Ketosis-prone type 2 diabetes mellitus (review of the literature)
https://doi.org/10.14341/2072-0351-5456
Abstract
Idiopathic ketosis-prone type 2 diabetes is intermediate between two major forms of diabetes mellitus. The aim of this review is to analyse factors contributingto its development and occurrence and to discuss approaches to differential diagnosis of this pathology.
About the Authors
Natalya Alexandrovna Molitvoslovova,
Endocrinological Research Centre, Moscow
Tatiana Vasil'evna Nikonova
Endocrinological Research Centre, Moscow
References
1. Winter W.E., Maclaren N.K., Riley W.J., Clarke D.W., Kappy M.S., Spillar R.P. Maturity-onset diabetes of youth in black Americans //N. Engl. J. Med. 1987; Р. 316: P. 285-291.
2. Umpierrez G.E., Casals M.M., Gebhart S.P., Mixon P.S., Clark W.S., Phillips L.S. Diabetic ketoacidosis in obese African-Americans //Diabetes. 1995;44: Р. 790-795.
3. Umpierrez G.E., Woo W., Hagopian W.A., Isaacs S.D., Palmer J.P., Gaur L.K., et al. Immunogenetic analysis suggests different pathogenesis for obese and lean African-Americans with diabetic ketoacidosis //Diabetes Care. 1999;22: Р.1517-1523.
4. Banerji M.A., Chaiken R.L., Huey H., Tuomi T., Norin A.J., Mackay I.R., et al. GAD antibody negative NIDDM in adult black subjects with diabetic ketoacidosis and increased frequency of human leukocyte antigen DR3 and DR4. Flatbush diabetes. Diabetes. 1994;43: Р. 741-745.
5. McFarlane S.I., Chaiken R.L., Hirsch S., Harrington P., Lebovitz H.E., Banerji M.A. Near-normoglycaemic remission in African-Americans with Type 2 diabetes mellitus is associated with recovery of beta cell function //Diabet Med. 2001;18: Р. 10-16.
6. Mauvais-Jarvis F., Sobngwi E., Porcher R., Riveline J.P., Kevorkian J.P., Vaisse C., et al. Ketosis-prone type 2 diabetes in patients of sub-Saharan African origin: Р. clinical pathophysiology and natural history of beta-cell dysfunction and insulin resistance //Diabetes. 2004;53: Р. 645-653.
7. Sobngwi E., Vexiau P., Levy V., Lepage V., Mauvais-Jarvis F., Leblanc H., et al. Metabolic and immunogenetic prediction of long-term insulin remission in African patients with atypical diabetes //Diabet Med. 2002;19: Р. 832-835.
8. Banerji M.A., Chaiken R.L., Lebovitz H.E. Long-term normoglycemic remission in black newly diagnosed NIDDM subjects //Diabetes. 1996; 45: Р. 337-341.
9. Gill G.V., Mbanya J.C., Ramaiya K.L., Tesfaye S. Diabetes in Africa: Р. epidemiology, management and healthcare challenges //Heart. 2008; 94(11): Р. 1376-1382.
10. Wilson C., Krakoff J., Gohdes D. Ketoacidosis in Apache Indians with non-insulin- dependent diabetes mellitus //Arch Intern Med. 1997;157: Р. 2098-2100.
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: Р. 198-201.
12. Nagasaka S., Ishikawa S., Itabashi N., Rokkaku K., Saito T.. Ketoacidosisonset type 2 diabetes in Japanese. Association with the widespread distribution of soft drinks and vending machines [Letter] //Diabetes Care. 1998;21: Р. 1376-1378.
13. Tan K.C., Mackay I.R., Zimmet P.Z., Hawkins B.R., Lam K.S. Metabolic and immunologic features of Chinese patients with atypical diabetes mellitus //Diabetes Care. 2000;23: Р. 335-338.
14. Yan S.H., Sheu W.H., Song Y.M., Tseng L.N. The occurrence of diabetic ketoacidosis in adults //Intern Med. 2000;39: Р. 10-14.
15. Hsin Yu .E., Guo H.R., Wu T.J. Factors associated with discontinuing insulin therapy after diabetic ketoacidosis in adult diabetic patients //Diabet Med. 2001;18: Р. 895-899.
