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[Clinical characteristics of primary immunodeficiencies in children from a tertiary hospital]
Dayeli Anani García-Torres1, Ana Paola Macías-Robles, Liliana Pérez-Jiménez
1Instituto Mexicano del Seguro Social, Centro Médico Nacional de Occidente, Hospital de Pediatría, Servicio de Alergia e Inmunología Clínica Pediátrica, Jalisco, México. dayeli_anani@hotmail.com.
Insights
Primary immunodeficiencies in Mexican children are predominantly antibody deficiencies, with X-linked agammaglobulinemia being most common. Immunoglobulin replacement therapy significantly reduces hospitalizations, improving patient outcomes.
Area of Science:
- Pediatric Immunology
- Clinical Genetics
Background:
- Primary immunodeficiencies (PIDs) are a group of inherited disorders affecting the immune system.
- These conditions predispose individuals to recurrent infections and autoimmune complications.
Purpose of the Study:
- To characterize the epidemiological and clinical features of primary immunodeficiencies diagnosed at a pediatric reference center in West Mexico.
- To evaluate the impact of treatment on patient outcomes.
Main Methods:
- A cross-sectional study was conducted on pediatric patients diagnosed with primary immunodeficiency.
- Data collected included demographics, diagnosis, clinical manifestations, and treatment outcomes.
Main Results:
- Sixty pediatric patients were analyzed, with a male predominance (78%).
- Antibody deficiencies were the most common category (46%), notably X-linked agammaglobulinemia (21%).
- Infectious processes, particularly pneumonia (30%), were the primary clinical presentation; immunoglobulin replacement therapy reduced hospitalizations by over 95%.
Conclusions:
- Understanding the local epidemiology of PIDs is crucial for improving diagnosis and management.
- Early diagnosis and effective therapies like immunoglobulin replacement can significantly enhance survival and quality of life for affected children.
Background:
Primary immunodeficiencies are diseases that are caused by one or more defects in the immune system.
Objective:
The purpose of the article is to describe the characteristics of the immunodeficiencies that were diagnosed in a pediatric hospital, which forms a reference center in the West of Mexico.
Methods:
A cross-sectional study of pediatric patients with primary immunodeficiency in a pediatric hospital in Guadalajara, Jalisco.
Results:
60 patients were registered, of which 21% were female, and 78% were male. Predominant immunodeficiencies of antibodies formed the largest group (46% of patients). X-linked agammaglobulinemia was the most frequent immunodeficiency (21%); other frequent diagnoses were common variable immunodeficiency, and IgG subclass deficiency. The average age of the patients that were diagnosed was of 6.3 years. The most common initial manifestations were infectious processes (46%), and pneumonia was the frequent diagnosis (30%). Autoimmune manifestations were observed in 13% of patients; 67% of the patients were found with immunoglobulin replacement therapy, with which a decrease in hospitalizations from 3.2 times to 0.13 times was observed. There were two deceased patients due to surgical complications of cardiac correction and intestinal reconnection.
Conclusion:
Knowing the behavior of primary immune deficiencies in our environment allows the opening of areas of opportunity in order to improve the survival and quality of life of our patients.
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