Biomaterial Database

[Clinical features, pathogenesis, and therapy of hereditary angioneurotic edema (author's transl)]. 1982

F Schindera, and E Struck, and W Spira

The hereditary angioneurotic edema is probably a more frequent disease than presumed until now. It is inherited as an autosomal dominant trait, and is due to diminished or functionally deficient C1 esterase inhibitor. Family history is negative in cases of spontaneous mutations. Two thirds of the patients are already affected in infancy with peripheral edema, facial edema, abdominal symptoms and/or edema of the glottis occuring spontaneously or secondary to trauma. Recently the prognosis has become better due to early diagnosis and the possibility of substitution therapy with C1 esterase inhibitor. A long-term prophylaxis may be achieved with synthetic androgens.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D011247	Pregnancy	The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.	Gestation,Pregnancies
D011379	Prognosis	A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations.	Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D003173	Complement C1s	A 77-kDa subcomponent of complement C1, encoded by gene C1S, is a SERINE PROTEASE existing as a proenzyme (homodimer) in the intact complement C1 complex. Upon the binding of COMPLEMENT C1Q to antibodies, the activated COMPLEMENT C1R cleaves C1s into two chains, A (heavy) and B (light, the serine protease), linked by disulfide bonds yielding the active C1s. The activated C1s, in turn, cleaves COMPLEMENT C2 and COMPLEMENT C4 to form C4b2a (CLASSICAL C3 CONVERTASE).	C 1 Esterase,C1 Esterase,C1s Complement,Complement 1 Esterase,Complement 1s,Complement Component 1s,C1s, Complement,Complement, C1s,Component 1s, Complement,Esterase, C 1,Esterase, C1,Esterase, Complement 1
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults
D000728	Androgens	Compounds that interact with ANDROGEN RECEPTORS in target tissues to bring about the effects similar to those of TESTOSTERONE. Depending on the target tissues, androgenic effects can be on SEX DIFFERENTIATION; male reproductive organs, SPERMATOGENESIS; secondary male SEX CHARACTERISTICS; LIBIDO; development of muscle mass, strength, and power.	Androgen,Androgen Receptor Agonist,Androgen Effect,Androgen Effects,Androgen Receptor Agonists,Androgenic Agents,Androgenic Compounds,Agents, Androgenic,Agonist, Androgen Receptor,Agonists, Androgen Receptor,Compounds, Androgenic,Effect, Androgen,Effects, Androgen,Receptor Agonist, Androgen,Receptor Agonists, Androgen
D000799	Angioedema	Swelling involving the deep DERMIS, subcutaneous, or submucosal tissues, representing localized EDEMA. Angioedema often occurs in the face, lips, tongue, and larynx.	Quincke's Edema,Urticaria, Giant,Angioneurotic Edema,Angioedemas,Angioneurotic Edemas,Edema, Angioneurotic,Edema, Quincke's,Edemas, Angioneurotic,Giant Urticaria,Giant Urticarias,Quincke Edema,Quinckes Edema,Urticarias, Giant