Antibody nature of an AHG (factor 8) inhibitor.
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Biomedical subjects
Publications and source records attributed to J Shuster.
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The authors hypothesized that patients with anxious or hostile depression may have a greater risk of mortality from coronary artery disease (CAD) than other depressed patients and therefore proceeded to assess the possible relationship between CAD risk factors and anger and anxiety in a sample of 138 depressed outpatients. The authors observed that increased anxiety scores were associated with higher cholesterol levels and with prolonged QTc intervals. Similarly, the patients with anger attacks tended to have higher cholesterol levels compared with the patients without these attacks. The study's findings partially support the authors' hypothesis that hostile or anxious depressed patients are at greater risk for CAD than other depressed patients.
BACKGROUND: Neuroblastoma has many characteristics which suggest that preclinical detection might improve outcome. The Quebec Neuroblastoma Screening Project was initiated to determine whether mass screening could reduce mortality in a large cohort of infants. As an early endpoint, we report whether screening could reduce the incidence of poor-prognosis neuroblastoma in children with advanced-stage disease over 1 year of age. METHODS: All 476,603 children born in the province of Quebec during the 5-year period of May 1, 1989, to April 30, 1994, were eligible for urinary assay of homovanillic acid and vanillylmandelic acid at 3 weeks and 6 months of age. Children with a positive screen were referred to one of four paediatric cancer centres in the province for uniform evaluation and treatment if necessary. Standardised incidence ratios (SIRs) were calculated for neuroblastoma in the province and two similar population-based controls, the state of Minnesota and the province of Ontario, during the same period of time and with similar ascertainment procedures. FINDINGS: Compliance with screening in Quebec province was 91% at 3 weeks (n = 425,816) and 74% at 6 months (n = 349,706). Through July 31, 1995, with a follow-up of the birth cohort of 15-75 months, 118 cases of neuroblastoma were diagnosed, 43 detected preclinically by screening, 20 detected clinically before screening at 3 weeks of age, and 55 detected clinically after 3 weeks of age having normal screens (52) or never screened (3). Retrospective analysis of stored samples confirmed that 49 of 52 patients missed by screening had levels of catecholamine metabolites that were too low to be detected at 6 months or earlier. Based on US Surveillance, Epidemiology and End Results data, 54.5 cases of neuroblastoma would have been expected in Quebec province during the study period, for an SIR of 2.17 (95% CI 1.79-2.57, p < 0.0001). For the two control groups, 43 and 80 cases of neuroblastoma were detected, respectively, compared with 37.9 and 85.4 expected, overall SIR 1.00 (not significant). SIRs for Quebec province by age at diagnosis in yearly intervals show a marked increased incidence under 1 year of age (SIR 2.85, 2.26-3.50), with no reduction in incidence in subsequent years. Limiting analysis to only patients diagnosed over 1 year of age with advanced-stage disease, 22 cases were detected in Quebec province versus 14.4 expected (SIR 1.52, 0.95-2.23). Data in the two control groups show no significant increase or decrease in any-stage disease in children under or over the age of 1 year, except for an increase in early-stage disease in Minnesota children over 1 year: 10 versus 3.8 expected (SIR 2.67, 1.27-4.58). INTERPRETATION: Screening for neuroblastoma increases the incidence in infants without decreasing the incidence of unfavourable advanced-stage disease in older children. It is unlikely that screening for neuroblastoma in infants will reduce mortality for this disease.
Several cases of hemophiliacs with AIDS have been reported. These case reports have prompted studies of asymptomatic healthy hemophiliacs. During these studies, we found four hemophiliacs who presented a distinct clinical syndrome identical to the lymphadenopathy syndrome in homosexuals which has been considered a prodrome to AIDS. Our results demonstrated that the AIDS related complex in hemophiliacs is characterized by recurrent herpes viral infections. Furthermore, the clinical and laboratory data in these patients suggest that this syndrome does not inexorably lead to AIDS, and may be a manifestation of a group of disorders in which AIDS represents one end of the spectrum.
