Search

Objective: The objectives of this article are to review the data about the consequences of thrombophilia testing and to think about its indications., Current Knowledge and Key Points: The indications of congenital thrombophilic testing have extended since the discovery of prevalent abnormalities, such as mutations of factor V or II genes. However, thrombophilia does not result in a significant increase in the risk of recurrence unlike the spontaneous occurrence of thrombotic events. The factor V Leiden mutation is associated with a moderate increase in recurrence rate, while the G20210A mutation of factor II is not associated with a significant increase in recurrence. Regarding the decrease in natural anticoagulants is concerned, there is no definite conclusion, although the decrease in antithrombin is suspected of being associated with an increase in recurrence., Future Prospects and Projects: Finally, identification of a constitutional thrombophilia most often do not influence the therapeutic decisions unless some rare abnormalities are found, such as a decrease in antithrombin, homozygous mutations in factors V or II genes or associations of thrombophilia. One must remember that antiphospholipid antibodies must be searched because their impact on recurrences is well-known. Diagnostic work-up for thrombophilia is not useful after a distal or a superficial venous thrombosis (except for antiphospholipid antibodies in case of distal venous thrombosis).

Introduction: Psychiatric adverse effects of fluoroquinolones are known for long, but can sometimes be missed. We analyse cases spontaneously reported to the French pharmacovigilance., Methods: Cases of psychiatric adverse effects with fluoroquinolones reported to the French pharmacovigilance system were analysed. The studied period was from January 1985 and June 2002. Data analysed included age, sex, adverse effect, fluoroquinolone, seriousness and evolution., Results: Five hundred ninety cases have been reported concerning 273 males and 316 females (sex unknown in 1 case). Mean age was 66 years (median: 70, range: 12-102). The most frequently reported psychiatric adverse effects were confusion (51%), hallucinations (27%), agitation (13%), delusion (12%), insomnia (8%), somnolence (4%) (several adverse effects could be associated in a single patient). Serious cases represented 21.7% (resulting in hospitalisation in most cases). Evolution was favourable in most cases (88.5%), and was unknown in 9.5% of cases., Conclusion: The number of cases reported during this period is moderate, but under-reporting probably interferes. The eventuality of this kind of adverse effect with fluoroquinolones should be kept in mind. Dose should be adjusted to renal function, especially in older patients.

Introduction: Painful bruising syndrome was described by Gardner and Diamond in 1955. It is marked by spontaneous bruising, without any biological abnormality, affecting young women with pathological mental context., Exegesis: We report three observations with painful bruising syndrome. In a patient, psychotherapy induced improvement in dermatological and articular manifestations. In other case, placebotherapy made clinical symptoms go away for a prolonged period., Conclusion: Some etiological hypotheses have been postulated for Gardner and Diamond syndrome. However, published cases speak in favour of psychogenic hypothesis. Somatic and psychological approach must be offered to these patients.

Introduction: The association between adult Henoch-Schonlein purpura and prostatic carcinoma is exceptional. We report a new case., Exegesis: The diagnosis of prostatic adenocarcinoma, suspected because of prostatic induration is made after anatomopathological study. The patient has all the clinical (purpura, polyarthralgia), biological (circulating monocmonal IgA), and histological (leukocytoclastic vasculitis, with IgA and C3 deposits) criteria of Henoch-Schonlein purpura too. The simultaneous appearance of vasculitis and neoplasia is known. The association between adult Henoch-Schonlein purpura and malignant neoplasm like lung carcinoma or lymphoma exists too. The initial events leading to the development of these vasculitis could be tumor antigens or abnormal IgA production., Conclusion: The simultaneous appearance of these two disease asks here the question of mechanisms implicated in these association.

Introduction: Distensibility is the ability of large elastic arteries to increase in diameter from diastole to systole. Pulse wave velocity (PWV) is one of the ways to measure this parameter. Several techniques, including QKd, are able to measure PWV., Purpose: QKd is the time interval between the Q wave on EKG and auscultation of the second Korotkoff's sound at the brachial artery. QKd is measured by a specific apparatus that registers ambulatory blood pressure as well as EKG (normal > 200 ms). Arterial distensibility seems to be able to predict cardiovascular morbidity and QKd has been demonstrated to predict such morbidity in a sample of elderly hypertensives. Currently the relationship between QKd and prognosis is under investigation in systemic sclerosis (ERAMS study)., Conclusion: QKd is a noninvasive ambulatory method that measures arterial distensibility as well as blood pressure.

