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Biomedical subjects

F Danon

Publications and source records attributed to F Danon.

At least 37 records · Page 2Linked to original sources
PubMed1990

C4 null alleles in childhood onset systemic lupus erythematosus. Is there any relationship with renal disease?

C4 genotyping was performed in 38 unrelated patients with systemic lupus erythematosus (SLE) aged 2-16 years at onset. Null alleles were found in 68% of patients. Ten patients had one null allele at the C4A locus and 11 others had one null allele at the C4B locus. One patient was homozygous for C4A*Q0, 2 homozygous for C4B*Q0 and 1 heterozygous C4A*Q0/B*Q0. The last patient was C4A*Q0B*Q0/B*Q0. Three patients, being heterozygous C2 deficient, had thus a combined C2 and C4 deficiency. A significant increase in C4 null alleles in SLE patients has been reported by different authors who have suggested that the C4 null alleles confer susceptibility to SLE. However, when considering only the 20 patients of French descent, no differences in gene frequencies were found between this group and the French population. Disease patterns were compared in patients with or without null C4. Renal involvement was more frequent in the C4A*Q0 or C4A*Q0/C4A*Q0 patients than in the patients without null C4 (9/11 vs 3/12). These data suggest that differences between these results and those of others might be due to clinical heterogeneity of the disease, as exemplified by the frequency of renal involvement in the different groups. In susceptible individuals the absence of one C4A gene product may predispose the patient to renal involvement.

Adolescent↗
PubMed1990

Antinuclear antibodies directed to a 200-kilodalton polypeptide of the nuclear envelope in primary biliary cirrhosis. A clinical and immunological study of a series of 150 patients with primary biliary cirrhosis.

Antinuclear antibodies giving a perinuclear fluorescence and directed to a 200-kilodalton polypeptide of the nuclear envelope have been described in primary biliary cirrhosis. The purpose of this study, based on a series of 150 patients with primary biliary cirrhosis, was to ascertain the prevalence of these antibodies and to compare patients with and without these antibodies. Antinuclear antibodies giving a perinuclear fluorescence were demonstrated in 43 of the 150 patients (29%); antibodies directed to the 200-kilodalton polypeptide of the nuclear envelope were found in 40 of these 43 patients. Asthenia, arthralgia, associated extrahepatic diseases, Raynaud's phenomenon, and other antinuclear specificities were significantly less common, and titers of antimitochondrial antibodies were significantly lower in patients with antibodies directed to the 200-kilodalton polypeptide of the nuclear envelope than in patients without these antibodies. Clinical outcome, liver tests, and histological lesions did not significantly differ in patients with and without these antibodies.

Antibodies, Antinuclear↗
PubMed1990

Reactivity of human monoclonal IgM with nerve glycosphingolipids.

We examined the reactivity of monoclonal IgM of sera from patients with neuropathy and monoclonal IgM, with or without antibody activity to myelin-associated glycoprotein (MAG), as well as sera from non-neurologic patients with Waldenström's macroglobulinaemia, with various nerve glycolipids extracts or with purified gangliosides. As expected from previous studies, all (five cases) anti-MAG IgM stained two glycolipids, the chemical characteristics of which corresponded to sulphated glucuronyl-paragloboside (SGPG) and sulphated glucuronyl-lactosaminyl-paragloboside (SGLPG). Five of 12 sera from patients with neuropathy whose IgM was devoid of anti-MAG reactivity stained nerve extracts greatly enriched (98%) with SGPG and SGLPG. Three of these five sera reacted with additional glycolipids and/or gangliosides. Two of 16 sera from patients with macroglobulinaemia without neuropathy reacted strongly with both SGPG and SGLPG. The latter finding as well as the detection of low titre of anti-sphingolipid antibodies in normal sera may cast a doubt on the pathogenetic significance of this antibody activity.

Antibodies, Monoclonal↗
PubMed1990

Characterization of human autoantibodies specific for lamin A.

We have characterized human autoimmune polyclonal antibodies reactive with lamin A, a 74 kDa peripheral protein of the nuclear envelope. Unlike other known antibodies to lamin A, the antibodies described here do not crossreact with the structurally related lamin C. These antibodies feature only chi light chains suggesting that their specificity is restricted to a limited number of epitopes. Based on the known amino acid sequence of human lamins A and C, the epitope(s) are most likely located in the 80 amino acid carboxyl tail of mature lamin A.

