Hereditary disorders of the protein C system in central artery and branch arteriolar occlusions]

BACKGROUND: Resistance to activated protein C (APC) is the most common hereditary defect in patients with venous thrombosis. There are conflicting reports on the prevalence of APC resistance in patients with arterial thrombosis, e.g. coronary arteries, compared to the APC resistance prevalence among the normal population. The prevalence of APC resistance in branch retinal artery occlusion (BRAO) and central retinal artery occlusion (CRAO) is unknown. PATIENTS AND METHODS: 29 consecutive patients with arterial retinal occlusions (BRAO, n = 12; CRAO, n = 17) were included in this prospective study over a 23-months-period. We searched for APC resistance, protein C or S deficiencies, as well as for acquired vascular risk factors. Factor-V-deficient plasma and genetic analysis with a PCR method were employed for APC resistance determination. Protein C and protein S activity were determined with functional tests. RESULTS: APC resistance was found in 3 of 29 patients (10.3%). Two of these patients had BRAO and one patient CRAO. Comparing this prevalence to the APC resistance prevalence within the normal population (9%), the difference was not statistically significant. 27 patients (93.1%) had one or more vascular risk factors (arterial hypertension = 19 [65.5%], hyperlipidaemia = 14 [48.2%], smoking = 7 [24.1%], diabetes mellitus = 5 [17.2%], carotid artery stenosis = 5 [17.2%]). CONCLUSIONS: We could not find an increased prevalence of APC resistance in patients with CRAO or BRAO when compared to the normal population.     

Retinal vascular occlusion and deficiencies in the protein C pathway.

PURPOSE: To report abnormalities in the protein C pathway and other vascular occlusion risk factors in patients with retinal vascular occlusion. METHODS: In a study, we investigated 76 consecutive patients who had in-patient evaluation of venous or arterial retinal vascular occlusion. All patients underwent comprehensive tests for coagulation disorders including determinations of protein C, protein S, lupus anticoagulants, and resistance to activated protein C and were screened for vascular disease risk factors. Resistance to activated protein C was confirmed by a polymerase chain reaction method to detect the specific factor V R506Q mutation. For comparative purposes, we also screened 209 consecutive inpatients with deep vein thrombosis from the same geographic region for resistance to activated protein C as well as protein C and protein S deficiencies. RESULTS: Ten (29%) of 35 patients with central retinal vein occlusion (CRVO) had factor V R506Q mutation. The factor V R506Q mutation was detected in four (19%) of 21 patients with branch retinal vein occlusion. The higher frequency in factor V R506Q mutation compared with the expected 9% mutation prevalence in a white population was highly significant for the central retinal vein occlusion group but not for the branch retinal vein occlusion group. In all patients with resistance to activated protein C, the factor V R506Q mutation was detected; 16 were heterozygous, one homozygous. No cases of lupus anticoagulants, protein C, or protein S deficiencies were detected. Forty (19%) of 209 patients with deep vein thrombosis were carriers of the factor V R506Q mutation. CONCLUSIONS: The prevalence of the factor V R506Q mutation is similar in patients with central retinal vein occlusion and patients with deep vein thrombosis and represents a relevant risk factor. Screening for this mutation is therefore recommended in all patients with central retinal vein occlusion.     

A large-scale, short-term, media-based weight loss program

BACKGROUND. This article describes the evaluation of a media-based weight loss and nutrition program. METHODS. Fifteen broadcasts were aired on a Chicago television news program over a three-week period in November of 1986. Some participants (n = 37) received the television program and an accompanying manual, and some (n = 37) received, in addition to the television and media interventions, encouragement to attend self-help groups dealing with obesity. RESULTS. Repeated measures analysis of variance tests were performed, and planned comparisons were conducted only if main effects were significant. At posttesting, those participants attending the self-help groups lost an average of more than nine pounds, whereas those provided only the television program and manual had decreased by less than a pound. Those attending the groups had significantly decreased their percent of dietary fat intake, significantly increased aerobic exercise, and had significantly more hopefulness, motivation, and stimulus control. DISCUSSION. The findings suggest that short-term mass media programs by themselves were probably not very effective, but when supplemented by a self-help manual and support groups may be able to produce significant short-term weight loss.     

Continuous infusion cisplatin and etoposide chemotherapy for cancer of unknown primary site (CUPS) in Taiwan, a region with a high prevalence of endemic viral infections

BACKGROUND: To evaluate the efficacy and toxicity of cisplatin/etoposide continuous infusion chemotherapy for cancer of unknown primary site in Taiwan, a region with a high prevalence of endemic viral infections. METHOD: Between April 1994 and February 1996, 20 patients with a diagnosis of CUPS were treated, including 15 males and five females, of average age 63.3 years (range 41-83 years). Continuous intravenous infusion of etoposide 80 mg/m2 and cisplatin 25 mg/m2 was given for 3 days every 3 weeks. Pretreatment tumor marker and viral serology studies were performed for baseline evaluation. Nearly two-thirds of the patients had poorly differentiated carcinoma. The average number of metastatic sites was 2.65 (range 1-4), with liver and lymph node involvement predominating. RESULTS: The overall response rate was 25% (95% CI 17.7-32.3%); 30.7% for poorly differentiated cancers and 25% for well differentiated cancers. Median survival was 4 months (range 1-12 months), 4.8 months for patients attaining partial response. Toxicity was moderate, grade 3 and 4 neutropenia occurred in 55% and grade 3 and 4 thrombocytopenia in 40%; other toxicities were mild. CA125 and CA199 were elevated in more than 50% of patients. Viral serology studies were not significantly different from those of the indigenous population. CONCLUSION: Etoposide and cisplatin combination chemotherapy has modest activity in patients with extensive CUPS and, at the schedule and dosage given, it is associated with moderate toxicity.      

Validating the EQ-5D with time trade off for the German population

The aim of this survey study was to derive the societal values of the general public for the EuroQol EQ-5D. Using the same protocol as previously used in the United Kingdom, we compared the German values with the British. In face-to-face interviews a sample of 339 individuals in northern Germany valued 15 different health states from a sample of 36 states. Values were derived using the York MVH protocol for time trade-off (TTO) and a visual analogue scale (VAS). Values for all 243 health states of the EQ-5D were estimated by a regression model. The VAS values revealed close a resemblance to the British VAS results. German TTO values were higher than the British. This was especially the case for the worse health states. The results suggest that the TTO values are more related to national variables than values derived by VAS. The use of the TTO values of this investigation makes it possible to anticipate these cultural differences in studies carried out in Germany.