Ann Pediatr Endocrinol Metab.  2022 Mar;27(1):73-77. 10.6065/apem.2040250.125.

Hypertriglyceridemia with acute pancreatitis in a 14-year-old girl with diabetic ketoacidosis

• Park H
• Kim MS
• Kim J
• Lee SM
• Cho SY
• Yoo EG
• Jin DK

• KMID: 2528201
• DOI: http://doi.org/10.6065/apem.2040250.125

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 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.

Keyword

Hypertriglyceridemia; Acute pancreatitis; Diabetic ketoacidosis

Figure

• 

  Fig. 1. Contrast-enhanced abdominal computed tomography (CT) imaging. The initial CT scan shows edematous pancreas (arrowhead) and adjacent fluid collection (arrows), suggesting grade D acute pancreatitis. (A) Axial image, (B) coronal image.

Reference

References

1. Edge JA, Hawkins MM, Winter DL, Dunger DB. The risk and outcome of cerebral oedema developing during diabetic ketoacidosis. Arch Dis Child. 2001; 85:16–22.

2. Rawla P, Sunkara T, Thandra KC, Gaduputi V. Hypertrigly-ceridemia-induced pancreatitis: updated review of current treatment and preventive strategies. Clin J Gastroenterol. 2018; 11:441–8.

3. Wolfgram PM, Macdonald MJ. Severe hypertriglyceridemia causing acute pancreatitis in a child with new onset type i diabetes mellitus presenting in ketoacidosis. J Pediatr Intensive Care. 2013; 2:77–80.

4. Wolfsdorf JI, Allgrove J, Craig ME, Edge J, Glaser N, Jain V, et al. ISPAD clinical practice consensus guidelines 2014. Diabetic ketoacidosis and hyperglycemic hyperosmolar state. Pediatr Diabetes. 2014; 15 Suppl 20:154–79.

5. Lutfi R, Huang J, Wong HR. Plasmapheresis to treat hypertriglyceridemia in a child with diabetic ketoacidosis and pancreatitis. Pediatrics. 2012; 129:e195–8.

6. Sharma PK, Kumar M, Yadav DK. Severe hypertriglyceridemia causing pancreatitis in a child with new-onset type-i diabetes mellitus presenting with diabetic ketoacidosis. Indian J Crit Care Med. 2017; 21:176–8.

7. Zaher FZ, Boubagura I, Rafi S, Elmghari G, Elansari N. Diabetic ketoacidosis revealing a severe hypertriglyceridemia and acute pancreatitis in type 1 diabetes mellitus. Case Rep Endocrinol. 2019; 2019:8974619.

8. Singla AA, Ting F, Singla A. Acute pancreatitis secondary to diabetic ketoacidosis induced hypertriglyceridemia in a young adult with undiagnosed type 2 diabetes. JOP. 2015; 16:201–4.

9. Hahn SJ, Park JH, Lee JH, Lee JK, Kim KA. Severe hypertriglyceridemia in diabetic ketoacidosis accompanied by acute pancreatitis: case report. J Korean Med Sci. 2010; 25:1375–8.

10. Yagnik PJ, Desai PH, Modem VM. Hypertriglyceridemia with acute pancreatitis in pediatric diabetic ketoacidosis: a case report. Cureus. 2019; 11:e3844.

11. Martin SS, Blaha MJ, Elshazly MB, Brinton EA, Toth PP, McEvoy JW, et al. Friedewald-estimated versus directly measured low-density lipoprotein cholesterol and treatment implications. J Am Coll Cardiol. 2013; 62:732–9.

12. Umpierrez G, Freire AX. Abdominal pain in patients with hyperglycemic crises. J Crit Care. 2002; 17:63–7.

13. Lin L, Quan H, Chen K, Chen D, Lin D, Fang T. ABCC8-related maturity-onset diabetes of the young (MODY12): a report of a chinese family. Front Endocrinol (Lausanne). 2020; 11:645.

14. Hussain K, Flanagan SE, Smith VV, Ashworth M, Day M, Pierro A, et al. An ABCC8 gene mutation and mosaic uniparental isodisomy resulting in atypical diffuse congenital hyperinsulinism. Diabetes. 2008; 57:259–63.

15. Hattersley AT, Greeley SAW, Polak M, Rubio-Cabezas O, Njølstad PR, Mlynarski W, et al. ISPAD clinical practice consensus guidelines 2018: the diagnosis and management of monogenic diabetes in children and adolescents. Pediatr Diabetes. 2018; 19 Suppl 27:47–63.

16. Kapoor RR, Flanagan SE, James CT, McKiernan J, Thomas AM, Harmer SC, et al. Hyperinsulinaemic hypoglycaemia and diabetes mellitus due to dominant ABCC8/KCNJ11 mutations. Diabetologia. 2011; 54:2575–83.

17. Huopio H, Otonkoski T, Vauhkonen I, Reimann F, Ashcroft FM, Laakso M. A new subtype of autosomal dominant diabetes attributable to a mutation in the gene for sulfonylurea receptor 1. Lancet. 2003; 361:301–7.

18. Cywinski JS, Walker FA, White H, Traisman HS. Juvenile diabetes mellitus associated with acute pancreatitis. Acta Paediatr Scand. 1965; 54:597–602.

19. Slyper AH, Wyatt DT, Brown CW. Clinical and/or biochemical pancreatitis in diabetic ketoacidosis. J Pediatr Endocrinol. 1994; 7:261–4.

20. Aboulhosn K, Arnason T. Acute pancreatitis and severe hypertriglyceridaemia masking unsuspected underlying diabetic ketoacidosis. BMJ Case Rep. 2013; 2013:bcr2013200431.