Antibody Specificity↗
PubMed1990

Peripheral polyneuropathies associated with monoclonal IgM. Antibody activity of monoclonal IgM and therapeutic implications.

Monoclonal IgM from patients with peripheral neuropathy react in nearly 80% of the cases with some components of myelin. The main target is myelin associated glycoprotein (50% of the cases); several types of glycolipids or gangliosides have been identified as the reactive antigen in the other cases. Anti-MAG IgM share little cross reactive idiotopes. Only primate antisera identified a combining site related public idiotope. In fact, these IgM use various light and heavy chains belonging to different variability subgroups. The preliminary results of an ungoing trial aimed to establish the benefit of plasmapheresis in these patients are discussed.

Adult↗
PubMed1990

[Peripheral neuropathy and plasmocytic proliferations. Apropos of 17 cases].

Seventeen cases of peripheral neuropathy with plasmocytic proliferation were grouped together to analyze the clinical manifestations and evolution of this association of symptoms. The plasmocytic proliferation was 5 times that of multiple myeloma, 9 times that of a solitary bone plasmacytoma and 3 times that of a solitary plasmacytoma. The follow-up lasted 1 to 18 years (mean, 8 years) for solitary plasmacytomas. Other cutaneous and endocrinological symptoms, organomegaly and edema were sometimes present. The evolution was relatively severe, except in 6 patients whose solitary plasmacytomas were successfully treated with radiation. Six patients died 2 to 8 years (mean, 5 years) after the onset of neuropathy as a result of a progression of the neuropathy and/or anasarca (4 cases), or acute leukemia (2 cases) abetted by long-term chemotherapy. The therapeutic approaches available, the nature of the plasmocytic proliferation and its association with the neuropathy and the other symptoms are discussed.

Adult↗
PubMed1990

[Serum antibodies in dogs with autoimmune disorders recognize 40 kd glycoprotein in the nucleus of mammalian cells].

We report a new antibody specificity in 15 sera recovered from a group of dogs developing systemic lupus erythematosus (SLE) or clinically related disorders. This antibody stains in a speckled fashion the nucleus of human Hep-2 cells. Immunodiffusion tests with saline extracts of rabbit thymus showed that all 15 sera generate a common precipitation line which crosses the lines from reference sera to Sm, SS-A/ro, SS-B/La, and RNP antigens. The target nuclear antigen is a 40 kD polypeptide (p40). An important property of p40 resides in its ability to bind specifically Wheat Germ Agglutinin lectin but not Concanavalin A, supporting the notion that the antigen is a glycoprotein bearing a N-acetylglucosamine moiety.

Animals↗
PubMed1989

Nucleolar proteins identified in human cells as antigens by sera from dogs with autoimmune disorders.

In the course of a systematic screening of sera from dogs suffering from autoimmune disorders, three sera were shown by indirect immunofluorescence to characteristically label the nucleoli and nucleoplasm of human cell lines (Hep-2 and HeLa). This pattern of staining persisted throughout the cell cycle, except for mitosis when the fluorescence was localized in extrachromosomal areas. By immunoblotting nuclear and subnuclear fractions, three polypeptides of 110,000, 95,000, and 45,000 Da apparent molecular weight were identified, which reacted with all three sera. By means of affinity purification, it was shown that an antibody specific for any one of the three proteins also reacts with the two others. This antigenic cross-reactivity suggested regions of structural homology shared by the three proteins. Indeed, treatment of nucleoli with high concentrations of DNase I containing residual proteolytic activity resulted in the disappearance of the 110- and 95-kDa proteins and the concomitant appearance of a doublet of 45-kDa proteins. Subnuclear localization studies indicated that all three polypeptides were located in both nucleoli and nucleoplasm. Significantly, the 110-kDa protein differs from the major nucleolar protein, nucleolin, by its electrophoretic mobility in two-dimensional gels, its location in nucleoli and in nucleoplasm, its absence in nucleolar organizer regions of chromosomes, and its differential solubility of DNase I. Therefore, the three antigenically related species reported in this study constitute a new class of nucleolar proteins.

