Zhonghua yi xue za zhi · 2007
[Clinical features of hereditary angioedema: analysis of 133 cases].
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Ren Hua-li, Zhang Hong-yu
Аннотация
OBJECTIVE: To investigate the clinical characteristics of hereditary angioedema (HAE).
METHODS: The clinical data of 133 cases with HAE from 40 unrelated families were analyzed retrospectively.
RESULTS: Recurrent extremity swelling and/or facial and genital edema were reported in all patients (100%); 76.7% of the patients recalled abdominal symptoms; ascites was found in all 6 ultrasound examined patients in acute episodes. The ages of the patients ranged from 1.5 to 70. with the mean age of the first episode of 18 +/- 7. The time between onset and diagnosis was 16 years. Eight of the 133 patients were recognized as sporadic cases, without definite family history. The serum C1-INH levels of 130 patients were low, only the C1-INH levels of 3 cases in 1 family were a little bit higher than the normal level. Only one of the 40 families was diagnosed as with type II HAE (HAE-II). 43.6% of the patients received prophylaxis with danazol. Danazol had a good efficacy in all patients and were well tolerated by most of them.
CONCLUSIONS: A rare autosomal dominant disease, and characterized by recurrent episodes of cutaneous swelling, abdominal pain, and laryngeal edema, HAE can be fatal. Abdominal symptoms are often underestimated. HAE-II is very rare in China. Prophylaxis with danazol is effective and can be well tolerated.
METHODS: The clinical data of 133 cases with HAE from 40 unrelated families were analyzed retrospectively.
RESULTS: Recurrent extremity swelling and/or facial and genital edema were reported in all patients (100%); 76.7% of the patients recalled abdominal symptoms; ascites was found in all 6 ultrasound examined patients in acute episodes. The ages of the patients ranged from 1.5 to 70. with the mean age of the first episode of 18 +/- 7. The time between onset and diagnosis was 16 years. Eight of the 133 patients were recognized as sporadic cases, without definite family history. The serum C1-INH levels of 130 patients were low, only the C1-INH levels of 3 cases in 1 family were a little bit higher than the normal level. Only one of the 40 families was diagnosed as with type II HAE (HAE-II). 43.6% of the patients received prophylaxis with danazol. Danazol had a good efficacy in all patients and were well tolerated by most of them.
CONCLUSIONS: A rare autosomal dominant disease, and characterized by recurrent episodes of cutaneous swelling, abdominal pain, and laryngeal edema, HAE can be fatal. Abdominal symptoms are often underestimated. HAE-II is very rare in China. Prophylaxis with danazol is effective and can be well tolerated.