Angioedema hereditary

Ruddy S, Gigli I, Sheffer A, Austen K The laboratory diagnosis of hereditary angioedema in Rose N, Richter M, Sehon A (eds) Proceedings of the Sixth International Congress of Allergology. Amsterdam, Excerpta Medica, 1968, pp 351-359. 

Fields T, Ghebrehiwet B, Kaplan AP Kinin formation in hereditary angioedema plasma evidence against kinin derivation from C2 and in support of spontaneous formation of bradykinin. J Allergy 82 Chn Immunol 1983 72 54-60. 

Curd J, Yelvington M, Burridge N Generation of bradykinin during incubation of hereditary angioedema plasma. Mol Immunol 1983 19 1365. 83... 

Curd J, et al PrekaUikrein activation and high-molec-ular-weight kininogen consumption in hereditary angioedema. N Engl J Med 1983 308 1050-1053. 

Local bradykinin generation in hereditary angioedema. J Allergy Clin Immunol 1999 104 1321-1322. 

Bas M. Bier H. Greve J, Kojda G, Hoffmann T Novel pharmacotherapy of acute hereditary angioedema with bradykinin B2-receptor antagonist icatibant. Allergy 2006 61 1490-1492. 

Bork K. Frank J, Grundt B, Schlattmann P. Nussberger J. Kreuz W Treatment of acute edema attacks in hereditary angioedema with a bradykinin receptor-2 antagonist (icatibant). J Allergy Clin Immunol 2007 119 1497-1503. 

Schneider L. Lumry W Vegh A, Wilhams A, Sch-malbach T Critical role of kallikrein in hereditary angioedema pathogenesis a chnical trial of ecallan-tide, a novel kaUikrein inhibitor. J Allergy Clin Immunol 2007 120 416-422. 

Dewald G, Bork K Missense mutations in the coagulation factor XII (Hageman factor) gene in hereditary angioedema with normal Cl inhibitor. Biochem Biophys Res Commun 2006 343 1286-1289. 

Martin L, Raison-Peyron N. Nothen M. Cichon S. Drouet C Hereditary angioedema with normal Cl inhibitor gene in a family with affected women and men is associated with the p.Thr328Lys mutation in theP12gene.J Allergy Chn Immunol 2007 120 975-977. 

Hereditary angioedema For the prevention of attacks of angioedema in males and females. 

Hereditary angioedema - Danazol prevents attacks of the disease characterized by episodic edema of the abdominal viscera, extremities, face, and airway that may be disabling... 

Danazol Synthetic androgen. Suppresses gonadotrophin production. Exhibits some weak androgenic activity Oral administration in the treatment of endometriosis, benign breast disorders, menorrhagia, premenstrual syndrome and hereditary angioedema... 

Stanozolol Synthetic androgen Treatment of some clinical presentations of Behfet s syndrome and management of hereditary angioedema... 

Mechanism of Action A testosterone derivative that suppresses the pituitary-ovarian axis by inhibiting the output of pituitary gonadotropins. Causes atrophy of both normal and ectopic endometrial tissue in endometriosis. Follicle-stimulating hormone (FSH) and luteinizing hormone (LH) are depressed in fibrocystic breast disease. Inhibits steroid synthesis and binding of steroids to their receptors in breast tissues. Increases serum levels of esterase inhibitor. Therapeutic Effect Produces anovulation and amenorrhea, reduces the production of est rogen, corrects biochemical deficiency as seen in hereditary angioedema. 

Hereditary angioedema PO Initially, 200 mg 2-3 times/day. Decrease dose by 50% or less at 1-3 mo intervals. If attack occurs, increase dose by up to 200 mg/day. 

Drug of choice for treating all types of hereditary angioedema in the elderly... 

Hereditary angioedema prophylaxis PO Initially, 2 mg 2 times/day. Decrease at 1-3 month intervals. Maintenance 2 mg/day. 

It is a synthetic steroid with anabolic and androgenic properties. Used in prophylactic treatment of hereditary angioedema, vascular manifestations of Behcebs syndrome. 

Danazol is a weak androgen and also has a series of other hormonal and anti-hormonal properties. It inhibits pituitary gonadotropin and has been used in the treatment of endometriosis, fibrocystic disease of the breast, idiopathic thrombocytopenic purpura, and hereditary angioedema. Its hepatotoxic effects include reversible rises in serum transaminases and cholestatic hepatitis a few cases of hepatocellular tumors have been reported. 

A 34-year-old woman who had taken danazol 400 mg/ day for 13 years for hereditary angioedema developed a mass in the right hypochondrium. Her alcohol intake was under 20 g/day. She had a large heterogeneous hepatic tumor, a well-differentiated hepatocellullar carcinoma in a non-cirrhotic liver. 

Cicardi M, Castelh R, Zingale LC, Agostoni A. Side effects of long-term prophylaxis with attenuated androgens in hereditary angioedema comparison of treated and untreated patients. J Allergy Clin Immunol 1997 99(2) 194-6. 

Church JA. Oxandrolone treatment of childhood hereditary angioedema. Ann Allergy Asthma Immunol 2004 92 377-8. 

Nzeako UC, Frigas E, Tremaine WJ. Hereditary angioedema a broad review for clinicians. Arch Intern Med. 2001 161 2417-2429. 

Duponchel C, Di Rocco C, Cicardi M, Tosi M. Rapid detection by fluorescent multiplex PCR of exon deletions and duplications in the Cl inhibitor gene of hereditary angioedema patients. Hum Mutat 2001 17(1) 61 70. 

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