PURPOSE: This study reviewed the Pediatric Oncology Group experience with phase II clinical trials in children (< 21 years of age) with refractory tumors. PATIENTS AND METHODS: Patients registered in Pediatric Oncology Group phase II studies were evaluated. Patients had to be < 21 years of age with recurrent and refractory measurable disease. Tumor types and response rates were determined. Death on therapy from either drug toxicity, progressive disease, infection, or hemorrhage was measured. Tumor-specific, disease-free survival curves were calculated by Kaplan-Meier analysis. RESULTS: Between 1984 and 1994, 2,465 patient entries were made on 45 phase II trials. Malignancies registered included acute lymphocytic leukemia (ALL) (16.7%), acute myeloid leukemia (AML) (12.0%), osteogenic sarcoma (7.8%), neuroblastoma (7.2%), astrocytoma (7.2%), medulloblastoma (7.1%), glioma (6.7%), ependymoma (6.1%), and others (29.2%). The overall response rate was 19.6% (CR + PR) for children entered on phase II trials. Tumor-specific response rates ranged from 62.1% (23/37) for children with Hodgkin's disease to no responses (0/23) in patients with hepatoblastoma. When comparing single versus multiagent trials, a significantly better initial response rate was seen in the latter studies. However, 5-year survival was comparable. Progression-free survival for all tumor histologies were 12.9% and 9.2% at 2 and 5 years, respectively. Death on study was seen in 11.6% of the patients; however, only three deaths were directly related to drug toxicity. There were no significant gender differences in regards to response, progressive disease, or death on study. CONCLUSION: Phase II studies conducted in children offer a considerable likelihood of therapeutic benefit without exposing these patients to untoward toxicity.
In study 1, laboratory and supervised blood or urine test data from actual cases were used to develop patient profiles. Seven diabetologists from the same institution rated the diabetic control of 125 profiles on a four-point scale (1 = poor, 2 = fair, 3 = good, 4 = excellent). Six of the 7 diabetologists demonstrated adequate intra- and interrater reliability. Study 2 assessed the reliability of judgments of diabetic control made by diabetologists working in two different settings. There were 9 raters from institution 1 and 8 from institution 2. The impact of the amount and type of information on judgment reliability was evaluated by developing two types of profiles. The test form contained only laboratory and supervised blood or urine test data similar to that utilized in study 1. The history form contained this information as well as other descriptive data typically available to diabetologists. The 17 diabetologists rated 125 anonymous profiles on each of two separate occasions approximately 1 wk apart. On one occasion they rated profiles presented on the test form. On the other occasion they rated profiles presented on the history form. As in study 1, the diabetologist raters demonstrated adequate intra- and interrater reliability. Intrarater reliability was somewhat better when rating test form profiles compared with history form profiles. Reliability was not higher within than between institutions. An analysis of the relative contribution of different diabetes control indices to the diabetologists' judgments indicated that HbA1 influenced raters' judgments at both institutions more than any other single variable.(ABSTRACT TRUNCATED AT 250 WORDS)
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Reticuloendothelial system Fc receptor function was measured in 10 patients with systemic lupus erythematosus (SLE) taking corticosteroids, 10 SLE patients not receiving corticosteroids and in 19 controls, 9 of whom were corticosteroid-dependent asthmatics and 10 of whom were healthy. Clearance studies were performed using autologous 51 Cr-labeled erythrocytes that had been sensitized with human IgG anti-Rh(D) [6,700 molecules/cell]. The clearance curves could be split into a fast (t1/2 fast) and a slow (t1/2 slow for any of the groups of patients. The results were correlated with serum C3, C4, DNA-binding, fluid phase 125I-Clq binding, a disease activity index, corticosteroid dose and duration of therapy with corticosteroids. The only significant correlation was an inverse correlation of C4 with the t1/2 slow in SLE patients not on corticosteroids (r=- 0.71, p less than 0.05). The t1/2 slow of the 3 SLE patients with active nephritis (86 +/- 40 min) was significantly different from the 17 SLE patients with inactive nephritis or normal renal function (37 +/- 5 min) (P less than 0.05). We conclude that there is no overall defect of Fc receptor function in our patients with SLE although there is decreased clearance in patients with active lupus nephritis.