Search PubMed⌕ Search

Biomedical subjects

B Wechsler

Publications and source records attributed to B Wechsler.

413 records · Page 23Linked to original sources
PubMedDate not supplied

Subacute bacterial endocarditis presenting as polymyalgia rheumatica or giant cell arteritis.

OBJECTIVE: To report on several patients with subacute bacterial endocarditis who were initially presumed, incorrectly, to have polymyalgia rheumatica or giant cell arteritis. METHODS: We report 3 cases of subacute streptococcal endocarditis mimicking giant cell arteritis in 2 cases and polymyalgia rheumatica in one. We reviewed the literature through Medline search of French and English-language articles published between 1966 and 2005 and found 5 similar cases. RESULTS: Shoulder and/or pelvic girdle pain was associated with neck or back pain in all patients. Scalp tenderness, bilateral jaw pain, amaurosis fugax were present in 2 patients. One patient had no fever. Two patients were treated with corticosteroids with initial good clinical response in one. Appropriate antibiotic therapy resulted in the rapid disappearance of rheumatic complaints in 2 patients and achieved a definitive cure of endocarditis in all cases. CONCLUSION: Rheumatologic symptoms may hinder the correct diagnosis of infective endocarditis in patients who present with a clinical picture suggesting polymyalgia rheumatica or giant cell arteritis. In such cases, blood cultures should be systematically drawn.

Adrenal Cortex Hormones↗
PubMedDate not supplied

The multiple clinical aspects of lupus.

New insights about the following aspects of lupus erythematosus are developed: (1) thrombotic and neurological manifestations associated with the presence of antiphospholipid antibodies; (2) cardiac complications, especially coronary ischemia, conduction abnormalities and valvular involvement; (3) pregnancy, with particular attention to fetal and maternal prognoses, Soulier-Boffa syndrome and congenital atrioventricular heart blocks; (4) subacute lupus erythematosus, bullous lupus and cutaneous manifestations associated with the lupus anticoagulant.

Abortion, Spontaneous↗
PubMedDate not supplied

[A case of autochthonous leprosy (author's transl)].

A case is reported of autochthonous leprosy in a French patient who had never lived in an endemic zone. The disease was of the lepromatous leprosy type and the liver, spleen, skin nerves and muscles were affected. Characteristic findings were Hansen's bacillus in nasal scrapings, a deficiency in cellular immunity, and the presence of cryoglobulins and circulating immune complexes. Autochthonous leprosy which has not been imported, and without signs of direct contagion, appears to be exceptionally rare. The authors review the clinical forms of leprosy and immunological findings.

Humans↗
PubMedDate not supplied

[Evolutive and prognostic aspects of Wegener's granulomatosis].

We have followed 45 patients affected with Wegener's granulomatosis and conducted a retrospective analysis of its evolutive aspects and prognostic factors. The study concerned 25 men and 20 women, with an age-range of 50 +/- 15 years at the onset of the disease. The time between occurrence of the first manifestations and the diagnosis was 16 +/- 21 months, the follow-up was 6.3 +/- 8 years. All patients except 3 were given steroids, associated in 39 cases with an immunosuppressor and in 9 cases with plasma exchanges. In 2 localized forms in the ENT area, remission was possible with cotrimoxazole alone. 15 deaths occurred, the causes of which are infection and renal insufficiency when death occurs early, and cardiovascular disorders and neoplasms when death occurs late. The survival probability is 93 p. cent at one year, 83 p. cent at 3 years and 75 p. cent at 5 years from the time of the first manifestations and 75 p. cent at one year, 53 p. cent at 3 years and 50 p. cent at 5 years from the time of the diagnosis. Relapses have been reported in 11 patients, some of which occurred several years after discontinuation of the treatment: they emphasize the need for prolonged monitoring of these patients. Presence of one of the poor prognostic factors: age 50 years (p less than 0.05), presence of renal insufficiency (p less than 0.05) or hemoglobinemia less than or equal to 10 g/cc (p less than 0.05), should be an indication for a more intensive treatment of Wegener's granulomatosis.

Adult↗
PubMedDate not supplied

[Horton's disease. Current problems].