16. Maldonado M., Hampe C.S., Gaur L.K., D'Amico S., Iyer D., Hammerle L.P., et al. Ketosis-prone diabetes: Р. dissection of a heterogeneous syndrome using an immunogenetic and beta-cell functional classification, prospective analysis, and clinical outcomes //J. Clin. Endocrinol Metab. 2003;88: Р. 5090-5098.
17. Pi-ero-Pilo-a A., Litonjua P., Aviles-Santa L., Raskin P. Idiopathic type 1 diabetes in Dallas, Texas: Р. a 5-year experience //Diabetes Care. 2001;24: Р. 1014-1018.
18. Maldonado M.R., Otiniano M.E., Lee R., Rodriguez L., Balasubramanyam A. Characteristics of ketosis-prone diabetes in a multiethnic indigent community //Ethn Dis. 2004;14: Р. 243-249.
19. Sobngwi E., Gautier J.F. Adult-onset idiopathic Type I or ketosis-prone Type II diabetes: Р. evidence to revisit diabetes classification //Diabetologia. 2002;45(2): Р. 283-285.
20. Sobngwi E., Mauvais-Jarvis F., Vexiau P., Mbanya J.C., Gautier J.F.. Diabetes in Africans. Part 2: Р. Ketosis-prone atypical diabetes mellitus //Diabetes Metab. 2002;28: Р. 5-12.
21. i-ero-Pilo-a A., Raskin P. Idiopathic Type 1 diabetes //J. Diabetes Complications. 2001; 15: Р. 328-335.
22. Dodu S.R. Diabetes in the tropics //Br. Med. J. 1967;2: Р. 747-750.
23. Oli J.M. Remittant diabetes mellitus in Nigeria //Trop Geogr Med. 1978;30: Р. 57-62.
24. Adadevoh B.K. «Temporary diabetes» in adult Nigerians //Trans R. Soc Trop. Med. Hyg. 1968; 62: Р. 528-530.
25. Ducorps M., Ndong W., Jupkwo B., Belmejdoub G., Thiolet C., Mayaudon H., et al. [Diabetes in Cameroon. Classification difficulties in Africa] //Med Trop (Mars). 1996; 56: Р. 264-270.
26. Umpierrez G.E., Kelly J.P., Navarrete J.E., Casals M.M., Kitabchi A.E. Hyperglycemic crises in urban blacks //Arch Intern Med. 1997; 157: Р. 669-675.
27. Banerji M.A., Lebovitz H.E. Remission in non-insulin-dependent diabetes mellitus: Р. clinical characteristics of remission and relapse in black patients. Medicine (Baltimore). 1990;69: Р. 176-185.
28. Umpierrez G.E., Clark W.S., Steen M.T. Sulfonylurea treatment prevents recurrence of hyperglycemia in obese African-American patients with a history of hyperglycemic crises. Diabetes Care. 1997; 20: Р. 479-483.
29. Kobayashi T., Itoh T., Kosaka K., Sato K., Tsuji K. Time course of islet cell antibodies and beta-cell function in non-insulin-dependent stage of type I diabetes //Diabetes. 1987; 36: Р. 510-517.
30. Kobayashi T., Nakanishi K., Murase T., Kosaka K. Small doses of subcutaneous insulin as a strategy for preventing slowly progressive beta-cell failure in islet cell antibody-positive patients with clinical features of NIDDM //Diabetes. 1996; 45: Р. 622-626.
31. Schiel R., Muller U.A. GAD autoantibodies in a selection-free population of insulin-treated diabetic patients: Р. indicator of a high prevalence of LADA? //Diabetes Res. Clin. Pract. 2000; 49: Р. 33-40.
32. Tuomi T., Groop L.C., Zimmet P.Z., Rowley M.J., Knowles W., Mackay I.R. Antibodies to glutamic acid decarboxylase reveal latent autoimmune diabetes mellitus in adults with a non-insulin-dependent onset of disease //Diabetes. 1993; 42: Р. 359-362.
