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[Primary immunodeficiency state in a child with the pulmonary hypertension syndrome]
Insights
A young child with recurrent infections and primary pulmonary hypertension was diagnosed with primary immunodeficiency. Postmortem analysis revealed suppressed cell-mediated immunity, contributing to severe lung and heart conditions.
Area of Science:
- Pediatric Immunology
- Cardiology
- Pulmonology
Background:
- A 2-year-old girl experienced recurrent viral and bacterial infections.
- Primary pulmonary hypertension was diagnosed at age 2.
Observation:
- Diagnostic cardiac catheterization was performed to rule out congenital heart disease.
- The patient unfortunately died during the procedure.
Findings:
- Postmortem examination showed increased beta-lymphocytes and plasma cells, with reduced T-lymphocyte zones.
- Pathology revealed primary immunodeficiency with suppressed cell-mediated immunity, lymph node hyperplasia, pneumosclerosis, and cardiac hypertrophy.
Implications:
- This case highlights a severe primary immunodeficiency presenting with pulmonary hypertension and cardiac complications.
- Understanding such rare conditions is crucial for early diagnosis and management in pediatric patients.
Abstract:
A girl aged 2 years and 10 months repeatedly suffered viral (thrice) and bacterial (colitis, salmonellosis, pneumonia 6 times) infections. At an age of 2 years primary pulmonary hypertension was diagnosed. Diagnostic catheterization was performed to exclude a congenital heart disease. The death occurred during the catheterization. An increase of beta-lymphocytes and plasma cells, a reduction of the T-lymphocyte zone were detected in the immunocompetent system during postmortem histological examination. The pathology diagnosis: primary immunodeficiency with a predominant suppression of cell-mediated immunity; hyperplasia of the lymph nodes with their plasmacytization; pneumosclerosis of all lobes of both lungs (a syndrome of primary pulmonary hypertension clinically); hypertrophy of the muscles of both atria and right ventricle, dilation of the heart cavities; acute heart insufficiency.