Biomedical subjects
Publications and source records attributed to B Górska.
We present here extensive clinical and biochemical data on thirteen SLOS (type I) patients with proven defect in cholesterol biosynthesis for further delineation of the classical SLOS phenotype at different patient ages.
Microtest in gel was compared to the traditional, immunohaematological methods. Blood samples of 1.032 persons were tested. From the results presented in this paper we conclude that Gel-test is specific, clear, easy to perform and more sensitive than the traditional methods. It can be used for red cell phenotyping and antibody detection as well. By this technique it was possible to establish the specificity of antibodies of Rh system not found by any other method. The advantage of this test is that nearly all unspecific reactions could be eliminated. The disadvantage concerned the high sensitivity in the detection of cold, clinically insignificant allo- and autoantibodies. Therefore, some modifications for the elimination of these unfavourable reactions were introduced.
In the paper the results are presented of an analysis of the causes of recurrent abdominal pain in 294 children hospitalized at the Gastroenterology Department, Childrens Health Centre, and the accepted diagnostic programme is evaluated. The children were referred to the CHC, as a rule with a suggestion of organic causes of recurrent abdominal pain. On the basis of carried out examinations the following was diagnosed: in 111 children organic causes (37.8% of the studied group), in 94 children recurrent abdominal pain of functional aetiology (32%), in 53 children psychological background (18%), and in 36 children mixed organic and functional background. These proportions between those groups is the result of the selection carried out at earlier stages of the diagnostic process. In the paper, the correctness and validity of the accepted diagnostic programme are also demonstrated.
The activity of monocytes in the ADCC test against human red blood cells sensitized with anti-Rh antibodies is unquestionable, however, a participation of lymphocytes in this reaction is still controversial. The aim of this paper was to test the cytotoxic activity of monocytes and lymphocytes isolated from human peripheral blood against erythrocytes coated with anti-C + D antibodies of Rh system. Due to some individual changes in the activity of mononuclears observed in the ADCC test, it was of interest to test how this activity changes with time, in individual healthy subjects. Both types of mononuclears were observed to damage the sensitized non-treated as well as papain-treated red cells. The cytotoxic activity of these cells, however, showed substantial deviation in the same person.
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Antibody-dependent lymphocyte cytotoxicity was compared in two test procedures. Human erythrocytes (group O R1R1 or R2R2) and mouse lymphoma cells (line L1210) were used as target cells. Anti-Rh (anti-C + D) serum obtained from a hyperimmunized blood donor and serum obtained from rabbit immunized with L1210 cells were used as the source of antibody specific for target cells. In both tests, lymphocytes (PBL) or mononuclear cells (MNC) isolated from heparinized or defibrinated blood were used as effectors. In both tests comparable results were obtained.
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The AutoAnalyser Technicon II system was adapted for screening red cells for the presence of warm-type autoantibodies. A significant decrease in optical density was recorded when the red cells of 38 patients with autoimmune haemolytic anaemia were tested using the bromelin-methylcellulose method. This was never seen with the red cells of 226 patients with other diseases or of healthy blood donors. The method is very simple and more sensitive than the manual direct antiglobulin test.
IgG subclasses of anti-Rh antibodies in the sera of 65 pregnant women were determined. The frequency of subclasses was: IgG1-87.6%, IgG2-23.0%, IgG3-56.9% IgG4-7.7%. In different cases of Rh incompatibility, no correlation was found between the intensity of the hemolytic disease of the newborn and the pattern of IgG subclasses in maternal antibodies. On the basis of the whole material analysis it was observed, however, that the more pregnancies with fetomaternal incompatibility the women had and the more severe the course of the hemolytic disease was in their babies, the more frequently the IgG3 sublcass was present in the pattern of antibodies and the less frequent were the IgG2 and IgG4 subclasses. We have put forward the hypothesis that at the time of strong stimulation of the immunological mechanisms, antibodies are produced by many cell clones and that their activity may decline and disappear with progressing development of clones specifically producing anti-Rh antibodies of the subclasses IgG1 and IgG3.
In 47 serum samples obtained from 31 women immunized with D antigen of the Rh system the level of anti-Rh D antibodies was measured by the papain and antiglobulin tests and the IgG subclasses of anti-Rh antibodies were determined. If the antibodies were of the IgG1 subclass or if this subclass prevailed in the IgG subclass pattern, the antibody level determined by the papain test of Löw was higher or equal to their level found in the antiglobulin test. In all cases, in which the antibodies titer was higher in the antiglobulin test, the antibodies were either of the IgG3 subclass exclusively, or this subclass prevailed quantitatively. This was due to partial inactivation of antibodies belonging to the IgG3 subclass by papain added to the serum in the Löw's test. In the test with papainized erythrocytes (called two-stage test) the IgG subclasses could not be differentiated, and the results obtained then were always higher than in the antiglobulin test. The differences in the reactions of antibodies performed routinely for diagnostic purposes can be explained by differences in immunoglobulin subclasses produced during immunization.
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Forty-four children with autoimmune haemolytic anaemia (AIHA) are described: 31 had acute, subacute or chronic disease with warm autoantibodies and 13 had acute or chronic anaemia with cold autoantibodies. The commonest forms were the acute and subacute types with warm autoantibodies and these were more frequent in young children, while chronic AIHA occurred mainly among children at puberty. In about 16% the anaemia was accompanied by a chronic disorder but in over 50% the anaemia was preceded by an acute infection or immunization. The former gave rise mainly to chronic anaemia, but the latter was associated with the acute and subacute forms. In general the prognosis was good and death was never caused by anaemia per se. The prognosis was worse in patientw with clinical features of thrombocytopenia and bleeding and with the immunological findings of free autoantibodies in the serum and a positive direct antiglobulin test. In acute and subacute forms, treatment with corticosteroids and sometimes with blood transfusions was effective. In chronic forms of the disease it was often necessary to give additional immunosuppressive drugs or/and to perform a splenectomy.
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