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Biomedical subjects

J A Spero

Publications and source records attributed to J A Spero.

At least 19 recordsLinked to original sources
PubMed1992

Repeated dose administration of desmopressin acetate in uncomplicated cardiac surgery: a prospective, blinded, randomized study.

The effects of single or repeated doses of desmopressin on blood loss were examined in uncomplicated cardiac surgery, while assessing the potential for thrombogenic side effects. Seventy patients undergoing elective coronary artery bypass grafting (CABG) were studied. Patients were randomized into three blinded groups: Group I received DDAVP (0.3 micrograms/kg), IV, after cardiopulmonary bypass (CPB) and 12 hours later in the Intensive Care Unit (ICU); Group II, DDAVP (0.3 micrograms/kg), IV, after termination of CPB and saline (placebo) 12 hours later in the ICU; Group III, saline (placebo) IV after CPB and 12 hours later in the ICU. Blood loss and bleeding time decreased for Group I at 24 hours (P < 0.04) when compared to Group III; however, blood product replacement, as well as intraoperative and total blood loss at 36 hours, were not different among treatment and control groups. There were four myocardial infarctions recorded in Group I, two in Group II, and one in Group III. These differences were not found to be statistically significant. It is concluded that in routine CABG the prophylactic use of single or repeat dose DDAVP does not effectively decrease blood loss or blood product replacement.

Bleeding Time↗
PubMed1989

Comparative efficacy of nonheated and heat-treated factor IX complex concentrate in treatment of hemophiliacs with inhibitors.

To determine if heat-treated factor IX complex concentrate is as effective as nonheated factor IX complex concentrate for treatment of subjects with hemophilia A and antifactor VIII antibodies (inhibitor patients), we have retrospectively reviewed consecutive home treatment records of ten inhibitor patients who had been receiving nonheat-treated factor IX complex concentrate (NHT-Konyne) and subsequently converted to heat-treated factor complex concentrate (Konyne-HT) when it was licensed in late 1984. Overall, 162 of 284 (57%) separate bleeding episodes treated with NHT-Konyne and 53 of 80 (66.3%) separate bleeding episodes treated with Konyne-HT required only one treatment course of approximately 60-75 U/kg. The distribution of bleeding sites and the absolute factor IX unitage required per treatment episode were similar for both preparations. These data suggest that the percentage of hemophilic inhibitor patients responding to factor IX complex concentrate remains at least 50%, as was reported several years ago in a controlled study, and that inhibitor bypass activity has not altered by heat treatment.

Antibodies↗
PubMed1989

Liver transplantation: intraoperative changes in coagulation factors in 100 first transplants.

Six intraoperative blood samples were obtained at intervals from each of 100 individuals undergoing their first liver transplants. The patients fell into the following diagnostic categories: postnecrotic cirrhosis 28, primary biliary cirrhosis 20, sclerosing cholangitis 19, miscellaneous diseases 14, carcinoma/neoplasia 12 and fulminant hepatitis 7. Coagulation factor values in the initial (baseline) blood samples varied by patient diagnosis. In general, all factor levels were reduced except factor VIII:C, which was increased to almost twice normal. The slight intraoperative changes in factors II, VII, IX, X, XI and XII suggested that a steady-state relationship existed between depletion (consumption/bleeding) and repletion (transfusion, transit from extra- to intravascular space), even in the anhepatic state. In contrast, there were rapid and very significant falls in factor VIII and fibrinogen and a less pronounced decrease in factor V, all reaching their nadirs in early to mid-Stage III. The cause of these coagulation changes appears to be activation of the fibrinolytic system.

Adolescent↗
PubMed1988

Prognostic importance of antibodies to human immunodeficiency virus by recombinant immunoassay and Western blot techniques in HIV antibody-positive hemophiliacs.

