COPART Risk Score, Endothelial Dysfunction, and Arterial Hypertension are Independent Risk Factors for Mortality in Claudicants.

OBJECTIVE: The COPART risk score consists of six variables to assess the prognosis of PAOD patients. The flow mediated dilation (FMD) quantifies endothelial function. The aim of this study was to evaluate the mortality prediction of these two variables in a long-term observation of claudicants. METHODS: 184 consecutive claudicants were included in a prospective observational study over a median observation period of 7.9 (IQR 7.2-8.7) years. The endothelial function was assessed on the day of study inclusion using brachial FMD. RESULTS: Three groups were assigned according to the COPART risk score: low risk (LR), n = 72 (39%); medium risk (MR), n = 59 (32%); and high risk (HR), n = 53 (29%). Overall survival rates differed among COPART risk score groups (p < .001, 5 year survival: LR group 83% [95% CI 74-92%]; MR group 73% [95% CI 62-84%]; HR group 57% [95% CI 43-70%]). Survivors had a significantly better median FMD than non-survivors (4.1% [IQR 1.2-6.4] vs. 1.3% [IQR 0.0-4.2]; p < .001). Also the FMD differed significantly among the three COPART risk groups (LR 4.0% [IQR 1.2-6.3], MR 2.3% [IQR 0.0-6.3], HR 1.7% [IQR 0.0-3.6]; p = .033). Finally, independent predictors for disease specific survival were COPART risk score (p = .033; MR group [HR 1.6], 95% CI 0.7-3.6; HR group [HR 2.7], 95% CI 1.2-5.8), FMD (p = .004; FMD ≤2.5 vs. >2.5, HR 2.6, 95% CI 1.4-4.9), and arterial hypertension (p = .039; HR 3.5, 95% CI 1.1-11.3). CONCLUSIONS: COPART risk score, FMD, and arterial hypertension are independent long-term mortality predictors in this group of claudicants. The best mortality assessment is provided by including all three predictors.

COPART Risk Score Predicts Long-term Mortality in Peripheral Arterial Occlusive Disease.

OBJECTIVES: The COhorte de Patients ARTériopathes (COPART) Risk Score is a risk score assessing the 1 year outcome of patients who received inpatient treatment because of their peripheral arterial occlusive disease (PAOD). The COPART Risk Score consists of six variables each of which is allocated a different number of points (age, history of myocardial infarction, C-reactive protein, ankle-brachial index, estimated glomerular filtration rate, medication with antiplatelet agents, statins and renin-angiotensin system inhibitors). METHODS: 129 consecutive claudicants were included in a prospective trial with an average follow up of 8.8 (± 0.7) years. All patients were hospitalized for their first endovascular procedure to the pelvic and/or femoropopliteal arteries. The endpoints were all cause mortality and cardiovascular (CV) death. The COPART Risk Score was calculated for the three patient cohorts (low risk: 52 patients [40.3%]; medium risk: 41 patients [31.8%]; high risk: 36 patients [27.9%]). RESULTS: During the follow up period 23.1% (n = 12) of patients in the low risk group, 34.1% (n = 14) of patients in the medium risk group, and 63.9% (n = 23) of patients in the high risk group died. CV death occurred in 11.5% in the low, 22.0% in the medium, and 41.7% in the high risk groups. The three groups differed significantly with regard to all cause and CV mortality (p < .0001 and p = .001). CONCLUSIONS: The COPART Risk Score is a suitable instrument to predict long-term all cause and CV mortality in claudicants preceding their first peripheral intervention.

Cystic medial necrosis Erdheim Gsell as a rare reason for spontaneous rupture of the ascending aorta.

Spontaneous rupture of the ascending aorta is a life-threatening condition requiring immediate intervention. The rupture usually leads to sudden death as a result of hemopericardium or hemothorax. The underlying histopathological condition in the cases described so far was mostly an atheromatous plaque. Some other rare underlying conditions were also described. We report a case of cystic medial necrosis Erdheim Gsell as a reason for fatal spontaneous rupture of the ascending aorta.

Are flow-mediated vasodilatation and intima-media thickness of the brachial artery associated with restenosis after endovascular treatment of peripheral arterial occlusive disease?

