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Biomedical subjects

N Magy

Publications and source records attributed to N Magy.

18 recordsLinked to original sources

[Value of eosinopenia in inflammatory disorders: an "old" marker revisited].

PURPOSE: The value of eosinopenia as a test in favour of an infectious disease was suggested by Schilling since 1929. We tried to verify this hypothesis with a prospective and multicentric study. PATIENTS AND METHODS: One thousand and thirty-eight patients were included (82 females and 56 males, means age: 71.8 years). Diagnoses were: 83 infectious diseases, 38 systemic diseases, 10 neoplasia and 7 miscellaneous. RESULTS: The mean value of eosinophils was 72/mm3 in bacterial infectious diseases and 214/mm3 in non infectious diseases (p < 0.01). When leukocytes were higher than 10,000/mm3 and eosinophils counts less than 40/mm3, predictive value for an infectious bacterial disease was 100% as well as specificity. Under same conditions, when protein C reactive was higher than 100mg x l(-1), the predictive value was 85% and the specificity was 57%. CONCLUSION: Our study shows that an inflammatory syndrome associated with hyperleucocytosis above 10,000/mm3 and eosinophils counts under 40/mm3 seems strongly related to bacterial infectious diseases.

Aged↗

[Liveoid vasculopathy with combined thrombophilia: efficacy of iloprost].

INTRODUCTION: Livedoid vasculopathy is characterized by early, focal painful purpuric lesions of the lower skin extremities without histologic finding of small vessel vasculitis. EXEGESIS: A 38-year-old man was seen in our unit for painful purpuric lesions of both feet localized on toes and external sides. Skin biopsy showed dermic vessel thrombosis and endothelial cell proliferation. Lupus anticogulant antibody was positive in association with a heterozygous factor V (Leiden) gene mutation (G1691A). Anticoagulation failed to relieve pain and cutaneous lesions. Intravenous iloprost, a prostacylcin analogous (Ilomedine) was dramatically and rapidly effective in our patient. CONCLUSION: Livedoid vasculopathy is a cutaneous affection related to vascular thrombotic events in which thrombophilia plays a central role. Iloprost might be an interesting alternative treatment of painful purpuric lesions when anticoagulant treatments are ineffective.

Adult↗

[Relative value of different antiphospholipid antibodies detected in a department of internal medicine: retrospective study of 124 patients].

PURPOSE: The value of antiphospholipid antibodies (aPL) detected in the sera of the patients of an Internal Medicine department is not univocal and is still much debated. To test the contribution of such new markers, we reviewed the records of patients having antiphospholipid antibodies detected between 1996 and 1997. METHODS: One hundred and twenty four patients, having at least one of these two aPL: lupus anticoagulant (LA), anticardiolipin antibodies (aCL), or one of these two anti-proteins: anti-beta 2glycoprotéin I antibodies (anti-beta 2GPI) or anti-prothrombin antibodies (aPT), were studied. LA was detected by a PTT-LA technique and aCL, anti-beta 2GPI and aPT by ELISA-sandwich techniques. For each patient we recorded sex, age, personal and familial history of thrombosis, fetal losses and systemic disease, the reason of aPL detection, the final diagnosis, activated partial thromboplastin time (aPTT), platelets count and type of aPL. RESULTS: The population was composed of 77 women (62%) and 47 men (38%) with a mean age of 54 years [12-92 years]. A thrombocytopenia was strongly correlated to aCL presence (OR = 6.15 et p = 0.03). The reason of aPL detection was venous thrombosis, recurrent fetal losses, systemic disease, infectious disease or fortuitous discovery of a prolonged aPTT. The final diagnosis was a systemic disease in 57% of cases, an infectious disease in 14.5%, a thrombosis in 4.5% and a neoplasia in 3%. LA was detected in 54% of patients, aCL in 39.5%, anti-beta 2GPI in 23% and aPT in 31%. No relationship between the aPTT value and the type of aPL could be established. CONCLUSION: Our study shows that familial histories of venous thrombosis or systemic disease are useful to enhance antiphospholipid antibodies detection; that LA is mostly associated to systemic and infectious diseases; that aCL and anti-beta 2GPI are predominant in case of venous thrombosis and that thrombocytopenia has to enhance aCL detection and the discussion about a possible APS.

Adolescent↗

[Myelodysplasias and systemic diseases. A non-fortuitous association].

