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Biomedical subjects

B Wechsler

Publications and source records attributed to B Wechsler.

At least 145 records · Page 8Linked to original sources
PubMed1997

Endomyocardial fibrosis in Behçet's disease.

OBJECTIVE: To report on four patients with Behçet's disease associated with endomyocardial fibrosis involving the right or the left ventricle. METHODS: Charts of more than 350 patients with Behçet's disease were reviewed. Endomyocardial fibrosis was confirmed because of cardiac failure in three patients and incidentally discovered by histological examination of an operative specimen in one patient. Echocardiography displayed bright echogen endocardium. Angiocardiography showed a reduced ventricular size. Electron beam computed tomography demonstrated a lowdense area involving the endocardium. Magnetic resonance imaging showed a mass of intermediate intensity on T1 weighted images. Diagnosis of endomyocardial fibrosis was based on histological study of a biopsy specimen in one patient and of an operative specimen in three. RESULTS: Six other similar cases of endomyocardial fibrosis complicating Behçet's disease were previously reported in the medical literature. Endomyocardial fibrosis predominantly involved the right ventricle. It can be considered a feature of Behçet's disease because: (a) no other cause was discovered; (b) arteritis, valvulopathy, and intraventricular thrombus were closely linked, and (c) all patients with endomyocardial fibrosis had vasculo-Behçet pattern. CONCLUSION: Endomyocardial fibrosis may be the sequelae of vasculitis involving endocardium or myocardium, or both and complicated with intraventricular thrombosis. Behçet's disease should be added to the list of causes of endomyocardial fibrosis.

Adult↗
PubMed1997

A peculiar form of amicrobial pustulosis of the folds associated with systemic lupus erythematosus and other auto-immune diseases.

Amicrobial pustulosis of the folds is a new entity which was initially described in association with systemic lupus erythematosus (SLE). We report four cases of this rare pustulosis occurring in the course of various auto-immune disorders. Dermatological features were similar in all patients. The primary lesions were small pustules which coalesced and formed erosive areas underlined with pustular lesions. They were localized in the folds. Three of the four patients had also isolated pustules on the scalp. Spongiform pustules were histologically present. A primary bacterial or fungal infection was ruled out by cultures on appropriate media. Serum calcium level was normal. Associated auto-immune diseases were: systemic lupus erythematosus, incomplete systemic lupus erythematosus, 'idiopathic' thrombocytopenic purpura and myasthenia gravis without thymoma (one of each). The relationship between this peculiar form of pustulosis and autoimmunity remains to be elucidated. The pustulosis did not respond to antibiotics but disappeared with systemic or topical corticosteroids.

Adolescent↗
PubMed1997

[Post-infectious systemic vasculitis: recovery without corticotherapy].

UNLABELLED: In the most cases the causes of systemic vasculitis are unknown and treatment is symptomatic (corticosteroids often associated with immunosuppressive agents). We report three cases of systemic vasculitis associated with infections for which dramatic improvement was observed without cortico-therapy (in two patients). CASE REPORT 1: A previously overweight 72-year-old woman was admitted because of a one-year history of fever, fourteen kilogram weight loss, vascular purpura, and polyneuropathy. Abnormal laboratory values included inflammatory syndrome [erythrocyte sedimentation rate (ESR): 80mm/first hour, thrombocytosis: 500,000/microliter, hypereosinophilia (1200/microliter) and positive perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) [anti-myelo-peroxydase antibodies: 30 U (normal < 7)]. Neuromuscular biopsy showed necrotizing vasculitis involving small and medium arteries. Further studies revealed a florid diverticulosis of the colon and no other severe visceral involvement. Treatment was started then with sigmoidectomy. Within six weeks her general condition improved dramatically without corticotherapy: regression of all systemic manifestations, the ESR normalized, and p-ANCA became negative. Ten months later she was still asymptomatic. CASE REPORT 2: An 50-year-old-man developed a progressive fifteen-kilogram weight loss (within 2 months), severe polyneuropathy of all four limbs. His ESR was 120 mm/first hour, and C-reactive protein 200 mg/l. Neuromuscular biopsy showed necrotizing vasculitis affecting small vessels in the nerve and no immune deposits. Stomatologic examination revealed a multiple foci of dental infections. The extraction of all these foci of infections associated with antibiotics improved dramatically all systemic manifestations (within eight weeks), once more without corticotherapy. Eight months later the patient remained asymptomatic. CASE REPORT 3: A 30-year-old-woman was admitted because of five-week history of fever, myalgias, polyarthritis, and cutaneous nodules in her limbs. Abnormal laboratory values included inflammatory syndrome, proteinuria of 0.7 g/day, and a significant rise in Chlamydia trachomatis antibodies titres from 1/64 to 1/256 over a 5 week period. She had a previous history of genital condyloma. The prednisone initialed (0.5 mg/kg/day) twelve days prior to admission was gradually reduced (stopped within 2 months) and treatment with doxycycline (200 mg/day) was initiated. Within six weeks of antibacterial treatment we assisted to a total regression of the initial clinical manifestations and laboratory values became normalized. Ten months later she remained asymptomatic. CONCLUSION: In systemic vasculitis, investigations in a search of foci of infections are of dual interest: possible etiologic agent like our case reports (strong evidence for an infectious association) and, from a therapeutic perspective, we must identify the microbes behind vasculitis syndromes, since treatment with corticosteroids may have serious consequences if the patient has an active infectious disease.