OBJECTIVE: Restenosis after percutaneous angioplasty of peripheral arteries is still an unsolved matter. Previous studies reported an association between flow-mediated dilatation (FMD), a marker of endothelial dysfunction, and restenosis after coronary angioplasty. This study evaluates the influence of FMD and brachial intima-media thickness (B-IMT) on restenosis after angioplasty of peripheral arteries. METHODS: One hundred and eighty-four patients (124 male) with claudication related to peripheral arterial disease participated in this trial. FMD and B-IMT were assessed before endovascular revascularisation. In a 12-month follow-up duplex ultrasound examinations were performed to detect restenosis. Finally 128 patients (91male, 37 female) were eligible for statistical analysis. RESULTS: Restenosis was found in 54 patients (42.2%). Mean FMD was 3.53 ± 3.56%, with no difference between the patients with restenosis (3.55 ± 3.64%) and those without (3.52 ± 3.48%; p = 0.716). B-IMT had a mean value of 0.326 ± 0.134 mm. B-IMT significantly differed between the patients with restenosis (0.326 ± 0.134 mm) and those without (0.256 ± 0.133 mm; p = 0.007). We confirmed that a B-IMT over 0.21 mm was an independent risk factor for restenosis [OR 2.9 (1.3-6.3)]. CONCLUSION: Endothelial dysfunction is not associated with restenosis. Conversely patients with enlarged B-IMT are at risk of restenosis after angioplasty of peripheral arteries.

Acute wiiitis representing as thrombosis of the inferior vena cava and left pelvic veins.

Deep venous thrombosis as a result of venous wall injury provoked by trauma is a common finding. It often occurs in patients with sportive overstraining, caused by over fatigue of the body structures. In 2007, the entity of "acute wiiitis" was first described in a letter to the New England Journal of Medicine. Acute wiiitis sums up all affections, mainly skeletal and muscle affections, provoked by playing Nintendo Wii, a very common and loved video-game system. Deep venous thrombosis as a consequence of Nintendo Wii has not been described so far. We present a patient with a massive free floating thrombus of the left pelvic veins originating from the gluteal veins and reaching into the inferior vena cava after playing Nintendo Wii.

Distinct levels of genetic diversity of Borrelia burgdorferi are associated with different aspects of pathogenicity.

Different species of pathogenic Borrelia show different symptoms and tick vector specificity. Even within regions where only one species is found, Lyme disease progresses very differently from one patient to another. Since Borrelia shows very little recombination either within or between species, alleles of a gene can be used to mark clones. The ospC gene is highly variable within each species and can be used to define groups of related clones. It has been previously shown that only four out of seventeen ospC groups of Borrelia burgdorferi sensu stricto cause invasive forms of the disease. Other groups cause erythema migrans, a skin rash at the site of the tick bite, but not invasive disease, while still other groups seem to be nonpathogenic to humans. In this study we extend the analysis of the ospC gene to the other pathogenic species, Borrelia garinii and Borrelia afzelii. Only two groups in B. afzelii and four groups in B. garinii cause invasive disease. Thus, only ten out of the 58 defined ospC groups cause invasive and presumably chronic Lyme disease.

Cases of Lyme borreliosis resistant to conventional treatment: improved symptoms with cephalosporin plus specific beta-lactamase inhibition.

We present four cases of verified late Lyme borreliosis with persistent symptoms and positive serology despite repeated courses of high-dose intravenous penicillin G and/or cephalosporins (including cefoperazone). The patients were now treated with cefoperazone 2 g plus sulbactam 1 g bid iv for 14 days. At the end of treatment, patients were symptom free and have remained so for the following 12 months. By then, IgG against Borrelia burgdorferi had decreased. It is concluded that the addition of beta-lactamase inhibitors to intravenous treatment could be beneficial in Lyme disease refractory to conventional treatment.

Infection with multiple strains of Borrelia burgdorferi sensu stricto in patients with Lyme disease.

OBJECTIVE: To assess human skin biopsy specimens from erythema migrans lesions for the presence of infection with multiple strains of the Lyme disease spirochete, Borrelia burgdorferi. DESIGN: Skin biopsy specimens were obtained prospectively from patients with erythema migrans. To determine allelic differences and strain identification of B burgdorferi, the biopsy specimens were analyzed by cold single-strand conformation polymorphism of an amplified fragment of the outer surface protein C (ospC) gene. Further single-strand conformation polymorphism patterns of amplified ospC genes from culture isolates were compared with polymerase chain reaction products obtained directly from erythema migrans biopsy specimens. SETTING: A private dermatology office and a university medical center outpatient department. PATIENTS: Sixteen patients presenting with erythema migrans. RESULTS: Two of the 16 patients in this cohort were infected with 2 B burgdorferi sensu stricto strains, as evidenced by 2 ospC alleles in their skin biopsy results. CONCLUSION: This is the first documented description of the existence of more than a single strain of B burgdorferi sensu stricto in a human specimen.

Cystic adventitial degeneration of the popliteal artery-the diagnostic value of duplex sonography.

