Abstract. JESUS, Adriana Almeida de et al. Associação de imunodeficiências primárias com doenças auto-imunes na infância. Rev. Bras. Reumatol. [online]. APDIP é uma associação sem fins lucrativos que foi criada com a finalidade de apoiar os doentes com imunodeficiências primárias em Portugal. A Imunodeficiência Combinada Grave é uma imunodeficiência primária rara, na qual existe a ausência combinada das funções dos linfócitos. T e dos linfócitos.

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The mean age was 4. Rev Alerg Mex ; It included patients diagnosed with any serious infection admitted to the PICU from January to Januaryand excluded those with a history of hospitalization for initial trauma or postoperative for other causes.

Arch Inst Primaeias Tunis ; In the present study, the most prevalent initial infection responsible for ICU admission was pneumonia, given equivalent surveys of general infections in children with PIDs Copyright and License information Disclaimer. Imunopediatria [cited Feb 01]; [homepage on the Internet].

imunodeficiencias primarias diagnostico

The data were obtained through the yearbook of admissions of the PICU and records were analyzed until the period of data collection, which took place from March to Mayi. Support Center Support Center.

The immunological investigation in patients with PIDs is individualized according to the signs and symptoms and must be performed in stages, according to the findings in each subject 2.

In general, PIDs are monogenic, hereditary diseases, which cause immunological changes and can express increased susceptibility to certain types of infections, tumors, or autoimmune diseases imunodrficiencias2.

Received Jun 28; Accepted Sep From January to December4. Results From January to January53 children were admitted to the PICU involved in the study with primary diagnosis of some form of severe infectious. Thus, even if the blood count and immunoglobulins have shown normal results in two other patients investigated, other humoral changes cannot be excluded, such as complement changes or qualitative deficit of antibodies, or even cellular changes, such as qualitative for phagocytes or lymphocytes, or other more specific.


Retrospective study that assessed the data records of patients with any severe infections admitted in the Pediatric Intensive Care Unit, covering a period from January to Januaryin order to confirm if they performed an initial investigation for PID with blood count and immunoglobulin dosage.

J Allergy Clin Immunol. J Clin Immunol ; Cell Mol Life Sci ; Coutinho A, Carneiro-Sampaio M.

The most prevalent diagnoses associated with hospitalization were pneumonia in 22 The present study has limitations due to the retrospective data collection, with analysis depending on the completion of the medical records of each patient. Rev Bras Alerg Imunopatol. Allergol et Immunopathol ; This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Pulmonary complications of primary immunodeficiencies. Two patients with low IgG levels alone were diagnosed with transient imunodeficienckas of infancy, and the patient with global deficit of antibodies is one of the four patients who died during hospitalization, not having the chance to complete the investigation.

Alergol Immnuopathol Madr ; Among the seven patients with initial screening, only two Flow cytometric analysis of the granulocyte respiratory burst: Clinical and laboratory characteristics of 75 patients with specific polysaccharide antibody deficiency imunodeficeincias.

Defesas vulneráveis : Revista Pesquisa Fapesp

These patients should be referred for further evaluation in immunology research centers, which require greater dissemination among pediatricians so that patients can be referred, investigated, and followed-up accordingly. Primary immune deficiencies presenting in adults: Primary immunodeficiencies PIDs are rare diseases considered in isolation; however, they comprise a set of over different changes already described and have an estimated prevalence of 1: Ear, nose, and throat manifestations in patients with primary immunodeficiencies.

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In this sense, the Jeffrey Modell Foundation, along with other American institutions, formulated warning signs to draw attention for the need to investigate a possible immune deficiency in this group of patients 5.

Ann Allergy Asthma Immunol ; In the present study, in the seven cases investigated, four had diagnostic confirmation of PIDs and another had low levels of all classes of antibodies, being highly suggestive of agammaglobulinemia with imunodeficienclas compatible with the mentioned survey.

Response to polysaccharide antigens in patients with ataxia-telangiectasia. Immune Deficiency Foundation [cited Feb]; [homepage on the Internet]. However, this association suggests that similar defects in immune response could be related to both diseases and that prospective studies are needed to elucidate imujodeficiencias hypothesis.

J Immunol Methods ; Diagnostic criteria for primary immunodeficiencies. Rheumatologic findings may be imunodeficciencias first manifestation of primary humoral immunodeficiencies.

The levels of antibodies were demonstrated in graphical representation of immunoglobulins by age Figure 1. Results were expressed by means of descriptive statistics.

Complement protein hereditary deficits during purulent meningitis: Infections are the main manifestations of PIDs. Our findings suggest the necessity of performing PID investigation in this group of patients.

Nossos achados sugerem a necessidade de investigar as IDP nesse grupo de pacientes. Among the 53 patients, only seven Hyper-IgM syndrome in members of two unrelated Chilean families: Nuestros hallazgos confirman la necesidad de investigar las IDP en ese grupo de pacientes.