Article in Japanese

The efficacy of systemic steroids for pulmonary granulomatous lesion in an infant with chronic granulomatous disease

Atsushi MIYAKE¹⁾, Kenji GOTOH¹⁾, Kiyohito OKUMIYA¹⁾, Kazuaki TATARA¹⁾, Yosuke NAKASHIMA¹⁾, Mariko TERAMACHI¹⁾, Yuhei TANAKA¹⁾, Yushiro YAMASHITA¹⁾

Chronic granulomatous disease (CGD) is a rare inherited disorder characterized by recurrent and life-threatening bacterial and fungal infections that usually begin early in life. It is caused by abnormality in neutrophils and other phagocytes of superoxide generating system. This paper reports a case of refractory pulmonary granulomatous lesions in a 14-day old boy with CGD who visited a local hospital presenting high fever. He was transferred to our hospital due to uncontrollable pulmonary infection. His illness was diagnosed as CGD by the detection of CYBB mutation and invasive aspergillus infection judged by a positive result from the aspergillus antigen test. Despite treatment with various types of antibiotics and antifungal agents, the pulmonary lesion and inflammatory indicators including C reactive protein and his number of white blood cells increased. His pulmonary granulomatous lesions were considered to be under hyper inflammatory status. Therefore prednisolone was administered at 1 mg/kg/day for 2 weeks. After steroid therapy, the inflammatory indicators decreased and the pulmonary granulomatous lesion contracted dramatically. This report indicates that the use of steroids might be helpful in controlling granulomatous lesions, which are refractory to antibiotics and antifungal therapy, in CGD patients.

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1) Department of Pediatrics and Child Health, Kurume University School of Medicine

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Received	March 10, 2016
Accepted	January 26, 2017

29 (1)：29─37,2017