Ann Pediatr Endocrinol Metab.  2019 Dec;24(4):257-261. 10.6065/apem.2019.24.4.257.

Two cases of ketosis-prone diabetes mellitus in Korean adolescents

• Hwang WB
• Kim JH
• Cho SM

• KMID: 2468729
• DOI: http://doi.org/10.6065/apem.2019.24.4.257

Abstract

In recent years, reports of diabetes mellitus (DM) cases that do not fit the traditional classification system have increased in prevalence. While insulin deficiency appears as type 1 DM (T1DM), the new type also has the clinical features of type 2 DM (T2DM); as such, this new type of DM is called ketosis-prone diabetes (KPD) and is correlated with findings of severe hyperglycemia and ketoacidosis. To provide a clear, clinical classification of DM, new classification systems are being studied. Among these, the Aβ system demonstrates the highest sensitivity and specificity in predicting clinical features and prognosis. We report 2 cases of KPD in Korean pediatric patients. The first patient was referred while in a state of diabetic ketoacidosis (DKA) and was considered to have T1DM. However, their blood glucose was well-controlled even with small doses of insulin, and the treatment was able to be changed to metformin therapy. The second patient seemed to be a typical case of T2DM because of his obesity and strong family history. However, blood glucose was not well-controlled with a regular diet, and ketosis occurred. After performing a glucagon stimulation test, both patients showed different clinical features that were finally diagnosed as type A-β+ KPD. The rapid and accurate diagnosis of KPD can reduce the duration of inappropriate insulin use and improve patients' quality of life. Further, the treatment of KPD children should be individualized according to each patient's lifestyle to preventing recurrent DKA.

Keyword

Ketosis-prone diabetes; Child; Korean

MeSH Terms

Adolescent*
Blood Glucose
Child
Classification
Diabetes Mellitus
Diabetes Mellitus, Type 1*
Diabetic Ketoacidosis
Diagnosis
Diet
Glucagon
Humans
Hyperglycemia
Insulin
Ketosis
Life Style
Metformin
Obesity
Prevalence
Prognosis
Quality of Life
Sensitivity and Specificity
Blood Glucose
Glucagon
Insulin
Metformin

Reference

References

1. Maldonado M, Hampe CS, Gaur LK, D'Amico S, Iyer D, Hammerle LP, et al. Ketosis-prone diabetes: dissection of a heterogeneous syndrome using an immunogenetic and beta-cell functional classification, prospective analysis, and clinical outcomes. J Clin Endocrinol Metab. 2003; 88:5090–8.

2. Balasubramanyam A, Nalini R, Hampe CS, Maldonado M. Syndromes of ketosis-prone diabetes mellitus. Endocr Rev. 2008; 29:292–302.

3. Balasubramanyam A, Zern JW, Hyman DJ, Pavlik V. New profiles of diabetic ketoacidosis: type 1 vs type 2 diabetes and the effect of ethnicity. Arch Intern Med. 1999; 159:2317–22.

4. Mauvais-Jarvis F, Sobngwi E, Porcher R, Riveline JP, Kevorkian JP, Vaisse C, et al. Ketosis-prone type 2 diabetes in patients of sub-Saharan African origin: clinical pathophysiology and natural history of beta-cell dysfunction and insulin resistance. Diabetes. 2004; 53:645–53.

5. Yoon B, Kim G, Bae JH, Yun YJ, Lee YH, Lee BW, et al. A case of autoantibody-positive ketosis-prone diabetes mellitus. The Journal of Korean Diabetes. 2016; 17:60–6.

6. Kim MK, Lee SH, Kim JH, Lee JI, Kim JH, Jang EH, et al. Clinical characteristics of Korean patients with new-onset diabetes presenting with diabetic ketoacidosis. Diabetes Res Clin Pract. 2009; 85:e8. –11.

7. Seok H, Jung CH, Kim SW, Lee MJ, Lee WJ, Kim JH, et al. Clinical characteristics and insulin independence of Koreans with new-onset type 2 diabetes presenting with diabetic ketoacidosis. Diabetes Metab Res Rev. 2013; 29:507–13.

8. Yu JS, Jin HJ, Ko JT, Kang HS. A case of severe diabetic ketoacdosis in a child with type 2 diabetes. J Korean Soc Pediatr Endocrinol. 2011; 15:46–50.

9. Dodu SR. Diabetes in the tropics. Br Med J. 1967; 2:747–50.

10. Adadevoh BK. "Temporary diabetes" in adult Nigerians. Trans R Soc Trop Med Hyg. 1968; 62:528–30.

11. Aizawa T, Katakura M, Taguchi N, Kobayashi H, Aoyagi E, Hashizume K, et al. Ketoacidosis-onset noninsulin dependent diabetes in Japanese subjects. Am J Med Sci. 1995; 310:198–201.

12. Tan KC, Mackay IR, Zimmet PZ, Hawkins BR, Lam KS. Metabolic and immunologic features of Chinese patients with atypical diabetes mellitus. Diabetes Care. 2000; 23:335–8.

13. Balasubramanyam A, Garza G, Rodriguez L, Hampe CS, Gaur L, Lernmark A, et al. Accuracy and predictive value of classification schemes for ketosis-prone diabetes. Diabetes Care. 2006; 29:2575–9.

14. Weir GC, Laybutt DR, Kaneto H, Bonner-Weir S, Sharma A. Beta-cell adaptation and decompensation during the progression of diabetes. Diabetes. 2001; 50 Suppl 1:S154–9.

15. Eizirik DL, Korbutt GS, Hellerström C. Prolonged exposure of human pancreatic islets to high glucose concentrations in vitro impairs the beta-cell function. J Clin Invest. 1992; 90:1263–8.

16. Gaba R, Gambhire D, Uy N, Gonzalez EV, Iyer D, Hampe CS, et al. Factors associated with early relapse to insulin dependence in unprovoked A-β+ ketosis-prone diabetes. J Diabetes Complications. 2015; 29:918–22.

17. Smiley D, Chandra P, Umpierrez GE. Update on diagnosis, pathogenesis and management of ketosis-prone Type 2 diabetes mellitus. Diabetes Manag (Lond). 2011; 1:589–600.