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Biomedical subjects

B Wechsler

Publications and source records attributed to B Wechsler.

At least 163 records · Page 9Linked to original sources
PubMed1996

Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases.

We made a retrospective evaluation of clinical and radiologic features, treatment, and outcome of Erdheim-Chester disease, a rare non-Langerhans cell histiocytosis. We had 7 patients coming from 3 French teaching hospitals and reviewed 52 cases from the literature. These cases were considered to have Erdheim-Chester disease when they had either typical bone radiographs (symmetrical long bones osteosclerosis) and/or histologic criteria disclosing histiocytic infiltration without features for Langerhans cell histiocytosis (no S-100 protein, no intracytoplasmic Birbeck granules). Ages at diagnosis ranged from 7 to 84 years (mean +/- SD = 53 +/- 14 yr) with a male/female ratio of 33/26. Bone pain was the most frequent clinical sign (28/59), mostly located in the lower limbs. Exophthalmos and diabetes insipidus were found in respectively 16/59 and 17/59 patients. General symptoms (fever, weight loss) and "xanthomas" (mainly located on the eyelids) were present in 11/59 patients. Retroperitoneal involvement was found in 17/59 patients. Skeletal X-ray showed typical osteosclerosis of the diaphysis of the long bones in 45/59 patients. Bone radiographs showed osteolytic lesions of the flat bones (skull, ribs) in 8 patients. Histologic diagnosis was performed after a bone biopsy (28 patients), a retroorbital biopsy (9 patients), and/or a biopsy of the retroperitoneal infiltration or the kidney (11 patients). Six of our 7 patients but only 5 of 52 patients from the literature had the complete histologic criteria, disclosing no Birbeck granules or S-100 immunostaining. In other cases, histologic results usually described a xanthogranulomatous infiltration by foamy histiocytes nested in fibrosis. Treatment was corticotherapy (20/59), chemotherapy (8/59), radiotherapy (6/59), surgery (3/59) and immunotherapy (1 patient). Twenty-two patients died after a mean follow-up of 32 +/- 30 mo (range, 3-120 mo). In conclusion, Erdheim-Chester disease may be confused with Langerhans cell histiocytosis as it sometimes shares the same clinical (exophthalmos, diabetes insipidus) or radiologic (osteolytic lesions) findings. However, it also appears to have distinctive features. Patients are older and have a worse prognosis than those with Langerhans cell histiocytosis, and the diagnosis relies on the association of specific radiologic and histologic findings.

Adolescent↗
PubMed1996

[Meningomyelitis disclosing Behçet's disease].

The authors report a patient who developed a meningo-myelitis attributed to Behçet disease. Spinal MRI assessed spinal involvement. Among neurological manifestations of Behçet disease, isolated spinal cord involvement is infrequent. Dramatic improvement of neurological symptoms was obtained after steroid and immunosuppressive treatment.

Adolescent↗
PubMed1995

[Antiphospholipid antibodies: cause of thrombosis or an epiphenomenon?].

Antiphospholipid antibodies (aPL) present in systemic lupus erythematosus and the primary antiphospholipid syndrome are a well-known risk factor for thrombosis. Most of them require the presence of a cofactor, beta 2-glycoprotein I for anticardiolipin antibodies, prothrombin for lupus anticoagulant. These aPL are of the "immune" type. APL are also found in various non-immunological conditions, in which repeated endothelial or membranous damages appear to be frequent, but thromboses are rare. Most of these aPL are cofactor-independent, except those induced by chlorpromazine, and might belong to "natural" antibodies.

Animals↗
PubMed1995

Pregnancy complicated by cerebral venous thrombosis in Behçet's disease.

Pregnancy and Behçet's disease are both disorders with increased risks of cerebral venous thrombosis. However, we describe the first case of central venous thrombosis during the pregnancy of a woman with Behçet's disease, revealed by headaches, visual obscuring, and a slight increase in cerebrospinal fluid opening pressure. Treatment with heparin and corticosteroids led to rapid amelioration.

Adrenal Cortex Hormones↗
PubMed1995

Cholangitis associated with paroxysmal nocturnal hemoglobinuria: another instance of ischemic cholangiopathy?

Ischemia is increasingly recognized as a cause of cholangiopathy. The aim of this study was to report a case of association of paroxysmal nocturnal hemoglobinuria with abrupt-onset cholangiopathy. Anomalies resembling sclerosing cholangitis were documented in a patient suffering from recurrent biliary pain. None of the conditions that have been associated with primary sclerosing cholangitis or other forms of cholangiopathy was present, but shortly thereafter, paroxysmal nocturnal hemoglobinuria occurred. Hepatic vein thrombosis later complicated the course of the disease. Because the fortuitous coincidence of these uncommon conditions is unlikely, this case indicates that paroxysmal nocturnal hemoglobinuria is a cause of ischemic cholangiopathy. Other thrombogenic conditions may also be implicated in some instances of apparently idiopathic cholangiopathy.