Much that has been written in the last few years concerning temporal arteritis has allowed us to better clarify the clinical picture, and for example frequency of hepatic involvement. The atypical forms of the disease such as those that involves large arteries, or those in which sedimentation rate is normal, are better known. It is in such atypical cases, particularly in a young patient, that an external carotid arteriography is most useful. Though the pathophysiology of the disease is not well understood, serologic and histologic arguments exist in favor of an autoimmune etiology, in which the antigen would be the alestin, found in the internal elastic lamina of the artery. Temporal arteritis is theorically well classified amongst the giant cell granulomatous arterities but there are some atypical forms difficult to distinguish from Takayasu's arteritis or even periarteritis nodosa.

Adrenal Cortex Hormones↗
PubMedDate not supplied

[Leiomyosarcoma of the inferior vena cava (author's transl)].

A further case of leiomyosarcoma of the inferior vena cava is described, bringing the total number reported in the published literature to 63. The tumor was located in the inter-renal segment of the vena cava, and was effectively removed by excision combined with venous reconstruction, enabling conservation of the renal veins. These malignant tumors occur more frequently in women, and their symptomatology varies according to their location in either the upper, middle or lower area of the inferior vena cava. Upper segment tumors are revealed by a Budd-Chiari syndrome or obstruction of the right atrium and are usually rapidly fatal. Middle and lower segment tumors can be treated surgically, if diagnosis is made early enough and cavography is conducted.

Budd-Chiari Syndrome↗
PubMedDate not supplied

[Cardiovascular involvement in Behçet's disease. (author's transl)].

35 cases of Behçet disease are presented including 16 vascular manifestations: 14 veinous thrombosis with 9 vena cava obstruction; 1 femoral aneurysm; 2 pulmonar arteries thrombosis; 3 pericarditis; 2 myocardial infarction. In all these cases corticosteroids and anti-coagulation therapy were instituted. With a follow-up from 5 to 36 months, only 5 relapsed in the five first months of therapy. The authors suggest systematic anticoagulation or anti-agregant therapy in Behçet disease.

Adult↗
PubMedDate not supplied

Gadolinium-enhanced MR of chronic dural sinus thrombosis.

Gadopentetate dimeglumine-enhanced MR imaging was performed in four patients with chronic dual sinus thrombosis. After injection, intense enhancement of the chronic thrombus was observed in all cases. On three-dimensional time-of-flight MR angiography, performed in one case, the occluded part of the superior sagittal sinus was not distinguishable from a normal sinus because of thrombus enhancement mimicking blood flow. Enhancement of the clot is best explained by organization of the thrombus, which is converted into vascularized connective tissue. It could lead to false-negative results in patients with chronic dural sinus thrombosis studied with contrast-enhanced MR or contrast-enhanced time-of-flight MR angiography techniques.

Adult↗
PubMedDate not supplied

Central nervous system involvement in relapsing polychondritis.

Central nervous system (CNS) involvement is a rare condition in relapsing polychondritis. We report a case of meningoencephalitis that revealed relapsing polychondritis in a 70-year-old woman. A vasculitic process is presumed. Patient was treated with steroids and dapsone, and no relapse was observed after a follow-up of 2 years.

Aged↗
PubMedDate not supplied

Malignant hypertension in antiphospholipid syndrome without overt lupus nephritis.

The antiphospholipid syndrome is usually defined by the association of a clinical manifestation (recurrent venous and/or arterial thrombosis, recurrent spontaneous miscarriages) and a biological abnormality (anticardiolipin antibody, lupus anticoagulant). We retrospectively analyzed the records of 5 patients (4 females, 1 male, aged 30 +/- 12 years) with antiphospholipid syndrome, primary (n = 1) or secondary to systemic lupus erythematosus (n = 4), who developed malignant systemic hypertension with renal insufficiency, in the absence of lupus nephritis. Before the episode of malignant hypertension, all patients had normal systemic blood pressure and renal function. During malignant hypertension the systolic pressure was 206 +/- 39 mmHg and the diastolic pressure 130 +/- 25 mmHg, peak serum creatinine was 204 +/- 95 mumol/l, daily proteinuria was 1.1 +/- 0.8 gr, and complement serum levels were normal in all patients. Renal angiography found normal proximal renal arteries. Renal biopsy showed ischaemic glomeruli without proliferative lesions (n = 5), focal intimal fibrosis either isolated (n = 3) or associated with thrombosis (n = 2) of the intrarenal vessels, and the absence of vasculitis. Immunofluorescence study did not reveal typical lupus deposits. Patients were treated with antihypertensive agents, increasing doses of prednisone (n = 3), and anticoagulant (n = 2) or anti-aggregant therapy (n = 1). After a mean follow-up of 6.8 +/- 5.2 years, 4 patients were still alive with normal blood pressure and renal function, whereas 1 patient died of a probable catastrophic antiphospholipid syndrome. Patients with antiphospholipid syndrome, primary or secondary to systemic lupus erythematosus, may develop malignant hypertension with renal insufficiency and intrarenal vascular lesions, in the absence of lupus nephritis.

