[Manubrio-sternal arthritis and behcet's disease: report of 3 cases]. / Arthrite manubrio-sternale et syndrome de Behçet: à propos de 3 observations.

Manubrio-sternal joint involvement in arthritis is described and may be evidenced by erosion, reactive sclerosis and ankylosis. Some reports of such involvement appear in the radiological literature in association with psoriatic arthritis and spondylarthropathy. Involvement of the manubrio-sternal joint in Behçet's disease is very uncommon. To our knowledge, only one case has been described. We report a case of manubrio-sternal arthritis in a women with Behçet's disease. Two others cases with suspicion of Behçet's disease are described.

Epiglottis involvement in a visceral leishmaniasis.

The visceral form of leishmaniasis caused by Leishmania infantum is frequently observed in Mediterranean countries, however otorhinolaryngeal involvement has only rarely been reported in the literature. We report here the case of a 23-year-old Cameroon man, resident in France since 1991, and presenting with recurrent epistaxis and an epiglottis tumoral lesion. The visceral form of leishmaniasis with hepatosplenomegaly and pancytopenia was diagnosed. Culture of the epiglottal lesion revealed L. infantum. Serological test for human immunodeficiency virus was negative and the patient was not on immunosuppressive drugs. Dramatic improvement was observed with antimony.

[Spinal cord compression caused by extramedullary hematopoiesis foci in the course of thalassemia]. / Compression médullaire par des foyers d'hématopoïèse extra médullaire au cours de la thalassémie.

BACKGROUND: Extramedullary haematopoiesis is a physiological response to chronic anemia, observed frequently during homozygous thalassemia. It is usually asymptomatic but can be manifested by compression of adjacent organs, particularly the spinal cord. CASE REPORT: A 44-year-old woman diagnosed with thalassaemia intermedia, was admitted for difficulties to walk and sphincter disturbances. Neurologic examination suggested spinal cord compression, which is confirmed by dorso-lumbar resonance magnetic imaging. The histology obtained by laminectomy led to the diagnosis of extramedullary hematopoiesis related to thalassemia. A radiotherapy enabled with good outcome. DISCUSSION: Spinal cord compressions by extramedullary hematopoiesis during thalassemia are uncommon (75 cases in the literature) but can induce severe sequelae if the diagnostic is not rapidly obtained. Magnetic resonance imaging is the gold standard allowing precise diagnosis and spreading of extramedullary hematopoiesis. Radiotherapy and more recently hydroxyurea are the first line treatment. CONCLUSION: This observation recalls that extramedullary hematopoiesis is a differential diagnostic of spinal cord compression in patients with thalassemia. A screening of paravertebral localization of extramedullary hematopoiesis should be performed in high risk thalassemic patients.

[Thymoma and hypogammaglobulinemia. Good's syndrome: apropos of a case and review of the literature]. / Thymome et hypogammaglobulinémie, le syndrome de Good: à propos d'un cas et revue de la littérature.

INTRODUCTION: Good's syndrome is defined as the association of thymoma, hypogammaglobulinemia, and repetitive bronchopulmonary infections. We report one case and review the clinical and immunological features of this syndrome. EXEGESIS: We describe the case of a 67-year-old man who presented with both severe bronchopulmonary infection resistant to antibiotherapy and thymoma. Definite diagnosis was based on the existence of hypogammaglobulinemia. Treatment with intravenous immunoglobulins led to positive outcome. CONCLUSION: Good's syndrome occurs in only 5% of parathymic diseases. To prevent further bronchial tube destruction and pulmonary surgery, it must be diagnosed and treated early.

[Cannabis-induced arteritis vs. Léo Buerger disease. Nosologic discussion apropos of two new cases]. / Artérite du cannabis versus maladie de Léo Buerger. Discussion nosologique à propos de deux nouveaux cas.

BACKGROUND: We observed two cases of juvenile endarteritis which might suggest a possible link between Winiwarter-Buerger disease and cannabis-induced endarteritis. CASE REPORTS: Our two patients were young men aged 18 and 20 years. Both developed acute distal ischemia of the lower or upper limbs with arteriographic evidence suggestive of Winiwarter-Buerger disease. Both smoked regularly but not excessively and both used cannabis regularly. In one case, the therapeutic response to withdrawal of cannabis was good. In the second, use of cannabis continued and arterial disease persisted. DISCUSSION: The cause of Winiwarter-Buerger disease remains elusive although smoking is undoubtedly involved in the pathogenic mechanism. Our two cases recall the cannabis-induced endarteritis described in the sixties in Kif smokers in North Africa. The main clinical and radiographical features in this condition are the same as in Winiwarter-Buerger disease. CONCLUSION: Winiwarter-Buerger disease and cannabis-induced endarteritis are 2 very similar conditions. The probably rare finding of juvenile endarteritis mimicking thromboangeitis obliterans should, in our opinion, be considered as a secondary and possibly toxic effect of cannabis.

