Abstract
This Memorandum discusses the problems and techniques involved in the detection of carriers of haemophilia A (blood coagulation factor VIII deficiency) and haemophilia B (factor IX deficiency), particularly with a view to its application to genetic counselling. Apart from the personal suffering caused by haemophilia, the proper treatment of haemophiliacs places a great strain on the blood transfusion services, and it is therefore important that potential carriers should have precise information about the consequences of their having children.The Memorandum classifies the types of carrier and describes the laboratory methods used for the assessment of coagulant activity and antigen concentration in blood. Particular emphasis is laid on the establishment of international, national, and laboratory (working) standards for factors VIII and IX and their calibration in international units (IU). This is followed by a detailed account of the statistical analysis of pedigree and laboratory data, which leads to an assessment of the likelihood that a particular person will transmit the haemophilia gene to her children. Finally, the problems and responsibilities involved in genetic counselling are considered.
MeSH Terms
Antigens/analysis
Blood Coagulation Tests/methods
Factor IX/standards
Female
Genetic Counseling
Genetic Linkage
Hemophilia A/diagnosis,genetics
Hemophilia B/diagnosis,genetics
Humans
Immunoassay/methods
Male
Pedigree
Probability
Statistics as Topic
Chemicals
Antigens
Factor IX
References (18)
18 references, click to expand
-
Linkage Between the X Chromosome Loci for Glucose-6-Phosphate Dehydrogenase Electrophoretic Variation and Hemophilia A.
Am J Hum Genet. 1965 Jul;17(4):320-4
PMID: 17948498
-
Sex chromatin and gene action in the mammalian X-chromosome.
Am J Hum Genet. 1962 Jun;14:135-48
PMID: 14467629
-
Heredity counselling for sex-linked recessive deficiency diseases.
Ann Hum Genet. 1958 Feb;22(2):144-52
PMID: 13509527
-
Effect of antihemophilic factor on one-stage clotting tests; a presumptive test for hemophilia and a simple one-stage antihemophilic factor assy procedure.
J Lab Clin Med. 1953 Apr;41(4):637-47
PMID: 13045017
-
A co-operative study for the detection of the carrier state of classic hemophilia.
N Engl J Med. 1977 Apr 28;296(17):959-62
PMID: 846542
-
The use of biochemical data in screening for mutant alleles and in genetic counselling.
Ann Hum Genet. 1974 Jan;37(3):315-26
PMID: 4812952
-
Detection of the carrier state in hereditary coagulation disorders. I.
Thromb Diath Haemorrh. 1968 Mar 31;19(1):279-303
PMID: 5652235
-
Immunoradiometric assay of factor VIII related antigen, with observations in 32 patients with von Willebrand's disease.
Br J Haematol. 1976 Jun;33(2):221-32
PMID: 1083743
-
Calculation of predictive odds for possible carriers of heamophilia.
Thromb Diath Haemorrh. 1975 Dec 15;34(3):740-7
PMID: 1209543
-
Probabilistic classification of hemophilia A carriers by discriminant analysis.
Thromb Res. 1976 May;8(5):683-95
PMID: 936111
-
Determination of the von Willebrand's disease antigen (factor VIII-related antigen) in plasma by quantitative immunoelectrophoresis.
J Lab Clin Med. 1975 Jul;86(1):152-9
PMID: 1080496
-
Evaluation of the detection rate of hemophilia carriers.
Thromb Res. 1975 Aug;7(2):339-50
PMID: 51518
-
Immunologic studies of antihemophilic factor (AHF, factor VIII). IV. Radioimmunoassay of AHF antigen.
J Lab Clin Med. 1972 Dec;80(6):822-33
PMID: 4634503
-
Blood-coagulation factor 8: genetics, physiological control, and bioassay.
Adv Clin Chem. 1965;8:189-236
PMID: 5321718
-
Extensions to pedigree analysis I. Likehood calculations for simple and complex pedigrees.
Hum Hered. 1975;25(2):95-105
PMID: 1150306
-
A general model for the genetic analysis of pedigree data.
Hum Hered. 1971;21(6):523-42
PMID: 5149961
-
Immunologic differentiation of classic hemophilia (factor 8 deficiency) and von Willebrand's dissase, with observations on combined deficiencies of antihemophilic factor and proaccelerin (factor V) and on an acquired circulating anticoagulant against antihemophilic factor.
J Clin Invest. 1971 Jan;50(1):244-54
PMID: 5543879
-
Quantitative estimation of proteins by electrophoresis in agarose gel containing antibodies.
Anal Biochem. 1966 Apr;15(1):45-52
PMID: 5959431