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Biomedical subjects

O Bletry

Publications and source records attributed to O Bletry.

At least 55 records · Page 3Linked to original sources
PubMed1991

[Effectiveness of intravenous immunoglobulins in polymyositis and dermatomyositis. An open trial in 15 patients].

Polymyositis (PM) and dermatomyositis (DM) are dysimmune diseases usually treated with corticosteroids and immunosuppressants. Human polyvalent immunoglobulins administered intravenously (IgIV) are known to be effective in some dysimmune diseases. Between August 1987 and September 1989 we conducted an open trial of IgIV in 15 patients (mean age 44 +/- 14 years) with either PM (12 cases) or DM (3 cases) associated with a collagen disease in 2 patients. In 14 of these 15 patients the conventional treatments (corticosteroids, immunosuppressants, plasmapheresis, total body irradiation, lymphopheresis) had failed. One patient was seropositive for picornavirus and received IgIV as initial treatment. IgIV infusions were given 4 +/- 3.9 years on average after the onset of PM or DM. Twelve of the 15 patients received another treatment, starting at least 6 weeks before IgIV and pursued without dosage increase, which consisted of corticosteroids (11 cases), methotrexate (5 cases) or plasmapheresis (1 case). Human polyvalent immunoglobulins for intravenous use were prescribed in doses of 2 g/kg/monthly course. All but two patients (1 course) received 3 to 6 courses on average. The IgIV infusions were well tolerated in 12 patients; 3 patients showed allergic manifestations which regressed. Therapeutic effectiveness was evaluated by muscle testing and by repeated assays of creatine phosphokinase (CPK). Clinical improvement, usually perceptible after the first course, was observed in 13/15 patients; it was associated with a more than 30 percent decrease of the initial CPK level in 13 patients and with a reduction of associated therapies in 9 patients. In the entire patient population a statistically significant lowering of mean CPK value was observed as early as in the first course (P less than 0.001). In view of their effectiveness, rapid action and safety, intravenous Ig infusions may be regarded as an interesting treatment in PM or DM patients.

Adrenal Cortex Hormones↗
PubMed1991

Efficacy of intravenous gammaglobulin therapy in chronic refractory polymyositis and dermatomyositis: an open study with 20 adult patients.

PURPOSE: Polymyositis and dermatomyositis are inflammatory muscular diseases of unknown cause. Many interventions are available to treat patients with these conditions including corticosteroids, immunosuppressive drugs, plasmapheresis, and total body irradiation. However, these therapies are not always effective, and they may be associated with certain serious side effects. An attempt was made to evaluate the efficacy of polyvalent intravenous immunoglobulin (IVIG) in patients with polymyositis or dermatomyositis refractory to traditional treatment. PATIENTS AND METHODS: Twenty patients (16 women and 4 men; mean age 43 [16 SD] years), 14 with chronic refractory polymyositis and six with dermatomyositis, received high doses of IVIG because of the failure of traditional treatments (prednisone [19], methotrexate [10], azathioprine [6], cyclophosphamide [3], cyclosporine [3], chlorambucil [1], plasmapheresis [8], lymphopheresis [1], and total body irradiation [1]). In one patient with positive results on picornavirus serologic testing, IVIG was the first treatment choice. IVIG therapy was given with prednisone in 15 patients, with methotrexate in six patients, and with plasmapheresis in one patient. There were no changes in treatment in the 2 months before the introduction of IVIG therapy and no increases in dose during this treatment. Preparations of polyvalent human intravenous gammaglobulins with increased intact immunoglobulin G were used. Thirteen patients received 1 g/kg daily for 2 days each month, and seven patients received 0.4 g/kg daily for 5 days each month. The mean duration of treatment was 4 months. RESULTS: Clinical assessment, which consisted of the measurement of proximal muscle power, and biochemical studies were carried out before each treatment period. Significant clinical improvement was noted in 15 of the 20 patients. Mean muscle power estimated for the 20 patients before and after IVIG therapy was statistically significantly reduced (p less than 0.01). Eighteen patients showed biochemical improvement, and two patients with normal initial serum creatine kinase levels showed clinical improvement. Mean creatine kinase levels for the 20 patients during IVIG therapy showed a statistically significant decrease from the first IVIG perfusions (p less than 0.01). Side effects of IVIG therapy were noted in four patients; however, these effects were mild. During IVIG therapy, steroid doses were significantly reduced from the second or the third IVIG infusion (p less than 0.05). CONCLUSION: IVIG is an efficacious new therapy for polymyositis and dermatomyositis and should play a role in the treatment of these diseases, replacing or reducing steroid and immunosuppressive medications.