Adult↗
PubMed1995

[Treatment of Still disease in adults with intravenous immunoglobulins].

In most cases, the treatment of adult's Still disease presents difficulties, in view of its undesirable side-effects. For this reason, we made an open trial of the effects of high-dose intravenous immunoglobulins (IVIg), a therapy with low iatrogen risk, and whose effectiveness in the treatment of other multisystemic diseases has been acknowledged. Seven patients suffering from adult Still's disease were given between one and eight IVIg infusions with a dose of 1 g/kg/day for two consecutive days. All seven responded positively, with clinical improvement. It lasted between 1 and 90 days in three patients, who subsequently relapsed; it has been continuing for an average of 13 months (2 to 24 months) in the other four patients. There were no clinical features making it possible to distinguish, after the trial, the patients who responded positively to intravenous immunoglobulins. This data, which needs to be confirmed with a controlled trial, provides hope of improved therapy for the one half of patients suffering from adult Still's disease who respond positively to intravenous immunoglobulins.

Adult↗
PubMed1995

[Uveitis: late complication of undiagnosed Whipple disease].

We report a case of severe chronic bilateral uveitis in a 60-year-old white man without neurological manifestations. Spondylarthritis and isolated malabsorption suggested the diagnosis of Whipple's disease. A normal duodenal biopsy delated the diagnosis, who was secondarily demonstrated an adenopathy biopsy and macrophage study on vitrectomy sample (macrophage distended by periodic acid-Schiff positive material). With 1 year follow-up the uveitis was rapidly and completely cured by an antibiotic association (rifampicin and trimethoprim-sulfamethoxazol). The characteristics of ocular involvement in Whipple's disease and treatment of the disease are discussed.

Anti-Bacterial Agents↗
PubMed1995

Urogenital manifestations of Wegener granulomatosis.

We report 8 patients with Wegener granulomatosis (WG) who suffered from symptomatic urogenital involvement including acute urinary retention related to prostatitis, orchitis, ureteral stenosis, bladder pseudotumor, and penile ulceration. Urogenital manifestations occurred as an isolated manifestation of WG in 4 patients, at the onset of the disease in 1 patient, and as the only symptom of relapse in 3. Data used to distinguish specific WG involvement from infection or cyclophosphamide urothelial toxicity are discussed. Four patients needed a surgical procedure consisting of suprapubic cystostomy for acute urinary retention, bilateral ureteral double J stents for bilateral ureteral stenosis, and prostate transurethral resection. Urogenital symptoms promptly resolved with medical therapy. High-dose corticosteroids and immunosuppressive drugs should be used as first-line therapy to avoid unnecessary surgery.

Adult↗
PubMed1995

M mode and Doppler echocardiographic assessment of left ventricular diastolic function in primary antiphospholipid syndrome.

BACKGROUND: High titres of serum antiphospholipid antibodies are a possible pathogenic factor for cardiac lesions in patients with systemic lupus erythematosus. OBJECTIVE: To test the hypothesis of a causal link between high titres of antiphospholipid antibodies in the serum and myocardial involvement in patients without systemic lupus erythematosus. PATIENTS AND DESIGN: 18 patients with primary antiphospholipid syndrome (recurrent fetal loss, arterial and/or venous thrombosis, high titres of antiphospholipid antibodies, and no criteria for systemic lupus erythematosus) were prospectively studied by cross sectional, M mode, and pulsed Doppler echocardiography, and compared with 18 healthy controls. The pulsed Doppler indices of left ventricular diastolic function included isovolumic relaxation time and four mitral outflow indices: peak velocity of early flow, peak velocity of late flow, early to late peak flow velocity ratio, and rate of deceleration of early flow. Four computerised M mode indices were also measured: peak rate of left ventricular enlargement in diastole, peak rate of posterior wall thinning, peak velocity of lengthening of the posterior wall, and velocity of circumferential chamber lengthening. RESULTS: Compared with controls, patients with primary antiphospholipid syndrome had higher values for isovolumic relaxation time and peak velocity of late mitral outflow and lower values for early to late mitral peak outflow velocity ratio, rate of deceleration of early mitral outflow, peak rate of left ventricular enlargement in diastole, peak rate of posterior wall thinning, peak velocity of lengthening of the posterior wall and velocity of circumferential chamber lengthening. CONCLUSION: This abnormal pattern reflects an impairment of myocardial relaxation and filling dynamics of the left ventricle in patients with primary antiphospholipid syndrome who were free of any clinically detectable heart disease. These data suggest that high serum titres of antiphospholipid antibodies may be associated with subclinical myocardial damage.

Adult↗