Cystical adventitial degeneration of the popliteal artery is a disorder which is difficult to diagnose, due to the similarity of the symptoms of people presenting with peripheral arterial occlusive disease (PAOD) or popliteal entrapment syndrome. The only thing that differs from patients suffering from PAOD is the lack of typical risk factors for arteriosclerosis. Typical diagnostic procedures like conventional angiography or magnetic resonance Imaging angiography can be negative, too and therefore misleading. The only which is crucial in the diagnosis of cystic adventitial degeneration of the popliteal artery is to know the morphological background of this disorder, namely that it is a cyst of the adventitia of the artery which leads to a dynamic exercise-dependent flow inhibition. We present a 57-year old white male who had a week's history of intermittent claudication in his left calf. He was lacking of typical risk factors for arteriosclerosis and on first examination all pulses in both lower extremities were palpable and Doppler index on both legs was >1. Only duplexsonography revealed a cystic formation impressing the left popliteal artery in the hight of the rift in the popliteal joint.

Oxidative stress during peripheral angioplasty. Implication for late restenosis?

BACKGROUND: Percutaneous transluminal angioplasty (PTA) is routine treatment for patients with peripheral arterial disease (PAD). The procedure induces local generation of reactive oxygen species (ROS), such as H2O2. Since these have been shown to stimulate vascular smooth muscle cell growth (VSMCG), we investigated peroxide levels in patients with PAD during PTA and related these results to late clinical outcome. METHODS: Thirty patients (17 male, 13 female, 20 Fontain stage II, 10 Fontaine stage IV, median age 68 years) undergoing PTA of a 2-6 cm stenosis of the femoral or popliteal artery were included. The procedure was performed successfully in all patients. At follow-up six months thereafter restenosis was evaluated by duplex sonography. Total peroxide concentrations were determined in plasma drawn before, 6, 24 and 48 hours after the procedure by the Operoxide activityO assay, which is based on the reaction of horseradish peroxidase with plasma peroxides, using tetramethylbenzidine as the chromogenic substrate. RESULTS: The median peroxide level before angioplasty was 280 mmol/L (range 47-549). Levels were higher in patients with advanced disease, in smokers and in patients with diabetes. In response to angioplasty, peroxide levels increased within 48 hours (p<0.001). Six months after the procedure, restenosis was observed in 10/30 (33 percent) of patients. Clinical outcome was not dependent upon baseline or postinterventional peroxide levels. CONCLUSIONS: Elevated peroxide levels are seen in patients with advanced arteriosclerotic disease and in those with diabetes, but are not predictive for late restenosis.

The PlA1/A2 polymorphism of platelet glycoprotein IIIa is not associated with deep venous thrombosis.

BACKGROUND: Platelet glycoprotein (GP) IIb/IIIa, a fibrinogen and von Willebrand factor binding membrane receptor, has an important role in platelet aggregation. A common leucine33-proline polymorphism (PlA1/A2) of the gene encoding the GP IIIa subunit is associated with platelet reactivity and has been proposed as a risk factor for atherothrombotic disease. The aim of this study was to investigate the role of this polymorphism for deep venous thrombosis (DVT). METHODS: We performed a case-control study including 206 patients with documented DVT and a sex- and age-matched group of 310 control subjects. GP IIIa genotypes were determined by restriction fragment analysis of amplimers containing the polymorphic site. RESULTS: A1/A1, A1/A2 and A2/A2 genotypes were found in 67.0, 31.6 and 1.5 percent of patients and 72.3, 25.8 and 1.9 percent of controls (p=0.35), PlA2 allele frequencies were 0.17 in patients and 0.15 in controls (p=0.92). Odds ratio of the PlA2 allele for DVT was 1.21 (95 percent CI 0.85-1.71, p=0.29) and remained insignificant after adjustment for factor V Leiden and prothrombin 20210A genotypes (1.22, 95 percent CI 0.86-1.75, p=0.27). CONCLUSIONS: Our data suggest that the PlA1/A2 polymorphism of GP IIIa is not associated with DVT.

Genetic evaluation of the common variant of the endothelial nitric oxide synthase (Glu(298)->

BACKGROUND: Nitric oxide is synthesized by endothelial nitric oxide synthase and plays a key role in adequate endothelial function. An important effect is the reduction of free radicals caused by cigarette smoking, which is the only established risk factor for thromboangiitis obliterans (TAO). In patients with TAO a genetic defect of endothelial nitric oxide synthase may therefore result in deteriorated endothelial function. The aim of our study was to evaluate whether a genetic defect of the common variant of endothelial nitric oxide synthase (Glu(298)-->Asp) can be found in a higher degree in patients with TAO compared to healthy controls. METHODS: We enrolled 42 patients with TAO and 149 healthy subjects. RESULTS: Nineteen patients (45.2%) showed homozygosity for Glu(298) versus 76 (51%) in the control group. The heterozygous status for Glu(298)-->Asp was found among 18 patients (42.9%) compared to 61 (40.9%) members of the control group, which was a nearly equal distribution. Homozygosity for the mutant Asp(298) was slightly elevated in the patient group with 11.9 versus 8.1% in the control group. Allele frequency for Asp(298) was 0.333. CONCLUSIONS: Our data do not show elevated homozygosity for the common variant Glu(298)-->Asp in patients with TAO compared to healthy controls. The limitation of our evaluation is the small number of patients, which is a general problem when evaluating patients with TAO, as this is not a common disease.

