Objective To determine the safety and efficacy of fresh frozen plasma (FFP) iniusion for the treat- ment of hereditary angioedema (FIAE). Methods The medical records of patients with HAE admitted to Peking Union ...Objective To determine the safety and efficacy of fresh frozen plasma (FFP) iniusion for the treat- ment of hereditary angioedema (FIAE). Methods The medical records of patients with HAE admitted to Peking Union Medical College Fiospital who had received FFP infusion during 2004 and 2010 were reviewed and PubMed database iFom 1966 to the present were searched using the following key words: hereditary angioedema and fresh frozen plasma. The patient's age, sex, body location of HAE attacks, the dose of FFP infusion, time of beginning to improvenaent, time to complete remission, complication, C 1 inhibitor activity, and outcome were analyzed. Results A total of 13 enrolled patients (7 male and 6 female) received 16 times of FFP infusion, in- cluding 2 patients undergoing FFP infusion in Peking Union Medical College Hospital and 11 patients re- ported in the literature. The mean dosage of FFP infusion was 586±337 mL. Two cases suffered from wors- ening abdominal pain and one case experienced skin rash. Only I patient had no improvement in symptom owing to transfusion related reaction. There was a definite improvement in symptom 49± 19 minutes after beginning FFP infusion. The remission time decreased from 61.7±27.0 hours to 3.3 (2.0, 12.0) hours after FFP infusion. FFP infusion was effective for both type I and type Ⅱ HAE. Conclusion FFP seems to be safe and effective for acute attacks of HAE.展开更多
HEREDITARY angioedema (HAE) is an autosomal dominant inherited condition which was initially described by Osier in 1888.1 Patients with HAE can develop rapid subcutaneous or submucosal edema involving the hands, fee...HEREDITARY angioedema (HAE) is an autosomal dominant inherited condition which was initially described by Osier in 1888.1 Patients with HAE can develop rapid subcutaneous or submucosal edema involving the hands, feet, limbs, face, intestinal tract, even larynx and trachea. The mortality of an acute attack of HAE without treatment was reported as high as 30%.2 HAE is caused by the deficiency of Cl esterase inhibitor (CIINH) which results in episodes of edema in parts of the human body,展开更多
文摘Objective To determine the safety and efficacy of fresh frozen plasma (FFP) iniusion for the treat- ment of hereditary angioedema (FIAE). Methods The medical records of patients with HAE admitted to Peking Union Medical College Fiospital who had received FFP infusion during 2004 and 2010 were reviewed and PubMed database iFom 1966 to the present were searched using the following key words: hereditary angioedema and fresh frozen plasma. The patient's age, sex, body location of HAE attacks, the dose of FFP infusion, time of beginning to improvenaent, time to complete remission, complication, C 1 inhibitor activity, and outcome were analyzed. Results A total of 13 enrolled patients (7 male and 6 female) received 16 times of FFP infusion, in- cluding 2 patients undergoing FFP infusion in Peking Union Medical College Hospital and 11 patients re- ported in the literature. The mean dosage of FFP infusion was 586±337 mL. Two cases suffered from wors- ening abdominal pain and one case experienced skin rash. Only I patient had no improvement in symptom owing to transfusion related reaction. There was a definite improvement in symptom 49± 19 minutes after beginning FFP infusion. The remission time decreased from 61.7±27.0 hours to 3.3 (2.0, 12.0) hours after FFP infusion. FFP infusion was effective for both type I and type Ⅱ HAE. Conclusion FFP seems to be safe and effective for acute attacks of HAE.
文摘HEREDITARY angioedema (HAE) is an autosomal dominant inherited condition which was initially described by Osier in 1888.1 Patients with HAE can develop rapid subcutaneous or submucosal edema involving the hands, feet, limbs, face, intestinal tract, even larynx and trachea. The mortality of an acute attack of HAE without treatment was reported as high as 30%.2 HAE is caused by the deficiency of Cl esterase inhibitor (CIINH) which results in episodes of edema in parts of the human body,