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S Vignes

Publications and source records attributed to S Vignes.

At least 19 recordsLinked to original sources

[Laryngeal amyloidosis: a rare cause of hoarseness].

INTRODUCTION: The larynx is a rare site of deposition for amyloidosis. Diagnosis may be delayed and evoked in patients with prolonged hoarseness. We have reported two cases of laryngeal amyloidosis. EXEGESIS: One man and one woman suffered from hoarseness during one and three years, respectively. Laryngoscopic examination showed diffuse infiltration of the larynx. Amyloidosis was confirmed by the characteristic Congo-red staining of laryngeal biopsies. The search for other localizations of amyloidosis was negative. No monoclonal plasma cell proliferation was detected. Both patients received endoscopic CO2 laser excision. In one case, chemotherapy was initially associated due to dystrophic plasma cells in bone marrow aspiration and then withdrawn because of clinical failure. With a 6-month follow-up, hoarseness remained stable. CONCLUSION: Laryngeal amyloidosis is an essentially localized disease revealed by hoarseness. Treatment is endoscopic by laser excision. In nearly half of the cases in the literature, it had to be repeated due to localized recurrent lesions. Long-term prognosis of localized laryngeal amyloidosis is better than systemic AL amyloidosis.

Amyloidosis↗

[Polyclonal B lymphocytosis and hyper-IgM: immunodeficiency and/or benign lymphoid proliferation associated with tobacco?].

PURPOSE: To study the association of polyclonal B-cell lymphocytosis with binucleated lymphocytes with clinical manifestations suggesting the existence of an immunodeficiency, to evaluate the effect of cigarette smoking on this 'benign lymphoid proliferation', to analyze the clonality of lymphocytes, to determine the levels of immunoglobulins (Ig) G, A, M. METHODS: Description and analysis of the results obtained in four patients and literature review. RESULTS: Polyclonal B-cell lymphocytosis is associated with both a decrease in IgA and IgG and an increase in IgM. Recurrent infectious episodes (bronchitis) were observed in two patients. Transient smoking cessation allowed a decrease in lymphocytosis and IgM levels in two patients. No hematological malignancy occurred during the follow-up, while biological abnormalities persisted. CONCLUSION: Persistent polyclonal B-cell lymphocytosis may be associated with minor clinical features of immunodeficiency. Smoking cessation may sometimes lead to a decrease in lymphocytosis and IgM.

Adult↗

Inhaled corticosteroids and Churg-Strauss syndrome: a report of five cases.

Churg-Strauss syndrome is an eosinophil-associated, small vessel granulomatous vasculitis, characterized by late onset asthma, upper airways disease, eosinophilia, and clinical manifestations of systemic vasculitis. Several cases of Churg-Strauss syndrome have been recognized in patients treated with cysteinyl leukotriene-receptor antagonists and weaned off systemic corticosteroids. These cases have led to a general warning on the possible development of Churg-Strauss syndrome after taking cysteinyl leukotriene-receptor antagonists. The authors report five cases of Churg-Strauss syndrome in severe steroid dependent asthmatics in whom inhaled corticosteroids allowed systemic corticosteroid withdrawal. It is concluded that physicians should monitor patients carefully when severe asthma is controlled with any substance allowing withdrawal from (or even avoidance) of systemic corticosteroids. Case-control studies should identify more precisely the risk factors of Churg-Strauss syndrome.

Administration, Inhalation↗

Percentage of glycosylated serum ferritin remains low throughout the course of adult onset Still's disease.

OBJECTIVE: To determine the evolution of levels of total serum ferritin and percentage of the glycosylated form in patients with adult onset Still's disease (AOSD) at the time of diagnosis and during follow up. METHODS: All patients with AOSD were tested at the time of diagnosis and during follow up. Total serum ferritin levels were analysed by immunoassay, and the percentage of glycosylated ferritin was determined by methods using Sepharose-Con A. RESULTS: 14 patients (eight women, six men) with AOSD were enrolled. At the time of diagnosis, mean (SD) age was 36 (16) years. Mean initial total serum ferritin was 6350 (1300) microg/l (normal <250 microg/l). The mean initial percentage of glycosylated ferritin was 14.7 (13)% (normal >50%). Mean follow up time was 37 (35) months. At the time of the last examination all patients were in remission except one, who presented a chronic articular form. Total serum ferritin remained high in this single patient and was normal in the 13 others, with a mean of 98 (73) microg/l. In all patients the percentage of glycosylated ferritin remained low, with a mean of 16 (16)%. CONCLUSION: Total serum ferritin is a marker of the active phase of AOSD. The percentage of glycosylated ferritin is low both in the active phase and in remission. Further studies are needed to confirm these data and to determine their specificity for AOSD before considering any possible use of a low percentage of glycosylated ferritin as a diagnostic tool in suspected AOSD, especially when atypical or previously treated.