PURPOSE: Myelodysplastic syndromes are clonal hematologic disorders, expanded from myeloid stem cells. A primitive immunologic disorder is discussed. This hypothesis could explain a non-casual association with systemic diseases. The aim of our study is to test this hypothesis. METHODS: We retrospectively investigated the data of 60 patients with myelodysplastic syndromes (group I) hospitalized in our unit from 1990 to 1999. The frequency of systemic disorders was screened and compared to controls (group II). Group II consisted of 120 patients matched for age and sex and hospitalized in the same hospital during the same period. RESULTS: Sixty patients were included (mean age: 83 years old). Myelodysplastic syndrome subtypes were refractory anemia with excessive blasts (52%), refractory anemia (43%) and sideroblastic anemia (5%). Fourteen cases of systemic manifestations were reported in group I (23%) and five in the controls (4%) (P < 0.0001). Systemic manifestations in group I included vasculitis in six cases (42%), polyarthritis in three cases (21%), systemic amyloidosis AA in two cases (14%), relapsing polychondritis in one case, pyoderma gangrenosum in one case and celiac disease associated with a systemic granulomatosis in one case. In the controls, vasculitis was present in four cases and polyarthritis in one. Median age at onset of myelodysplastic syndrome was not influenced by the association with systemic disorders which, in return, have not influenced the myelodysplastic syndromes' subtypes. Myelodysplastic syndromes succeeded to systemic manifestations in 71.4% of cases and could not be attributed to immunosuppressive therapy. CONCLUSIONS: The association of myelodysplastic syndromes with systemic manifestations seems not to be casual. It raises the hypothesis of a primitive immunological disorder in both diseases. Moreover, the description of two cases of systemic amyloidosis and one case of pyoderma gangrenosum might suggest an additional disorder of macrophages or granular cells.

Age of Onset↗

[Tick-born encephalitis].

INTRODUCTION: Tick-borne encephalitis (TBE), a disease contracted through tick bites, is caused by a Flavivirus. Its geographical distribution comes from the geographical distribution of the reservoir of infection--i.e., mainly the tiny mammals living in the forests and bushes. The endemic area spreads from the Rhine to the Urals, from Scandinavia to Italy and Greece. CURRENT KNOWLEDGE AND KEY POINTS: Symptoms usually evolve in three phases: at first a nonspecific phase with fever and myalgia, then an afebrile phase, and finally a phase with neurological manifestations, such as meningitis, meningoencephalitis and/or myelitis, and fever. Motor neurological sequelae are possible. The cases occurring in the East are characterized by their greater severity compared to those occurring in the West. The diagnosis, easily established given a history of a tick bite in an endemic area, is confirmed by the presence of specific IgM in the blood and/or cerebral spinal fluid. FUTURE PROSPECT AND PROJECTS: There is no specific treatment. Prevention consists of individual prophylactic measures (self-examination and systematic extraction of ticks after exposure, use of repellents), and in immunization. The vaccine, prepared from inactivated viruses, should be used for target populations, that is, for people exposed to tick bites during their professional or leisure outdoor activities.

Diagnosis, Differential↗

[Nail manifestations of systemic diseases].

General diseases-associated nail disorders may be classified into 5 types according to their clinical impact. Type I refers to nail disorders considered as peculiar features among the various symptoms of general disease such as peri-ungueal vascular dilatation, onycholysis, Beau lines, opaque nails or melanonychia. Type II refers to nail disorders with a strong clinical value such as Terry's nails, Muerhrcke's lines or koilonychia. Type III refers to nails disorders presenting as significant syndromes of several diseases such as yellow-nail syndrome of subungueal splinter haemorrhages, and type IV to specific disorders considered as true keys for diagnosis such as digital hippocratism, Bazex's acrokeratosis, Koenen's tumor, or nail hypoplasia. In type V are included drug-induced nails disorders especially after chemiotherapy or antiretroviral therapy.

Autoimmune Diseases↗

Amyloid arthropathy revealed by RS3PE syndrome.

Amyloid arthropathy is a form of primary AL amyloidosis with a monoclonal component in the blood and/or urine, and RS3PE syndrome is acute edematous polysynovitis in subjects older than 60 years. A 74-year-old man was diagnosed with both disorders. He was admitted for benign acute polyarthritis of the hands and feet and reported carpal tunnel symptoms predominating on the right. A synovial biopsy at the right wrist disclosed deposits that stained with Congo red even after potassium permanganate treatment (positive Wright's test). Articular AL amyloidosis was diagnosed. The symptoms resolved under glucocorticoid therapy alone, casting some doubt on their relationship with the amyloidosis. Roentgenograms showed geodes, a feature not present in RS3PE. Whether RS3PE may be among the possible presentations of articular amyloidosis is discussed.

Aged↗

[Wegener's disease and central diabetes insipidus].

Vasculitis with central diabetes insipidus is a rare condition which must be recognized for proper management. We report a case of Wegener's granulomatosis with diabetes insipidus and review the literature concerning the clinical and radiological features of this association. We examined the data supporting the efficacy of cyclophosphamide in diabetes insipidus. Diabetes insipidus is inaugural in 46% of cases. Wegener's granulomatosis has no distinctive features when associated with diabetes insipidus. MR imaging reveals an enlargement of the posterior pituitary in 100% of cases. Cyclophosphamide is effective in 80% of cases. In Wegener's granulomatosis, pituitary vasculitis is a rare condition. MR imaging is the most accurate method for pituitary exploration. Cyclophosphamide is effective in the treatment of vasculitis lesions in Wegener's granulomatosis.

Aged↗

[Withdrawing corticosteroid therapy: the stress of the last milligrams...].

