Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Long-term recombinant interferon-γ treatment in 2 cases of osteopetrosis

장기간 인터페론 감마로 치료한 골화석증 2례

  • Kim, Dong-Yun (Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School) ;
  • Han, Dong-Kyun (Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School) ;
  • Baek, Hee-Jo (Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School) ;
  • Jung, Sung-Taek (Department of Orthopedic Surgery, Chonnam National University Hwasun Hospital, Chonnam National University Medical School Chonnam) ;
  • Kook, Hoon (Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School) ;
  • Hwang, Tai-Ju (Department of Pediatrics, Chonnam National University Hwasun Hospital, Chonnam National University Medical School)
  • 김동연 (전남대학교 의과대학, 화순전남대병원 소아과학교실) ;
  • 한동균 (전남대학교 의과대학, 화순전남대병원 소아과학교실) ;
  • 백희조 (전남대학교 의과대학, 화순전남대병원 소아과학교실) ;
  • 정성택 (전남대학교 의과대학, 화순전남대병원 정형외과학교실) ;
  • 국훈 (전남대학교 의과대학, 화순전남대병원 소아과학교실) ;
  • Received : 2007.08.17
  • Accepted : 2007.10.12
  • Published : 2007.11.15
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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)-${\gamma}$ have been attempted in patients with milder clinical types. In addition, IFN-${\gamma}$ 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-${\gamma}$ therapy that resulted in improved blood counts and no further pathological fractures.

골화석증은 뼈파괴세포의 기능의 이상과 백혈구의 과산화물 생성 이상을 특징으로 하는 드문 골경화성 질환으로, 유전방법이나 발병나이, 증상, 증상의 정도 등에 따라 몇 가지 아형으로 분류된다. 악성형 골화석증의 궁극적 치료 방법은 조혈모세포이식이지만, 조직형 일치 공여자가 없는 경우나 비교적 양성인 만성형 골화석증에서는 스테로이드나 carcitriol, 인터페론 감마 등의 치료를 시도해 볼 수 있다. 특히 인터페론 감마 치료는 골흡수를 증가시키고, 조혈 기능과 백혈구 기능을 향상시킨다고 보고되고 있다. 본 저자들은 약 6년, 3년 동안 인터페론 감마 치료 후 각각 수혈, 골절과 같은 다른 합병증 없이 조절되고 있는 골화석증 2례에 대해 보고하는 바이다.

Keywords

References

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