Hereditary angioedema （HAE） is an autosomal dominant condition that affects one in about 50 000 persons, characterized by recurrent episodes of subcutaneous or submucosal swelling involving the hands, feet, limbs, f...Hereditary angioedema （HAE） is an autosomal dominant condition that affects one in about 50 000 persons, characterized by recurrent episodes of subcutaneous or submucosal swelling involving the hands, feet, limbs, face, intestinal tract, even larynx and trachea.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
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 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
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 swelling involving the hands, feet, limbs, face, intestinal tract, even larynx and trachea.
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 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.
