Moulias R, Brecy H, Ollier M P, Roualdes M, Maïza M, Hartmann L
Nouv Presse Med. 1977 Jun 18;6(25):2233, 2235-8.
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.
同一家族的四名个体患有C1酯酶抑制剂功能缺陷。其中三人的遗传性血管性水肿表现为腹部发作、阵发性发作且类似外科急腹症。这些发作与内脏或黏膜水肿的发展有关。急性发作期间总补体(及其各组分)的测定,以及发作间期的动态补体研究,是提示可能诊断的一种简单方法。只有C1酯酶抑制剂功能活性的评估才能提供确切证据。熟悉这些急性腹部发作的临床和生物学特征,有助于避免对这些患者进行反复、不必要的手术。鉴于血管性水肿患者可能同时存在特定病变,应在外科监护下进行药物治疗。此外,发作可能在任何时候扩散,导致声门水肿,需要进行气管切开术或立即插管。