33. Tuomi T., Carlsson A., Li H., Isomaa B., Miettinen A., Nilsson A., et al. Clinical and genetic characteristics of type 2 diabetes with and without GAD antibodies //Diabetes. 1999; 48: Р. 150-157.
34. Pozzilli P., Di Mario U. Autoimmune diabetes not requiring insulin at diagnosis (latent autoimmune diabetes of the adult): Р. definition, characterization, and potential prevention //Diabetes Care. 2001; 24: Р.1460-1467.
35. Groop L., Bottazzo G.F., Doniach D. Islet cell antibodies identify latent type I diabetes in patients aged 35-75 years at diagnosis //Diabetes. 1986; 35: Р. 237-41.
36. Borg H., Gottsater A., Fernlund P., Sundkvist G. A 12-year prospective study of the relationship between islet antibodies and beta-cell function at and after the diagnosis in patients with adult-onset diabetes. Diabetes. 2002; 51: Р. 1754-1762.
37. Sobngwi E., Choukem S.P., Agbalika F., Blondeau B., Fetita L.S., Lebbe C., Thiam D., Cattan P., Larghero J., Foufelle F., Ferre P., Vexiau P., Calvo F., Gautier J.F. Ketosis- prone type 2 Diabetes Mellitus and Human Herpes Virus 8 Infection in Sub- Sahara Africans //JAMA. 2008;299(23): Р. 2770-2776.
38. Balasubramanyam A., Nalini R., Hampe S.C., Maldonado M. Syndromes of Ketosis- Prone Diabetes Melitus //Endocr.Rev.2008;29(3):Р. 292-302.
39. Ramaswami N., Lakshmi K.G., Maldonado M., Hampe S.C, Rodriguez L., Garza G., Lernmark A., Balasubramanyam A. HLA Class II Alleles Specify Phenotypes of Ketosis-Prone Diabetes //Diabetes Care. 2008; 31(6): Р. 1195-1200.
40. Boutin P., Gresh L., Cisse A., Hara M., Bell G., Babu S., et al. Missense mutation Gly574Ser in the transcription factor HNF-1alpha is a marker of atypical diabetes mellitus in African-American children //Diabetologia. 1999; 42: Р. 380-381.
41. Mauvais-Jarvis F., Boudou P., Sobngwi E., Riveline J.P., Kevorkian J.P., Villette J.M., et al. The polymorphism Gly574Ser in the transcription factor HNF-1alpha is not a marker of adult-onset ketosis-prone atypical diabetes in Afro-Caribbean patients //Diabetologia. 2003;46: Р. 728-729.
42. Shimajiri Y., Sanke T., Furuta H., Hanabusa T., Nakagawa T., Fujitani Y., et al. A missense mutation of Pax4 gene (R121W) is associated with type 2 diabetes in Japanese //Diabetes. 2001; 50: Р. 2864-2869.
43. Sosa-Pineda B., Chowdhury K., Torres M., Oliver G., Gruss P. The Pax4 gene is essential for differentiation of insulin-producing beta cells in the mammalian pancreas //Nature. 1997; 386: Р. 399-402.
44. Mauvais-Jarvis F., Smith S.B., Le May C., Leal S.M., Gautier J.F., Molokhia M, et al. PAX4 gene variations predispose to ketosis-prone diabetes //Hum. Mol. Genet. 2004; 13: Р. 3151-3159.
45. Maldonado M.R., Otiniano M.E., Lee R., Rodriguez L., Balasubramanyam A. Ethnic differences in beta-cell functional reserve and clinical features in patients with ketosis-prone diabetes //Diabetes Care. 2003; 26: Р. 2469.
46. Rasouli N., Elbein S.C. Improved glycemic control in subjects with atypical diabetes results from restored insulin secretion, but not improved insulin sensitivity //J.Clin. Endocrinol. Metab. 2004; 89: Р. 6331-6335.
47. Pitteloud N., Philippe J. Characteristics of Caucasian type 2 diabetic patients during ketoacidosis and at follow-up //Schweiz Med Wochenschr. 2000; 130: Р.576-582.
48. Ramos-Roman A.M., Pinero-Pilona A., Beverley A-H., Raskin P. Comparison of type 1, type 2, and atypical ketosis-prone diabetes at 4 years of diabetes duration //J. of Diab. and its Complic. 2006; 20: Р. 137-144.