Adrenal Cortex Hormones↗
PubMed1997

[Magnetic resonance angiography: fields of exploration, main indications and limitations].

Magnetic resonance angiography (MRA) has become a widely accepted technique with regards to the other available noninvasive techniques in the diagnosis of vascular disease. This paper proposes a review of the different indications of this technique in arterial and venous diseases. Among several MRA pulse sequences, the most frequently used until today consisted of a time-of-flight technique which provided angiograms without any injection of intravascular contrast medium. It required to be performed in a plane perpendicular to the main axis of the vessel to be optimal. New techniques, such as contrast medium bolus-enhanced acquisitions allow examination of vascular segments in a plane parallel to their course (coronal for the aorta and lower limb arteries). An increasing number of clinical applications has raised since the implementation of MRA techniques on MR devices; some of them are widely accepted, whereas some others remain under the scope of extensive validation. With a high level of accuracy in grading carotid artery stenosis, MRA is now routinely used in cerebral arterial occlusive diseases and has in part replaced contrast angiography. MRA of the venous system of the brain plays a major role in the diagnosis and follow up of dural venous thrombosis. Other vascular brain diseases, such as vascular malformations, yet have limited uses. Carotid artery dissections are fairly demonstrated with MRA, which can be used for diagnosis as well as for follow-up. The accuracy of MRA in the diagnosis of venous thrombosis of the cervical/mediastinal veins has been reported as high as 100%. Moreover, MRA allows a precise assessment of collateral vessels in case of complete cervical/mediastinal venous thrombosis.

Abdomen↗
PubMed1996

[Planned pregnancy program in systemic lupus erythematosus].

OBJECTIVES: Maternal and fetal risk is high during pregnancy for young women with systemic lupus erythematosus. We analyzed outcome after a planned pregnancy program for these patients. METHODS: Between 1982 and 1994, 58 pregnancy were planned in 34 women with systemic lupus. Nine of them had renal and 5 central nervous system involvement; antiphospholipid syndrome was present in 8; steroid therapy was given in 24, immunosuppressors in 2 and plasma exchange in 1. At diagnosis of pregnancy, prednisone was prescribed (at least 10 mg/d), associated with aspirin in all non-symptomatic patients with antiphospholipid antibodies followed by heparin at pre partum. Heparin was used in case of antiphospholipid syndrome. Women with anti-SSA or B antibodies and no past history of congenital atrioventricular block were not given any specific treatment. RESULTS: An acute lupus flare-up occurred in 27% of the cases including 6% in post partum. The flare-up was mild in all cases and treatment had to be changed in half of the cases. There were 9 early abortions, 1 induced abortion for congenital malformation, 2 fetal deaths, 28 premature deliveries and 18 term deliveries. Cesarean section was indicated in 8 cases. Severe neonatal infection occurred in 2 premature infants and 1 other was growth retarded. Cutaneous neonatal lupus was observed in 2 infants. No atrioventricular blocks occurred. CONCLUSION: Fetal death or very premature birth were more frequent in patients with antiphospholipid syndrome. When pregnancy is planned in women with systemic lupus erythematosus, live birth rate reaches 96% after exclusion of early and therapeutic abortions. This rate is close to the rate in the general population. The high rate of premature birth is the main risk, but there were no maternal nor neonatal deaths.

Abortion, Spontaneous↗
PubMed1996

Demonstration of efficacy of combining corticosteroids and colchicine in two patients with idiopathic sclerosing mesenteritis.

