Objective To determine the safety and efficacy of fresh frozen plasma(FFP) infusion for the treatment of hereditary angioedema(HAE).Methods The medical records of patients with HAE admitted to Peking Union Medical Col...Objective To determine the safety and efficacy of fresh frozen plasma(FFP) infusion for the treatment of hereditary angioedema(HAE).Methods The medical records of patients with HAE admitted to Peking Union Medical College Hospital who had received FFP infusion during 2004 and 2010 were reviewed and PubMed database from 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 improvement,time to complete remission,complication,C1 inhibitor activity,and outcome were analyzed.Results A total of 13 enrolled patients(7 male and 6 female) received 16 times of FFP infusion,including 2 patients undergoing FFP infusion in Peking Union Medical College Hospital and 11 patients reported in the literature.The mean dosage of FFP infusion was 586±337 mL.Two cases suffered from worsening abdominal pain and one case experienced skin rash.Only 1 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 Ⅰand type Ⅱ HAE.Conclusion FFP seems to be safe and effective for acute attacks of HAE.展开更多
A pregnant woman diagnosed with type 1 angioedema seeks care at a public hospital for planning the delivery. This report presents ways to prevent and manage an acute HAE crisis during childbirth and early postpartum w...A pregnant woman diagnosed with type 1 angioedema seeks care at a public hospital for planning the delivery. This report presents ways to prevent and manage an acute HAE crisis during childbirth and early postpartum without the availability of first-line medications, such as plasma-derived human C1-INH concentrate.展开更多
Hereditary angioedema secondary to C1-inhibitor deficiency is a rare autosomal dominant disorder characterized by a deficiency of C1 esterase inhibitor.An eight-year-old girl showed periorbital painless swelling, diag...Hereditary angioedema secondary to C1-inhibitor deficiency is a rare autosomal dominant disorder characterized by a deficiency of C1 esterase inhibitor.An eight-year-old girl showed periorbital painless swelling, diagnosed as ethmoiditis. A craniofacial scan did not evidence a paranasal sinus involvement, C1INH levels were undetectable, with low C4 levels: 7.6 mg/dl and C1INH: <8.46 mg/dl. The genetic study identified a rare mutation of the C1INH gene. This clinical report is of relieve because paediatric cases described in literature are rare, did not presented a positive family history, and received a diagnosis after many attacks. Furthermore our girl received a prompt diagnosis of HAE at the first attack of angioedema.展开更多
Hereditary angioedema (HAE) is an autosomal dominant condition that affects one in about 50 000 persons,characterized by recurrent episodes of subcutaneous or submucosal
Background: Hereditary angioedema (HAE) is a primary immunodeficiency disorder characterized by C1 complement inhibitor deficiency and unregulated activation of complement. Aspirin hypersensitivity is related to an in...Background: Hereditary angioedema (HAE) is a primary immunodeficiency disorder characterized by C1 complement inhibitor deficiency and unregulated activation of complement. Aspirin hypersensitivity is related to an increase in the amount of leukotrienes with eosinophil and mast cell activation and increased levels of glandular kallikrein with upregulated local conversion of bradykinin. Both conditions can be present in the same patient. Objectives: We present five patients with HAE;they were all being treated in similar ways according to the therapeuthic options available in our institute (danazol). However, three of them had recurrent episodes of angioedema;in these cases, it was identified aspirin hypersensitivity as a cause of poor disease control. A review of the literature is included. Case Presentation: We present the cases of four females and one male (age range 21 - 58 years) with type I HAE. Subjects were all ISSSTE beneficiaries (state workers) treated at the National Medical Center “20 de Noviembre”. Aspirin hypersensitivity was identified in three patients. Elimination of NSAIDs along with dietary elimination of high salicylate-containing foods improved control of angioedema crisis (severe and/ or recurrent episodes). Discussion: Aspirin hypersensitivity was identified as a factor for poor control in our patients with HAE. Such cases improved with dietary elimination of high salicylate-containing foods and avoidance of NSAIDs. Conclusions: This is the first report of patients with HAE and aspirin hypersensitivity as a cause of poor control. We recommend a deliberate search of these comorbidities, especially in cases of poor disease control. Further studies are needed to continue the investigation on this topic.展开更多
Hereditary angioedema is a rare but life-threatening disease, usually resulting from upper respiratory tract traumas and stress. In this case report, we present the management of a 14-year-old female patient who was d...Hereditary angioedema is a rare but life-threatening disease, usually resulting from upper respiratory tract traumas and stress. In this case report, we present the management of a 14-year-old female patient who was diagnosed with hereditary angioedema and scheduled to undergo transurethral resection of bladder (TURB) procedure for bladder tumor. She was on prophylactic danazol treatment and prior to the operation the dose of danazol was increased. On the day of the operation, patient was given C1-IHN concentrate and was sedated. In conclusion, hereditary angioedema is a rare disease in which multidisciplinary and aggressive approach during anesthesia would yield successful results.展开更多
Abdominal involvement in angioedema is often a challenge to diagnose.Acute onset abdominal pain is its most common presenting symptom,and misdiagnosis may lead to unnecessary surgical intervention.Familiarity with the...Abdominal involvement in angioedema is often a challenge to diagnose.Acute onset abdominal pain is its most common presenting symptom,and misdiagnosis may lead to unnecessary surgical intervention.Familiarity with the types and presentations of angioedema can be invaluable to clinicians as they consider the differential diagnoses of a patient presenting with abdominal pain. Detailed personal and family histories,careful physical examination of the patient,combined with knowledge of angioedema types,can help clinicians perform their diagnostic evaluation.An accurate diagnosis is essential in order to provide appropriate treatment to patients with angioedema.Depending upon the diagnosis,treatment may be the avoidance of provoking factors(such as allergens or medications),inhibiting histamine-provoked reactions,or treating C1 esterase inhibitor deficiency.展开更多
文摘Objective To determine the safety and efficacy of fresh frozen plasma(FFP) infusion for the treatment of hereditary angioedema(HAE).Methods The medical records of patients with HAE admitted to Peking Union Medical College Hospital who had received FFP infusion during 2004 and 2010 were reviewed and PubMed database from 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 improvement,time to complete remission,complication,C1 inhibitor activity,and outcome were analyzed.Results A total of 13 enrolled patients(7 male and 6 female) received 16 times of FFP infusion,including 2 patients undergoing FFP infusion in Peking Union Medical College Hospital and 11 patients reported in the literature.The mean dosage of FFP infusion was 586±337 mL.Two cases suffered from worsening abdominal pain and one case experienced skin rash.Only 1 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 Ⅰand type Ⅱ HAE.Conclusion FFP seems to be safe and effective for acute attacks of HAE.
