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Biomedical subjects

J Goasguen

Publications and source records attributed to J Goasguen.

At least 55 records · Page 3Linked to original sources

[Acquired angioneurotic edema caused by acquired deficiency of C1 esterase inhibitor disclosing lymphoproliferative syndrome. Apropos of a case, review of the literature].

One case of acquired angio-neurotic oedema is described and discussed with the other cases recorded in literature since Caldwell's one in 1972. This entity is characterized by: --the late onset of angio-oedema but its presence only in about half cases, --a complement deficiency resulting from the lack of C1-esterase inhibitor, --the absence of familial identical cases, --the great frequency of associated illnesses overall lympho-proliferative diseases, --the therapeutic response to either etiologic treatment of the associated disease or to the symptomatic effect of drugs used in hereditary angio-neurotic oedema.

Aged↗

A new prognostic classification of chronic lymphocytic leukemia derived from a multivariate survival analysis.

Survivals of two series of CLL patients (99 from a retrospective series and 196 from a prospective series) were studied separately. The three main staging systems (Rai, Binet, Rundles) agreed well, but as far as survival is concerned, too many stages are defined. The authors performed a Cox multivariate analysis of survival in order to isolate important prognostic factors at diagnosis and to use them to build a simple three-stage classification. Thrombopenia and anemia appeared as the most important risk factors. Among the nonanemic and nonthrombopenic patients, the number of involved areas was clearly related to prognosis in the authors' two series. This study allowed the authors to propose a new classification in three prognostic groups. Group C: anemia (Hb less than 10 g) and/or thrombopenia (platelets less than 100,000/mm3); about 15% of the patients; median of 2 years. Group B: no anemia, no thrombopenia, three or more involved areas (counting as one each of the following: axillary, cervical, inguinal, lymph nodes, whether unilateral or bilateral, spleen and liver); about 30% of patients; median of 7 years. Group A: no anemia, no thrombopenia, less than three involved areas; about 55% of patients; the survival of this group does not seem different from that of the French population of the same age and sex distribution. This three-stage classification only requires clinical examination and routine hemogram, has a good prognostic value which was confirmed on the series of Montserrat and Rozman (146 patients), and should therefore be helpful in planning new clinical trials.

Aged↗

[Cytochemical and autoradiographic ultrastructural study of the hairy cell].

The ribosomal nature of the Hairy cell's Ribosome lamella Complex is given by cytochemical ultrastructural reactions. Using Autoradiography after tritiated uridine incubation no labelling was observed on the nucleolus and in the ribosome lamella Complex. Abnormalities in protein synthesis are thus demonstrated.

Autoradiography↗

[Quantitation of myelofibrosis in blood diseases by electronic image analysis (author's transl)].

Normal and pathologic reticulin networks colored black by silver nitrate can be automatically quantitated by electronic image analysis. By using this technique, different parameters can be obtained, such as the average density, the surface of network meshes, the thickness of the fibers, the complexity of the reticulum, and the heterogeneity of the myelofibrosis distribution. All of these parameters were obtained in 83 osteomedullar biopsies of blood diseases (primary splenomegaly, chronic myeloid leukemia, polycythermia vera, acute leukemia, and aplastic anemia). We have shown that there is no relation between the different parameters obtained and the medullary richness, hematopoietic center, or patient survival. On the other hand, the histomorphometric parameters can be used to distinguish acute leukemia and chronic myeloid leukemia myelofibrosis, while the parameters in primary splenomegaly are shown to be very heterogeneous.

Acute Disease↗

[Associated myasthenia and disseminated lupus erythematosus. A report on two cases and complete review of the published literature (author's transl)].

Two patients aged 47 and 48 years respectively were found to have associated immunity disorders: myasthenia and DLE in the first case, and erythroblastic anemia, myasthenia, a lupus syndrome, and a thymoma in the second case. The association of myasthenia and DLE has been reported 39 times in the published literature (20 times only if stricter biological criteria are applied). The association does not increase the severity of the patient's condition and a thymoma is not present more frequently. Studies on the major histocompatibility complex and lymphocyte levels are still insufficient in this context: the haplotype was HLA A1 B8 in three cases out of seven. The physiopathological data available cannot confirm the possibility of a common pathogenesis in which the thymus and lymphocytes could play a determining role.

Antibodies↗

[Rare etiology of hemiplegia with young adult: paradoxical embolism (author's transl)].

Paradoxical embolism is due to the passage of an embolic material from the deep veins of the lower extremities or pelvis, into the systemic circulation through an abnormal intracardiac communication. Only the angiographic and hemodynamic diagnosis practised on two young patients with a cerebral embolism, can explain the mechanism. The diagnosis is based on the arterial embolism, the venous thrombosis with or without pulmonary embolism, the abnormal communication favoring right-to-left shunting. The rising of right atrial pressures permits this shunting. This high pressure can result from a pulmonary embolism, a high blood pressue due to an effort, or an embolism that might coil up over the tricuspid valve.

Adult↗

The gene(s) controlling the synthesis of C1 esterase inhibitor is not linked to the genes of the HLA system and is not on the 6th chromosome.

Certain genes of the complement system are carried by the 6th chromosome and are sometimes linked to particular genes of the HLA system. This study shows that in 15 patients suffering from hereditary angioneurotic oedema the gene(s) controlling the synthesis of C1 esterase inhibitor is not linked to the genes HLA-A or B and is not on the 6th chromosome.

Angioedema↗

[Hodgkin's disease: study of delayed hypersensitivity of 64 non treated patients. Value of epicutaneous test using croton oil].

Cellular immunity in 64 patients with Hodgkin's disease was studied during diagnosis before any treatment. The functional deficiency of the thymodependent lymphocyte was demonstrated in vivo by skin tests and, in vitro, by the test of inhibition of leucocyte migration to phytohemagglutininin and spontaneous rosette formation, to sheep red cells. This deficiency correlates with the clinical spread and the histological severity. The relationship between skin anergy and biological tests was found. The epicutaneous test using croton oil possesses non-specific inflammatory activity. It is negative this test is negative, skin reactivity to specific antigens is reduced. The croton tests help interpretation of skin responses to specific antigens and thus assessment of the immune state.

Adolescent↗

[T.R.H. test in 93 cases of thyroid disease].

The authors used the stimulation of TSH test by intravenous injection of TRH in 93 patients. They obtained a negative or weak response in thyrotoxicosis, toxic adenomas, and Means syndrome, and a normal response with non-extinctive hot nodules. The response was positive and high in primary hypothyroidism. The results are sometimes paradoxal in hypothalamo-pituitary pathology even after hypophysectomy: a delayed and elevated response is in favour of a pituitary reserve of non-utilised TSH. In non-functional goitres and thyroiditis, the sometimes positive responses obtained are an indication for substitutive and depressive therapy.

Adenoma↗

[Not Available].

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France↗