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A Case of Chronic Granulomatous Disease

Journal of the Korean Pediatric Society 1992;35(5):704-712.
Published online May 15, 1992.
A Case of Chronic Granulomatous Disease
So Young Lee1, Dae Chul Kim1, Sung Hee Oh1, Hahng Lee1, Hyang Eun Sohn2, Wha Soon Chung2, Young Hyeh Ko3, Moon Hyang Park3
1Department of Pediatrics, College of Medicine, Hanyang University, Seoul, Korea
2Department of Clinical Pathology, College of Medicine, Hanyang University, Seoul, Korea
3Department of Pathology, College of Medicine, Hanyang University, Seoul, Korea
만성 육아종성 질환 1례
이소영1, 김대철1, 오성희1, 이항1, 손향은2, 정화순2, 고영혜3, 박문향3
1한양대학교 의과대학 소아과학교실
2한양대학교 의과대학 임상병리학교실
3한양대학교 의과대학 조직병리학교실
Abstract
Chronic granulomatous disease (CGD) is a hereditary disorder of neutrophil with defective killing and digesting of ingested catalase-positive bacteria or fungi in spite of normal phagocytosis. Dissemination of ingested live organisms results in multiple granuloma in reticuloendothelial system and multiple abscesses manifested by recurrent pus-draining suppurative lymphadenopathy, subcutanous abscess, or impetiginous skin rahses, pheumonitis, and hepatosplenomegaly, ultimately resulting in death. Two cases of CGD from South Korea were mentioned in the world literature 10) and three cases were reported in a Korean literature 8,9), suggesting that Korean population is certainly not free from the genes of CGD and more cases are expected to be identified. The authors report a 2 years old Korean male child who presented with multiple suppurative cervical lymphadenitis, draining pus at the sites of incision and drainage, generalized papulonodular rashes on extremities, and chronic pneumonic infiltrations. The past medical history revealed that the patient had been treated for neonatal meningitis, frequent upper respiratory infections, pneumonia, recurrent suppurative unhealing cervical lymphadenitis where staphylococcus was once isolated, assumed BCGitis, generalized papulonodular skin lesions. Laboratory investigation revealed Serratia marcescens growing from a draining cervical lymph node and acid-fast bacilli from a skin biopsy sample obtained after 8 months therapy wiith isoniazid and rifampin for assumed BCGitis. The diagnosis of CGD was confirmed by a stimulated NBT test, using a endotoxin-coated coverslip according to Ochs and Igo. The test revealed no NBT positive neutrophil, in the patient while over 90% NBT-positive neutrophils both in his father and his younger male sibling and 50% NBT-positive neutrophils in his mother, suggesting a sex-linked recessive inheritance. Serratia marcesens infection on the cervical area was resolved with parenteral antibiotic therapy and oral Trimethoprim-Sulfamethoxazole(TMP-SMZ) over 3 weeks, and frequent thorough dressing of the draining sitesl The skin lesions on extremities also improved slowly without other special therapeutic intervention. At 4 weeks after discharge on prophylactic oral TMP-SMZ following 6 weeks of hospitalization, he returned with a recurrent cervical abscess with Candida albicans identified from the drained pus, responding to 3 weeks course with Amphotericin B. Thereafter, he is followed on an out-patient basis without any further major infectious event while on oral prophylactic TMP-SMZ over the 15 months of followup until the time of this report and maintaining a good general healthe with completely resolved pulmonary infiltration, skin rash, and pus-draining cervical lymphadenitis and with a height at 10~25th percentile and a weight at 25~50th percentile. In conclusion, a relatiely simple stimulated NBT slide test could confirm the diagnosis of CGD in a 2 years old Korean male child and oral prophylaxis with TMP-SMZ could maintain a good general health for 15 months of follow-up after intense antibacterial and antifungal therapy for generalized infections at presentation.
Key Words: Chronic granulomatous disease, Stimulated NBT test, Trimethoprim-Sulfamethoxazole


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