文摘A pregnant woman diagnosed with type 1 angioedema seeks care at a public hospital for planning the delivery. This report presents ways to prevent and manage an acute HAE crisis during childbirth and early postpartum without the availability of first-line medications, such as plasma-derived human C1-INH concentrate.
文摘Hereditary angioedema secondary to C1-inhibitor deficiency is a rare autosomal dominant disorder characterized by a deficiency of C1 esterase inhibitor.An eight-year-old girl showed periorbital painless swelling, diagnosed as ethmoiditis. A craniofacial scan did not evidence a paranasal sinus involvement, C1INH levels were undetectable, with low C4 levels: 7.6 mg/dl and C1INH: <8.46 mg/dl. The genetic study identified a rare mutation of the C1INH gene. This clinical report is of relieve because paediatric cases described in literature are rare, did not presented a positive family history, and received a diagnosis after many attacks. Furthermore our girl received a prompt diagnosis of HAE at the first attack of angioedema.
文摘Hereditary angioedema (HAE) is an autosomal dominant condition that affects one in about 50 000 persons,characterized by recurrent episodes of subcutaneous or submucosal
文摘Background: Hereditary angioedema (HAE) is a primary immunodeficiency disorder characterized by C1 complement inhibitor deficiency and unregulated activation of complement. Aspirin hypersensitivity is related to an increase in the amount of leukotrienes with eosinophil and mast cell activation and increased levels of glandular kallikrein with upregulated local conversion of bradykinin. Both conditions can be present in the same patient. Objectives: We present five patients with HAE;they were all being treated in similar ways according to the therapeuthic options available in our institute (danazol). However, three of them had recurrent episodes of angioedema;in these cases, it was identified aspirin hypersensitivity as a cause of poor disease control. A review of the literature is included. Case Presentation: We present the cases of four females and one male (age range 21 - 58 years) with type I HAE. Subjects were all ISSSTE beneficiaries (state workers) treated at the National Medical Center “20 de Noviembre”. Aspirin hypersensitivity was identified in three patients. Elimination of NSAIDs along with dietary elimination of high salicylate-containing foods improved control of angioedema crisis (severe and/ or recurrent episodes). Discussion: Aspirin hypersensitivity was identified as a factor for poor control in our patients with HAE. Such cases improved with dietary elimination of high salicylate-containing foods and avoidance of NSAIDs. Conclusions: This is the first report of patients with HAE and aspirin hypersensitivity as a cause of poor control. We recommend a deliberate search of these comorbidities, especially in cases of poor disease control. Further studies are needed to continue the investigation on this topic.
文摘Hereditary angioedema is a rare but life-threatening disease, usually resulting from upper respiratory tract traumas and stress. In this case report, we present the management of a 14-year-old female patient who was diagnosed with hereditary angioedema and scheduled to undergo transurethral resection of bladder (TURB) procedure for bladder tumor. She was on prophylactic danazol treatment and prior to the operation the dose of danazol was increased. On the day of the operation, patient was given C1-IHN concentrate and was sedated. In conclusion, hereditary angioedema is a rare disease in which multidisciplinary and aggressive approach during anesthesia would yield successful results.
文摘Abdominal involvement in angioedema is often a challenge to diagnose.Acute onset abdominal pain is its most common presenting symptom,and misdiagnosis may lead to unnecessary surgical intervention.Familiarity with the types and presentations of angioedema can be invaluable to clinicians as they consider the differential diagnoses of a patient presenting with abdominal pain. Detailed personal and family histories,careful physical examination of the patient,combined with knowledge of angioedema types,can help clinicians perform their diagnostic evaluation.An accurate diagnosis is essential in order to provide appropriate treatment to patients with angioedema.Depending upon the diagnosis,treatment may be the avoidance of provoking factors(such as allergens or medications),inhibiting histamine-provoked reactions,or treating C1 esterase inhibitor deficiency.
