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Published on: April 21, 2012
Clinical manifestations of 42 Moroccan patients with chronic granulomatous disease
Assma Dably1, Ibtihal Benhssaein1,2, Jalila El Bakkouri
1Laboratory of Clinical Immunology, Inflammation and Allergy LICIA, Faculty of Medicine and Pharmacy, Hassan II University, Casablanca, Morocco.
Background:
Chronic granulomatous disease (CGD) is a primary immunodeficiency due to alterations in the oxidative metabolism of phagocytic cells. This condition is characterized by serious and recurrent infections caused by pyogenic bacteria, particularly Staphylococcus aureus, and fungal pathogens such as Aspergillus. These infections are associated with granuloma formation and inflammatory manifestations.The aim of our study was to report the clinical characteristics, microbiological aspects and outcomes, and prognosis of a cohort comprising 42 Moroccan patients suffering from CGD.
Methods:
A total of 42 patients were diagnosed for family history, consanguinity, and both clinical and laboratory findings.The diagnosis was confirmed by assessing neutrophil oxidative burst activity, using either the nitroblue tetrazolium (NBT) test or the dihydrorhodamine (DHR) test.
Results:
The cohort comprised children from 34 different families, including 12 siblings. The age of onset ranged from 4 days to 13 years, with the diagnosis being established between the ages of 25 days and 13 years. The predominant clinical manifestations were skin infections, lymphadenopathy, pneumonia, BCGitis, liver abscess, pulmonary aspergillosis, and inflammatory colitis. The most frequently isolated germs were Aspergillus, Serratia, and Staphylococcus. Among the total of 42 patients, 17 fatalities occurred, with aspergillosis being identified as the primary cause of their deaths.
Conclusions:
In this study, the clinical characteristics and isolated microorganisms correspond to the pathogens known to be important in CGD. Lung infections represent the most prevalent complication and significantly contribute to high mortality rates, particularly in the case of Aspergillus pneumonia, which is known for its tendency to disseminate. Additionally, BCGitis has been frequently observed in countries where the BCG (Bacille Calmette-Guérin) vaccination is routinely administered. Enterocolitis emerges as the most common inflammatory complication in clinical settings. Unfortunately, CGD remains largely unknown in Morocco, highlighting the urgent need to raise awareness among doctors. This increased awareness could facilitate early diagnosis and improve patient prognosis.
Insights
Chronic granulomatous disease (CGD) is a severe immunodeficiency causing recurrent infections. Aspergillosis was the leading cause of death in Moroccan CGD patients, highlighting the need for increased awareness and early diagnosis.
Area of Science:
- Immunology
- Genetics
- Pediatrics
Background:
- Chronic granulomatous disease (CGD) is a primary immunodeficiency affecting phagocytic cell oxidative metabolism.
- CGD leads to severe, recurrent infections from bacteria like Staphylococcus aureus and fungi such as Aspergillus.
- Infections in CGD patients are associated with granuloma formation and inflammatory responses.
Purpose of the Study:
- To describe the clinical features, microbiology, outcomes, and prognosis of 42 Moroccan patients with CGD.
- To highlight the specific challenges and patterns of CGD in a Moroccan cohort.
- To inform healthcare professionals about CGD in Morocco.
Main Methods:
- Diagnosis involved family history, consanguinity, clinical and laboratory findings.
- Neutrophil oxidative burst activity was confirmed using nitroblue tetrazolium (NBT) or dihydrorhodamine (DHR) tests.
- A cohort of 42 patients from 34 families was analyzed.
Main Results:
- The cohort included children diagnosed between 25 days and 13 years, with onset from 4 days to 13 years.
- Common manifestations included skin infections, lymphadenopathy, pneumonia, BCGitis, liver abscess, pulmonary aspergillosis, and inflammatory colitis.
- Aspergillus, Serratia, and Staphylococcus were the most frequent isolates; 17 of 42 patients died, primarily from aspergillosis.
Conclusions:
- Clinical and microbiological findings align with known CGD pathogens.
- Pulmonary infections, especially Aspergillus pneumonia, are a major cause of mortality.
- BCGitis and enterocolitis are significant complications; CGD is under-recognized in Morocco, necessitating greater physician awareness for improved diagnosis and prognosis.
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