경구 스테로이드 치료와 우유 섭취 제한으로 호전된 폐 혈철 침착증의 증례
A Case of Pulmonary Hemosiderosis that was Dissolved by an Oral Prednisolone and the Milk Avoidance
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저자들은 발열, 호흡 곤란 및 창백함을 보이고, 폐렴 및 중증 패혈증 의심 하에 항생제 투여, 적혈구 수혈, 고용량 스테로이드 및 정맥 면역 글로불린 치료에도 불구하고 악화가 반복된 29개월 환아에 대해 폐 조직 검사 및 기관지 폐포 세척술을 시행하여 폐 혈철 침착증을 진단하였다. 환아에서 우유 및 우유 성분 특이 IgE 항체가 검출되지 않았으나 우유 섭취와의 연관성을 토대로 우유 섭취 제한을 권고한 결과 스테로이드 투여 후 호전된 증상이 더 이상 재발하지 않음을 경험하였고, 이후 우유 특이 IgG 검사상 양성 소견을 확인하였기에 폐 혈철 침착증의 원인으로 Heiner 증후군이 의심되는 증례를 보고하는 바이다.
Abstract
A 29-month-old boy presented with fever, dyspnea, and paleness. He was initially diagnosed with pneumonia and severe sepsis. Although he was treated with intravenous antibiotics and high dose methylprednisolone, dyspnea and paleness recurred two times. Under suspicion of pulmonary hemosiderosis, we performed video-assisted thoracoscopic lung biopsy and bronchoalveolar lavage on him and found hemosiderin-laden macrophages in both specimens. Despite thorough history and laboratory examination, we could not find any pathologic or serologic evidence for primary and secondary causes of pulmonary hemosiderosis except for one that indicating Heiner’s syndrome. After taking oral prednisolone he showed improvement of anemia and dyspnea, which was maintained by milk avoidance. Based on the history and the existence of immunoglobulin G antibodies against milk components, we are considering it as the case of Heiner’s syndrome.
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