Portal de Periódicos da CAPES

HEMOPTISE EM CRIANÇA: UM ACHADO DA SÍNDROME DE MUNCHAUSEN

2018; Volume: 19; Issue: 4 Linguagem: Inglês

10.5935/1676-0166.20180024

1676-0166

Letícia G. Cabrini, Letícia F. Legnani, Letícia B. de Souza, Bruna P. da Trindade, Débora Carla Chong‐Silva,

Hemoglobinopathies and Related Disorders

Objective: primary immunodeficiencies (PID) are common, but under diagnosed diseases.The Jeffrey Modell Foundation developed warning signs to help physicians identify PID.The aim of this study was to evaluate ten warning signs application in an outpatient reference center in Southern Brazil.Method: cross-sectional study applying ten warning signs in patients followed in a reference center for PID treatment between 1994 and 2013.Warning signs were adapted by experts to Brazilian reality based on the original by Jeffrey Modell Foundation.Results: forty five patients with PID were evaluated; 32 (71.1%) had humoral deficiencies, 7 (15.6%)immunodeficiency associated to genetic syndrome, 3 (6.7%)congenital phagocyte defect, 1 (2.2%)SCID and 2 (4.4%) unclassified.Forty four (98%) showed one or more warning signs.The more frequently warning sign was two or more pneumonia within 1 year (71%); next was four or more new ear infections within 1 year (32%); then, 1 or more severe systemic infection (27%), recurrent intestinal infections/chronic diarrhea (27%) and a family history of PID (20%).Conclusion: PID are common and underrecognised by health professionals.Suspicion is essential to diagnosis and ten warning signs have been a helpful tool to investigate patients for PID.