Adult↗
PubMed1990

Capillary microscopy during eosinophilic fasciitis in 15 patients: distinction from systemic scleroderma.

PURPOSE: Eosinophilic fasciitis (EF) is a newly recognized syndrome that bears much discussion in regard to its distinction from progressive systemic sclerosis (PSS). In vivo microscopic examination of the nailbed capillaries has elicited the description of a characteristic vascular pattern seen in PSS dermatomyositis, and mixed connective tissue disease. To clarify the capillaroscopic aspects of this syndrome and to seek criteria distinguishing it from PSS, we performed nailbed capillary microscopy in 15 patients with EF and compared the results of this examination with those in 98 patients with PSS and those in 75 normal control subjects. PATIENTS AND METHODS: The diagnosis of EF was made in 15 patients aged 25 to 69 years (average 43 years) who had an acute course, with painful edema and subcutaneous sclerotic induration sparing the extremities. There was a peripheral hypereosinophilia in all 15 patients. Twelve underwent muscle or deep cutaneous biopsy, including the fascia. Nine of these had fascial thickening, and an inflammatory cell infiltrate was observed in eight. The diagnosis of PSS was made in 98 patients, according to the usual criteria. Seventy-five normal control subjects were examined. All the capillaroscopic examinations were performed by one observer. RESULTS: None of the patients in the EF group had a scleroderma-like capillaroscopic pattern. Thirteen had normal results of capillary microscopy. Two had a nonspecific organic microangiopathic picture. In the group of 98 patients with PSS, 89 had numerous megacapillaries (p less than 0.001), seven had a nonspecific organic microangiopathic pattern, and two had normal findings (p less than 0.001). In the whole group of 75 control subjects, the features were normal. CONCLUSION: Our results show a clear distinction between the results of capillary microscopy in cases of EF and PSS. The normal pattern in EF seems to be another argument for its differentiation from PSS.

Adult↗
PubMed1990

A method for the in vivo investigation of the serotonergic 5-HT2 receptors in the human cerebral cortex using positron emission tomography and 18F-labeled setoperone.

Following previous validation in baboons, we have studied the characteristics of [18F]setoperone as a radioligand for investigating serotonergic 5-hydroxytryptamine2 (5-HT2) receptors in the normal, unmedicated human brain with positron emission tomography (PET); subjects orally pretreated with therapeutic amounts of ketanserin, sulpiride, or prazosin were also studied to evaluate the specificity and sensitivity of [18F]setoperone brain specific binding. In controls (n = 10), the tracer showed a clear-cut retention in both frontal cortex and striatum (known to contain a high density of 5-HT2 receptors) relative to cerebellum (known to be devoid of 5-HT2 receptors). In the seven young controls (20-39 years old), the frontal cortex/cerebellum and striatum/cerebellum ratios increased during the first hour to reach similar values of 2.53 +/- 0.12 and 2.38 +/- 0.11 (mean +/- SEM), respectively, and were essentially stable during the second hour. Pretreatment with ketanserin (a 5-HT2 blocker) significantly reduced the frontal cortex/cerebellum ratio to 0.7-1.0 at 65 min, whereas the striatum/cerebellum ratio was significantly, but only partially, reduced. During sulpiride treatment (a D2 blocker), the frontal cortex/cerebellum ratio was not altered, whereas the striatum/cerebellum ratio was significantly, but only partially, reduced. With prazosin pretreatment (an alpha 1-adrenergic blocker), neither the frontal cortex/cerebellum nor the striatum/cerebellum ratio was modified. These data in humans with PET demonstrate that [18F]setoperone labels with high sensitivity and selectivity 5-HT2 receptors in the frontal cortex; in striata, however, binding is to both 5-HT2 and D2 receptors. The deproteinated-to-whole plasma radio-activity concentration ratio increased with time following injection. The mean percentage of intact [18F]setoperone, in deproteinated plasma, was 82, 74, 53, 45, 30, and 22% at 5, 10, 20, 30, 60, and 110 min following injection, respectively. These data indicate that [18F]setoperone (a) is significantly bound to plasma proteins and (b) is significantly metabolized into several labeled metabolites that are much more hydrophilic than setoperone and, hence, presumably do not cross the blood-brain barrier. These results suggest the suitability of [18F]setoperone data for modeling of 5-HT2 receptor binding in brain.

