Korean Journal of Pediatrics 2007;50(11):1129-1133.
Published online November 15, 2007.
Long-term recombinant interferon-γ treatment in 2 cases of osteopetrosis
Dong-Yun Kim1, Dong-Kyun Han1, Hee-Jo Baek1, Sung-Taek Jung2, Hoon Kook1, Tai-Ju Hwang1
1Departments of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School Chonnam, Korea
2Departments of Orthopedic Surgery, Chonnam National University Hwasun Hospital, Chonnam National University Medical School Chonnam, Korea
장기간 인터페론 감마로 치료한 골화석증 2례
김동연1, 한동균1, 백희조1, 정성택2, 국훈1, 황태주1
1전남대학교 의과대학, 화순전남대병원 소아과학교실
2전남대학교 의과대학, 화순전남대병원 정형외과학교실
Correspondence: 
Hoon Kook, Email: hoonkook@chonnam.ac.kr
Abstract
Osteopetrosis, a rare osteosclerotic bone disease characterized by a defect in osteoclast function and the reduced generation of superoxide by leukocytes, can be classified into several types based on their mode of inheritance, age of onset, severity, and associated clinical symptoms. Stem cell transplantation is the only curative therapy for the infantile malignant type, although alternative treatments, such as corticosteroids, calcitriol, and interferon (IFN)-γ have been attempted in patients with milder clinical types. In addition, IFN-γ therapy has been reported to increase bone resorption and hematopoiesis and to improve leukocyte function. Here, we present the cases of two patients with osteopetrosis who benefited from either 3 or 6 years of INF-γ therapy that resulted in improved blood counts and no further pathological fractures.
Key Words: Osteopetrosis, Osteoclast, Interferon-γ


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