[Congenital dyserythropoietic anemia type I. Report of pair of siblings (author's transl)].
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Biomedical subjects
Publications and source records attributed to T Kuroume.
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This is the first case of two siblings with congenital dyserythrobpoietic anemia (CDA) type I to be reported from a Japanese family. Both of these cases showed characteristic morphological aberration of erythroid precursors, ineffective erythropoiesis, and negative acidified serum test. The ultrastructural study of erythroblasts also revealed characteristics quite compatible with CDA type I.
Human fetal lymphoid cells from the thymus, blood, spleen, liver, and kidney of twelve 6-to-24-wk-old fetuses were studied for their ability to respond to thymosin by the alteration of E-rosette forming cells (E-RFC) and EAC-rosette forming cells (EAC-RFC). The preincubation of spleen cells with 100 micrograms thymosin-V1 effected a statistically significant increase in E-RFC, while no changes were observed in the lymphoid cells from blood or other organs. EAC-RFC assay revealed some increases in blood, thymus, and spleen; however, they were not statistically significant. Our results suggest that the fetal spleen may play an important role in the development of primitive immunocytes into mature cells and that this development is affected by thymic hormone.
Two protein fractions of rice, grain, glutelin and globulin, were prepared by dilute alkali and salt extraction, respectively. The globulin fraction was separated into G1-1, G1-2, and G1-3 fractions by Sephadex G-200 column chromatography. The allergenic activities and lymphocyte-stimulating properties of these fractions were investigated by the radioallergosorbent test (RAST) with sera from 6 individuals who showed immediate skin reaction to soluble rice extract and by 3H-thymidine incorporation tests with 5 subjects with indurated skin reaction of delayed onset. All fractions were found to be reactive with specific IgE antibody, and G1-1 and G-2 revealed lymphocyte-stimulating activity. RAST inhibition revealed considerable cross-reactivity of IgE antibody with the glutelin and globulin fractions. When the glutelin and globulin fractions were heated at 60 degrees C for 1 hr, 100 degrees C for 2 min, or 100 degrees C for 10 min, RAST activities were reduced to 40%-70% of native. On the other hand, lymphocyte-stimulating activities of the globulin fraction heated at 60 degrees C for 1 hr or 100 degrees C for 2 min were enhanced up to 6 times of native activities, while those of identically treated glutelin fractions remained unchanged.
Plasma obtained from patients with MCNS in active stage suppressed the 3H-TdR incorporation of autologous or homologous lymphocytes stimulated with PHA-P and E rosette formation, while plasma from patients in remission did not. The depressed mitogenic response of lymphocytes from patients in the active stage significantly improved when the patient's plasma was substituted for the plasma from inactive MCNS or healthy persons and fetal calf serum. In the preliminary experiment, it was seen that the inhibitory factors were heat stable at 56 degrees C, unadsorbable to charcoal powder and retained in both the permeable and impermeable fractions of collodion bag.
An enzymatic hydrolysate of bovine casein were studied for molecular size and antigenic properties. As determined by Sephadex G-25 gel filtration, polypeptide fractions having molecular weights greater than 1,000 were not detected in the soluble fraction of the casein hydrolysate. Antigenicity of the fraction was not detected by heterologous passive cutaneous anaphylaxis in guinea pig-rabbit antiserum system mediated by immunoglobulin G types of antibody (guinea pig passive cutaneous anaphylaxis) and its inhibition test. Furthermore, it was not detected by rat long latency homologous mediated by immunoglobulin E antibody and its inhibition test. To prepare anticasein immunoglobulin E serum for rat long latency homologous passive cutaneous anaphylaxis, immunization of rats was by single subcutaneous injection of casein with aluminum hydroxide. Antiserum with highest passive cutaneous anaphylaxis titer (1:64) against bovine casein was obtained by a dose of 2,000 micrograms of the protein at day 12.
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Human lymphocyte colonies were grown in semi-solid culture in the presence of staphylococcal protein A. The best results were obtained when 1 X 10(6) lymphocytes were cultured for 5 to 7 days with 25 to 50 microgram of protein A per dish. A lineal relationship was observed between the number of cells seeded and the number of colonies developed. An increased number of colonies was seen in cord blood culture compared with that in child and adult peripheral blood. The addition of 5 X 10(-5) M of 2-ME to the culture approximately doubled the colony counts. The cells harvested from the colonies contained about 70% of E-RFC and 20% of EAC-RFC.
Homocytotrophic antibodies (HCA) were produced in rats by primary injection of streptokinase-streptodornase (SK-SD) after the antigen was absorbed on alminum hydroxide gel. The properties of rat SK-SD-specific HCA were qualitatively similar to SK-SD skin-sensitizing antibodies found in sera of human beings, namely, homocytotropic activity (48 h homologous PCA reaction), heat lability at 56 degrees C, and inactivation by reduction with 2-mercaptoethanol followed by alkylation with iodoacetamide. In neutralization tests, D-SK-SD, a low molecular hapten-like substance contained in SK-SD preparation, absorbed the antibody against ND-SK-SD antigen-specifically. The neutralizing activity mostly resided in fraction 1 of D-SK-SD separated through DEAE-Sephadex A-25 ion-exchange column, opposing the inhibitory activity of fraction 3 on lymphocyte transformation. The possible implication of these findings in the immunological treatment of streptococcal disorders is discussed.