Adult↗
PubMed1989

[Splenectomy can cure thrombocytopenia of systemic lupus erythematosus].

Between 1976 and 1988, 7 patients with systemic lupus erythematosus (SLE) underwent splenectomy for severe peripheral thrombocytopenia (platelet count below 30,000/mm3) which had resisted corticosteroid therapy (n = 7), high-dose immunoglobulins (n = 4), vincristine (n = 2), danazol (n = 1), cyclophosphamide and plasma exchanges (n = 1). No infective or non-infective complication of splenectomy was observed. One patient died of intracerebral haemorrhage 3 years later, after several recurrences of thrombocytopenia. The 6 survivors have normal platelet counts (more than 150,000/mm3) after splenectomy, over a mean follow-up period of 80 months. Two of these 6 patients never had recurrent thrombocytopenia and have not received any treatment for 17 to 102 months. Four patients are still under prednisone in doses lower than 30 mg/day. Among these, 2 had recurrent thrombocytopenia 2 and 60 months respectively after splenectomy and responded favourably to another course of corticosteroids associated, in one case, with cyclophosphamide. Thus, splenectomy is an effective treatment of the thrombocytopenia associated with SLE. However, the possibility of early or late recurrences makes it necessary to reserve splenectomy to those cases where medical treatment has failed or is contra-indicated.

Adolescent↗
PubMed1989

[AIDS in elderly patients. Apropos of 22 cases observed in the Paris region].

If geriatric AIDS is defined as the occurrence of this disease in individuals over 60 years old, it represents about 5% of the cases reported to the Direction Générale de la Santé by the end of 1988. We retrospectively analyzed 22 clinical cases of geriatric AIDS observed between 1985 and 1987, i.e. 21% of the cases reported at that time. In 55% of them, infection resulted from contaminated blood transfusions. Initially, hospitalization was indicated due to a significant deterioration of the patient's general condition, however, neuropsychiatric disorders and intellectual degeneration were present in 18% of the cases. Biological anomalies at the time of admission were classical with the exception of a high frequency of leukopenia often associated with anemia or thrombopenia. During the evolution of the disease, opportunistic infections were very common (90% of the cases). However, the major characteristic of this clinical form of AIDS is the high percentage (55%) of patients suffering from major neurological and psychiatric disorders, including subacute encephalitis in more than half of these patients. The prognosis is very bad, with an average survival time of 4.3 months from the time of diagnosis (median 2.5 months). The cumulative effect of the delay in diagnosing AIDS during the first hospitalization and the classical seriousness of encephalitis suffice to explain the very poor prognosis in patients whose age can play a role in altering the immune response.

AIDS Dementia Complex↗
PubMed1989

[AL amyloidosis and primary fibrinolysis. Study of the mechanism of fibrinolysis].

A 45 years old woman with AL amyloidosis presented with a hypofibrinogenemia (fibrinogen 100 mg/dl) without severe bleeding. There was laboratory evidence of fibrinolysis with shortened euglobulin lysis time, decreased alpha-2 plasmin inhibitor and decreased plasminogen. The mechanism of this primary fibrinolysis remains unclear, since there is no enhancement of the tissue-type plasminogen activator. Analysis of the 8 cases related in the literature of excessive fibrinolysis associated with amyloidosis demonstrated improvement of bleeding manifestations and abnormal fibrinolysis following the administration of antifibrinolytic agents.

Amyloid↗
PubMed1989

[Prognostic factors of hypereosinophilic syndrome. Study of 40 cases].

