Journal of the Korean Pediatric Society 1979;22(2):148-157.
Published online February 15, 1979.
A Case of Chronic Granulomatous Disease (Autopsy case).
Kil Sun Kong1, Sung Sook Cho1, Don Hee Ahn1, Keun Chan Sohn1, Joo Kyun Park2, Hyo Sook Park2, Je Keun Ji3
1Department of Pediatrics, National Medical Center.
2Department of Pathology, National Medical Center.
3Department of Pathology, College of Medicine, Seoul National University.
慢性 肉芽腫性 疾患
孔吉善1, 趙星淑1, 安敦姬1, 孫槿贊1, 朴柱堅2, 朴孝淑2, 池提根3
1國立醫療院 小兒科
2國立醫療院 病理科
3서울大學校 醫科大學 病理學敎室
Abstract
A 3 year and 3 months old boy with recurrent infections since his age of 5 months was presented with clinical data and autopsy findings. He was the 4th product of healthy parents. His elder brother died of recurrent perianal abscess and sepsis at his age of 3 years. His 2nd elder sister died on the 14th day of life probably from the complication of BCG vaccination. Beginnig with perianal abscesses at his age of 5 months, he has been continuously suffering from recurrent infections such as arthritis, ostomyelitis, pneumonia, epididymitis, subcutaneous abscesses and perianal abscesses. In spite of meticulous supportive and aggressive antibiotic therapy persistent positive cultures for staph. Aureus, klebsiella, E. Coli, Enterococcus and coliform bacilli from different sited were noted. Erythrocyte sedimentation rate of 25 to 40 were constant. White cell count varied frem 15500 to 33400 with polymorphonucleocytes predominance. NBT test showed persistent low scoring of 2% throught the course. He finally died of pneumonia and empyema. At postmortem examination, multiple abscesses and grnulomas of right lung and multipe granulomas in the liver, spleen, lymph node, bone, marrow, adrenal gland, kidney and intestinal wass were noted. At microscopic examination histiocytic granulomas with lipid containing histiocyte infiltrations were noted in every organs described including brain.




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