Hypogammaglobulinaemia-Allen and Hadden MMIBALTJORH the presence of the hypogammaglobulinaemia gene on the X chromosome.The X is a long chromosome, with about 150 cross-over units, and linkage cannot be detected between genes approaching 50 cross-over units apart.The appearance of close linkage must probably be understood as a matter of chance, but this family can nevertheless be used as an illustration of how informative a real close linkage with a marker gene could be (Fig. 4).II 2 is heterozygous XgaXg, for she is Xg(a +) and has Xg(a -) children.To her two sons, III 3 and III 4, she has given Xg gene and her hypogammaglobulinaemia gene ; but to her daughter, III 2, she has given her Xga gene and, barring crossing over, a gene not determining hypogammaglobulinaemia.III 2 should therefore not be a carrier: that she appears not to be, by having two normal sons, is probably a matter of chance; for the close linkage invoked in this demonstration, we must repeat, in all probability does not exist. SummaryTwo adult brothers, aged 25 and 27, were found to have "congenital" hypogammaglobulinaemia associated with steatorrhoea.Their clinical state has responded to parenteral gammaglobulin replacement, but steatorrhoea persists in mild degree and is not responsive to a gluten-free regime.Both brothers have had severe intermittent anaphylactic-like reactions to the injected gammaglobulin.Members of the immediate family were found to have hypergammaglobulinaemia.The classification of these cases is discussed, and the incidence of steatorrhoea in hypogammaglobulinaemia reviewed.A genetic model of a possible inheritance pattern using the Xg blood-group system is constructed.
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Yarnell et al. (1964) studied this question.
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