[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. It can be subsequently explained by a functional defect of the C1 esterase inhibitor or alpha2-neuraminoglycoprotein.
D000293:Adolescent; D000799:Angioedema / Q000097:blood / Q000150:complications / Q000235:genetics* / Q000628:therapy; D003174:Complement C1 Inactivator Proteins; D003175:Complement C2 / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D003165:Complement System Proteins / Q000032:analysis*; D003937:Diagnosis, Differential; D004487:Edema / Q000209:etiology; D004950:Esterases / Q000037:antagonists & inhibitors; D005260:Female; D005767:Gastrointestinal Diseases / Q000175:diagnosis / Q000209:etiology*; D006023:Glycoproteins / Q000097:blood; D006801:Humans; D007819:Laryngeal Edema / Q000209:etiology; D008297:Male; D009438:Neuraminic Acids / Q000097:blood; D010375:Pedigree; D013997:Time Factors
D003174:Complement C1 Inactivator Proteins; D003175:Complement C2; D003181:Complement C4; D006023:Glycoproteins; D009438:Neuraminic Acids; D003165:Complement System Proteins; D004950:Esterases
Les manifestations abdominales de l'oedème angioneurotique héréditaire. Intérêt de l'exploration du système du complément (a propos de vingt-neuf familles)