A 4-year-old Thai boy with RUL consolidation (Page 2/2) |
| Prepared by...
Kakanang Jantarapagdee, M.D.
Sudawan Siriaksorn, M.D.
Thirasak Borisuthibandit, M.D.
Muthita Trakultivakorn, M.D.
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| Department
of Pediatrics, Faculty of Medicine, Chiang Mai University |
| Course in the hospital and Discussion:
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The result of NBT test suggested that he had chronic granulomatous disease (CGD), confirmed by DHR-123 assay which indicated that the disease was transmitted as X-linked inheritance. Patients with CGD were susceptible to infection with catalase-positive organisms e.g. S. aureus, B. cepacia, S. marcescens, C. violaceum, Nocardia spp. or Aspergillus spp. |
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The causative agent was not identified from the initial investigations except Candida spp. in BAL fluid. Because this organism may be one that colonized in his oropharynx and contaminated tip of the bronchoscope, open lung biopsy was performed. |
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Histological findings of his lung tissue were shown in Figure 6 (A-B). The granulomatous inflammation of lungs in patients with CGD are usually secondary to infection especially from Aspergillus spp. or sarcoidosis (reported cases). The former was more common and response to antifungal therapy, while the latter was less common and response to corticosteroid therapy.
| Figure 6: The staining for AFB, PAS, GMS were negative. |
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(A) Histological finding revealed the non-caseating, non-necrotizing granulomatous inflammation of lung parenchyma |
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(B) Numerous RBC filled in alveolar space. |
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After 4-week-course of oral itraconazole therapy. His clinical and chest x-ray were markedly improved (Figure 7 A-B). Aspergillus spp. was the most propable causative agent. Antifungal therapy had been continued until his chest x-ray returned to normal before it was switched to prophylactic dosage. |
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| Figure 7: |
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(A) Chest x-ray after 4-week-course, |
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(B) after 4-month-course of oral itraconazolehis CXR revealed marked improvement. |
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He was given co-trimoxazole prophylaxis for bacterial infection. His mother was educated about keeping him away from getting any live-attenuated vaccines. The genetic counseling was also done. He was planned to undergo hematopoietic stem cell transplantation when he is free from infection and a matched-donor is available. |
