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Biomedical subjects

L Intrator

Publications and source records attributed to L Intrator.

At least 55 records · Page 3Linked to original sources
PubMed1986

Spontaneous recurrent fetal wastage and autoimmune abnormalities: a study of fourteen cases.

Case records of 14 women with spontaneous recurrent fetal wastage were analyzed. Venous and/or arterial thrombosis developed in nine patients and vasospastic symptoms in eight. Every patient had some kind of autoantibodies: prothrombin activation inhibitor was present in seven cases, anticardiolipin in five, and, antimitochondrial antibodies in two. Antinuclear antibodies were present in seven cases, and significant titer of antibodies to single-stranded DNA in nine cases. With a mean follow up of 6.5 years no patient developed systemic lupus erythematosus. It is suggested that such women have a peculiar form of vasculitis.

Abortion, Habitual↗
PubMed1985

Immunoblastic lymphoma involving the bone marrow in a patient with alpha chain disease. Clinical and immunoelectron microscopic study.

A patient is reported who had disseminated immunoblastic proliferation that emerged during the course of alpha chain disease. This proliferation was characterized by overt marrow invasion together with osseous and neurologic manifestations. On immunoelectron microscopic study, the malignant immunoblasts displayed varying degrees of cytoplasmic maturation and constituted a morphologic spectrum of alpha-chain-synthesizing cells, ranging from immature blasts without endoplasmic reticulum development to relatively mature plasmablasts; alpha chain was not expressed at the surface of these cells. The general features of the overt malignant stage of alpha chain disease are reviewed in reference to this unusual case. The implications of the cellular findings are discussed with regard to the maturation stage of malignant immunoblasts.

Adult↗
PubMed1982

Impaired IgG synthesis in patients with the nephrotic syndrome.

Immunoglobulin synthesis by pokeweed mitogen-stimulated lymphocytes from 62 nephrotic patients and 18 healthy controls was studied. Defective IgG production was observed in patients with the nephrotic syndrome related to minimal change disease, mebranous glomerulonephritis and membranoproliferative glomerulonephritis. The mean values of in vitro IgG synthesis were 20%, 61% and 32%, respectively, of that obtained in the control group. At the same time, serum IgG levels were significantly decreased in each group of patients. In minimal change disease, reduced IgG production and serum levels were fully reversible after recovery (off steroid therapy). The data indicate that a cellular defect of antibody production is a common and eventually transitory phenomenon associated with the acquired hypogammaglobulinemia found in patients with a variety of glomerulopathies.

Adult↗
PubMed1980

Circulating immune complexes in patients with gram negative septic shock.

In order to explain complement components abnormalities observed during septic shock, circulating immune complexes (C.I.C.) were searched for in sera from 34 patients with gram negative sepsis by two different methods: polyethylene glycol precipitation test based on physical properties of C.I.C. and C1q deviation test based on the property of radiolabelled C1q to react with C.I.C. Serum immunoglobulins (IgG, IgA, IgM) and complement components (C1q, C3, C4) levels were simultaneously determined. Seventeen patients with minimal haemodynamic abnormalities had normal or increased levels (except C4 at 62% of normal) and in eleven cases both tests for C.I.C. were simultaneously positive. Seventeen patients with severe septic shock had a decrease in IgG, IgM C1q, C3 and C4 and none had both tests for C.I.C. simultaneously positive (P less than 10(-4)). The disappearence of C.I.C. in patients with severe septic shock associated with evidence of complement activation suggests their involvement in the pathogenesis of septic shock in man.

Antigen-Antibody Complex↗
PubMed1980

[Evaluation of C3d in primitive chronic glomerular nephritis (author's transl)].

Plasma levels of C3d, which is liberated by enzymatic cleavage of C3, are determined in 97 patients with primitive chronic glomerular nephritis. This level is indicative of an abnormally high consumption of C3. In all cases where C3 is low, C3d is found at abnormally high levels. When the level of C3 is normal, C3d may, however, be elevated: a more subtle interpretation of the pathologic significance of C3 in immune disease is thus possible.

Complement C3↗
PubMed1979

[Measurements of serum C3d in primitive chronic glomerular nephropathies (author's transl)].

Measurement of serum C3 does not provide precise informations concerning an eventual consumption of this complement component during an immunological process. An increased synthetic rate may compensate an accelerated catabolism. The study of breakdown products of C3, such as C3d is a more sensitive approach of the role of complement in some immunological disorders. Therefore C3d was measured in the serum of patients with chronic non systemic glomerular diseases. High values of serum C3d were found in all cases of hypocomplementemic glomerulonephritis. Circulating C3d was also increased to a lower extent, in patients with normocomplementemic nephritis such as minimal change disease, mesangial nephritis with IgA deposits and membraneoproliferative (type I) glomerulonephritis. The data suggested the involvement of complement in a number of glomerulonephritis. Participation of complement in immunological disorders particularly in chronic non systemic glomerulonephritis could require a reevaluation when functional tests are performed in addition to static measurements.

