Sturge-Weber 증후군 환아에서 발생한 영양실조성 전격성 제 1형 당뇨병
Malnutrition-related Fulminant Type 1 Diabetes in a Patient with Sturge-Weber syndrome
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초록
전격성 당뇨병은 특발성 제 1형 당뇨병의 아형으로 1주 이내의 급격히 발생한 증상을 보이며, 제 1형 당뇨병과 관련된자가항체가 없는 것이 특징이다. 일본에서 처음 보고된 이래 국내에서는 성인에서 몇몇 보고된 예가 있으나, 소아에서는 2예가 보고되었을 뿐이다. 영양실조성 당뇨병은 과거 열대지역의 젊은 연령층에서 보고되었고, 저체중 및 케톤산증을 동반하지 않는 고혈당을 보이는 것이 특징이며, 영양불균형과 관련되어 있다. 저자들은 Sturge-Weber 증후군을가진 15세 여아에서 장기간의 영양불균형 상태에서 전격적으로 발생한 '영양실조성 전격성 당뇨병'이 발병한 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.
Abstract
Fulminant type 1 diabetes is a subtype of idiopathic type 1 diabetes and is characterized by a short duration of symptom onset and an absence of anti-islet autoantibodies. It has not been common since first reported in Japan, and only two childhood cases are reported in Korea. Malnutrition-related diabetes is associated with malnutrition and characterized by low body weight and hyperglycemia without ketoacidosis. Here we report a case of malnutrition-related fulminant diabetes in a 15-year-old girl with Sturge-Weber syndrome.
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