Response of Variant Hereditary Angioedema Phenotypes to Danazol Therapy
نویسندگان
چکیده
منابع مشابه
Response of variant hereditary angioedema phenotypes to danazol therapy. Genetic implications.
Hereditary angioedema (HAE), an auto-somal dominant disorder characterized by attacks of episodic edema is associated with decreased functional levels of the C1 esterase inhibitor. Approximately 85% of patients have lowered antigen levels of a normal inhibitor protein. 15% of patients have normal or elevated antigenic levels of functionless protein. We have examined the response to danazol ther...
متن کاملLong-term efficacy of danazol treatment in hereditary angioedema.
BACKGROUND No systematic study has been published yet on the long-term efficacy of attenuated androgens in hereditary angioedema (HAE). Our aim was to conduct a follow-up study in two (German and Hungarian) cohorts of HAE patients (45 and 39 patients, respectively) undergoing uninterrupted treatment for 6 years with similar (starting dose 128 ± 78 mg per day and 136 ± 70 mg per day, respectivel...
متن کاملHereditary angioedema without deficiency of C1 inhibitor: response to therapy
Results Nineteen patients have been identified (2M:17F; 20-60 years old). The following therapies were oriented: combined contraceptive substitution for progestagen (10/19); treatment with progestagen (2/19); tranexamic acid (15/19): 1250mg (2), 1000mg (1), 750 (5), 500 mg (4), 250 mg (1); oxandrolon (5/19) (0.5 mg-5mg/day), danazol 200mg/day (1/19) and combined therapy woth oxandrolon and tran...
متن کاملFuture Therapy for Pediatric Hereditary Angioedema
Hereditary angioedema (HAE) due to C1-inhibitor (C1-INH) deficiency is characterized by recurrent attacks of swelling with symptoms typically beginning in childhood. The introduction of targeted therapies for prevention and treatment of attacks has been of significant benefit to patients, including children, with HAE. However, the ideal of a safe, convenient therapy that prevents all attacks ha...
متن کاملHepatocellular Carcinoma in a Noncirrhotic Liver after Long-Term Use of Danazol for Hereditary Angioedema
We report a 57-year-old male who was treated with high-dose danazol for hereditary angioedema for more than 30 years; he developed hepatocellular carcinoma in the absence of cirrhosis. Despite surgical resection, he had a recurrence and received sorafenib, but had a poor skin tolerance. Such tumors arising after danazol are infrequent, and this case is highly unique due to the minor lesions fou...
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ژورنال
عنوان ژورنال: Journal of Clinical Investigation
سال: 1979
ISSN: 0021-9738
DOI: 10.1172/jci109449