A 5-year-old male patient was admitted to Children Medical Center (CMC) with inflammation and swelling in his left mandible and wrist without a history of trauma. In the past, he had suffered from several episodes of pneumonia which started at the age of seven months. On admission, laboratory findings included erythrocyte sedimentation rate (ESR) 84 mm/h, WBC count 12100/mm3 (61% neutrophils, 39% lymphocytes), hemoglobin 11.3 gr/dl and thrombocyte 386000/mm3. As the CRP analysis displayed 20 mg/dl, cephalexin (150 mg/kg/day) was initiated. In his roentgenogram, osteolytic lesions in the distal metaphase of hand and maxillary bone were observed. Considering history of several infections and multifocal osteomyelitis, bone biopsy was performed and his immune system function was evaluated. In the bone biopsy, non-necrotizing granulomatoid lesions were seen. The induration of purified protein derivative reaction was 10 mm diameter. Besides, HIV, hepatitis B surface antigen (HBs), rheumatoid factor and brucella agglutination tests were all negative. The serum IgG level was 1650 mg/dl (normal: 441–1135 mg/dl). IgM and IgA were in high normal range at 250 and 175 mg/dl, respectively. Because no defect was found in his humeral and cellular immunity, the phagocytic cells function was tested with a nitroblue-tetrazolium (NBT) slide test. Based on his hematological and immunological tests (NBT = 0), CGD was considered as underlying disease in this case. Regarding his NBT test, antibiotic therapy was changed from cephalexin to co-trimoxazole (20 mg/kg/day, iv) plus (along with) interferon-γ (50 microgram/m2 every other day). After two weeks of treatment, the patient's condition improved and he was discharged with prescription of both cephalexin (100 mg/kg/day) and co-trimoxazole (10 mg/kg/day) to be taken orally as prophylaxis.
The patient was readmitted to our center after eight months with a tender mass in his right upper quadrant (RUQ) (Fig 1). On admission, his major complaint was severe dyspnea, a persistent cough and also chest and abdominal pain in epigastric area which was started 10 days ago. He was placed on antibiotic therapy including cephalexin (100 mg/kg/day). A computerized tomography (CT) scan of the chest and abdomen was performed which revealed the hypodense area in liver (Fig 2). Adjacent to this opacity, involvement of lower right ribs and reaction to soft tissue were also observed, indicating ribs osteomyelitis. After sonography guided drainage of the above-mentioned hepatic abscess, a sample was sent to the Mycology Department in Tehran University of Medical Sciences. The microscopic examination of clarified specimen with KOH 10% indicated the branched, septated and dichotomous mycelia (Fig. 3). The remaining specimen was also cultured on Brain Heart Infusion agar (BHI), Sabouraud's dextrose agar (S) and Sabouraud's containing 0.005% chloramphenicol (Sc). The S and Sc culture media were incubated at 25°C and BHI at 37°C. The colonies grew rapidly, attaining the diameter of 5 cm within 5 days and their color was bluish green. Cellophane tape preparations and slide cultures demonstrated septated, branched and hyaline hyphae with rough-walled conidiophores and radiated conidial heads. Based on these microscopic and macroscopic findings, Aspergillus fumigatus was determined as causative agent in this case. Deoxycholate Amphotericin B (1 mg/kg/day, iv), interferon-γ (50 microgeram/m2 every other day, sc) and rifampicin (10 mg/kg/day) were administered with diagnosis of invasive aspergillosis. The only adverse event observed during treatment was hypokalemia, which was adjusted by administration of potassium chloride 15%. One month after initiation of antifungal therapy, his follow-up CT scan of the abdominal and thoracal region demonstrated relative resolution of hepatic abscess. After four weeks of intravenous treatment, the patient's clinical condition improved. He was discharged upon his parents' responsibility while continuing taking rifampicin (10 mg/kg/day) for two more weeks as a treatment in addition to co-trimoxazole (5 mg/kg/day) and itraconazole (4 mg/kg/day) as long term prophylaxis.