Guía de práctica clínica: tamizaje, diagnóstico y manejo de episodios agudos y profilaxis del angioedema hereditario Parte I: Enfoque diagnóstico / Clinical practice guidelines: screening, diagnosis and management of acute events and prophylaxis of hereditary angioedema Part I: Diagnostic approach
Arq. Asma, Alerg. Imunol
; 4(4): 382-393, out.dez.2020. ilus
Article
in Es
| LILACS
| ID: biblio-1382033
Responsible library:
BR2717.9
RESUMEN
El angioedema hereditario (AEH) es una enfermedad genética rara, con una prevalencia aproximada entre 1 por cada 50.000 habitantes, caracterizada por episodios de edemas a nivel subcutáneo y de mucosas (abdominal, genitourinario, respiratoria), siendo potencialmente mortal cuando hay afectación de la laringe. En Perú se estiman 600 pacientes con AEH. El AEH se puede clasificar del siguiente modo con deficiencia del inhibidor de C1 (tipos I y II), y sin deficiencia del inhibidor de C1 (denominado anteriormente tipo III). El diagnóstico de laboratorio incluye prueba de complemento C4, prueba cuantitativa y cualitativa para inhibidor de C1 esterasa, y estudios genéticos.
ABSTRACT
Hereditary angioedema (HAE) is a genetic rare disease with a prevalence of approximately 1 per 50,000 inhabitants, characterized by episodes of edema at the subcutaneous level and mucous membranes (abdominal, genitourinary, respiratory), being potentially fatal when there is involvement of the larynx. In Peru, there are an estimated 600 patients with HAE. HAE can be classified as follows with C1 inhibitor deficiency (types I and II), and without C1 inhibitor deficiency (previously called type III). Laboratory diagnosis includes C4 complement test, quantitative and qualitative tests for C1 inhibitor esterase, and genetic studies. In this first part of the Clinical Practice Guide, we present the recommendations for the diagnostic approach of HAE.
Key words
Full text:
1
Index:
LILACS
Main subject:
Peru
/
Mass Screening
/
Clinical Laboratory Techniques
/
Diagnosis
/
Angioedemas, Hereditary
Type of study:
Diagnostic_studies
/
Guideline
/
Qualitative_research
/
Risk_factors_studies
/
Screening_studies
Limits:
Humans
Country/Region as subject:
America do sul
/
Peru
Language:
Es
Journal:
Arq. Asma, Alerg. Imunol
Journal subject:
Biologia
/
Cincia
Year:
2020
Type:
Article