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Biomedical subjects

B Wechsler

Publications and source records attributed to B Wechsler.

At least 37 records · Page 2Linked to original sources
PubMed2004

[Bilateral optic neuropathy revealing systemic sarcoidosis].

INTRODUCTION: Although ocular involvement concerns about 25 to 60% of patients with systemic sarcoidosis, papilledema is a rare event. EXEGESIS: A 43-year-old man presented with left visual loss related to papillitis. Despite high-dose corticosteroids, visual loss became rapidly bilateral. Thoracic CT-scan showed mediastinal and parenchymal involvement. Histological study of lung, bronchial and lymphadenopathy biopsies displayed giant-cells granuloma without necrosis. Diagnosis of sarcoidosis complicated with optic neuropathy was made. Therapy combining prednisone, methotrexate and infliximab was started without significant improvement of visual acuity. CONCLUSION: This report underlines the potential severity of optic neuropathy in sarcoidosis.

Adult↗
PubMed2003

[Chronic severe uveitis: classification, search for etiology and therapeutic approach].

PURPOSE: Severe chronic and refractory uveitis is a major diagnostic and therapeutic challenge for ophthalmologists and internists. Molecular tools, such as PCR but also new imaging techniques, have significantly changed the diagnostic approach during the last 10 years. Presumed and empirical diagnosis should be excluded in the face of atypical clinical presentations. CURRENT KNOWLEDGE AND KEY POINTS: A retrospective study based on 927 consecutive patients presenting with severe uveitis between 1991-1996, has recently defined the epidemiological characteristics and the visual outcome of this group of patients. An associated condition was determined in 67.5% of cases, divided in 4 different subgroups: infectious uveitis; uveitis associated with a systemic disease; eye-limited, presumed immune-mediated disorder and idiopathic eye-limited disorder. The management of patients with sight-threatening forms of uveitis is efficiently performed in collaboration with internists and depends on a complete diagnostic procedure and a well-adapted treatment. FUTURE PROSPECTS AND PROJECTS: Extensive work-up is mandatory when the therapeutic response seems atypical with resistance to corticosteroids and classical immunosuppressive drugs. Infectious uveitis should be excluded in severe and intractable forms of uveitis. Thereafter, new therapeutic strategies based on type I interferon and anti-TNF molecules can be proposed in order to decrease the potential risk of blindness in this young group of patients.

Chronic Disease↗
PubMed2003

[Bilateral internal carotid thrombosis: search for strangulation, even very ancient].

INTRODUCTION: We report 2 cases of bilateral carotid thrombosis secondary to repeated attempts of strangulation. EXEGESIS: The first case was discovered in a 31-years-old woman who complained of psychiatric manifestations, memory loss and aphasia. Subsequently she developed hemiplegia which worsened under low dose aspirin. The second case occurred in a 41-years-old woman who presented with sudden hemiplegia and aphasia. Diagnosis of bilateral carotid thrombosis based on angiocomputerized tomography or angioRMI data which suggested dissection. Thrombosis location was similar: bilateral, symmetric, close to the carotid bifurcation. Both women had atherosclerosis risk factors: current smoking, use of estrogen containing pill, dyslipidemia and/or familial cardiovascular history. Search for thrombophilia was negative. Both women had been victims of intimate partner violence with repeated attempts of strangulation, but they were stopped 8 and 6 years before the diagnosis of carotid thrombosis. Several private interviews were necessary before knowing the exact cause. CONCLUSION: Symmetrical bilateral thrombosis of internal carotids near the bifurcation should lead to evoke repeated attempts of strangulation, even if there are arterial risk factors, and especially if the patient is a middle-aged female. The therapy is not codified but prolonged anticoagulation is generally prescribed.

Adult↗
PubMed2003

[Neonatal lupus syndrome: review of the literature].

PURPOSE: Neonatal lupus syndrome include skin lesions, hematological and hepatic disorders, and congenital heart block (CHB) in the absence of severe cardiac malformation. This rare disorder is closely linked to transplacental transport of anti-SSA/Ro and anti-SSB/La maternal antibodies. CURRENT KNOWLEDGE AND KEY POINTS: The prevalence of CHB in newborns of anti-Ro/SSA positive women with known connective tissue disease is 2% and the risk of recurrence ranges from 10 to 17%. Skin and systemic lesions are transient, whereas CHB is definitive and is associated with significant morbidity and mortality (estimated at 16-19%). A pacemaker must be implanted in 2/3 of cases. Myocarditis may be associated or may appeared secondarily. Mothers of children with CHB are usually asymptomatic or have Gougerot-Sjögren, or undifferentiated connective tissue disease. Mothers of children with cutaneous manifestations may present with more severe disease and systemic lupus erythematosus. In anti-Ro/SSA positive pregnant women, echocardiograms should be performed at least every 2 weeks from 16 to 24 weeks gestation. Electrocardiogram should be performed for all children. FUTURE PROSPECTS AND PROJECTS: The efficiency of prophylactic treatment of CHB is not established. Therapy for CHB detected in utero is not standardized and involves fluorinated steroids (especially betamethasone).

Causality↗
PubMed2003

Questions about dexamethasone use for the prevention of anti-SSA related congenital heart block.

