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Ann Pediatr Endocrinol Metab > Accepted Articles
DOI: https://doi.org/10.6065/apem.2040250.125    [Accepted] Published online May 14, 2021.
Hypertriglyceridemia with acute pancreatitis in a 14-year-old girl with diabetic ketoacidosis
Hyojung Park1  , Min-Sun Kim2  , Jiyeon Kim2  , Sae-Mi Lee3,4  , Sung Yoon Cho2  , Eun-Gyong Yoo5  , Dong-Kyu Jin2 
1Department of Pediatrics, Seongnam Citizens Medical Center, Seongnam, Korea
2Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
3GC Genome, GCLabs, Yongin, Korea
4Department of Laboratory Medicine, Kangwon National University School of Medicine, Chuncheon, Korea
5Department of Pediatrics, College of Medicine, Pochon CHA University, Seongnam, Korea
Address for correspondence:  Sung Yoon Cho
Email: nadri1217@naver.com
Received: November 16, 2020   Revised: January 18, 2021   Accepted: February 1, 2021
Abstract
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 seven-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 normal range after 6 days of admission without anti-lipid medication. Considering not too low C-peptide levels, 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 maturity-onset diabetes of the young (MODY) 12. This is the first reported case of HTG induced AP with DKA in a patient with MODY. In addition, we reviewed the literatures 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 consequence of AP. We recommend considering AP in all DKA patients presenting with severe HTG to ensure early and proper management.
Keywords: Hypertriglyceridemia; Acute pancreatitis; Diabetic ketoacidosis
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