The course of the disease is diverse and unpredictable, even within a single patient over their lifetime. This disease may be similar in its presentation to other forms of angioedema resulting from allergies or other medical conditions, but it is significantly different in cause and treatment. When HAE is misdiagnosed as an allergy it is most commonly treated with steroids and epinephrine, drugs that are usually ineffective in treating a HAE episode. Other misdiagnoses have resulted in unnecessary exploratory surgery for patients with abdominal swelling and other HAE patients report that their abdominal pain was wrongly diagnosed as psychosomatic.
|Published (Last):||4 June 2011|
|PDF File Size:||4.66 Mb|
|ePub File Size:||5.74 Mb|
|Price:||Free* [*Free Regsitration Required]|
A review of the reported defects in the human C1 esterase inhibitor gene producing hereditary angioedema including four new mutations. Clin Immunol. Acquired angioedema. Allergy Asthma Clin Immunol.
Acquired deficiency of the inhibitor of the first component of complement: report of five additional cases with commentary on the syndrome. J Allergy Clin Immunol. Adverse effects of danazol prophylaxis on the lipid profiles of patients with hereditary angioedema. AE 3rd. Increased vascular permeability in C1 inhibitor-deficient mice mediated by the bradykinin type 2 receptor. Ambiguous genitalia in a term female infant due to exposure to danazol in utero.
Angioedema due to acquired C1-inhibitor deficiency: a bridging condition between autoimmunity and lymphoproliferation. Autoimmun Rev. Schweiz Med Wochenschr. Angiotensin-converting enzyme inhibitor associated angioedema. Immunol Allergy Clin North Am. Acta Med Scand Suppl. Bradykinin-mediated angioedema. C1 esterase inhibitor deficiency, airway compromise, and anesthesia.
Anesth Analg. C1 inhibitor deficiency: consensus document. Clin Exp Immunol. Characterisation of a new C1 inhibitor mutant in a patient with hepatocellular carcinoma. Mol Immunol. Clinical management of hereditary angio-oedema in children. Pediatr Allergy Immunol. Clinical studies of sudden upper airway obstruction in patients with hereditary angioedema due to C1 esterase inhibitor deficiency. Arch Intern Med.
Detection of C1 inhibitor mutations in patients with hereditary angioedema. Revista Brasileira de Alergia e Imunopatologia. Disease Control CDC. Inactivated hepatitis B virus vaccine. Ecallantide for the treatment of acute attacks in hereditary angioedema. Effects of short-term and long-term danazol treatment on lipoproteins, coagulation, and progression of atherosclerosis: two clinical trials in healthy volunteers and patients with hereditary angioedema.
Clin Ther. Epsilon aminocaproic acid therapy of hereditary angioneurotic edema: A double-blind study. Five novel mutations in the C1 inhibitor gene C1INH leading to a premature stop codon in patients with type I hereditary angioedema.
Hum Mutat. Hereditary angioedema and chronic urticaria: is there a possible association? Frequent de novo mutations and exon deletions in the C1 inhibitor gene of patients with angioedema. Gompels MM. C1-inhibitor deficiencies hereditary angioedema : where are we with therapies? Curr Allergy Asthma Rep. Hepatocellular adenomas in patients taking danazol for hereditary angio-oedema. Hereditary and acquired C1-inhibitor deficiency: biological and clinical characteristics in patients.
Medicine Hereditary angio-oedema treated with Eaminocaproic acid. Hereditary angioedema guidelines Giavina-Bianchi P et al. Hereditary angioedema in women. Hereditary angioedema with normal C1-inhibitor activity in women.
Hereditary angioedema: a current state-of-the-art review, IV: short-and long-term treatment of hereditary angioedema: out with the old and in with the new? Ann Allergy Asthma Immunol. Hereditary Angioedema: a current state-of-the-art review, V: attenuated androgens for the treatment of hereditary angioedema.
Hereditary angioedema: a current state-of-the-art review, VI: novel therapies for hereditary angioedema. Hereditary angioedema: a decade of human C1-inhibitor concentrate therapy.
Hereditary angioedema: a rare but potentially lethal disease. Hereditary angioedema: an appraisal of cases. Hereditary angioedema: long-term treatment with one or more injections of C1 inhibitor concentrate per week.
Int Arch Allergy Immunol. Hereditary angioedema: optimal therapy. Hereditary angioedema: the clinical syndrome and its management in the United States. Hereditary angioedema.
Ann Emerg Med. Hereditary angioneurotic edema: two genetic variants. Hereditary angioneurotic edema. Icatibant, a new bradykinin-receptor antagonist, in hereditary angioedema. International consensus algorithm for the diagnosis, therapy and management of hereditary angioedema.
Long term therapy of hereditary angioedema with danazol Ann Intern Med. Long-term danazol prophylaxis does not lead to increased carotid intima-media thickness in hereditary angioedema patients. Management of hereditary angio-oedema with low-dose danazol. Management of hereditary angioedema in pediatric patients. Acute pancreatitis associated with hereditary angioedema. Gastroenterol Hepatol. Nanofiltered C1 inhibitor concentrate for treatment of hereditary angioedema.
Oestrogen-dependent hereditary angio-oedema with normal C1 inhibitor: description of six new cases and review of pathogenic mechanisms and treatment.
Oxandrolone treatment of childhood angioedema. Pediatric hereditary angioedema due to C1-inhibitor deficiency. Plasma bradykinin in angio-oedema. Recurrent episodes of skin angioedema and severe attacs of abdominal pain induced by oral contraceptives or hormone replacement therapy.
Self-administration of intravenous C1-inhibitor therapy for hereditary angioedema and associated quality of life benefits. Side effects of long-term prophylaxis with attenuated androgens in hereditary angioedema: comparison of treated and untreated patients.
Spontaneous activation of the first component of human complement C1 by an intramolecular autocatalytic mechanism. The effect of long-term danazol prophylaxis on liver function in hereditary angioedema in hereditary angioedema-a longitudinal study. The effects of fetal exposure to Danazol. The humanistic burden of hereditary angioedema: Impact on Hereditary angioedema guidelines Giavina-Bianchi P et al.
Allergy Asthma The long-term safety of danazol in women with hereditary angioedema. Fertil Steril. Therapeutic agents for hereditary angioedema. Tranexamic acid therapy in hereditary angioneurotic edema.
Tranexamic acid: preoperative prophylatic therapy for patients with hereditary angioneurotic edema. Transient adrenogenital syndrome due to exposure to danazol in utero. Treatment of acute edema attacks in hereditary angioedema with a bradykinin receptor-2 antagonist Icatibant. Treatment of hereditary angioedema with danazol. Treatment of hereditary angioneurotic oedema with tranexamic acid. A random double-blind cross-over study.
Acta Med Scand. Type 1 hereditary angiooedema. Variability of clinical presentation and course within two large kindreds. Monatsh Prakt Dermatol. Ultrasonography in the diagnosis and monitoring of ascites in acute abdominalattacksofhereditaryangioneuroticoedema.