Medical Literature - 1975

A study of complement functions in a family with hereditary angioneurotic edema

Casali P, Rugarli C, Zanussi C. 11/1975 Bollettino dell Istituto Sieroterapico Milanese

Nov 20;54(5):405-408.

A study of some complement functions was performed in two patients affected with hereditary angioneurotic edema (HAE) – mother and daughter – from a Southern Italy family (Apulia) and in three of their relatives. A decreased level of the C1 esterase plasma inhibitor was found in both the patients, who were treated with tranexamic acid with a slight, but definite improvement. However, the disease could not be traced beyond two generations in the pedigree and a couple of ascendants was found who were free from HAE both from the clinical standpoint and from that of the laboratory evaluation of complement functions. The possibility that HAE arose in the family through a mutational event is discussed.

Not available online.

Hereditary angioedema: the use of fresh frozen plasma for prophylaxis in patients undergoing oral surgery

Jaffe CJ, Atkinson JP, Gelfand JA, Frank MM. 6/1975 Journal of Allergy & Clinical Immunology


Six patients with hereditary anagioedema (HAE) undergoing 7 episodes of dental surgery received transfusions with fresh frozen plasma one day before surgery. Although the morbidity observed in these patients following similar procedures had been high, no significant complications of surgery were noted with this therapy. Thus, fresh frozen plasma infusion appears to provide a safe and effective method of prophylaxis in patients with HAE. Following infusion of fresh frozen plasma, serum levels of C4 esterase inhibitor (C1EI) rose transiently, and then fell to preinfusion levels within 1 to 12 days. In all but one patient the rise in C4 was greater than could be accounted for by the amount of C4 infused. In no patient did the level of C1EI or C4 rise to within the normal range. The data raise the question of the role of C1EI in the pathogenesis of angioedema in these patients.

Available online at:

Hereditary angioneurotic oedema

Levi L. 7/1975 Klinische Wochenschrift

Jul 15;53(14):679-684

Three families with hereditary angioneurotic oedema (HANE) are described. Serum CI-INH and C4 immunochemical determinations were performed on 23 members of the families: in 6 persons low CI-INH and C4 levels were found with typical symptoms of HANE, and in one asymptomatic subject. Normal ranges of both proteins for adults, children and newborns are given for comparative purposes. The importance of the early diagnosis and treatment is emphasized.

Not available online.

Observations on the ultrastructure of lesions induced in human and guinea pig skin by C 1 esterase and polypeptide from hereditary angioneurotic edema (HANE) plasma

Willms K, Rosen FS, Donaldson VH. 7/1975 Clinical Immunology & Immunopathology


Intradermally injected preparations of purified Cls̄ and polypeptide from plasma of persons with hereditary angioneurotic edema (HANE) induced venular lesions with ultrastructural changes of the endothelium similar to changes induced by histamine (1). Treatment of test animals with an antihistaminic did not block increased vascular permeability about injections of HANE polypeptide or bradykinin. Animals depleted of C3 demonstrated decreased change in vascular permeability about injections of Cls̄, but responses to histamine, bradykinin and HANE polypeptide were not blocked. Patients with HANE excreted larger amounts of histamine in their urine than normals; this increase was most pronounced as an epidsode of edema was subsiding.

Available online at: (small fee)

The relationship between hemolytic and immunodiffusion methods for measurement of C4 in patients with immunologic disorders

Yam P, Petz LD, Cooper NR. 9/1975 American Journal of Clinical Pathology


A comparison of radial immunodiffusion and functional (hemolytic) assays for C4 revealed a highly significant correlation between the two methods in patients with a variety of immunologic disorders. Immunodiffusion technics are more convenient than hemolytic assays for most clinical laboratories. C4 assays are useful for the presumptive diagnosis of hereditary angioneurotic edema, whereas C3 is usually normal in such patients. C4 is usually more sensitive than C3 in a variety of immunologic disorders, although in some patients the opposite is true, especially in hypocomplementemic nephritis.

Not available online.

Funding for Canadian Hereditary Angioedema Network has been generously provided by unrestricted grants from:


CSL Behring


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