| 1. |
- Holmberg, Lars, et al.
(författare)
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Prenatal diagnosis of hemophilia B by an immunoradiometric assay of factor IX
- 1980
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Ingår i: Blood. - 0006-4971. ; 56:3, s. 397-401
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Tidskriftsartikel (refereegranskat)abstract
- An immunoradiometric assay of factor IX was developed based on homologous antibodies that arose in a hemophilic patient. With this assay, 11 of 12 patients with severe hemophilia B had factor IX antigen levels below 1 U/dl and 6 patients with mild hemophilia B had various levels. Factor IX antigen in 8 fetuses (16th-20th gestational week) aborted for therapeutic reasons ranged from 1.8 to 10.0 U/dl. Six amniotic fluids contained 0.28-1.2 U/dl factor IX antigen. Using the immunoradiometric assay, we could diagnose hemophilia B prenatally in one fetus at risk. No factor IX antigen (< 0.2 U/dl) was detectable in the fetoscopic sample. After termination of the pregnancy, analysis of blood from the abortus confirmed the diagnosis of severe hemophilia B. We conclude that very sensitive immunologic assays, such as the one described here, will prove useful in prenatal diagnosis of severe hemophilia B, since determination of factor IX activity in fetoscopic samples is unrealiable because of possible contamination with thromboplastic material.
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| 2. |
- Ljung, R, et al.
(författare)
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Ganglioneuroma with an uncommon location in a six-year-old girl
- 1984
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Ingår i: Acta Paediatrica Scandinavica. - 0001-656X. ; 73:3, s. 3-411
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Tidskriftsartikel (refereegranskat)abstract
- A 6-year-old girl with a neurogenic bladder disease is described. Additional gait disturbances and mild but progressive foot deformities led to the diagnosis and surgical treatment of a ganglioneuroma located exclusively intraspinally. It is suggested that the tumor originated from remnant neural crest cells, probably early in life or even prenatally.
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| 3. |
- Nilsson, I. M., et al.
(författare)
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Suppression of Secondary Antibody Response by Intravenous Immunoglobulin in a Patient with Haemophilia B and Antibodies
- 1983
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Ingår i: Scandinavian Journal of Haematology. - : Wiley. - 0036-553X. ; 30:5, s. 458-464
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Tidskriftsartikel (refereegranskat)abstract
- A 39‐year‐old patient, suffering from severe haemophilia B and antibodies against factor IX, has twice been treated with extracorporeal protein A‐Sepharose adsorption followed by conventional substitution therapy in combination with immunosuppression (cyclophos‐phamide). On both occasions, separated by a 2‐year interval, the same procedure was followed except that, on the second, administration of i.v. immunoglobulin (Gammonativ, KabiVitrum) was added. Within a week of the first treatment the patient developed a 15‐fold increase in the antibody titre. Following the second treatment described here, no secondary antibody response could be detected, and after a further 12 weeks only traces of antibodies are demonstrable. It seems that antibody synthesis was suppressed by the i.v. immunoglobulin. No evidence was found to demonstrate that the effect was due either to a non‐specific suppression of the immune and reticuloendothelial systems or to the action of interfering antibodies. It has not yet been established whether or not the protein A‐Sepharose adsorption technique, or the immunosuppressive treatment, contributed in any way to the result. The observations suggest a new approach to the treatment of haemophiliacs with antibodies of the high‐responding type.
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