Iran Hereditary Angioedema Registry (IHAER)
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About HAE
Hereditary angioedema (HAE) is a rare autosomal dominant disorder caused by a deficiency in quantitiy (Type I) or function (Type II) of complement1 esterase inhibitor (C1-INH), characterized by subcutaneous and/or submucosal edema attacks involving different organs. HAE could give rise to fatal laryngeal edema, acute abdominal pain leading to unnecessary emergency surgeries, and considerably reduced quality of life.
Aim of  HAE Registry
Rare diseases registries are valuable tools for: 1) increasing understanding around the natural history, risk, and outcomes; 2) supporting research on genetic, and molecular basis; 3) as well as development of treatment protocols for a rare disease. Moreover, it facilitates connections among affected patients, families, and clinicians.
Considering  the fact that HAE is rare neglected but potentially life-threatening disorder, Iran National Hereditary Anagioedema Registry (IHAER) was established in 2006 for the first time in Iran at Immunology, Asthma and  Allergy Research Institute (IAARI)  to achieve the above mentioned goals.
Registry Design
The patients referred to IAARI with symptoms suggestive of HAE are investigated using a questionnaire ( recording demographic and clinical) and laboratory tests. The patients with a definite diagnosis of HAE (type I, II) based on low serum levels of C4 and C1INH quantity/function accompanying with suggestive clinical findings enter this Registry. Moreover, genetic analysis is available to reconfirm laboratory results. 
A Brief Report
Among more than 700 HAE suspected cases referred to IAARI, a number of 82 patients (females:43, males:39) with definite diagnosis of HAE have been registered in IHAER from 2006 to 2016.
 
Achievements:
  • Registered in Ministry of Health and Medical Education of Iran
  • In collaboration with more than 40 Clinical Immunologists experts all over Iran
  • More than 12 published article and abstracts
  • Presenting award granted abstracts in more than 6 prominent international congresses like EAACI, ESID, IPIC, ERS, GUF
  • Performing HAE Genetic Diagnosis
  • Authoring the “National Instruction of HAE Diagnosis and Management for Healthcare providers”
  • Providing patients with informative materials and HAE ID card for emergencies
 
Future Perspectives
  • Establishment of a comprehensive registry software
  • Collaboration with international counterparts
  • Promoting diagnostic methods and facilitating treatment access for patients
 
2016/4/5


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