Asymptomatic intracardiac thrombi and primary antiphospholipid syndrome.

Intracardiac thrombi have rarely been reported in patients with the antiphospholipid syndrome. We describe a new case revealed by systematic echocardiography in an asymptomatic woman who consulted for mild thrombocytopenia. Our case is characterized by a past history of migraine, fetal loss and psychiatric disturbances and by a high level of antiphospholipid antibodies (anticardiolipin, anti-beta(2)-glycoprotein I and lupus anticoagulant). Echography, in the absence of any cardiovascular symptom, showed bulky right intra-atrial thrombi requiring surgical excision. Histopathological analysis revealed the fibrino-cruoric nature of the lesion without myxoma. This case shows that, when faced with a mild thrombocytopenia associated with antiphospholipid antibodies, echocardiography may reveal a life-threatening and completely asymptomatic intracardiac thrombosis.

Successful surgical management of a ruptured abdominal aortic aneurysm in a renal transplant patient. A case report.

With extended indications for renal transplantation and increasing survival, it can be expected that atherosclerotic vascular disease in the post-transplant patient will become more frequent. The authors report a case of a ruptured abdominal aortic aneurysm in a renal transplant recipient. A temporary axillo-femoral shunt was used to maintain perfusion of the renal graft during aortic cross-clamping. They review the literature and discuss the available methods for preserving renal function.

Migraine and coeliac disease.

The pathophysiology of migraine remains unclear. To incriminate a comorbid factor is always hypothetical, even if suppression of this factor appears to prevent the migrainous attacks. In our patient, treatment of coeliac disease coincided with total disappearance of severe migraine attacks. Moreover, the coeliac disease was first revealed during the evaluation of a migraine with aura.

Neutron powder diffraction study (T= 4.2-300 K) and polarization analysis of YBaCuFeO5+δ.

The crystal and magnetic structures of the perovskite YBaCuFeO5+δhave been studied in the temperature rangeT = 4.2-300 K by powder neutron diffraction. In addition to the antiferromagnetic ordering transition atTN = 442 K, a commensurate-incommensurate magnetic transition is detected atTN' = 190 K. Below this temperature, two sets of satellite peaks surround the (1/2,1/2,1/2) magnetic peak atd âˆ¼ Å, collapsing into a single set of satellites below 155 K. Polarization analysis has been performed to confirm the magnetic nature of the (1/2,1/2,1/2)±satellites.

Prevalence and clinical significance of IgG isotype anti-beta 2-glycoprotein I antibodies in antiphospholipid syndrome: a comparative study with anticardiolipin antibodies.

To investigate the prevalence, significance, and specificity of IgG isotype anti-beta 2-Glycoprotein I antibodies (a-beta 2-GPI) in antiphospholipid syndrome (APS), we developed an enzyme-linked immunosorbent assay (ELISA) for the detection of IgG-a-beta 2-GPI and tested sera from 61 patients with autoimmune disorders (AID), 39 patients with APS and 22 patients with systemic lupus erythematosus without APS, 139 patients with various infectious diseases (hepatitis C virus infection, human immunodeficiency virus infection, Q fever, Mediterranean spotted fever, syphilis) and 97 healthy control subjects. Using irradiated plates coated with human beta 2-GPI, we showed that in the sera of patients with AID, optical densities from the coated wells were significantly higher than those from the noncoated ones (p = 0.0001). In this assay, intra-assay and inter-assay variation coefficients ranged between 4% and 10%. Clinical evaluation showed that IgG-a-beta 2-GPI were found in 23 of 61 patients with AID but in only one patient with an infectious disease. The presence of the IgG-a-beta 2-GPI in association with APS (p = 0.005) was statistically significant with high specificity (98%) and positive predictive value (87.5%) but with low sensitivity (54%), and was significantly associated with venous thrombosis (p = 0.0025). In addition, the IgG-a-beta 2-GPI levels were highly correlated with those of anticardiolipin antibodies (aCL) (p < 0.001). In contrast to a-beta 2-GPI, aCL were found with a high prevalence (40%) in patients with infectious diseases. Because of their high specificity, anti-beta 2-GPI antibodies appear to be useful tools in the evaluation of the risk of APS. However, because of their low sensitivity, their detection needs to be associated with that of aCL.

M-CSF serous rate evolution during post-transplantation days in a heterotopic heart graft model mice.