Antibodies to specific HIV viral antigens were measured by ELISA recombinant proteins representing gag and env amino acid sequences of human immunodeficiency virus (HIV) (E. I. du Pont de Nemours, Wilmington, DE) and by a Western blot system using biotinavidin detection (Biotech Research Labs, Rockville, MD) on 36 HIV antibody-positive hemophiliacs (HTLV-III ELISA, du Pont) on whom date of seroconversion was known and on whom serial samples where available between 1977 and 1986, representing 2-8 years following seroconversion. The 36 included 9 acquired immunodeficiency syndrome (AIDS) and 27 non-AIDS (7 AIDS-related complex (ARC); 4 other HIV class IV, 16 asymptomatic) patients. The development of AIDS was preceded 1-4 years by loss or lack of antibody to gag (p15, 24, or 55) and/or to pol (p31, 53, or 64), each p less than 0.001, compared with non-AIDS patients. Correlation between Western blot and recombinant assays was good except in one Western blot p24 (gag) only seroconverter who showed strong reactivity to env by recombinant assay. In conclusion, HIV antibody patterns appear to show prognostic significance in HIV-infected hemophiliacs.

Antibodies, Viral↗
PubMed1987

1986 update of HIV seroprevalence, seroconversion, AIDS incidence, and immunologic correlates of HIV infection in patients with hemophilia A and B.

A cohort of 181 patients with hemophilia A (149) and hemophilia B (32) cared for at the Hemophilia Center of Western Pennsylvania was followed to determine human immunodeficiency virus (HIV) seroprevalence, seroconversion rate, and clinical and immunologic correlates of HIV infection. By December 1986, 82 (45%) were HIV seropositive, and of these, ten (12%) had developed AIDS, 28 (34%) had symptomatic HIV infection (CDC class III, IV), of whom 14 (17%) had AIDS-related complex (ARC), and 44 (54%) had asymptomatic HIV infection (CDC class II). The HIV seropositive group included 82% of those treated with factor VIII concentrate (97% severe, 5% moderate), 48% of those treated with factor IX concentrate (92% severe, 8% moderate), 10% of those treated with cryoprecipitate (67% severe, 33% moderate), and none of those treated with fresh frozen plasma. Based on 77 serially sampled HIV seropositive hemophiliacs (1977 to 1986), peak seroconversion occurred in 1982, with 14% (11 of 77) occurring since 1984. With increasing time from seroconversion, both T4 lymphocyte number and function (the latter measured by growth in soft agar [T colony assay]) progressively declined; T4 number declined to 135 +/- 26/mm3 (SEM), and colony count declined 1193 +/- 537 (control 3851 +/- 387) by 5 years after seroconversion. In those developing AIDS, total T4 fell below 100/mm3 (33 +/- 8/mm3) at diagnosis. In this cohort, the overall AIDS incidence is 5.5% (12% among the HIV seropositive) and in those seropositive 5 or more years, the AIDS incidence approaches 32%.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Liver transplantation in hemophilia A.

Four patients with hemophilia A have undergone liver transplantation in our institution, three successfully. The first was a 21-year-old man with chronic active hepatitis (CAH) in whom the effects of previous abdominal operations prevented the satisfactory technical insertion of the new liver. He died intraoperatively. The second patient was a 15-year-old boy with CAH who began to synthesize factor VIII coagulant activity (F VIII:C) within 18 hours of successful liver transplantation and has continued to do so for almost 2 years (F VIII:C range 0.89 to 3.20 U/mL). The first 2 months of his postoperative course were complicated by infections, but since that time he has done well and has returned to school. The third patient was a 48-year-old man with portal fibrosis and severe ascites. He synthesized F VIII:C (range 0.96 to 1.50 U/mL) within six hours after reestablishment of circulation through the new liver. His postoperative course was complicated by numerous infections, and he died with sepsis and an acquired immunodeficiency-like syndrome 4 months after transplantation. The fourth patient was a 47-year-old mild hemophiliac with CAH who produced adequate factor VIII:C levels following transplantation (range 0.79 to 2.80 U/mL). These patients demonstrate that liver transplantation in hemophiliacs with end-stage liver disease may be lifesaving and results in correction of the F VIII:C deficiency and associated hemorrhagic tendency.