49. Robertson R.P. Chronic oxidative stress as a central mechanism for glucose toxity in pancreatic islet beta cells in diabetes //J. of Biol. Chemistry. 2004; 279: Р. 42351-42354.
50. Sobngwi E., Gautier J.F, Kevorkian J.P., Villette J.M., Riveline J.P., Zhang S., Vexiau P., Leal S.M., Vaisse C., Mauvais-Jarvis F. High prevalence of glucose-6- phosphate dehydrogenase deficiency without gene mutation suggests a novel genetic mechanism predisposing to ketosis-prone diabetes //J. of Clin. Endocrinol. Metab. 2005;90: Р. 4446-4451.
51. Gosmanov A.R., Umpierrez G.E., Cuervo R., Karabell A.H., Thomason D.B. Beta cell function and muscle insulin signaling in obese African Americans with atypical diabetes //Diabetes. 2003; 52: Р. 287.
52. Stride A., Hattersley A.T. Different genes, different diabetes: Р. lessons from maturity- onset diabetes of the young //Ann. Med. 2002; 34: Р. 207-216.
53. Leahy J.L., Bonner-Weir S., Weir G.C. Beta-cell dysfunction induced by chronic hyperglycemia. Current ideas on mechanism of impaired glucose-induced insulin secretion //Diabetes Care. 1992; 15: Р. 442-455.
54. Rossetti L., Giaccari A., DeFronzo R.A. Glucose toxicity //Diabetes Care . 1990;13: Р. 610-630.
55. Yki-Jдrvinen H. Glucose toxicity //Endocr. Rev. 1992; 13: Р. 415-431.
56. Kahn S.E. Clinical review 135: Р. the importance of beta-cell failure in the development and progression of type 2 diabetes //J. Clin. Endocrinol. Metab. 2001; 86: Р. 4047-4058.
57. Porte D.Jr., Kahn S.E. Beta-cell dysfunction and failure in type 2 diabetes: Р. potential mechanisms //Diabetes. 2001; 50: Р. 160-163.
58. Volinic J.L., Lee J.H., Eto K., Kaur V., Thomas M.K. Overexpression of the coactivator bridge-1 results in insulin deficiency and diabetes //Mol. Endocrinol. 2006; 20: Р. 167-182.
59. Melloul D., Marshak S., Cerasi E. Regulation of pdx-1 gene expression //Diabetes. 2002; 51: Р. 320-325.
60. Harmon J.S., Gleason C.E., Tanaka Y., Oseid E.A., Hunter-Berger K.K., Robertson R.P. In vivo prevention of hyperglycemia also prevents glucotoxic effects on PDX-1 and insulin gene expression //Diabetes. 1999; 48: Р. 1995-2000.
61. McKinnon C.M., Docherty K. Pancreatic duodenal homeobox-1, PDX-1, a major regulator of beta cell identity and function //Diabetologia. 2001; 44: Р. 1203-1214.
62. Robertson R.P., Olson L.K., Zhang H.J. Differentiating glucose toxicity from glucose desensitization: Р. a new message from the insulin gene //Diabetes. 1994;43: Р. 1085-1089.
63. Osman A.A., Pendergrass M., Koval J., Maezono K., Cusi K., Pratipanawatr T., et al. Regulation of MAP kinase pathway activity in vivo in human skeletal muscle //Am. J. Physiol. Endocrino.l Metab. 2000; 278: Р. 992-999.
64. Cefalu W.T. Insulin resistance: Р. cellular and clinical concepts //Exp Biol Med (Maywood). 2001; 226: Р. 13-26.
65. Gosmanov A.R., Umpierrez G.E., Karabell A.H., Cuervo R., Thomason D.B. Impaired expression and insulin-stimulated phosphorylation of Akt-2 in muscle of obese patients with atypical diabetes. Am. J. Physiol. Endocrinol. Metab. 2004; 287: Р. 8-15.
