Gorham 병으로 진단된 9세 남아에서 파미드로네이트 치료 경험 1례
Pamidronate Treatment in 9-Year-Old Boy Diagnosed with Gorham Disease
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초록
Gorham 병은 골에서의 혈관 증식으로 인한 골기질의 손실을 특징으로 하는 매우 드문 질환이다. 적립된 치료법은 없으며 약물 치료, 수술 요법, 방사선 치료 등이 사용되고 있다. 비스포스포네이트 등의 골흡수억제제는 골용해를 억제하는 효과와 신생혈관 억제 작용이 있어 Gorham 병의 치료에 사용되고 있다. 저자들은 반복적인 혈흉을 보여 시행한 늑골 생검에서 Gorham 병으로 진단된 9세 환아의 증례 1례를 보고하는 바이다. 환아는 파미드로네이트 치료 이후 골병변 진행하지 않고 혈흉도 2년 이상 재발하지 않은 상태로 경과 관찰 중이다.
Abstract
Gorham disease is a rare disorder characterized by proliferation of vascular channels resulting in destruction and resorption of osseous matrix. There is no standard treatment defined for this disease, and variable therapies such as medical, surgical, and radiation therapy have been used. Antiresorptive medication, such as bisphosphonate, is used in Gorham disease because they suppress the course of osteolysis and angiogenisis. We report a 9-year- old boy with Gorham disease, who was presented with recurrent hemothorax and treated by pamidronate. After treatment, he showed no recurrence of hemothorax for more than 2 years.
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