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[Pulmonary symptoms of primary immunodeficiency diseases]
E Pérez Ruiz1, J Pérez Frías, F J García Martín
1Departamento de Pediatría, Hospital Materno-Infantil (Carlos Haya) Málaga.
Insights
Pediatric primary immunodeficiency disease (PID) often presents with severe pulmonary issues, including recurrent pneumonia and bronchial responsiveness. Early diagnosis by pediatric pulmonologists is crucial for better patient outcomes.
Area of Science:
- Immunology
- Pediatric Pulmonology
- Genetics
Context:
- Primary immunodeficiency disease (PID) compromises the immune system, increasing susceptibility to infections.
- Pulmonary sites are particularly vulnerable due to the concentration of immune components.
- Pediatric patients with PID face significant risks of severe and recurrent respiratory infections.
Purpose:
- To investigate the spectrum of pulmonary manifestations in pediatric patients diagnosed with primary immunodeficiency disease (PID).
- To highlight the role of pediatric pulmonologists in the early detection and management of PID.
Summary:
- A study of 44 children under 14 with PID revealed that antibody deficiencies were most common (61.3%).
- Pulmonary issues affected 61.3% of patients, including bronchial responsiveness, recurrent pneumonias, bronchiectasis, and severe/opportunistic pneumonias.
- Respiratory symptoms were the initial presentation in 70.3% of affected children.
Impact:
- Emphasizes the critical role of pediatric pulmonologists in recognizing PID symptoms.
- Highlights the need for prompt diagnosis to improve prognosis in children with PID.
- Underscores the significant pulmonary burden associated with primary immunodeficiency diseases in children.
Objective:
Patients who lack major components of the immune system carry an increased risk for severe and recurrent pulmonary infections at those respiratory sites were the deficient component would, in the normal state, have its greatest concentration. We report different pulmonary manifestations in pediatric patients with primary immunodeficiency disease (PID).
Patients And Methods:
We studied 44 children younger than 14 years old, who were diagnosed of PID in our pediatric department between January 1990 and May 1996.
Results:
Antibody deficiencies were the most frequent disorders (27/44; 61.3%) followed by PID associated with or secondary to other disorders (10/44; 22.7%) and defects of phagocyte function (5/44; 11.3%). Twenty-seven patients (61.3%) showed relevant pulmonary manifestations that required assistance in the division of pediatric pulmonology. Bronchial responsiveness was seen in 17/27, 11/27 had recurrent pneumonias with development of bronchiectasis in 7/27. Opportunistic or severe pneumonias leading to acute respiratory failure were diagnosed in 9/27. Necrotizing pneumonias leading to development of pneumatoceles, cavities or abscesses was seen in 3/27 with the same rate for lymphoid interstitial pneumonia. Respiratory symptoms were the first manifestations of PID in 19/27 (70.3%).
Conclusions:
The findings of the study emphasize the responsibility of the pediatric pulmonologists in avoiding the delayed diagnosis of PID since the prognosis depends on the precocity of diagnosis.