1/10. alpha1-Antitrypsin deficiency and liver disease in children.This report describes the clinical, biochemical, and hepatic morphologic findings in ten children with severe serum alpha1-antitrypsin deficiency. Genetic protease inhibitor (Pi) phenotyping, using acid-starch gel and crossed antigen-antibody electrophoresis, demonstrated Pi phenotype ZZ in all our cases. In eight patients, manifestations of liver disease appeared during the first year of life. The case reports show that alpha1-antitrypsin deficiency should be suspected in any child with neonatal hepatitis, unexplained hepatomegaly or splenomegaly, or cirrhosis. In our report, one infant is normal at age 6 months, and one infant had progressive hepatic damage that culminated in liver failure and death at age 6 months. The variable clinical course and prognosis for infants with severe alpha1-antitrypsin deficiency is well illustrated by these two infants.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
2/10. In utero development of a warm-reactive autoantibody in a severely jaundiced neonate.BACKGROUND: The fetus and neonate are widely considered to be immunologically immature. However, there are rare case reports of RBC alloantibody and autoantibody development. STUDY DESIGN AND methods: This report describes the case of a severely jaundiced full-term boy neonate presenting at birth with an IgG warm-reactive autoantibody. RESULTS: Mother and neonate were both blood group A, D . The mother had a negative antibody screen at 18 weeks' gestation and a negative DAT and antibody screen at the time of delivery. The neonate was born with a strongly reactive DAT (IgG) and a panreactive eluate. The serum also contained a panreactive antibody, and all crossmatches were incompatible. The neonate had a bilirubin of 12.5 mg per dL at birth, which peaked at 22.5 mg per dL. However, there was no overt evidence of hemolysis, as evidenced by normal serial Hct levels and reticulocyte counts. The neonate responded well to phototherapy and did not require either simple or exchange transfusion. The neonate's warm-reactive autoantibody maintained its original strength of reactivity on follow-up testing performed at 2 weeks and 2 months of age. CONCLUSIONS: This report describes a rare case of apparent in utero RBC autoantibody development. The fetal/neonatal immune response to blood group antigens is reviewed.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
3/10. Maternal ABO-mismatched blood for intrauterine transfusion of severe hemolytic disease of the newborn due to anti-Rh17.BACKGROUND: Clinically significant antibodies to high-incident antigens present a challenge in hemolytic disease of the newborn. Antigen-negative blood may be difficult to obtain for intrauterine transfusion (IUT). In these instances, maternal blood is de facto compatible regardless of an ABO mismatch. CASE REPORT: A group B/D-- woman with a history of hemolytic disease of the newborn due to anti-Rh17 (titer 256) presented to the obstetrical clinic at 12 weeks gestation for management of her third pregnancy. She consented to donate blood for possible IUT. STUDY DESIGN AND methods: Washed maternal packed cells were suspended in saline to 75 percent Hct and irradiated before transfusion. The fetus was transfused via the intrahepatic vein. RESULTS: Ultrasound examination at 19 weeks indicated a hydropic fetus. The fetal blood group was O Rh , direct antiglobulin test 4 , and hemoglobin 22 g per L. A total of 368 mL of maternal blood was transfused during seven procedures. Labor was induced at 38 weeks, and a 2560-g male infant was delivered by Caesarian-section due to fetal distress. The infant grouped as B Rh , direct antiglobulin test negative. No group O red blood cells were detected. The hemoglobin level was 143 g per L rising to 209 g per L at discharge 3 days later. The indirect bilirubin was 55 micromol/L and remained stable during the hospital stay. phototherapy was discontinued after 1 day, and the infant was discharged without an exchange or top-up transfusion. CONCLUSIONS: Maternal ABO-mismatched blood is an alternate source for IUT in instances when antigen-compatible allogenic blood is unavailable.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
4/10. Hemolytic disease of the newborn due to anti-C and anti-G masquerading as anti-D.After several transfusions with D-negative blood, an O Rh-negative women was apparently sensitized to the C and D antigens. In her prenatal workup, it became evident that she had in fact not been sensitized to D but to G, which initially appeared as anti-D plus anti-C. This sensitization pattern is an unusual occurrence in itself. Moreover, the fetus was affected significantly and was delivered at 32 1/2 weeks with moderate hemolytic disease of the newborn. Consideration is given to points regarding current methods of screening Rh-negative women for transfusion, the use of anti-Rh immune globulin in patients sensitized to anti-C and anti-G, medicolegal implications, and continuous attention to the risk-benefit ratio in decisions to use transfusions.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
5/10. A new therapeutic antibody removal method using antigen-positive red cells. II. Application to a P-incompatible pregnant woman.Therapeutic antibody removal using antigen-positive red cells was applied to a pregnant woman who has high titered IgG anti-P and had lost all of 4 previous fetuses in P-incompatible pregnancy. The treatment was commenced at the 6th week of gestation and was intensively performed 93 times up to the 35th week. The volume of plasma treated was 2.3 1 per procedure on the average and totalled 215 1. The antibody titer was kept at a low level. At the end of the 35th week of gestation a female infant was delivered by cesarean section. The baby did not require an exchange transfusion and encountered no complications.- - - - - - - - - - ranking = 5keywords = antigen (Clic here for more details about this article) |