Adolescent↗
PubMed1987

Antibody responses to immunization of patients with hemophilia with and without evidence of human immunodeficiency virus (human T-lymphotropic virus type III) infection.

Antibody responses after immunization with 23-valent pneumococcal polysaccharide and trivalent influenza virus vaccines were evaluated in 30 adults with hemophilia and in 17 healthy controls. The 30 patients with hemophilia included 13 who were human immunodeficiency virus (HIV) antibody positive with acquired immune deficiency syndrome (AIDS) or AIDS-related complex (group 1), 11 who were asymptomatic HIV antibody positive (group 2), and six who were asymptomatic HIV antibody negative (group 3). Sera were obtained before and 4 weeks after immunization, and levels of antibody were measured by enzyme-linked immunoassay or by hemagglutination inhibition assay. All three groups of patients with hemophilia showed significantly higher preimmunization geometric mean titers of antibodies (groups 1 and 2, fivefold, group 3, 2.8-fold higher), with little increase after pneumococcal vaccine, when compared with controls. Defective humoral responses were noted in groups 1 and 2, with depressed antibody responses after influenza vaccine, significantly elevated levels of IgG and IgM, and depressed blastogenic responsiveness to pokeweed mitogen. Group 3 demonstrated normal responses to pokeweed mitogen, normal antibody responses to influenza vaccine, and normal level of IgG and IgM, although levels of IgG and IgM were higher than those of controls. These data suggest that humoral immune abnormalities are found frequently in patients with hemophilia who are HIV antibody positive. Further, prolonged administration of blood products, regardless of the recipient's HIV status, appears to be associated with polyclonal activation of B cells for T-independent but not T-dependent antigens.

Acquired Immunodeficiency Syndrome↗
PubMed1986

Acute renal failure due to high-grade obstruction following therapy with epsilon-aminocaproic acid.

An 18-year-old man with mild factor VIII deficiency developed hematuria and, subsequently, acute renal failure due to high-grade urinary obstruction by clots during therapy with cryoprecipitate, epsilon-aminocaproic acid, and acetazolamide administered for ocular trauma. Discontinuation of therapy with the latter two agents and induction of a brisk diuresis with intravenous (IV) fluid therapy resulted in return of renal function concomitant with spontaneous clot passage. A review of previous literature suggests that hemophiliacs may be more susceptible than nonhemophiliacs to high-grade urinary obstruction due to clot formation when epsilon-aminocaproic acid is administered during episodes of hematuria. Acute flank pain, fever, and delayed dense nephrograms on IV pyelogram are characteristic of the syndrome and distinguish it from other forms of acute renal failure associated with epsilon-aminocaproic acid.

Acute Kidney Injury↗
PubMed1986

General surgery in adult hemophiliacs.

From 1976 to 1984, 23 operations were performed on 22 patients with hemophilia (18 patients with factor VIII and four with factor IX deficiency). Elective procedures included resection of abdominal aortic aneurysm, liver transplantation, vagotomy/pyloroplasty, insertion of Mousseau-Barbin tube, colectomy, cholecystectomy, inguinal herniorrhaphy (four patients), colonoscopy/polypectomy, mediastinoscopy, arteriovenous fistula for dialysis, anal fistulectomy, and miscellaneous skin and soft-tissue procedures (five patients). Emergency operations were appendectomy (two patients), repair of bleeding liver biopsy site, and repair of an incarcerated inguinal hernia. There were two deaths (9%) within 30 days of operation, neither directly caused by the coagulopathy. Four patients had bleeding after surgery, which was treated with additional cryoprecipitate or factor concentrate. There were no nonhemorrhagic complications. Before operation, appropriate replacement therapy with factor VIII concentrate, cryoprecipitate, or fresh-frozen plasma was provided. Coagulation factor levels were measured before operation and monitored daily after operation. Generally, factor levels were raised to at least 1.0 U/ml and maintained at greater than 0.5 U/ml for 7 to 14 days after operation. However, when patients were treated with fresh-frozen plasma, plasma exchange was performed and factor levels of approximately 0.35 U/ml were achieved before surgery. We conclude that operations in patients with hemophilia can be accomplished safely with careful monitoring of coagulation factor levels and appropriate replacement therapy.

