Congenital Hypogammaglobulinaemia with Steatorrhoea in Two Adult Brothers
- 22 August 1964
- Vol. 2 (5407), 486-490
- https://doi.org/10.1136/bmj.2.5407.486
Abstract
Two adult brothers, aged 25 and 27, were found to have "congenital" hypogammaglobulinaemia associated with steatorrhoea. Their clinical state has responded to parenteral gamma-globulin 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 gamma-globulin. Members of the immediate family were found to have hyper-gammaglobulinaemia. The classification of these cases is discussed, and the incidence of steatorrheea in hypogammaglobulinaemia reviewed. A genetic model of a possible inheritance pattern using the Xg blood-group system is constructed.Keywords
This publication has 17 references indexed in Scilit:
- The occurrence of rheumatoid factor and other abnormalities in families of patients with agammaglobulinemiaArthritis & Rheumatism, 1962
- Hypogammaglobulinæmia, Steatorrhœa and Megaloblastic AnæmiaPublished by Oxford University Press (OUP) ,1962
- A SEX-LINKED BLOOD GROUPThe Lancet, 1962
- [Hypothrepsia secondary to chronic hypoproteinemia involving the beta and gamma globulin fractions greatly improved by a gluten-free diet].1962
- Congenital AgammaglobulinaemiaBMJ, 1961
- [Congenital agammaglobulinemia with steatorrhea in two heterozygotic twins of different sex].1959
- Acquired Hypogammaglobulinemia in an AdultNew England Journal of Medicine, 1956
- Studies in serum proteins: Agammaglobulinemia in the adultAmerican Journal Of Medicine, 1955
- Absence of Serum Gamma Globulins in an AdultNew England Journal of Medicine, 1954
- AGAMMAGLOBULINEMIA1952