The gene(s) controlling the synthesis of C1 esterase inhibitor is not linked to the genes of the HLA system and is not on the 6th chromosome.

Certain genes of the complement system are carried by the 6th chromosome and are sometimes linked to particular genes of the HLA system. This study shows that in 15 patients suffering from hereditary angioneurotic oedema the gene(s) controlling the synthesis of C1 esterase inhibitor is not linked to the genes HLA-A or B and is not on the 6th chromosome.

[The molecular expression of C1 esterase inhibitor in hereditary angioneurotic oedema. Study of 32 families (author's transl)].

Seventy seven patients from 32 families suffereing from angioneurotic oedema were studied and a single theory of the disorder is suggested. The functional abnormality of alpha2 neuraminoglycoprotein (NGP) or of C1 esterase inhibitor is related involvement of the structure gene (s) controlling its synthesis. Particular immunochemical characteristics of the inhibitor seen in severals families and remaining constant through a number of generations would indicate the existence of a phenomenon of functional haploidism in these heterozygous patients.

An investigation of the complement system in patients with periodic disease (results from 29 cases).

The complement system was investigated in 29 patients suffering from authentic periodic disease. A statistically significant increase in C4, also in total complement and C3 could be demonstrated. It is possible that the increase in C4 was due to the macrophages which are always present in the infiltrates of periodic disease.

[Abdominal manifestations in hereditary acute angioneurotic oedema. Value of study of the complement system (author's transl)].

Four individuals of the same family suffered from a functional deficiency in C1 esterase inhibitor. In three of them, the manifestations of hereditary angioneurotic oedema were abdominal, paroxystic and pseudo-surgical. They were related to the development of visceral or mucosal oedema.

[Abdominal manifestations of hereditary angioneurotic edema. Importance of the exploration of the complement system (apropos of 29 families)].

Abdominal manifestations are almost constantly present (85% of cases) in the current form of hereditary Quincke's disease. In some cases, these abdominal manifestations occur even when cutaneomucosal edema is not present which leads to unwarranted often repeated and sometimes dangerous surgery. Apart from a story of heredity diagnosis of such troubles is possible, provided the total complement has been assayed to note its sharp fall.