BACKGROUND: Mothers with anti-SSA/Ro antibodies who have had a previous fetus with congenital heart block (CHB) have a risk of recurrence estimated to be up to 16%. OBJECTIVE: To improve the management of these "high risk patients" by determining (a) whether or not prophylactic treatment is efficient; (b) whether or not fluorinated steroids (betametasone and dexamethasone) that do cross the placenta in an active form are safe for the fetus; and (c) which prophylactic treatment should be used. METHODS: Retrospective study performed on seven mothers sent to a university hospital owing to a past history of one (six mothers) or two children (one mother) with CHB. RESULTS: 13 subsequent pregnancies occurred. No CHB was observed. All four pregnancies in women treated with 10 mg/day prednisone were uneventful. Three pregnancies in women receiving no steroids resulted in two early spontaneous abortions and one live birth. The six pregnancies in women treated with dexamethasone (4-5 mg/day) ended in one early and one late spontaneous abortion, two stillbirths, and two live births with intrauterine growth restriction and mild adrenal insufficiency. A histological study of one stillbirth disclosed intrauterine growth restriction and marked adrenal hypoplasia. CONCLUSION: Adverse obstetric outcomes were often seen here and major concerns have been raised by paediatricians about the safety of fluorinated steroids, owing to the results of animals studies, retrospective data, and randomised trials. Because fluorinated steroids have not been shown to improve prophylactic treatment of CHB in pregnant women at high risk, their use is questionable.

Adrenal Insufficiency↗
PubMed2003

Variability of disease progression in a family with autosomal recessive CMT associated with a S194X and new R310Q mutation in the GDAP1 gene.

Charcot-Marie-Tooth (CMT) with autosomal recessive (AR) inheritance is a heterogeneous group of inherited motor and sensory neuropathies. Six genes and five additional loci have been identified that are responsible for either demyelinating or axonal forms of the disease. The gene encoding the ganglioside-induced-differentiation-associated protein 1 (GDAP1) has been associated with both demyelinating and axonal phenotypes. We report a detailed clinical, electrophysiological, and genetic study of two siblings from a Moroccan ARCMT family who are compound heterozygotes for the already described S194X and a new R310Q mutation in the GDAP1 gene. The electrophysiological data are compatible with an axonal form of the disease. The phenotype included hoarse voice and paralysis of the diaphragm. This study shows the variability of the phenotype associated with mutations in GDAP1 gene in terms of associated signs and severity.

Adult↗
PubMed2002

[Interferons and Behçet's disease].

BACKGROUND: Behçet's disease is a vasculitis of unknown origin. Various therapeutic agents have been proposed. Interferon alpha 2a or 2b appear to be the most promising. CURRENT KNOWLEDGE AND KEY POINTS: Numerous anecdotal reports and some short series have shown the efficacy of interferon in some cases previously resistant to conventional therapy, particularly to immunosuppressors. Posology, depending on the author, may vary from three million three times a week to 18 million three times a week. Interferon alpha is useful in cases of ocular, muco-cutaneous and articular involvement. Data is lacking for digestive, vascular and neurological manifestations. FUTURE PROSPECTS AND PROJECTS: Multicenter trials will nevertheless be required to determine the duration of therapy, risk of relapses, associated therapy and the optimal time to commence interferon treatment.

Behcet Syndrome↗
PubMed2002

[ANCA associated glomerulonephritis related to benzylthiouracil].

INTRODUCTION: Vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA) have been reported in patients suffering from Graves' disease treated with anti-thyroid drugs and especially propylthiouracil (PTU). EXEGESIS: We report a case of Graves' disease treated with benzylthiouracil (Basdène). This therapy was complicated by acute renal insufficiency due to crescentic glomerulonephritis associated with pANCA. After benzylthiouracil withdrawal and under corticosteroids, renal insufficiency, biological inflammation and pANCA levels decreased. CONCLUSION: Similar vasculitis associated with pANCA secondary to anti-thyroid drugs, especially propylthiouracil, were described. This suggests a causal relation between drug and vasculitis. To our best knowledge, it is the first case of vasculitis secondary to benzylthiouracil.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2002

[Health and welfare of dairy cows in different housing programs].

The objective of this study was to evaluate the effect of two Swiss animal welfare friendly housing programs, 'regular access to outdoor facilities' (RAUS) and 'improved loose housing systems' (BTS) on health and welfare of dairy cows. During two years, the following indicators of health and welfare were observed on 136 randomly selected dairy farms: injuries around the joints, callosities, lameness, teat injuries, cleanliness, behaviour during lying and rising, body condition score, and incidence of medical treatments. Farms participating in both housing programs (BTS + RAUS) and farms with the program RAUS were compared to traditional farms that did not participate in a program. Housing system and management practice were recorded as additional factors influencing health and welfare of cows. Cows on farms participating in BTS + RAUS had, on average, fewer injuries around the hocks and fewer callosities at the carpal joints than cows in farms which did not participate in a housing program. Lameness was most frequent in cows of farms without housing program, followed by cows in tie stalls with the program RAUS and cows in loose housing systems with BTS + RAUS. There were fewer antibiotic treatments in farms with BTS + RAUS compared to the other farms.

Animal Husbandry↗
PubMed2002

[Neurological manifestations of Behçet's disease].

The neurological complications of Behçet's Disease are observed in 5 to 35 percent of cases depending of series. They included vascular and central nervous system manifestations. Vascular involvement is dominated by cerebral venous thrombosis marked by benign intracerebral hypertension. Prognosis is favourable with steroids and anticoagulation. Arterial involvement (thrombosis or aneurysm) are seldomly observed but increasingly with angio-MRI. Parenchymal involvement is dominated by meningoencephalitis eventually associated with fever. Myelitis and cranial nerve palsy are seldomly encountered. Cerebrospinal fluid is abnormal with lymphocytosis or pleiocytosis. MRI has a high sensitivity with hypersignals in the brainstem, basal ganglia, internal capsule and thalamus. Prognosis of central nervous system involvement is severe. Initially recovery is frequent, but severe impairment occurs due to relapse. In our experience, therapy including steroids, immunosuppressive drugs and colchicine improve short term and long term prognosis due to significant reduction of relapse in patients with good observance.

Anti-Inflammatory Agents↗