Hereditary angioneurotic edema (HAE) is an autosomal dominant disease that results from the deficiency of C1 esterase inhibitor (C1-INH) function. Urgent proper intervention of the upper airway is warranted to save the life of patients with the disease, which often causes acute airway obstruction. We present, with a review of literature, a case of 37-year old woman with HAE of larynx.
Adult ; Airway Obstruction ; Angioedema ; Angioedemas, Hereditary* ; Female ; Hereditary Angioedema Types I and II ; Humans ; Larynx*

No abstract available.
Angioedema* ; Angioedemas, Hereditary* ; Humans ; Omalizumab*

As a recognized rare and highly fatal disease, hereditary angioedema (HAE) is difficult to diagnose and characterized by recurrent edema involving the head, limbs, genitals and larynx, etc. Diagnosis of HAE is not difficult. However, low incidence and lack of clinical characteristics lead to difficulty of doctors on timely diagnosis and correct intervention for HAE patients. Therefore, it is crucial to improve the awareness of this disease and prevent its recurrence. for HAE patients. In view of absent cognition of doctors and the general public on HAE, patients often suffer from sudden death or become disabled due to laryngeal edema which cannot be treated in time. Thus, based on the Internet mobile terminal platform, the team set up an all-day rapid emergency response system which is provided for HAE patients by setting up "one-click help". The aim is to offer optimization on overall management of HAE and designed the intelligent follow-up management to provide timely assistance and specialized suggestion for patients with acute attacks.
Humans ; Angioedemas, Hereditary/drug therapy*

As a recognized rare and highly fatal disease, hereditary angioedema (HAE) is difficult to diagnose and characterized by recurrent edema involving the head, limbs, genitals and larynx, etc. Diagnosis of HAE is not difficult. However, low incidence and lack of clinical characteristics lead to difficulty of doctors on timely diagnosis and correct intervention for HAE patients. Therefore, it is crucial to improve the awareness of this disease and prevent its recurrence. for HAE patients. In view of absent cognition of doctors and the general public on HAE, patients often suffer from sudden death or become disabled due to laryngeal edema which cannot be treated in time. Thus, based on the Internet mobile terminal platform, the team set up an all-day rapid emergency response system which is provided for HAE patients by setting up "one-click help". The aim is to offer optimization on overall management of HAE and designed the intelligent follow-up management to provide timely assistance and specialized suggestion for patients with acute attacks.
Humans ; Angioedemas, Hereditary/drug therapy*

No abstract available.
Angioedema* ; Angioedemas, Hereditary* ; Complement C1 Inhibitor Protein* ; Complement C1s*

Acquired C1 inhibitor deficiency is a rare syndrome which usually presents with episodes of angioedema. The association of lupus erythematosus and angioneurotic edema has been previously reported but is usually related to hereditary C1 inhibitor deficiency. Currently the relationship between acquired C1 inhibior deficiency and lupus erythematosus is being discussed. So we report the case of a previously healthy 28-year-old woman, who developed simultaneously and acquired angioneurotic edema and systemic lupus erythematosus.
Adult ; Angioedema ; Angioedemas, Hereditary* ; Female ; Humans ; Lupus Erythematosus, Systemic

Hereditary angioedema (HAE) is rare disorder due to C1-inhibitor deficiency (C1-INH) that are debilitating and may be life-threatening. HAE is a lack of consensus concerning diagnosis, therapy, and management, particularly in Vietnam. In this case report, we report a 40-year-old male patient with typical clinical symptoms and family history but he showed normal C4 level, and we could not measure C1q and C1-INH level. However, the diagnosis of HAE can be made based on typical clinical symptoms and the favorable prophylactic response to danazol treatment. Based on these findings, we suggest that he has type I HAE, although he showed normal C4 level.
Adult ; Angioedemas, Hereditary ; Consensus ; Danazol ; Humans ; Male ; Vietnam

Hereditary angioedema (HAE) is a rare autosomal dominant disease that usually occurs in adolescence and early adulthood. It is characterized by recurrent non-pitting edema involving the skin and intestinal tract, especially the extremities and face. It is not associated with urticaria and pruritus. The cause is known to be the deficiency of C1 inhibitor. We herein report a 7-year-old girl with HAE who had recurrent episodes of swelling of the extremities and face without urticaria and pruritus. Her great grandmother had suffered from the same symptoms. The level of serum C4 was 8.01 mg/dL (normal: 10-40 mg/dL). The level of C1 inhibitor was 5.0 mg/dL (normal: 18-40 mg/dL). To our knowledge, this is the first pediatric case with typical clinical symptoms of HAE and C1 esterase inhibitor deficiency in Korea.
Adolescent ; Angioedema ; Angioedemas, Hereditary ; Complement C1 Inhibitor Protein ; Edema ; Extremities ; Humans ; Korea ; Pruritus ; Skin ; Urticaria

Hereditary angioedema is a rare genetic disease transmitted with a autosomal dominant trait result of a quantitative or functional defect of Cl inhibitor. We report three cases of heriditary angioedema from the same famly respectively the 35-year-old sister, the younger bn)ther of 32, and the younger sister of 20 The older sister died due to her airway being obstructel by acute laryngeal edema, and her you igirbrother and sister experienced recurrent episodes oswelling on the hand and forearm. The ore we examined the two patients serum complement levils to confirm diagnosis. In both, the r;ults showed decreased levels of Cl inhibitor and C4 which are the evidence of hereditary a giedema. The younger sister has been administered danazol (600mg/day) to prevent angioedem or 8 weeks, and we have not yet found a recurrenc of he symptoms. However, well cont ol the dosage of danazol because amenorchea has developec.
Adult ; Angioedema ; Angioedemas, Hereditary* ; Complement System Proteins ; Danazol ; Diagnosis ; Forearm ; Hand ; Humans ; Laryngeal Edema ; Siblings

C1 esterase inhibitor deficiency with consequent angioedma is an uncommon condition. Nonhereditary C1 inhibitor deficiency includes underlying disorders; lymphoproliferative disorder, autoimmune disease, hypereosinophilia, drug-induced, allergic, and idiopathic forms. The as sociation of hereditary C1 esterase inhibitor deficiency with systemic lupus erythematosus has been previously described. We experienced a case with transiently decreased C1 inactivator activity and angioedema in lupus nephritis. This present case is a previously healthy 22-year-old woman, who developed intermittent facial angioedema and decreased urine amount. After steroid treatment, the C1 inactivator activity was recovered and angioedema was disappeared.
Angioedema* ; Angioedemas, Hereditary ; Autoimmune Diseases ; Female ; Humans ; Lupus Erythematosus, Systemic ; Lupus Nephritis* ; Lymphoproliferative Disorders ; Young Adult