초록
Kwashiorkor는 단백질 결핍으로 인한 영양실조로 저알부민혈증, 설사, 피부염, 부종을 증상으로 나타낸다. 단백질-열량 결핍은 면역결핍을 초래하고 이는 감염에 대한 감수성을 높임으로써 생명을 위협하는 상태가 될 수 있다. 치료는 조기 영양공급, 감염증 치료가 주이며, 치료 경과 중 확장성 심근증이 나타날 수 있으며 이 경우 예후가 안 좋다. Kwashiorkor는 주로 빈곤한 나라에서 생긴다고 알려져 왔으며, 기술적으로 진보한 나라에서 치명적인 결과까지 낳은 예는 보고된 바가 없었다. 치명적인 kwashiorkor 1례를 경험하였기에 보고하는 바이다. 환아는 가난때문이 아니라 부모의 잘못된 생각으로 2개월 동안 곡물에 태운 곡식가루만 먹었으며, 설사, 전신 부종, 홍반, 저체온증 등을 주소로 입원하였다. 면역 결핍이 있는 상태였으며, 혈액 배양 검사상 Alcaligenes Xylosoxidans가 동정되었다. 단백질 보충식이 및 항생제 치료, 정주용 면역글로불린치료, 미세영양소 보충하였으나 확장성 심근병증, 다발성 장기 부전으로 사망하였다.
Kwashiorkor is a syndrome of severe protein malnutrition, which manifests itself in hypoalbuminemia, diarrhea, dermatitis, and edema. It can be life-threatening due to associated immune deficiency and an increased susceptibility to infections. Kwashiorkor should be treated early with nutritional support and the control of infection. Dilated cardiomyopathy may develop during the treatment and in such cases a poor prognosis is expected. Kwashiorkor has been known as a common disease of poor countries. To date, in fact, there has been no report of kwashiorkor leading to death in technically advanced countries. We here report a fatal case of a baby girl admitted with kwashiorkor. She had been fed only with cereal grain mixed with juice, without any protein supplement, for 2 months. This diet was deficient not because of poverty, but due to the illiteracy of her parents. The patient suffered from diarrhea, whole body edema, hypothermia, and dermatitis. Laboratory findings revealed an immune-deficient state featuring leukopenia and decreased immunoglobulin. Blood and urine cultures revealed Alcaligenes Xylosoxidans growth. The patient was fed frequent small amounts of protein-containing formula and intravenous albumin and micronutrients were administered for nutritional support. She was also treated with intravenous immunoglobulin and antibiotics in order to control infection. Nevertheless, she developed dilated cardiomyopathy and multi-organ failure and died. We review this case in light of the literature.