant effect on plasma levels of factor VIII and IX but may be associated with enhancement of fibrinolytic activity, resulting in increased bleeding frequency and requiring more clotting factor infusions. Therefore, danazol is not a viable alternative in the treatment of hemophilia.

Blood Coagulation Factors↗

Hypersensitivity reaction to a metabolite of cyclophosphamide.

Generalized urticaria and fever were noted in a patient with IgA (kappa) myeloma after intravenous cyclophosphamide. Intradermal skin testing revealed no reaction to cyclophosphamide and its analog, isophosphamide. However, phosphoramide mustard, a principle metabolite of cyclophosphamide, evoked an immediate wheal-and-flare response. Subsequent therapy with isophosphamide was well tolerated. These findings suggest that acute hypersensitivity reactions to cyclophosphamide are due to its metabolites and can be delineated with skin testing.

Cyclophosphamide↗

Blood: use and abuse of whole blood and fractions.

Guidelines for the replacement of blood and various components of blood in cases of hemorrhage, anemia, thrombocytopenia, granulocytopenia, and coagulation disorders are presented. Complications of various therapies, such as the transmission of hepatitis B in the transfusion of pooled plasma, and methods of calculating the number of units of a particular component needed in a particular patient are considered.

Anemia↗

Nonsteroidal anti-inflammatory drugs in the treatment of hemophilic arthropathy.

This study was undertaken to investigate the safety and efficacy of nonaspirin, nonsteroidal anti-inflammatory drugs (NSAIDs) in controlling the pain and stiffness of hemophilic arthropathy. Data were collected from eight adult hemophilic patients in successive double-blind controlled studies using choline magnesium trisalicylate (CMT) and ibuprofen (IPF). In seven patients, neither drug consistently affected bleeding times, platelet aggregation studies, frequency of joint hemorrhages, or frequency of factor infusions. In one patient, platelet aggregation appeared to be affected by CMT and bleeding time became markedly prolonged after IPF therapy. Three patients taking CMT and four patients taking IPF reported greater relief of pain and stiffness with the drugs than with placebos. Our results suggest that IPF and CMT can benefit the hemophiliac and that they can be used safely in most hemophilic patients under closely supervised conditions.

Adult↗

Response of blood coagulation parameters to elevated endogenous 17 beta-estradiol levels induced by human menopausal gonadotropins.

To elucidate the relationship between estrogen and thrombosis, we studied blood coagulation parameters in women whose ovaries were stimulated with human menopausal gonadotropins (hMG). Daily hMG administration over 1 to 2 weeks in seven anovulatory women increased plasma 17 beta-estradiol levels fivefold over the pretreatment value. Of the coagulation parameters, the fibrinogen level increased significantly from an initial value of 248 +/- 11.7 mg/dl (mean +/- SEM) to 353 +/- 32.2 mg/dl after hMG treatment (P less than 0.05), with a significant positive correlation between estrogen and fibrinogen levels (r = +0.762). In addition, a thrombokinetics study showed that the maximal rate of change in optical density of the prothrombin time and activated partial thromboplastin time was significantly increased, suggesting that the coagulation factors involved in extrinsic, intrinsic, and common pathways could be increased by estrogen. Antithrombin III levels decreased gradually during hMG administration. Thus, increased endogenous estrogen levels appear to induce the so-called "hypercoagulable state" through both an increase in coagulation factors in the coagulation cascade system and a decrease in antithrombin III, a potent natural inhibitor of activated coagulation factors. Patients on a regimen of hMG treatment for induction of ovulation serve as excellent models for the study of alteration of "natural" estrogen-mediated coagulation parameters.

Adult↗

Prevalence of type B and non-A, non-B hepatitis in hemophilia: relationship to chronic liver disease.

To assess the relationship of liver dysfunction and hepatitis markers in hemophilic patients treated with factor VIII or IX concentrates, we studied 103 patients with hemophilia A and B for 6-36 mo. Elevated serum alanine aminotransferase was noted in 79% of the patients, with 51% of the patients showing persistent elevation for longer than 6 mo. Thirteen patients (12%) were HBsAg-positive, with 8 patients showing persistence of HBsAg and abnormal serum alanine aminotransferase for more than 6 mo. Overall, anti-HBs was detected in 77% of patients. Twelve episodes of acute hepatitis were documented in 10 patients during 36 mo. Six episodes were due to hepatitis B virus. The remaining 6 episodes were due to non-A, non-B hepatitis with negative HBsAg and absence of seroconversion to hepatitis B virus, hepatitis A virus, cytomegalovirus, and Epstein-Barr virus. In the six episodes of non-A, non-B hepatitis, the incubation period was less than 10 days in 3 patients and 30 days in 2 patients. In all cases with non-A, non-B hepatitis, the illness was symptomatic, but mild. Serum alanine aminotransferase returned to normal within 4 mo in 2 patients, but in 3 patients serum alanine aminotransferase persisted longer than 6 mo. One patient developed an acute B hepatitis 40 wk after non-A, non-B hepatitis. Thus, infection with the hepatitis B virus still remains prevalent as a cause of acute hepatitis in hemophiliacs receiving commercial factor concentrates, and accounts for chronic liver dysfunction in patients with persistent HBs antigenemia. In addition, acute non-A, non-B hepatitis, appears to be relatively common in hemophiliacs, and non-A, non-B virus may account for many cases of persistent liver dysfunction in these patients.

Acute Disease↗

Musculoskeletal problems in hemophilia.

Spontaneous deep muscle and joint bleeding is a common characteristic of severe hemophilia, often causing damage to the musculoskeletal system. The development of concentrated replacement factors, however, has made home care programs possible, helped prevent deformity, and improved surgical management.

Bone Diseases↗