Animals↗
PubMed1989

Gamma heavy chain "disease": heterogeneity of the clinicopathologic features. Report of 16 cases and review of the literature.

This review underscores the diversity of the clinical manifestations and hematopathological features of gamma heavy chain disease based on the detailed report of 16 patients evaluated in our chemical department, the analysis of 12 cases diagnosed in our laboratory, and the study of 81 cases previously reported. This condition is defined by the presence in the serum of immunoglobulin molecules composed of deleted gamma heavy chains devoid of light chains. The production by the monoclonal B cells of these peculiar proteins appears to result from multiple defects (deletions, insertions, and mutations) in both heavy and light chain genes leading to abnormal mRNA splicing. Gamma heavy chain disease is currently underdiagnosed. The diagnosis established by immunoelectrophoresis using specific antisera combined, in some instances, with the immunoselection procedure, can easily be missed on serum electrophoretic patterns: a narrow abnormal band suggestive of a monoclonal component was found in only 10 of our 28 cases. The amount of heavy chain disease protein in urine ranges from trace to 20 g/day and is usually moderate. Gamma heavy chain disease most often presents as a lymphoproliferative disorder featured by lymphadenopathies, splenomegaly, and constitutional symptoms. Extra-hematopoietic tumor localizations, such as cutaneous or subcutaneous involvement or thyroid tumor, may occur. Autoimmune disorders, notably rheumatoid arthritis and autoimmune hemolytic anemia or thrombocytopenic purpura, are frequent (26% of cases). There is no specific histological pattern. The most frequent is a pleomorphic malignant lymphoplasmacytic proliferation mainly seen in bone marrow and lymph nodes. Some cases present with a predominantly plasmacytic proliferation or chronic lymphocytic leukemia. Other patients are affected with non-Hodgkin lymphoma of various morphologic types. Immunocytologic studies showed that a gamma heavy chain disease protein may occur in the context of a double monoclonal lymphoproliferative process or in various B or T cell malignancies that are not directly involved in the production of the abnormal immunoglobulin. In some patients, the histologic appearance of the enlarged lymphoid organs showed only a moderate lymphoplasmacytic infiltration of uncertain malignancy. More important, some patients showed no evidence of an underlying lymphoproliferative disorder after several years of follow-up. The clinical course of gamma heavy chain disease varies from an asymptomatic state to a rapidly progressive malignancy. The choice of therapy should entirely rely on the underlying clinicopathologic features, without taking into account the presence of the abnormal protein.(ABSTRACT TRUNCATED AT 400 WORDS)

Adult↗
PubMed1988

Bronchoalveolar lymphocytosis in patients with tropical spastic paraparesis associated with human T-cell lymphotropic virus type 1 (HTLV-1). Clinical, immunologic, and cytologic studies.

STUDY OBJECTIVE: To determine the features of pulmonary involvement in patients with tropical spastic paraparesis associated with human T-cell lymphotropic virus type 1. DESIGN: Nonrandomized prospective case series. SETTING: Tertiary care units in two university medical centers. PATIENTS: Consecutive sample of 21 patients with tropical spastic paraparesis associated with human T-cell lymphotropic virus type 1 infection. INTERVENTIONS: Chest roentgenogram and bronchoalveolar lavage were done in all patients. Fifteen patients had pulmonary function tests. Alveolar T-lymphocyte subsets were analysed in 10 patients and thoracic computed tomographic scans were done in 10 patients. MEASUREMENTS AND MAIN RESULTS: All patients were free of clinical pulmonary symptoms and had normal chest roentgenograms. Thoracic computed tomographic scans were normal in 9 of 10 patients and showed mild interstitial pneumonitis in 1. Pulmonary function tests were within the normal range in 13 patients and showed a mild restrictive syndrome in 2. Eighteen patients had increased absolute numbers of alveolar lymphocytes (mean, 77 +/- 39 X 10(3) lymphocytes/mL; range, 13.5 X 10(3) to 259 X 10(3) lymphocytes/mL). Sixteen patients had percentages of alveolar lymphocytes higher than 20% of all alveolar cells (mean, 33.5 +/- 12.7; range, 9 to 69). In all 10 patients tested, 64.2% +/- 13.2% of alveolar lymphocytes were CD8+ cells. CONCLUSIONS: Excessive absolute numbers and percentages of alveolar lymphocytes were observed in 18 and 16 patients, respectively. Most alveolar lymphocytes were CD8+ cells.

