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P Godeau

Publications and source records attributed to P Godeau.

At least 235 records · Page 13Linked to original sources

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↗

Leiomyosarcoma of the inferior vena cava. Experience with 7 patients and literature review.

Leiomyosarcoma of the inferior vena cava (IVC) is a rare malignant tumor originating in the smooth muscle of the media. Although rare, it is the most common malignancy in the IVC. One hundred and six cases have been reported thus far in the world literature, usually as isolated case reports. Clinical, radiologic, and therapeutic management and follow-up, including 7 additional cases, have been reviewed and summarized. Clinical manifestations are dependent upon the location of the tumor. The main symptom was a palpable mass for a tumor in segment I, abdominal pain for segment II, the presence of Budd-Chiari syndrome for segment III. Segment II was the most frequent site of leiomyosarcoma of the IVC, alone (n = 41) or with other segments (n = 39). Before laparotomy, clinical recognition was difficult or impossible. Recently, however, newer imaging modalities including ultrasound and CT scan have permitted earlier diagnosis. Metastases, when diagnosed, were either present at diagnosis (n = 20) or appeared as the disease progressed (n = 18). Metastatic disease frequently involved the liver, lung, lymph nodes, or bone. The small number of patients alive without metastases (16/113) must be analyzed all the more carefully because these patients were followed for less than 2 years. When prolonged follow-up is possible, the number of patients alive without neoplastic disease is significantly reduced. We found the prognosis of patients with LMS of the IVC to be poor. Diagnosis was made at autopsy for 27 patients. Among the 86 patients with follow-up information, 59 died within a mean of 16 months, and 26 were alive 25 months after the diagnosis. The main prognostic factor is topography, particularly the highest level of extension of the tumor. The upper-segment tumors have the poorest prognosis. The best therapeutic management is difficult to recommend because most of the cases in the literature did not include a sufficient follow-up. Given the very small number of patients completely free of neoplastic disease after sufficient follow-up, it seems unlikely that leiomyosarcoma of the IVC can now be cured. Patients who received a combination of surgery, radiotherapy and chemotherapy remained free of disease for longer periods. The unanswered question is: what is the best timing for each of these treatments? We recommend diagnosis of leiomyosarcoma of the IVC through biopsy guided by ultrasonography or computed tomographic scan. Therapeutic management should include large doses of chemotherapy preoperatively with or without radiotherapy to reduce tumor size.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Neurosarcoidosis].

Explore the source record for details and available documents.

Central Nervous System Diseases↗

[Severe pulmonary artery involvement of Takayasu arteritis. 3 cases and review of the literature].

Three cases of pulmonary arterial disease were identified out of a cohort of 75 cases of Takayasu's disease. In the first case, pulmonary hypertension considered to be idiopathic caused massive haemoptysis and the death of a 48 year old Spanish woman; autopsy revealed characteristic stenoses of the common carotid arteries. In the second case, haemoptysis led to pneumonectomy in a 23 year old West Indian woman with a diagnosis of agenesis of the right pulmonary artery. Disease of the supraaortic vessels occurred several months later and a retrospective diagnosis of Takayasu's disease of the pulmonary artery was made. In the third case, systematic pulmonary angiography in a 41 year old French woman referred for Takayasu's disease of the supraaortic arteries showed severe stenosis of the right pulmonary artery. She developed severe haemoptysis four years later which led to an attempted balloon angioplasty of the right pulmonary artery and embolisation of a branch of the right coronary artery thought to be the cause of the haemoptysis. This was complicated by posterior wall myocardial infarction but the haemoptysis did not recur thereafter. These three cases and a review of the literature show that the pulmonary lesions of Takayasu's disease occur mostly on the main right pulmonary artery but they may be more distal and involve the pulmonary arterioles. A coronaro-broncho-pulmonary collateral circulation may develop distal to the stenosed segments. The main complications are pulmonary hypertension and massive haemoptysis. Surgery is possible in cases of stenosis of the right main pulmonary artery. The potential role of endoluminal angioplasty is discussed.

Adult↗

[Pulmonary hyalinizing granuloma. Apropos of 2 new cases].

Pulmonary hyalinising granuloma are nodular or localised fibrosing lesions of the pulmonary parenchyma and are single or multiple. We report two new cases of this disorder which is rare, as only 62 cases have been published in the literature. It is a pathology with few symptoms, sometimes revealed by general signs. Radiologically there are nodules which are most often round or oval, intra-parenchymal, well demarcated, single or multiple. The histological appearance is characteristic: the centre of the granuloma consists of a network of dense collagen fibres which are lamellar separated by clear spaces; the periphery is the seat of rich cellular infiltration of plasmocytes and lymphocytes in the peri-vascular region. Most often there is a spontaneous benign outcome. The frequent association of pulmonary hyalinising granuloma in fibrotic disorders and the similarity in the histological appearance leads to the hypothesis of a common pathogenesis in these disorders.

Adult↗

[Vasculitis and neoplasms. 14 cases].

Fourteen cases of vasculitis associated with a neoplasm are reported. The vasculitides were classified as: leukocytolytic vasculitis 7, periarteritis nodosa 4, purpura rheumatica 1, cutaneous granulomatous vasculitis 1, microvasculitis nervosa 1. The neoplasms were diagnosed as: 10 hemopathies (including 4 cases of refractory anemia) and 5 solid tumors (1 patient had 2 tumors). The cutaneous manifestations (purpura, papules, subcutaneous nodules, etc.) and fever were the most common, while joint and neurological involvement were the rarest. The evolution of the vasculitis was usually corticosensitive and independent of the underlying neoplasm. A review of the literature revealed that these vasculitides (primarily leukocytolytic) were frequently associated with dysplastic myelogenous syndromes and hairy cell leukemia, while bronchopulmonary and colonic neoplasms were the most common solid tumors. The mechanisms giving rise to these vasculitides are discussed.