66. Pierre Choukem S.P., Sobngwi E., Fetita L.S., Boudou P., De Kerviler E., Boirie Y., Hainault I., Vexiau P., Mauvais-Jarvis F., Calvo F., Gautier J.F. Multitissue Insulin Resistance Despite Near-Normoglycemic Remission in Africans with Ketosis-Prone Diabetes //Diabetes Care. 2008;31(12): Р. 2332-2337.
67. Kitabchi A.E., Umpierrez G.E., Murphy M.B., Barrett E.J., Kreisberg R.A., Malone J.I., et al. Hyperglycemic crises in patients with diabetes mellitus //Diabetes Care. 2003; 26: Р. 109- 117.
68. Umpierrez E.G, Smiley D., Kitabchi E.A. Narrative Review: Р. Ketosis-Prone Type 2 Diabetes Mellitus //Ann. Intern. Med. 2006;144: Р. 350-357.
69. Aguilera E., Casamitjana R., Ercilla G., Oriola J., Gomis R., Conget I. Adultonset atypical (type 1) diabetes: Р. additional insights and differences with type 1A diabetes in a European Mediterranean population //Diabetes Care. 2004; 27: Р. 1108-1114.
70. Fajans S.S. Scope and heterogeneous nature of MODY. Diabetes Care. 1990; 13: Р. 49-64.
71. Fajans S.S., Bell G.I., Polonsky K.S. Molecular mechanisms and clinical pathophysiology of maturity-onset diabetes of the young //N. Engl. J. Med. 2001; 345: Р. 971-980.
72. Winter W.E., Silverstein J.H. Molecular and genetic bases for maturity onset diabetes of youth //Curr. Opin. Pediatr. 2000; 12: Р. 388-393.
73. Froguel P., Velho G. Molecular genetics of maturity-onset diabetes of the young //Trends Endocrinol Metab. 1999; 10: Р. 142-146.
74. Frayling T.M., Evans J.C., Bulman M.P., Pearson E., Allen L., Owen K., et al. Beta-cell genes and diabetes: Р. molecular and clinical characterization of mutations in transcription factors //Diabetes. 2001; 50: Р. 94-100.
75. Barman K.K., Premalatha G., Mohan V. Tropical chronic pancreatitis //Postgrad Med. J. 2003; 79: Р. 606-615.
76. Mohan V., Premalatha G., Pitchumoni C.S. Tropical chronic pancreatitis: Р. an update //J. Clin. Gastroenterol. 2003; 36: Р. 337-346.
77. Imagawa A., Hanafusa T. Fulminant Type 1 Diabetes Mellitus //Endopcrine Journal. 2006; 53(5): Р. 577-584.
78. Cho Y.M., Kim J.T., Ko K.S., Koo B.K., Yang S.W., Park M.H., Lee H.K., Park K.S. Fulminant type 1 diabetes in Korea: Р. high prevalence among patients with adult-onset type 1 diabetes //Diabetologia. 2007;50: Р. 2276-2279.
79. Moreau C., Drui D., Arnault-Quary G., Charbonnel B., Chaillous L., Cariou B. Fulminant type 1 diabetes in Caucasians: Р. A report of three cases. Diabetes Metab. 2008;34(5): Р. 529-532.
80. Imagawa A., Hanafusa T., Uchigata Y., Kanatsuka A., Kawasaki E., Kobayashi T., Shimada A., Shimizu I., Maruyama T., Makino H. Different contribution of class II HLA in fulminant and autoimmune type 1 diabetes mellitus //Diabetologia. 2008;51: Р. 524-526.
81. Nakamura T., Nagasaka S., Kusaka I., Yatagai T., Yang J., Ishibashi S. HLADR- DQ haplotype in rapid-onset type 1 diabetes in Japanese //Diabetes Care. 2003;26: Р. 1640-1641.
82. Hermann R., Knip M., Veijola R. et al. Temporal changes in the frequencies of HLA genotypes in patients with type 1 diabetes- indication of an increased invironmental pressure? //Diabetologia. 2003;46: Р. 420-425.
Review
For citations:
Molitvoslovova, N.A., Nikonova T.V. Ketosis-prone type 2 diabetes mellitus (review of the literature). Diabetes mellitus. 2009;12(3):65-69. (In Russ.) https://doi.org/10.14341/2072-0351-5456
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