[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. The measurement of total complement (and of its fractions) during the acute episode, as well as dynamic complement studies between attacks, represent a simple method for indicating the probable diagnosis. Only estimation of the functional activity of C1 esterase inhibitor provides definite evidence. Familiarity with the clinical and biological characteristics of these acute abdominal episodes makes it possible to avoid repeated, unnecessary operations in these patients. They should be treated medically, under surgical surveillance, since a patient with angioneurotic oedema may have nevertheless a specific lesion, in addition. In addition, the episode may spread at any time, resulting in oedema of the glottis requiring tracheotomy or immediate intubation.
D000006:Abdomen, Acute / Q000209:etiology*; D000328:Adult; D000799:Angioedema / Q000150:complications / Q000175:diagnosis / Q000201:enzymology / Q000235:genetics*; D002648:Child; D003172:Complement C1; D003165:Complement System Proteins / Q000032:analysis; D003937:Diagnosis, Differential; D004950:Esterases / Q000037:antagonists & inhibitors; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D012877:Skin Manifestations
D003172:Complement C1; D003165:Complement System Proteins; D004950:Esterases
Manifestations abdominales aiguës de l'oedème angioneurotique héréditaire. Intérêt de l'exploration du système du complément.