The immediate skin reaction (IR) with streptokinase-streptodornase (SK-SD) was found with a high incidence in children with renal disease and was confirmed to be mediated by a specific IgE antibody against SK-SD by means of the radioallergosorbent test (RAST). This test revealed high radiocounts in children with renal disease who showed the IR and there was a close correlation between the RAST counts and the intensity of the IR. Furthermore, the RAST counts in the severe nephrotic syndrome belonging to corticosteroid dependent and resistant group were far higher than those in the corticosteroid sensitive group. From these results, a determination of the RAST counts against SK-SD seemed to help evaluate the severity of some nephrotic syndromes and the specific IgE antibody may be involved in the pathogenesis of the disease.
A girl aged 14 years, who showed local skin lesions and generalized reactions such as fever, hepatosplenomegaly, hypergammaglobulinaemia and pancytopenia following mosquito bites, has been evaluated with various immunological methods. The intracutaneous tests, P-K tests and RAST confirmed the presence of specific IgE antibody against mosquito antigens, but no precipitating antibody was detected by either the double immunodiffusion or immunofluorescent antibody techniques. As far as the type IV reaction is concerned, the skin test failed to show a positive delayed skin reaction, but the lymphocyte incorporation of 3H-TdR exhibited a maximal response with smaller doses than the controls.
Firstly, it was reported that phytohemagglutinin P (PHA-P) can induce the immediate skin reaction besides the delayed one. 70 out of 80 cases (87.5%) gave positive immediate reactions with 1 mug of PHA-P. There was a close correlation between the radioallergosorbent test (RAST) counts against PHA-P and the total IgE value or erythematous diameter of the immediate skin reactions. Absorption with anti-IgE or PHA-P reduced the RAST counts against PHA-P to 79 and 56% of their initial values, respectively, but did not effect the RAST counts against steptokinase-streptodornase (SK-SD). These results suggest that the immediate skin reaction might be induced by specific IgE antibody against PHA-P. Secondly, using three fractions of PHA-P obtained by Sephadex G-200 gel filtration, skin test and RAST in humans, ACA in rats, and 3H-thymidine incorporation into human leukocytes, were carried out and data obtained that showed some discrepancy between them. That is, on skin testing, it was shown that all of the three fractions (fr.) had the ability to elicit the immediate and delayed skin reaction in humans and rats with the order of fr. 1, 2 and 3, and it was confirmed by RAST. On the other hand, the leukocyte-stimulating activity resided in fr. 1 and 2, while fr. 3, which seemed to be a group of subunits with a molecular weight of around 35,000, had almost no activity. These findings have been discussed in view of the earlier studies on the structure of PHA-P
Using the 3H-thymidine incorporation test, the lymphocyte-stimulating activity of streptokinase-streptodornase (SK-SD, Varidase) and its fractions were evaluated. The dose-response curves of all the preparations except the third fraction had two peaks with the maximum around 5 and 40 mug. The dialyzable fraction of Sk-SD (D-SK-SD) occupying approximately half of the original sample had almost no lymphocyte-stimulating activity; and conversely, it had a potent inhibitory effect on the 3H-thymidine incorporation into lymphocytes that normally occurs with non-dialyzable SK-SD (ND-SK-SD), PPD and PHA-P. Therefore, it will be desirable to remove the dialyzable fraction from the commercial SK-SD preparation when it is used for the determination of the capacity of cellular immunity against tsk-sd.
Three cases of milk sensitivity and two of soybean sensitivity are presented, suggesting that both pre-natal and post-natal sensitization may have been involved in the production of eczematous manifestations of early infancy. Hemagglutinating antibody titers against lactalbumin and soybean in the amniotic fluid were unusually high. This suggests that hemagglutinating antibody estimation against foods in the amniotic fluid may possibly predict infantile allegic manifestations in the newborn.
Streptokinase-streptodornase (SK-SD) induced immediate and delayed skin test reactions in a high proportion of patients with disorders in which beta-streptococcal infection may be involved. The P-K test and absorption experiments with antigen or anti-IgE suggested that the immediate reaction was mediated by specific IgE antibody against SK-SD. Furthermore, the ratio of specific IgE antibody against SK-SD to total IgE was roughly calculated to be 20 percent. Finally, the RAST technique was applied to detect specific IgE antibody against SK-SD which showed high radiocounts bound to SK-SD-coupled particles in the sera of nephrotic patients who had a strong immediate reaction against SK-SD and a severe disease state.
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Using the scratch test with self-made chrysanthemum pollen extract of 32,700 PNU/ml, a positive response was elicited in 60 of 316 patients (18.9%) with allergic rhinitis and bronchial asthma, and was positive in 42.5% adults with allergic rhinitis. On the other hand a 4.7% positive response was obtained in 84 non-allergic subjects. With the intracutaneous test, a threshold value was determined in 7 cases with 0.327 PNU/ml; 12 with 3.27 PNU/ml; 8 with 32.7 PNU/ml; and 8 with 327 PNU/ml, as opposed to one positive in 84 controls with 327 PNU/ml (1.3%). P-K tests were successfully done in 15 out of 16 cases. Furthermore, the results of in vitro neutralization tests using chrysanthemum and other compositae pollen extracts indicated the absence of sharing antigenic determinants between them. Provocation tests were conducted in 4 cases of allergic rhinitis and 3 cases of bronchial asthma with positive response in all the patients. From these results it was found that chrysanthemim pollinosis indeed exists in Japan, particularly in the mountainous districts.
In 10 newborn breast-fed infants with eczematous manifestations, sensitivity to egg was confirmed by indirect elimination and provocation tests in the mother and direct provocation and elimination tests in the infant in the weaning period. In almost all of the mothers hemagglutinating antibody titers against egg were high in the amniotic fluid. The role of intra-uterine sensitization to food in the etiology of eczematous manifestations in newborn breast-fed infants is discussed.