Sclerosing mesenteritis is an uncommon condition of unknown etiology. It is likely to be the fibrous evolution of mesenteric panniculitis. It often has an indolent course but may be complicated by progressive bowel obstruction. The treatment of the symptomatic forms is not well established. The observations of two women (20 and 65 years old) with a relentless downhill course of biopsy-proved sclerosing mesenteritis are described. A treatment regimen with corticosteroid therapy (initially 1 mg/kg/day) and colchicine (1 mg/day) led, in both cases, to a rapid improvement. Abdominal computer tomography showed reduction in the tumor size. The combination of corticosteroids and colchicine is helpful in the management of symptomatic sclerosing mesenteritis. Follow-up with abdominal computed tomography is useful in evaluating the therapeutic impact.

Adult↗
PubMed1996

Long-term MR follow-up of cerebral lesions in neuro-Behçet's disease.

To study the long-term evolution of cerebral lesions in neuro-Behçet's disease, MRI was carried out on 12 patients, with follow-up from 1.5 to 6 years (mean 3.5 years). On the first MRI, 66 lesions in all were found; each patient had 1-10 lesions (mean 5.5). There were 30 (46%) lesions in the brain stem, 18 (27%) in the basal ganglia region and 18 (27%) in the periventricular white matter. Of these 22 (33%) were small, 31 (47%) medium-size and 13 (20%) large lesions. On the last MRI, 60 lesions were found: each patient had 1-10 lesions (mean 5). At this time 31 lesions (52%) were in the brain stem, 13 (22%) in the basal ganglia region and 16 (26%) in the periventricular white matter. There were 41 (68%) small, 13 (22%) medium-size and 6 (10%) large lesions. About 40% of the lesions disappeared, 35% reduced in size and 25% remained unchanged. No lesion had enlarged. Of the 60 final lesions 20 (34%) were not observed on the first study. Small new lesions were found in 5 of 12 patients (42%), and were asymptomatic. Medium-size or large new lesions were found in 2 patients (17%) who had stopped steroid treatment and had a neurological relapse. Enlargement of the ventricular system or worsening of initial cerebral atrophy was observed in 9 of 12 patients. Appearance of small lesions and worsening of cerebral atrophy on long-term follow-up suggest the possibility of subclinical progression of cerebral vasculitis and should be considered in the prognosis of neuro-Behçet's disease.

Adolescent↗
PubMed1996

[Pleuropulmonary manifestations of systemic vasculitis].

Pleuropulmonary manifestations of systemic vasculitis are common, polymorphic and of ambiguous significance: the same pulmonary lesion may reveal a specific manifestation of vasculitis as well as a therapy-induced complication, especially infection which may favor per se a flare-up. Two questions will be successively studied: what are the pleuropulmonary characteristics of Wegener's granulomatosis, Churg-Strauss syndrome, periarteritis nodosa, Behçet's disease, Takayasu's disease and temporal arteritis? What are the major adverse effects that may occur in the course of a treated systemic vasculitis?

Humans↗
PubMed1996

[The heart and antiphospholipid antibodies. Personal experience and review of the literature].

Since the recognition of the antiphospholipid syndrome, a great number of cardiac manifestations have been reported in association with these antibodies: valvular disease, coronary artery disease, cardiomyopathy and intracardiac thrombosis. However this association raises numerous questions related to the pathogenic role of antiphospholipids, their prognostic significance and their frequency in a non-selected population with a definite cardiac manifestation. In view of the literature and our personal experience, it seems necessary to distinguish two kinds of situations. During systemic lupus and primary antiphospholipid syndrome (which must be systematically looked for in patients with history of thrombo-embolic disease), antiphospholipids antibodies certainly play a role in the occurrence of cardiac manifestations, but the precise place of thrombosis has to be best defined along with immunologic/inflammatory mechanisms. On the other hand, in a non-selected population, antiphospholipids antibodies may just be the consequence of the cardiac lesion and do not seem to have prognostic implications. This distinction, actually hypothetical, should be supported on the basis of distinct specificities of antiphospholipids antibodies and especially their dependence on beta 2-glycoprotein I, which would help to distinguish the harmful antibodies from those which probably just appear as an epiphenomenon.

Antibodies, Anticardiolipin↗
PubMed1996

Prospective and serial study of primary amyloidosis with serum amyloid P component scintigraphy: from diagnosis to prognosis.