Hypothenar hammer syndrome caused by posttraumatic aneurysm of the ulnar artery.

The so-called hypothenar hammer syndrome is a rare entity caused by lesions of the ulnar artery secondary to repetitive trauma to the hypothenar eminence, typically found in persons working with vibrating tools. Its clinical symptoms are pain, stiffness and whitening of the smitten fingers, sometimes in combination with Raynaud's syndrome. Angiographic evaluation of the smitten forearm and hand reveals occlusions, kinking, vasospasm and stenoses of the arteries in the hand and fingers. An aneurysm of the ulnar artery causing the hypothenar hammer syndrome is an even more rare morphological finding. The difficult aspect of treating a hypothenar hammer syndrome is to reopen the occluded vessels. Eventually, circulation deteriorates and skin lesions of the fingers may occur. The advantage of an isolated aneurysm of the ulnar artery is that normal circulation can be restored by vascular surgery, for example, with a vessel interponate. Surgical removal of the isolated aneurysm helps to prevent microembolism to the distal arteries and consequent deterioration of peripheral circulation. We report a young patient who presented with clinical symptoms of the hypothenar hammer syndrome and an aneurysm of the distal ulnar artery, diagnosed by magnetic resonance angiography. The only likely cause of the aneurysm was a bicycle accident some months prior to the occurrence of the aneurysm. The patient underwent vascular surgery and has been free of symptoms during six months of follow-up. A control magnetic resonance angiography performed one month after surgery revealed a normal vascular morphology.

Impaired endothelial-dependent and endothelium-independent vasodilatation in patients with thromboangiitis obliterans.

BACKGROUND: Endothelial dysfunction is the key process in the development of atherosclerosis. The aim of our study was to evaluate endothelial dysfunction measured by the noninvasive technique of Celermajer that plays a role in the pathogenesis of thrombangitis obliterans. METHODS: A total of 36 patients with thrombangitiis obliterans ([TAO]; mean age 44.9 ± 1.3 years) were compared with 30 healthy individuals (mean age 36.1 ± 1.8 years). High frequency ultrasound was used to measure changes in response to reactive hyperemia (leading to flow-mediated endothelium-dependent dilatation) and in response to 0.4 mg sublingual nitroglycerin ([NTG]; leading to NTG-induced, endothelium-independent dilatation). RESULTS: Patients with TAO showed a lower but statistically not significant flow-mediated dilatation and a statistically significant reduced NTG-induced vasodilatation than the control group. CONCLUSION: Our results suggest that both mechanisms play a role in patients with TAO, the endothelium-independent impaired vasodilatation even in a more significant way than the impaired endothelium-dependent vasodilatation.

The role of 2-18F-fluoro-2-deoxy-D-glucose positron emission tomography in the diagnosis of giant cell arteritis of the temporal arteries.

OBJECTIVE: As one of the diagnostic criteria for giant cell arteritis affecting the temporal arteries (temporal arteritis) is still biopsy-proven vasculitis of the affected artery, the aim of our study was to evaluate the value of a non-invasive procedure, 2-(18)F-fluoro-2-deoxy-d-glucose (FDG) positron emission tomography (F-18-FDG-PET), in the diagnosis of Horton's disease. METHODS: During a period of 10 months, 22 consecutive patients with the clinical diagnosis of giant cell arteritis and a positive hypoechogenic halo in duplex sonography were re-examined with F-18-FDG-PET. Six patients had giant cell arteritis involving both the large arteries and the temporal arteries; five patients showed giant cell arteritis only in the large arteries without concomitant involvement of the temporal arteries, and the remaining 11 patients showed only involvement of the temporal arteries. All patients were examined by sonography and F-18-FDG-PET, which was performed before treatment with corticosteroids. RESULTS: All patients with positive signs of giant cell arteritis in duplex sonography, i.e. a hypoechogenic halo in the large arteries (thoracic, subclavian, axillary, iliac, aorta), also showed elevated FDG uptake in the same vessels, with complete agreement in the anatomical distribution of changes. When positive sonography was limited to the temporal arteries, FDG-PET was completely negative in the temporal arteries and all other arterial locations. CONCLUSION: PET is not yet suitable for the diagnosis of temporal arteritis and therefore cannot replace invasive biopsy. F-18-FDG-PET is well suited to the demonstration of giant cell arteritis in arteries exceeding 4 mm in diameter.