Adolescent↗

[Still's disease in adults: treatment with intravenous immunoglobulins].

INTRODUCTION: Adult onset Still's disease is a rare systemic disorder of unknown etiology occurring in young adults. The diagnosis is based upon Yamaguchi criteria. Treatment is difficult and not well codified. CURRENT KNOWLEDGE AND KEY POINTS: Non steroidal anti-inflammatory drugs (salicylates, indomethacin) are used as first-line therapy but are not efficient. Steroids are needed in 80% of cases to control systemic manifestations of adult onset Still's disease. Immunosuppressive agents, such as methotrexate, are necessary when a high dose of steroids are required. The use of intravenous immunoglobulin was rarely reported, in particular in patients refractory to non steroidal anti-inflammatory drugs. Intravenous immunoglobulin was administered at 2 g/kg of body weight during two or five days. Infusion was given monthly for four-six cycles. Long-term remission was obtained in half of the patients. Precise mechanisms of action of intravenous immunoglobulin in adult onset Still's disease remain unclear. FUTURE PROSPECTS AND PROJECTS: Intravenous immunoglobulin may represent a new treatment, particularly in patients refractory to non steroidal anti-inflammatory drugs before the use of steroids. Further prospective works are needed to confirm these preliminary optimistic data.

Adult↗

[Evolutive visceral malaria and hyperimmune palustral splenomegaly: a difficult distinction].

One case of hyperreactive malarial splenomegaly is reported for a Comores Island patient living in France and having thus lost his protective immunity. Plasmodium falciparum was detected in a bone marrow aspiration, whereas peripheral venous blood samples were negative. A three-month treatment of sulfadoxine + pyrimethamine was effective with a complete regression of splenomegaly and biological disorders.

Adult↗

[Pseudotumorous presentation of neuro-Behçet: role of the withdrawal of colchicine?].

BACKGROUND: Neurological involvement occurs in 10 to 28% of patients with Behçet's disease. CASE REPORT: We report a case of neurological pseudotumoral presentation of Behçet's in a patient with a long standing disease treated with low dose of prednisone and colchicine (1 mg/day), 2 months after withdrawal of colchicine. CONCLUSION: Neurological manifestations during Behçet disease can be secondary to direct central nervous system involvement (encephalitis, encephalomyelitis) or vascular angitis (essentially cerebral venous thrombosis and, rarely, intracranial aneurysms). Neurological pseudotumoral presentation is rarely reported.

Anti-Inflammatory Agents↗

[Role of corticosteroid therapy in non-malignant diseases].

INTRODUCTION: As short-term corticosteroid therapy is widely used in clinical practice, it is important to determine its precise indications and limits of use. CURRENT KNOWLEDGE AND KEY POINTS: Duration of short-term corticosteroid therapy is arbitrarily considered to be up to 21 days. Anti-inflammatory, antiproliferative or analgesic actions represent the main pharmacological features of steroids. They are related to the interactions of steroids with cytokines and immune cells. Results of randomized double-blind and uncontrolled clinical studies were included in this review. Furthermore, clearly demonstrated results that were obtained more particularly in neurology, otorhinolaryngology, pneumology, infectious diseases, rheumatologic and traumatic processes are summarized. FUTURE PROSPECTS AND PROJECTS: Indications for short-term corticosteroid therapy are well established. However, further clinical studies are required, as current prescription of corticosteroid is still empirical in the management of most diseases.

Adrenal Cortex Hormones↗

[Still disease].

Adult onset Still's disease is a systemic disorder of unknown etiology. The diagnosis is difficult and based upon Yamaguchi's criteria after exclusion of infectious diseases, hematologic process or autoimmune diseases. Clinical manifestations are various. Functional prognosis depends essentially on articular involvement. Vital prognosis depends on either hepatic failure or hematological or infectious complications, or amyloidosis. Ferritinemia is an important biological parameter which is not included in current criteria. Treatment is not well codified but steroids represent the most efficient therapy to control fever and systemic manifestations. Search for new treatments and specific markers of adult onset Still's disease are needed.

Adult↗

[Current indications of low-molecular-weight heparins].

Low-molecular-weight heparins (LMWH) have different properties than non-fractionated heparins. Bioavailability is better after subcutaneous administration and a longer halflife allows a single daily injection. Efficacy is equivalent for postoperative thromboembolism prophylaxis for all types of surgery. In most reports, low-molecular-weight heparins are more effective than non fractionated heparin and lead to fewer episodes of hemorrhage and thrombocytopenia. For treatment of deep vein thrombosis, low-molecular-weight heparins are as effective as non-fractionated heparins after two, or even one, daily injection without hemostasis tests. Other on-going studies are evaluating LMWH indications in pulmonary embolism, artery disease, pregnancy or other medical conditions.

Cerebrovascular Disorders↗