Aged↗

[Hydatid cyst of the interventricular septum. Contribution of magnetic resonance imaging].

Cardiac localisation of hydatid cysts is rare representing 0.5 to 2% of all clinical forms of this condition. Hydatid cysts are usually observed in the left ventricle and involvement of the interventricular septum is exceptional. The principal diagnostic and preoperative investigations are serology and imaging by echocardiography and computed tomography, which can give conflicting results. The authors report a case of hydatid cyst of the interventricular septum in which computed tomography was non-contributory whereas echocardiography (the key investigation in all cases of hydatid cyst) associated with nuclear magnetic resonance imaging provided particularly accurate preoperative information. The latter investigation also allowed diagnosis of pulmonary, hepatic, splenic and renal involvement of the disease.

Adult↗

[Randomized double-blind study of delayed-action verapamil as a single dose versus 2 doses daily in patients with moderate hypertension].

The antihypertensive action of sustained release verapamil is, in the majority of cases, obtained with a dosage of 240 mg per day. In certain patients, it is necessary to increase the daily dose to 360 or even 480 mg. This dosage increase most often leads to twice daily administration. In the present trial (18 patients included), the efficacy 360 mg of sustained release verapamil was compared double-blind with that of 360 mg as two divided doses. The antihypertensive action of sustained release verapamil as a single dose was shown to be identical to that of two daily doses. It is therefore felt to be unnecessary to increase the number of daily doses in parallel with dosage increases. There is absolutely no doubt that single dose monotherapy leads to better compliance.

Delayed-Action Preparations↗

[Pulmonary arterial hypertension and systemic diseases].

Certain system diseases which include vascular involvement may be accompanied by pulmonary artery hypertension (PAHT). This chiefly concerns connective tissue disorders, notably scleroderma, CREST syndrome and mixed connective tissue disease and, to a lesser extent, systemic lupus erythematosus. The onset of PAHT in relation to vasculitis of the great vessels or necrosing angeitis is rare. It may result from pulmonary artery vasoconstriction of hypoxic origin during diffuse interstitial pulmonary fibrosis, a thrombo-embolic mechanism, a vasomotor phenomenon equivalent at pulmonary level to Raynaud's syndrome, and above all from pulmonary vasculitis, the commonest mechanism. The incidence of PAHT is such connective tissue disease is probably underestimated since it is a complication often unrecognised clinically. It is a major cause of death in such conditions, certain of which nevertheless enjoy a benign reputation. With the exception of rare cases of favourable outcomes under the influence of corticosteroids and immunosuppressants in cases of mixed connective tissue disease, no treatment has in fact been shown to be effective in terms of survival.

Connective Tissue Diseases↗

[Isolated recurrent superficial steroid-induced phlebitis of the lower limbs in young women. A new entity].

The authors report 3 personal cases of recurrent, strictly superficial phlebitis of the lower limbs which developed in young women and responded dramatically to corticosteroid therapy. The phlebitis was oestrogen dependent: it has started during pregnancy and was exacerbated in the second part of the menstrual cycle. Clinically, the disease presented as red, dilated veins and/or isolated inflammatory nodules mimicking acute nodular panniculitis. Histology confirmed the diagnosis by showing a major polymorphous inflammatory infiltrate in the venous wall and extending well beyond it, and an intraluminal occasionally occlusive thrombus. A search for thrombosis-inducing diseases, such as disorders of coagulation or fibrinolysis, connective tissue disease, neoplasia or infraction, was negative. Since the phlebitis was strongly inflammatory, frequently recurred and resisted numerous treatments, systemic corticosteroid therapy was instituted, resulting in a dramatic improvement. The steroids were then successfully replaced by antiplatelet agents or, because of the oestrogen dependence, cyproterone maleate. After a mean follow-up period of 2 years, the patients are doing well.

Adrenal Cortex Hormones↗

[Chronic constrictive pericarditis responsible for an increase of CA 125 levels. Two cases].

Despite its poor specificity, CA 125 is a widely used tumoral marker. Significant rises in serum CA 125 levels are found mainly in ovarian cancer, but they also occur in other malignant or benign pathologies such as chronic liver diseases. However, in benign pathologies the CA 125 concentrations hitherto reported did not exceed 850 U/ml. The authors report two cases of chronic constrictive pericarditis with ascites and pleural effusions in which a very high increase of serum CA 125 levels (above 1,200 U/ml) was directly related to the presence of pleural effusions.

Adult↗

[Value of "bolus" cyclophosphamide injections in Behçet's disease. Experience of 17 cases].

Seventeen patients with Behçet's disease were treated with intermittent intravenous bolus injections of cyclophosphamide. Twelve patients had severe eye lesions, 7 had central neurological disorders and 2 had both ocular and central nervous system involvement. Cyclophosphamide was given during 4 weeks on average, in mean doses of 922 +/- 127 mg. Each patient received an average of 10 +/- 5 injections. The drug was generally well tolerated; only one patient developed moderate and transient hepatocytolysis. Visual acuity was improved in 12 of the 22 eyes with active lesions, and signs of inflammation disappeared in all cases. Neurological improvement was observed in 5 of the 7 patients affected, with complete regression of the disorders in 2 of them. In 16 patients who were also under corticosteroid therapy, steroid dosage could be significantly reduced by 44 +/- 11 mg per day.

Adrenal Cortex Hormones↗