Safety Of Plasma-derived C1-inhibitor Treatment In Pediatric Patients With Hereditary Angioedema Due To C1-inhibitor Deficiency – A Long-term Survey
نویسندگان
چکیده
منابع مشابه
Pediatric hereditary angioedema due to C1-inhibitor deficiency
Hereditary angioedema (HAE) resulting from the deficiency of the C1 inhibitor (C1-INH) is a rare, life-threatening disorder. It is characterized by attacks of angioedema involving the skin and/or the mucosa of the upper airways, as well as the intestinal mucosa. In approximately 50 per cent of cases, clinical manifestations may appear during childhood. The complex management of HAE in pediatric...
متن کاملTreatment of hereditary angioedema with plasma-derived C1 inhibitor
BACKGROUND Plasma-derived C1 inhibitor (C1-INH) concentrate is a treatment option for acute hereditary angioedema (HAE) attacks and is considered the standard-of-care in many countries, although it is not yet available in the United States. Studies are still being conducted to establish its safety and efficacy as required by the FDA. OBJECTIVE To review the medical literature to determine if ...
متن کامل[Hereditary angioedema due to C1-esterase inhibitor deficiency].
The paper presents a case of family oedema by shortage of C1-esterase inhibitor, characterized by appearance at an early age (8 months), participation in some attacks of nasal mucous membrane, with nasal obstruction and sinusal manifestations and also the abnormally long period, up to 9 days, of some localized oedemas. The crises were absent during pregnancy. The study of four generations of th...
متن کامل163 The Efficacy of Human Plasma-Derived C1-Inhibitor Concentrate Used for Prophylactic Treatment in Patients With Hereditary Angioedema Due to C1-Inhibitor Deficiency
vidual rhinoconjunctivitis symptom scores. The primary efficacy endpoint, the daily CS during the pollen period while on treatment, was analyzed using a repeated measures ANCOVA model, as were the above secondary efficacy endpoints. The safety of the treatment was documented by means of adverse event reporting, laboratory data and physical examination findings. Results: The 300IR group showed a...
متن کاملShort-term prophylaxis in hereditary angioedema due to deficiency of the C1-inhibitor--a long-term survey.
BACKGROUND Hereditary angioedema is a potentially life-threatening disorder, because edema occurring in the mucosa of the upper airways can lead to suffocation. The management of HAE consists of avoiding the triggering factors, prophylaxis, and the acute treatment of edematous episodes. Medical procedures can also provoke edematous attacks, and therefore, short-term prophylaxis (STP) is recomme...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Journal of Allergy and Clinical Immunology
سال: 2017
ISSN: 0091-6749
DOI: 10.1016/j.jaci.2016.12.758