6/10. New variant of congenital dyserythropoietic anemia with trilineage myelodysplasia.We report the case of a male infant with a variant of congenital dyserythropoietic anemia (CDA), who developed severe hyperbilirubinemia on the day of birth, subsequent severe anemia, and hyperferritinemia. bone marrow and laboratory examinations revealed features of CDA including trilineage myelodysplasia and erythroblasts with a binucleated nuclear morphology and ineffective erythropoiesis. The CDA in this patient was assumed to be a new variant type because of: the lack of internuclear chromatin bridges in the erythroblasts with abnormal nuclear morphology; a negative acid serum test; the presence of erythrocyte antigen I, and the effect of splenectomy. Trilineage myelodysplasia in CDA is not known. An abnormality in the stem cells was suggested to be the cause of CDA in this case.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
7/10. Hemolytic disease of the newborn caused by maternal anti-Dib: a case report in taiwan.Possibly the first case of hemolytic disease caused by anti-Dib in a Chinese infant is reported. The infant developed jaundice soon after birth; based on study, the jaundice has been diagnosed as a result of maternal anti-Dib which was most likely induced by previous pregnancies. The phenotype of the mother's red cells was Di (a b-). The frequency of the Dia antigen among Chinese in taiwan is 3.2%. In Orientals, hemolytic disease of the newborn caused by maternal anti-Dib is likely to be more severe than that caused by anti-Dia.- - - - - - - - - - ranking = 1keywords = antigen (Clic here for more details about this article) |
8/10. Clincial and edidemiological studies on hepatitis b in children.Sera from children with various clinical categories of hepatitis were examined for hepatitis b surface (HBs) antigen (Ag) by radiommunoassay and for antibody to HBsAg (anti-HBs) by the passive hemagglutination test. It was found that frequencies of HBsAg and anti-HBs in 56 cases of acute hepatitis were 55% (31/56) and 11% (6/56), respectively. All of 4 patients with chronic hepatitis were found to have persistent HBs antigenemia. As for hepatitis in infant under the age of 6 months HBs Ag was detected in 2 each of 31 cases of neonatal hepatitis and of 15 cases of anicteric hepatitis. The data suggest the importance of HB virus as an etiologic agent of acute and chronic hepatitis in children in this country, however, other agents were supposed to be responsible for hepatitis in early infancy. Anti-HBs Ab was first detected in 15% of a group of children age from 3 to 5 years in Rishiri Island and in 12% of children aged from 6 to 9 years in Sapporo City. Frequency increased gradually through school age in both areas. Thus, HB virus infection seems to be common in the childhood population in urban as well as rural area of Hokkaido district.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
9/10. The role of the fetal immune system in the pathogenesis of RhD-hemolytic disease of newborns.Data in the literature and author's research regarding the role of the immune reaction of fetuses and newborns in the pathogenesis of RhD conflict and hemolytic disease of newborns was analyzed. In this disease, the immune response of fetuses and newborns is shown to develop under the effects of maternal antigens, including RhD IgG, which cross the placenta. One of the results is the formation of immune complexes (ICs) between the maternal antigens and fetal IgM. In the intensive immune reaction, these ICs are removed from the infants at a high rate. As a result, the intensity of erythrocyte destruction, the degree of anemia and hyperbilirubinemia decrease. Various forms of HDN are of different intrauterine duration: from a few days in the icteric form without anemia to a month or more, in the hydropic form. In the latter form, decompensation of the immune system develops; extravascular erythroclasia by macrophages is replaced by intravascular lysis of erythrocytes. We suggest some methods to determine the fetal condition and a cure for the most severe cases of HDN, as well as a way of decreasing RhD-sensitization in women. These suggestions may be of interest to specialists in pediatrics and obstetrics and may be of clinical use.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
10/10. Severe neonatal alloimmune thrombocytopenia due to anti-HPA-3a.BACKGROUND AND OBJECTIVES: Neonatal alloimmune thrombocytopenia is usually attributable to HPA-la antibodies. We report a case of parental platelet antigen incompatibility associated with a severe neonatal thrombocytopenia secondary to alloimmunization to HPA-3a. MATERIALS AND methods: Platelet antibodies were detected by the monoclonal antibody-specific immobilization of platelet antigens, genotyping of the platelets by PCR, and HLA typing by serologic procedures and PCR. RESULTS: Genotyping of maternal and paternal platelets confirmed the incompatibility in the HPA-3a system. It is noteworthy that the mother is of the HLA type DRB3*0101, is ABO-incompatible with her husband, and also has HLA class I antibodies. CONCLUSION: Severe neonatal thrombocytopenia associated with HPA-3a alloimmunization is infrequent and all the factors mentioned above could have played a role in the severity of the disease.- - - - - - - - - - ranking = 2keywords = antigen (Clic here for more details about this article) |
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