BACKGROUND: On heterotopic heart graft in mice, aged 7 weeks (C3H and B57), we investigate the variations of Macrophage Colony stimulating factor serous rate. The macrophage colony stimulating factor (M-CSF) is a cytokine involved in the immune response during transplantation. METHODS: Five groups were determined, group 1 with a heterotopic transplant without immunosuppressive treatment (N=24); group 2 with a heterotopic transplant and Corticoid treatment after the graft (N=29); group 3 with a heterotopic transplant and cyclosporine treatment after the graft (N=34); group 4 with an isogenic transplant (N=31) and group 5 undergoing a laparotomy (N=31). The mice are sacrificed at D4, D7, D10 or D14 and the M-CSF dosage are done by ELISA method. RESULTS: The serous rate of M-CSF is stable in the group with an isogenic transplant or with only a laparotomy. But in the group with a heterotopic transplant the M-CSF values increase (x1.5). If we use an immunosuppressive treatment the raising of M-CSF is less important. When we have a rejection graft, the serous rate of M-CSF increases but not significantly (Mann-Whitney test). CONCLUSIONS: We conclude M-CSF seems to be a reliable index of disorder during immune response, but is not a good marker of the rejection.

[Calcified aortic valvular disease associated with adult progeria]. / Valvulopathie aortique calcifiée associée à une progeria de l'adulte.

Adult's progeria or Werner's syndrome is a rare condition of autosomal-recessive inheritance, characterized by an apparent acceleration of many of the processes associated with aging. We describe the cardiovascular findings in a 44 year-old man with this disorder. Slightly elevation of urinary hyaluronic acid level contributes to the diagnosis.

[Postoperative transparietal Doppler ultrasonographic study of the internal mammary artery graft flow with respect to quality of the underlying myocardium]. / Etude en écho-doppler transpariétal postopératoire du flux du greffon mammaire interne en fonction de la qualité du myocarde sous-jacent.

The authors studied flow in the internal mammary artery by Doppler ultrasonography after bypass surgery of the left anterior descending artery to determine the correlation between the flow pattern and the quality of the distal run off. A pulsed Doppler was used to record flow from the right and left internal mammary arteries in the first, second and third intercostal spaces and the supraclavicular fossa. Only the best quality recordings with the highest amplitudes were retained for analysis. Forty-nine patients (average age 61 +/- 10 years), 43 men and 6 women, were included and were examined between the 10th and 15th postoperative days. All had stenosis of the left anterior descending on coronary angiography: three subgroups were identified ad the time of evaluation: (I) revascularisation of an infarcted zone with important angiographic and echographic sequellae. (II) revascularised zones with slight wall motion abnormalities. (IIIa) revascularisation of myocardium with no abnormality (including a subgroup of 5 patients (IIIb) characterised by a postoperative low output state and echocardiographic changes not present before surgery). Significant changes were observed in the flow patterns of the different groups. (I) an exclusively systolic flow (diastolic velocity time integral = 2.85 cm), (II) systolodiastolic flow (diastolic velocity time interval = 9 cm) similar to that in group IIIb, and IIIa predominantly diastolic flow (diastolic velocity time integral = 15.2 cm). The amplitude of diastolic flow in the mammary artery graft was therefore related to previous ischaemia of the revascularised myocardium; detection of stenosis by Doppler ultrasonography should therefore take into account the previous history of the patient.

[Paraneoplastic arterial thrombosis. Apropos of 2 cases]. / Thromboses artérielles paranéoplasiques. A propos de deux cas.

The search for a cancer is part of the classical investigation of unexplained venous thrombosis. Arterial thrombosis associated with neoplasia is more rare. The authors report two cases in which arterial thrombosis was the final event of their malignant disease. The first case had abacterial thrombotic endocarditis and disseminated intravascular coagulation at the origin of multiple thrombotic complications. The initially unknown cancer was a pancreatic adenocarcinoma. The second case presented with acute occlusion of the iliac artery after ablation of a malignant melanoma. Despite embolectomy with a Fogarty catheter and effective anticoagulation, the thrombosis recurred several times at the same site. The clinical features and the mechanisms of these two cases suggestive of Trousseau's syndrome are discussed.

Swan-Ganz catheter induced pulmonary artery perforation during cardiac surgery concerning two cases.

Two case reports describing patients having cardiac operations under extra corporeal circulation are presented. At the completion of the operation, a massive hemoptysis occurred in both patients after a Swan-Ganz catheter had perforated the pulmonary artery. A hemostasis lobectomy was then immediately required. The immediate and long term prognosis seems satisfactory. This is an unusual but serious complication. The incidence of this complication varies between 0.06 and 0.2%. The more frequently related risk factors include people over the age of 60, pulmonary artery hypertension, anticoagulant therapy, hypothermia and manipulation of the heart by the surgeon. When this accident occurs, many authors suspect the balloon. An early diagnosis is essential in the case of a major or even a minor hemoptysis, because this complication may be a lethal one as the mortality rate may reach 50%. According to us, the appropriate therapy which would reduce this mortality is a surgical one (hemostasis lobectomy).