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논문 기본 정보

자료유형
학술저널
저자정보
Park Hyojung (Department of Pediatrics Seongnam Citizens Medical Center Seongnam Korea) Kim Min-Sun (Department of Pediatrics Samsung Medical Center Sungkyunkwan University School of Medicine) Kim Jiyeon (Department of Pediatrics Samsung Medical Center Sungkyunkwan University School of Medicine) Lee Sae-Mi (GC Genome GCLabs Yongin Korea Kangwon National University School of MedicineChuncheon Korea) Cho Sung Yoon (Department of Pediatrics Samsung Medical Center Sungkyunkwan University School of Medicine) Yoo Eun-Gyong (Department of Pediatrics College of Medicine Pochon CHA University Seongnam Korea) Jin Dong-Kyu (Department of Pediatrics Samsung Medical Center Sungkyunkwan University School of Medicine)
저널정보
대한소아내분비학회 Annals of Pediatirc Endocrinology & Metabolism Annals of Pediatric Endocrinology & Metabolism 제27권 제1호
발행연도
2022.3
수록면
73 - 77 (5page)
DOI
10.6065/apem.2040250.125

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Diabetic ketoacidosis (DKA) is a medically fatal condition in poorly controlled hyperglycemia or newly diagnosed diabetes mellitus. Severe hypertriglyceridemia (HTG) is an uncommon complication of DKA and can be associated with acute pancreatitis (AP). We present the clinical manifestations, laboratory findings, and management of AP associated with HTG in a 14-year-old girl with DKA. The patient, with a 7-year history of type 2 diabetes presented with epigastric pain, 1 month after stopping insulin injection. DKA, severe HTG, and AP were diagnosed based on the laboratory and imaging tests. She recovered from DKA after conventional treatment for DKA, and her triglyceride (TG) level was reduced from 10,867 mg/dL to the normal range after 7 days of admission without antilipid medication. Given that her C-peptide level was not too low and considering her negative diabetes-related antibodies and high TG level, targeted gene panel sequencing was performed on the genes associated with diabetes and HTG. We identified a heterozygous mutation, c.4607C>T (p. Ala1537Val), in ABCC8 related to maturityonset diabetes of the young (MODY) 12. To our knowledge, this is the first reported case of HTG-induced AP with DKA in a patient with MODY. In addition, we reviewed the literature for pediatric cases of HTG with DKA. In patients with DKA, timely awareness of severe HTG related to insulin deficiency is crucial for improving the consequences of AP. We recommend considering AP in all DKA patients presenting with severe HTG to ensure early and proper management.

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