Complement C3↗
PubMed1979

Acquired von Willebrand's syndrome with IgM inhibitor against von Willebrand's factor.

This report describes a patient without evident underlying disease, in whom an acquired von Willebrand's syndrome was discovered before surgery. Coagulation abnormalities included a borderline bleeding time, a low retention of platelets on glass beads, decreased levels of factor VIII procoagulant activity (VIIIAHF), factor VIII-related antigen (VIIIAg), and ristocetin-induced agglutination cofactor (VIIIVWF). After cryoprecipitate infusion the patient did not have the expected rise and there was no secondary increment in VIIIAHF. The patient was treated with prednisone for three weeks without significant improvement in the laboratory findings. Spontaneous resolution was observed long after this therapy. The haemostatic abnormalities were attributable to the presence of an inhibitor directed against VIIIVWF. The inhibitor was found in the IgM fraction. Its autoimmune nature is probable although we failed to demonstrate any inhibitory effect of Fab obtained from the patient's purified IgM. Despite the lack of inhibitory effect against VIIIAHF and VIIIAg, the low levels of all three activities of the factor VIII complex could be explained by the short half-life of immune complexes between factor VIII and the inhibitor.

Aged↗
PubMed1979

A monoclonal macroglobulin with antinuclear activity.

Serum containing a monoclonal IgM protein from a patient with Waldenstroms' macroglobulinaemia gave intense immunofluorescent staining of kidney nuclei. The Fab mu fragments of this immunoglobulin were obtained. The IgM and Fab fragments reacted in vitro with kidney nuclei using unfixed cryostat sections of rat or mouse kidney. After treatment of the patient with chemotherapy, the monoclonal IgM disappeared, and no more antinuclear activity could be detected in the serum. The results strongly suggest that this IgM protein had antinuclear activity.

Adult↗
PubMed1979

[An atypical form of Waldenstrom's macroglobulinaemia with complete remission lasting for more than two years after multiple chemotherapy (author's transl)].

An authentic case of Waldenstrom's macroglobulinaemia without a tumoural syndrome is reported, with initial signs of peripheral pancytopenia. Complete and persistent remission appears to have followed multiple sequential chemotherapy. This very satisfactory and, according to data in the published literature, very rare result suggests that this type of treatment should be prescribed for all patients with macroglobulinaemia with poor prognostic clinical and histological characteristics.

Adult↗
PubMed1978

[Treatment of hereditary angioneurotic edema with androgens].

Hereditary angioedema (HANE) is a rare, life-threatening disease due to the deficiency of C1 inhibitor (C1 Inh). Androgen therapy has been recently shown to be effective for prophylaxis of Hane attacks. Since life-long androgen therapy may be hazardous, this study was designed to define the minimal doses required for effectiveness. Ten patients from six different families were treated during cumulative 73 months by danazol and/for methandrostenolone. One tablet/day of either drug was the minimal requirement to prevent any attack in all patients. When 3 tablets/day were given, complement abnormalities were simultaneously rapidly reversed. When 1 tablet/day was given the biological effect was barely detectable, except for C2. Serum C2 levels may, therefore, represent the best criteria of androgen therapy effectiveness. Thus, an excellent clinical result can be obtained with much lower doses of androgens than previously stated. This result seemed important with respect to the serious dose-dependent risk of androgens.

Adult↗
PubMed1978

[HLA and erythrocyte genetic markers in a family with hereditary angioneurotic edema (author's transl)].

The study deals with a family of 22 members spreading over four generations; 14 members suffer from hereditary angio-neurotic edema; all of them have been typed for 30 antigens of the A and B loci in the HLA System and for ABO and Rhesus erythrocyte markers. There is no connection between the disease and any of the markers considered. As for the HLA system, there should be at least 7 chromosomic recombinants to account for the relationship with one of the haplotypes involved.

ABO Blood-Group System↗
PubMed1977

Modifications of factor VIII related antigen in hemophiliacs with acute hepatitis and sub-clinical liver disease.

Modifications of Factor VIII related antigen (F, VIII R. A.) were investigated in three hemophilic patients during the course of post transfusion hepatitis. F. VIII R. A. was found to be elevated and correlated with the increase of the S.G.P.T. During the acute phase of the disease the Factor VIII related protein was tested for its ristocetin cofactor activity, its electrophoretic mobility on crossed antigen-antibody electrophoresis and its elution pattern on Sepharose 4B columns; all these properties proved to be abnormal. Liver function tests and F. VIII R. A. were measured in 36 multitransfused hemophiliacs. There was a significant increase of the F. VIII R. A. in the plasma of patients with abnormal liver function tests. Only a few of the multitransfused hemophiliacs had clinical symptoms of hepatitis, although 72% had elevated transaminase levels.

Acute Disease↗