Forty patients with hypereosinophilic syndrome (HES), defined according to the criteria established by Chusid et al., were studied retrospectively in order to determine the prognostic factors in this heterogeneous disease. A myeloproliferative syndrome (MPS) was diagnosed 17 times when splenomegaly and/or a greater than 5 times the normal vitamin B12, level existed. Cutaneous-visceral involvement was almost always present (95%), but could appear after several years of evolution. Twenty-three patients had cardiac involvement, determined by clinical, radiological, electrocardiographic and echocardiographic examinations; 12 of these had endomyocardiac fibrosis. The overall survival rate was 80% at 5 years and 42% at 10 and 15 years. Among the factors evaluated that are capable of influencing this survival, 5 were found to impair the prognosis; presence of an MPS; non-response of the hypereosinophilia to corticoids; existence of a cardiopathy; being male; and an elevated maximum eosinophilia; the last two factors were significantly associated with the cardiopathy. Because hematological or cardiac anomalies are not always present at the time of the first examination, HES patients require very close surveillance.

Actuarial Analysis↗
PubMed1989

[Ungueal capillaroscopy in fasciitis with eosinophilia: a distinctive feature of systemic scleroderma. Apropos of 15 cases].

Eosinophilic fasciitis (EF) is a recently described disease whose distinction from progressive systemic sclerosis (PSS) is still being discussed. PSS has a characteristic microcirculation pattern. We performed nailfold microscopy on 15 patients with EF and compared the results to those of 98 PSS patients and 75 normal control subjects. EF patients have a normal microcirculation pattern (13/15) or discrete, non-specific anomalies: none had the typical capillary pattern associated with PSS and associated diseases. The findings of this study justify making a distinction between EF and PSS and demonstrate that nail fold microscopy can be a useful tool for an early differential diagnosis between these two disorders.

Adult↗
PubMed1989

[Encapsulating peritonitis in periodic disease. Apropos of a case studied by x-ray computed tomography].

A case of encapsulating peritonitis complicating the course of familial Mediterranean fever is reported. This encapsulating peritonitis was responsible for abdominal pain and had a "pseudocystic" appearance on ultrasonography and computed tomography. Ultrasound guided aspiration produced a yellowish fluid rich in proteins and poor in cells. Surgical operation revealed a congested appearance of the peritoneum associated with a richly fibrinous appearance of the external wall of the mass. The differential diagnosis is discussed.

Adult↗
PubMed1988

[Wegener's granulomatosis in elderly subjects. 37 cases].

The clinical, biochemical and prognostic characteristics of 37 patients with Wegener's granulomatosis were studied in relation to age. Eleven patients were older than 60 years when the first sign of the disease appeared. Except for the fact that the renal lesions seemed to be more severe in these patients, there was no significant clinical or biochemical difference between them and patients under 60. Five patients over 60 years of age died during the first 2 years of Wegener's granulomatosis. Death was due to infection in 3 cases, to acute renal failure as complication of intra-alveolar haemorrhage in 1 case and to metastatic cancer in 1 case. Age-related comparisons of survival rates showed that the probability of surviving was lower in elderly subjects: 78 per cent at 1 year and 39 per cent at 3 and 5 years, as opposed to 96 per cent at 1 and 3 years and 84 per cent at 5 years in younger subjects (P less than 0.01). This poor prognosis is probably due to the renal lesions which constitute an important cause of death in the published series. Rapidly progressive glomerulonephritis being amenable to an early and intensive treatment, elderly patients should be treated as energetically as younger patients.

Adrenal Cortex Hormones↗
PubMed1988

[Cardiac involvement in Behçet's disease. 12 cases].

Fifteen cardiac manifestations observed in 12 of a series of 196 patients with Behçet's disease are reported. In this retrospective study conducted in an internal medicine department, 5 cases of pericarditis and 4 cases of myocardial infarction were encountered. The other cardiac manifestations were observed only once. They included ventricular aneurysm, endomyocardial fibrosis of the right heart, aortic insufficiency, mitral valve insufficiency, mitral valve prolapse, and right heart failure consecutive to pulmonary arterial hypertension. There was no relationship between the severity of cardiac lesions and that of the extracardiac manifestations of the disease. Comparison of these data with those found in the literature showed that pericarditis is the most frequent pathology, but it usually regresses rapidly. Lesions of the coronary arteries, with or without myocardial infarction, consist of stenosis, occlusion or pseudoaneurysm requiring surgical treatment. Myocardial lesions (with the possibility of pseudoaneurysm) and endomyocardial right heart fibrosis are exceptional but fairly characteristic of Behçet's disease which they should suggest. Hughes-Stovin's syndrome may be complicated by pulmonary arterial hypertension, although death is generally caused by massive haemoptysis.

Adult↗