Medical Literature - 1975

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.

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

Jun;55(6):386-393

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: jacionline.org/article/0091-6749%2875%2990077-9/abstract

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.

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

Jul;4(2):174-188.

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: sciencedirect.com/science/article/pii/0090122975900537 (small fee)