OBJECTIVE: The purpose of this study was to assess the value of the serum amyloid P (SAP) component scintigraphy in patients with primary amyloidosis (AL). MATERIAL AND METHODS: Pure human SAP labeled with iodine-123 (123I-SAP) was given intravenously to 24 patients with biopsy-proven systemic amyloidosis (15 without multiple myeloma = group 1, and 9 with multiple myeloma = group 2) and to 6 patients with multiple myeloma without any clinical or biological signs of amyloidosis (group 3). Whole-body images as well as regional views and tissue retention levels were obtained after 24 hours. Our study was approved by the institutional review committee and all individuals gave informed consent and were prospectively studied (median 13 months, range 1 to 47 from the date of the scintigraphy to May 1995). RESULTS: Organ localization of 123I-SAP, indicating the presence of substantial visceral amyloid deposits, was observed in all patients in group 1 and 2. The organ uptake of 123I-SAP included the spleen (1 patient was splenectomized) in 20 of 23 cases (87%), the liver in 15 of 24 (60%), and the kidneys in 6 of 24 (25%). Myocardial 123I-SAP was never seen although 13 out of the 24 patients had clinical or echographic data for amyloidosis. Twenty-four hour tissue retention was significantly elevated in all patients (group 1 and group 2): 55.66% +/- 19.16% in group 1 and 34.37% +/- 24.92% in group 2, as compared with normal levels < 24%. The sensitivity of the technique was 79% when only organ uptake was considered but reached 100% when tissue retention was also considered. The 24-hour tissue retention might be correlated with the severity of the amyloidosis: mean survival in patients with tissue retention greater than 50% was 11.3 months versus 24.5 months in patients with levels less or equal to 50%. Five of the 6 patients with multiple myeloma without evidence of amyloidosis had abnormal 123I-SAP imaging and 24-hour tissue retention levels. In 2 of them, amyloidosis was secondly detected. In the 9 patients who had two scintigraphies, variations in 24-hour tissue retention values were in accordance with the clinical evaluation. CONCLUSIONS: Spleen and liver distribution of amyloidosis is mostly revealed by 123I-SAP scintigraphy in patients with AL amyloidosis. The uptake of 123I-SAP appeared in proportion to the quantity of amyloidosis present in different tissues, and the relative quantity of amyloid deposits in the myocardium, carpal tunnel, digestive tract, and kidneys was often small and seldom visualized by 123I-SAP scintigraphy. In contrast 24-hour tissue retention levels were abnormal in all cases of known AL amyloidosis. This may be a positive argument for the diagnosis of amyloidosis when histopathological tests are normal. Tissue retention levels appear important as they may be correlated with survival.

Aged↗
PubMed1996

[Oculopharyngeal myopathies: value of myotomy of the superior sphincter of the esophagus. Apropos of a case].

Since 3 years, a 74 year-old man suffered of swallowing impairment, weight loss, bilateral ptosis and proximal muscular weakness. Electron microscopy disclosed intranuclear tubular filaments and confirmed the diagnosis of oculopharyngeal muscular dystrophy. Upper oesophageal sphincter myotomy was performed with complete improvement. Four months after surgery, swallowing disorders were not recurrent and weight gain was substantial.

Aged↗
PubMed1996

Risks of ovulation-induction therapy in systemic lupus erythematosus.

We report on four women with systemic lupus erythematosus who developed two types of complications after ovulation-induction therapy for primary or secondary infertility. Primary infertility was associated with endometriosis in one patient. Three had previously known systemic lupus erythematosus. All had inactive disease at onset of ovulation-induction therapy. Three patients developed symptoms consistent with moderate lupus flare a few weeks after the onset of ovulation-induction therapy. One patient developed inferior vena cava and unilateral left renal vein thrombosis. No patient became pregnant. A high oestrogen level induced by ovulation-induction therapy may explain the occurrence of lupus flare in patients with prior inactive lupus. All our patients had prior asymptomatic antiphospholipid antibodies. One patient developed a major thrombotic event. The presence of antiphospholipid antibodies increases the thrombotic risk related to ovulation-induction therapy. We conclude that ovulation-induction therapy should be restricted to patients with long-standing inactive systemic lupus erythematosus. A preventive increase of the corticosteroid dosage should be proposed in addition to heparin or antiaggregant therapy for those with prior asymptomatic antiphospholipid antibodies, or with heparin therapy for those with prior antiphospholipid antibody-related events.

Adult↗