Profile and prognosis of adolescents and adults with primary immunodeficiencies in the public health service in Brazil
| Autor: | Maria Conceição Santos Moraes, Fernando Samuel Sion, Licia Clara Fernandes Couto Da Silva, Carlos Alberto Morais de Sá, Fernanda Maia Vidal, Albertina Varandas Capelo, Norma de Paula Motta Rubini |
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| Jazyk: | angličtina |
| Předmět: |
Pulmonary and Respiratory Medicine
Pediatrics medicine.medical_specialty Severe combined immunodeficiency business.industry Immunology Treatment dropout Disease medicine.disease Bioinformatics Public health service Combined immunodeficiencies Meeting Abstract medicine Immunology and Allergy business Autoinflammatory Disorders |
| Zdroj: | The World Allergy Organization Journal |
| ISSN: | 1939-4551 |
| DOI: | 10.1186/1939-4551-8-s1-a134 |
| Popis: | Results 33 patients were analyzed: 55% female, 70% Caucasian, 27% Black, and 3% Asian, with ages between 12 and 64 years (mean = 36.7, SD = 11) and length of follow-up between 1 and 17 years (mean = 8.84, SD = 11.29). The profile and frequency of PIDs was as follows: Predominantly antibody deficiencies (55%), Combined immunodeficiencies less profound than generally severe combined immunodeficiency (18%) Combined immunodeficiencies associated with features or syndromic (15%), Complement deficiencies (9%), and Autoinflammatory disorders (3%). The average time between onset of symptoms and diagnosis of PID ranged between 1 and 28 years (mean = 10.83, SD = 7.07). Compliance with treatment was good in 76% of patients, partial in 12% and 12% of treatment dropout. Survival between the onset of clinical manifestations and the end of the study ranged between 1 and 47 years (mean = 21.52, SD =13.79). Throughout the study period, one patient, with Wiskott-Aldrich syndrome and partial adherence to treatment, died due to septicemia and there were two deaths from cardiovascular disease. |
| Databáze: | OpenAIRE |
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