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[Liver abscess in chronic granulomatosis of childhood (author's transl)]
Abstract:
A thirteen-year-old boy with an abscess of the liver and recurrent infections since infancy is described. Aspergillus fumigatus could be isolated from this surgical treated abscess. Polymorphonuclear leukocytes (PMN) and monocytes from the patient show a normal phagocytosis, whereas the oxygen metabolism and the intracellular killing of kastalase-positive microorganismus (Staphylococcus aureus, Candida albicans) are strikingly decreased. In contrast, the catalase-negative Pneumococcus pneumoniae is rapidly killed. The iodination (131 J) of PMN is despite phagocytosis also markedly reduced. Myeloperoxidase could be detected in the phagocytes histochemically. No morphologic abnormalities of PMN have been found by electron microscopy. A defect of the humoral of cellular immunity has been excluded. The characterized dysfunction of PMN and monocytes as well as the clinical features of the patient are consistent with a diagnosis of chronic granulomatous disease of childhood.
Insights
This study describes a boy with recurrent infections and a liver abscess, revealing impaired immune cell function. His condition suggests a diagnosis of chronic granulomatous disease, impacting his ability to fight certain infections.
Area of Science:
- Immunology
- Pediatric Infectious Diseases
Background:
- Recurrent infections and liver abscesses in a young patient suggest an underlying immune deficiency.
- Previous evaluations excluded humoral or cellular immunity defects.
Observation:
- A patient presented with recurrent infections since infancy and a liver abscess from which Aspergillus fumigatus was isolated.
- Polymorphonuclear leukocytes (PMN) and monocytes exhibited normal phagocytosis but decreased oxygen metabolism and intracellular killing of catalase-positive microorganisms.
- Catalase-negative bacteria (Pneumococcus pneumoniae) were rapidly killed, and PMN iodination was reduced despite phagocytosis.
Findings:
- The patient's immune cells demonstrated a specific defect in killing catalase-positive microbes.
- Histochemical analysis confirmed myeloperoxidase presence in phagocytes, with no morphological abnormalities observed via electron microscopy.
- The observed immune dysfunction, coupled with clinical symptoms, strongly indicates chronic granulomatous disease.
Implications:
- This case highlights the diagnostic challenges and specific immune deficits associated with chronic granulomatous disease (CGD).
- Understanding these cellular dysfunctions is crucial for accurate diagnosis and management of CGD in pediatric patients.
- The findings underscore the importance of evaluating oxidative metabolism in phagocytes for diagnosing immunodeficiencies affecting microbial killing.