Adult↗
PubMed1988

Impaired T-lymphocyte-dependent immune responses to microbial antigens in patients with HIV-1-associated persistent generalized lymphadenopathy.

T-cell mediated and humoral responses directed to microbial antigens were investigated, at the time of the initial visit, in a group of 139 patients with HIV-1-related persistent generalized lymphadenopathy (PGL) enrolled in a longitudinal study. In vivo and in vitro cell-mediated responses to tuberculin were lower in patients than in controls. Differences were not significant for candidin and streptococcal antigen in vitro, whereas higher responses were observed in the patient group for cytomegalovirus antigen. Following immunization, a subgroup of patients did not have a significantly raised serum antitetanus antibody level, whereas in vitro lymphocyte proliferative responses to tetanus toxoid were lower than in controls. No association was found between these abnormalities and other immunological parameters, including the blood level of CD4+ lymphocytes. Lower responses to most microbial antigens were observed in patients with HIV-1-related symptoms in addition to lymphadenopathy, or the patients who progressed to AIDS in the 2 years following the study. Moreover, intravenous drug users showed higher responses than homosexual patients, possibly because of the influence of previous infections on immunological responses to microbial antigens.

Acquired Immunodeficiency Syndrome↗
PubMed1988

Antinuclear autoantibodies specific for lamins. Characterization and clinical significance.

In 11 patients, sera displaying a ringlike nuclear immunofluorescent staining on sections of rat liver tissue were shown by Western blotting to contain antibodies to lamins. Sera from 8 patients contained autoantibodies reacting with lamin B, whereas sera from the other 3 patients reacted with lamins A and C. All patients (9 women and 2 men) had a chronic autoimmune disorder, which rarely fulfilled the usual criteria for a diagnosis of systemic lupus erythematosus. The disorder was characterized by acute or chronic (active or granulomatous) hepatitis; steroid-responsive blood cytopenia, often associated with a circulating anticoagulant, or anticardiolipin antibodies, or both; and cutaneous leukocytoclastic angiitis or probable brain vasculitis. Eight patients had at least two of these three conditions. Antilamin autoantibodies may thus be a marker for an unusual subset of autoimmune diseases.

Adult↗
PubMed1988

Autoantibodies to 200 kD polypeptide(s) of the nuclear envelope: a new serologic marker of primary biliary cirrhosis.

Fifty sera which gave ring-like nuclear staining in immunofluorescence on rat liver tissue sections were characterized. Using immunoprecipitation and Western blotting we showed that 10 of the sera contained antibodies to 200 kD polypeptide(s) of nuclear envelope. Clinical and biological data were available for nine of the patients. Strikingly, all of these patients suffered from primary biliary cirrhosis with eight of them having anti-mitochondrial antibodies. As no control serum displayed such a reactivity, anti-200 kD polypeptide(s) antibodies can be considered as a new marker specific of a subset of primary biliary cirrhosis, being present even when anti-mitochondrial antibodies are absent. The exact identity of the target remains to be established, since several polypeptides of similar molecular weight have been reported to belong to the nuclear envelope.

Adult↗
PubMed1988

Isotypic distribution of anti-double-stranded DNA antibodies: a diagnostic evaluation by enzyme-linked immunosorbent assay.

Anti-native DNA antibodies were studied using an immunoglobulin class-specific enzyme-linked immunosorbent assay (ELISA) in 450 sera, virtually all of which were antinuclear antibody positive. ELISA was positive in about 85% of systemic lupus erythematosus (SLE) sera, usually at high titer and for two or three isotypes. Virtually all sera with antibodies of the three main classes were collected from SLE patients. Very high titers were unique to SLE. In contrast, low-titer antibodies of a single, mostly IgM, class were found in certain patients without evidence of autoimmune disease or with non-SLE autoimmune diseases. The isotypy and titer of the antibodies hence appear to be critical parameters for a correct interpretation of results. Under these conditions, ELISA seems to be usable as single screening test for the demonstration of anti-DNA antibodies.

Autoantibodies↗