Adolescent↗
PubMed1986

AIDS retrovirus antibodies in hemophiliacs treated with factor VIII or factor IX concentrates, cryoprecipitate, or fresh frozen plasma: prevalence, seroconversion rate, and clinical correlations.

Antibodies to the AIDS retrovirus, specifically to human T cell lymphotropic virus, type III, and AIDS-associated retrovirus, were detected with increasing prevalence in a population of 190 hemophiliacs from western Pennsylvania between 1981 and 1984: 7.7% in 1981, 20.0% in 1982, 45.5% in 1983, and 62.5% in 1984. The seropositive included approximately three fourths of those receiving factor VIII concentrate, nearly one third of those receiving factor IX concentrate, nearly one fifth of those receiving cryoprecipitate, and none of those receiving fresh frozen plasma. The seroconversion rate, determined on 43 seropositive hemophiliacs from this group who were serially sampled, was 0% in 1977, 4.7% in 1978, 4.9% in 1979, 2.6% in 1980, 10.5% in 1981, 52.9% in 1982, 87.5% in 1983, and 100% in 1984. Of 27 seropositive for three or more years (since 1982 or before), four (15%) have developed AIDS and seven (26%), diffuse lymphadenopathy (ARC); of 16 seropositive for less than three years, none has developed AIDS and three (19%) have developed ARC. The mean time from seroconversion to onset of ARC, 0.8 +/- 0.2 years (SEM), was shorter (P less than .001) than the time to onset of AIDS, 4.1 +/- 0.6 years. These findings confirm the widespread presence of AIDS retrovirus and support the association of these retroviruses with the acquired immunodeficiency syndrome and related conditions.

Acquired Immunodeficiency Syndrome↗
PubMed1985

Patterns of gastrointestinal hemorrhage in hemophilia.

Peptic ulcer has been reported to be the cause of bleeding in 53%-85% of hemophiliacs with gastrointestinal hemorrhage (GIH). The management of GIH in hemophiliacs during the past decade has been affected by the availability of plasma concentrates, an increasing occurrence of chronic liver disease, and widespread use of endoscopic procedures. To determine the present patterns of GIH, we reviewed our experience at the Hemophilia Center of Western Pennsylvania during the last 10 yr. Twenty-five (10.3%) of 243 hemophiliacs experienced 41 episodes of GIH. The severity of hemophilia and a history of retroperitoneal hemorrhage were significant risk factors for GIH. Duodenal ulcer (22%), unknown site (22%), and gastritis (14%) were the three most common diagnoses. The use of fiberoptic endoscopy resulted in the recognition of diagnoses such as gastritis, esophagitis, Mallory--Weiss syndrome, and esophageal varices. Red cell transfusion requirements of hemophiliacs with GIH were no different than those of nonhemophiliacs with GIH (p greater than 0.05). The amount of factor VIII replacement used by hemophiliacs with GIH correlated with the severity of gastrointestinal bleeding (p less than 0.01), but not with the cause of gastrointestinal bleeding (p greater than 0.05). In conclusion, hemophiliacs develop GIH secondary to a variety of causes as do nonhemophiliacs. Fiberoptic endoscopy, after correction of factor VIII level to 0.40 U/ml, is a safe and valuable diagnostic procedure in hemophiliacs. The specific etiology of GIH in hemophiliacs should be aggressively sought and appropriate specific therapy provided.

Adolescent↗