Suppression of the hypothalamo-pituitary-adrenal axis (HPA) is a classic complication of corticosteroid therapy. There is some controversy over the conditions necessary to recover normal HPA activity. Most agree that the total duration of treatment, the maximal dose, the total cumulative dose and the the rate of withdrawal are all important factors in predicting HPA suppression. Recent studies provide evidence contradicting this opinion. It is now demonstrated that HPA activity is completely recovered as soon as the prednisone dose falls below 5 mg per day, that adrenal function is totally blocked for doses above 7.5 mg per day, and that an intermediary response to synactene is obtained for daily doses of 7.5 to 5 mg. It is thus reasonable to conclude that corticosteroid therapy can be safely withdrawn when the daily dose is less than 5 mg and, if a higher dose is given, a temporary increment would be indicated in case of stress.

Adrenal Cortex Hormones↗

Effects of corticosteroids on HCV infection.

The risk factors for clinical recurrent hepatitis C in liver transplant recipients are not clearly defined. It has been suggested that the corticosteroids included in the treatments of patients undergoing allograft rejection might induce acute hepatitis by increasing HCV replication. In this study we investigated the effects of corticosteroid boluses on HCV viremia in liver allograft recipients treated for acute rejection. Since we had previously developed a model of HCV replication in peripheral blood mononuclear cells (PBMC) in vitro, we also studied the effects of corticosteroids on HCV replication in vitro. A transient peak of HCV viremia was observed in patients treated with corticosteroid boluses for an acute allograft rejection. In the cell cultures, corticosteroids induced an increase of the total amount of viral RNA detectable. Our results demonstrate that corticosteroids induce an increase of hepatitis C virus replication in vivo and in vitro.

Adrenal Cortex Hormones↗

[Treatment of recurrent ulceration with low doses of thalidomide. Pilot study in 17 patients].

PURPOSE: Though thalidomide in a dosage of 100 mg/day is the standard treatment for recurrent oral and genital ulcers (OGU), its toxicity would be less important with lower dosage, while its efficacy would be identical. Furthermore, duration of treatment might be a major risk factor for the development of subsequent polyneuropathy. To determine the dosage of thalidomide leading to the best efficacy/toxicity ratio, a pilot study was conducted from 1993 to 1996. METHODS: Seventeen patients with OGU (mean age: 43 years, sex-ratio: 12:5) were included in the study and presented either recurrent oral ulcerations (8 patients), oro-genital ulcerations (3 patients), Behçet disease (4 patients), or recurrent OGU associated with leukemia (2 patients). The initial dosage of thalidomide was 50 mg/day (1 tablet) for 1 month. If the patient's condition improved, the dosage was reduced to one tablet every other day for 1 month and one tablet every 3 days thereafter. Nerve conduction studies (EMG) were performed at inclusion in the study and every 6 months thereafter. RESULTS: Among the 17 patients, remission was observed in ten patients within the first month of treatment and the condition of seven patients improved. Complete remission was observed in six patients after a 2-month treatment and in one patient after 4 months. A 200-mg/8 days dosage induced prolonged remission in 12 patients. Among them, ten patients received a 150-mg dosage over 8 days thereafter and disease relapsed in four of them. Among the six patients who received a 100-mg dosage over 8 days, only one relapse was observed. EMG showed a decrease in sensory nerve action potentials in six patients after 8 months and a half on average. Only three patients had to discontinue their treatment due to the occurrence of either paresthesia (2 patients) or areflexia (1 patient). Our study shows that initially a 50-mg/day dose is efficacious in the treatment of OGU and that administration of one tablet every 2 or 3 days is efficacious in more than 60% of the patients to maintain remission. CONCLUSION: A dosage of 50 mg/day is initially efficacious in most cases, provided that the patient is carefully followed up to allow early detection of potential peripheral neuropathy.

Adult↗

Acute dysautonomia secondary to autoimmune diseases: efficacy of intravenous immunoglobulin and correlation with a stimulation of plasma norepinephrine levels.

Acute dysautonomia is a disorder characterized by severe sympathetic and parasympathetic failure with relative preservation of motor and sensory function. The disease is considered to be idiopathic in most cases, but there is now a trend towards considering the disorder as an uncommon variant of Guillain Barré syndrome. We report two cases of acute dysautonomia which did not fulfill the criteria of the idiopathic form. The first case was associated with Sjögren's syndrome and the second with thyroiditis and antiganglioside antibodies which were correlated with the severity of the disease. Intravenous gammaglobulin (IVGG) was effective in both cases, as has been reported for the idiopathic form, and in one case the treatment was associated with an increase in the supine and standing plasma norepinephrine levels, thus substantiating the positive effects of IVGG on the orthostatic blood pressure and heart rate. We conclude that the spectrum of acute dysautonomia is superimposable on that of the inflammatory peripheric neuropathies and should include both the idiopathic form and dysautonomia with autoimmune associated disorders. IVGG are effective and seems to act by increasing plasma norepinephrine levels.

Acute Disease↗