Purpose: Aortoduodenal fistulas are the most frequent aortoenteric fistulas. They may be primary (occurring after aneurysms of the native aorta) or secondary (occurring after aortic prosthesis). Aortoduodenal fistulas are a rare complication of aortic prostheses. They may be caused by prosthesis infection or due to inadequate prosthesis., Methods: We report seven observations that emphasize issues pertaining to either diagnosis or therapy., Results: The delay of occurrence is variable, with a mean of 3 years as reported in the literature. Clinical picture includes upper digestive tract hemorrhage, sometimes fever, abdominal pain or mass. Though difficult, diagnosis can be achieved through gastric endoscopy or CT-scan. Additional diagnostic procedures are often not useful and should not be numerous. Surgical procedures help guide the diagnosis and constitute the main part of the treatment with suture of the duodenum and vascular prosthesis. According to previous works, our observations including prolonged follow-up of the patients suggest that the best vascular treatment is extra-anatomic axillo-bifemoral bypass, while simple suture and prosthesis replacement lead to poor results., Conclusion: Mortality related to aortodigestive fistulas is high (five out of seven patients in the present study) and requires prevention, including more particularly delicate surgery and antibiotic therapy in case of any episode of infection. Aortoduodenal fistulas must be suspected whenever a patient with aortic prosthesis has digestive bleeding or unexplained fever.

Introduction: Familial auto-immune hepatitis is unusual. We report a case in which hepatitis was associated with a deficiency in the C4 component of the complement., Exegesis: Type 1 auto-immune hepatitis A was diagnosed in a 38-year-old woman presenting with systemic lupus erythematosus. Her daughter had to undergo a splenectomy for immunologic thrombocytopenic purpura when she was 9 years old. When she turned 13, she further developed type 1 autoimmune hepatitis. During follow-up (4 and 8 years, respectively), both patients had a mild deficiency in C4., Conclusion: C4 deficiency is not only frequently observed in relatives of patients with auto-immune hepatitis, but also in familial systemic lupus. This abnormality may have had a crucial pathogenic role in these two patients.

Introduction: Some viral infections are associated with deep venous thrombosis. We report a case of deep venous thrombosis in an adult with varicella. He had neither known predisposing factors for thrombosis nor thrombophilia., Exegesis: Transient significant level of antiphospholipid antibodies and lupus circulating anticoagulant were observed. There was no evidence of thrombophilia. Deep venous thrombosis has been mostly associated with varicella in children. A transient protein S deficiency was present in almost all cases, though it was sometimes related to an anti-protein S antibody. This association is exceptional in adults. Some viruses such as herpesvirus and HIV are responsible for endothelium dysfunction, but this is still unclear in the case of varicella-zoster virus., Conclusion: In our observation, endothelium activation or antiphospholipid antibodies might be responsible for thrombosis.

Membrane thrombomodulin (TM) is a very efficient natural anti-thrombin glycoprotein with anticoagulant properties expressed on endothelial cell surface. Circulating plasmatic thrombomodulin (TMp) detected by enzyme immunoassay in plasma is considered as a cell marker of endothelial injury. The TMp levels are increased in many conditions (diabetes mellitus, atheromatous disease...). In cases of collagen vascular diseases, where vascular endothelium damage is suspected, TMp is increased particularly in systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). It is noteworthy that the TMp level is correlated with disease activity. Since TMp is a non specific marker of endothelial damage, it may be of interest as a useful marker for the supervision of these diseases. Further studies are needed on larger series. TMp level change during spontaneous evolution or under treatment will help determine wether TMp is a predictor and prognostic marker of these systemic diseases.

The authors describe the third case in the literature of superior mesenteric artery's thrombosis occurring in the course of the Behçet's syndrome according to Mason and Barnes classification. Clinical manifestations in the patient were represented by intestinal angor. The literature data concerning vascular involvement in Behçet's disease, particularly thrombosis are reviewed. The value of noninvasive paraclinic arterial digestive explorations is discussed.

We report on eight cases of amiodarone pulmonary toxicity. Main clinical symptoms are acute/subacute dyspnea or progression in some cases. Amiodarone responsibility is difficult to ascertain. Several arguments can be presented: clinical symptoms with dyspnea and/or fever and/or cough, interstitial or in diffusing capacity for carbon monoxide, abnormal broncho-alveolar lavage cytopreparation smear with increased percentage of lymphocytes and polymorphonuclear leucocytes in typical cases; trans-bronchoscopic lung biopsy failed to provided information on amiodarone toxicity in the two patients where biopsy were performed. Differential diagnosis is an essential step to eliminate other possible causes ie pulmonary micro-organism infections, cancer or pulmonary oedema secondary to heart failure. In one case acute pulmonary toxicity occurred early, after introduction of amiodarone, with a proposed immuno-allergic mechanism. In other cases, chronic amiodarone deposition in the lungs can explain clinico-radiologic features. In six cases improvement was observed after discontinuation of therapy within a 6-months period.

The human immunodeficiency virus (HIV) may be responsible for several types of vasculitis: leucocytoclastic vasculitis, granulomatous angiitis, angiitis associated with lymphoproliferative syndromes or necrotizing vasculitis including periarteritis nodosa (PAN). We report a case of PAN in a 62-year old HIV1-positive woman. The patient had no co-occurrent hepatitis B virus infection and was negative for antinuclear antibodies. She presented with sicca syndrome, necrotic purpura, myalgias and polyneuropathy. Skin, muscle and nerve biopsies showed signs of necrotizing vasculitis. Multiple microaneurysms typical of PAN were present on branches of the abdominal aorta. The symptoms due to vasculitis regressed after treatment with corticosteroids in bolus injections and plasmapheresis. AZT was not given owing to intolerance. The literature on vasculitis associated with HIV infection is reviewed.