Adolescent↗
PubMedDate not supplied

Monthly intravenous pulse cyclophosphamide therapy in Wegener's granulomatosis.

OBJECTIVE: To study the long term effects of monthly intravenous cyclophosphamide therapy in Wegener's granulomatosis. METHODS: Fourteen consecutive patients with active Wegener's granulomatos treated with a first-line combination of high-dose prednisone and monthly intravenous pulse cyclophosphamide were retrospectively studied. RESULTS: One patient died from septicemia complicating severe leukopenia after the first pulse. At 8 months after instituting intravenous pulse cyclophosphamide therapy, failure was observed in 6 other patients. Between month 16 and 18, 2 other patients relapsed when the time between 2 pulses was lengthened. Five patients developed cyclophosphamide-related side-effects: infection (n = 2), amenorrhea (n = 1), alopecia (n = 2) and vomiting (n = 2). Except for one fatal infection, no major side-effect of intravenous cyclophosphamide therapy was observed. At the end of the study, all patients were off intravenous cyclophosphamide therapy with more than 6 months of followup. The 6 responders were in remission on low-dose prednisone or without treatment. CONCLUSION: A combination of high-dose prednisone and intravenous cyclophosphamide may achieve long-term remission in 42% of patients with Wegener's granulomatosis. Responders to intravenous cyclophosphamide therapy had less extensive disease than non-responders.

Adolescent↗
PubMedDate not supplied

Acquired hemophilia: a rare complication of Sjögren's syndrome.

Acquired hemophilia is associated with diverse conditions such as post-partum period, autoimmune diseases, cancers, and lymphoproliferative diseases. We report a case of acquired hemophilia occurring in Sjögren's syndrome which was treated with corticoid therapy. A review of the different treatments of acquired hemophilia is presented.

Adrenal Cortex Hormones↗
PubMedDate not supplied

Intravenous immunoglobulin in adult Still's disease refractory to non-steroidal anti-inflammatory drugs.

OBJECTIVE: Adult onset Still's disease (AOSD) is a systemic disorder with an uncertain outcome at the time of diagnosis. The clinical response to nonsteroidal antiinflammatory drugs (NSAIDs) is often unsatisfactory in adult patients. Chronic use of steroids is frequently required, but may result in severe side effects. We report the results of an uncontrolled, unblinded trial of intravenous immunoglobulin (IVIG) in 7 patients with AOSD. METHODS: Seven consecutive patients unresponsive or poorly responsive to NSAIDs were enrolled. None of them had received steroids previously. AOSD was defined by the Yamaguchi criteria (J Rheumatol 1992; 19: 424). IVIG were administered every 4 weeks. NSAIDs were initially maintained. A positive response was defined by the disappearance of fever and arthritis within 2 weeks after the first IVIG infusion. RESULTS: Two patients failed to respond. Five patients initially considered to be good responders were given a total of 4 to 6 IVIG infusions. One of them relapsed at the time of the fourth IVIG infusion. The four others had a favourable clinical and biological course. At the time of evaluation 3 patients were symptom-free and off therapy, while one was still receiving low dose NSAIDs. CONCLUSION: This short, uncontrolled, unblinded study suggests that IVIG might represent a potential alternative to classical steroid therapy in patients with AOSD refractory to NSAIDs. These preliminary results need to be confirmed, however, in a double-blind randomized study.

Adult↗