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Beta-cell dysfunction

MedGen UID:
370119
Concept ID:
C1969875
Finding
HPO: HP:0006279

Conditions with this feature

Diabetes mellitus, ketosis-prone
MedGen UID:
1381503
Concept ID:
C3837958
Disease or Syndrome
In addition to classic type 1 (see 222100) and type 2 (see 125853) diabetes mellitus, atypical presentations are seen, particularly in populations of African ancestry. Ketosis-prone diabetes, the most common atypical form, is characterized by an acute initial presentation with severe hyperglycemia and ketosis, as seen in classic type 1 diabetes, but after initiation of insulin therapy, prolonged remission is often possible with cessation of insulin therapy and maintenance of appropriate metabolic control. Metabolic studies show a markedly blunted insulin secretory response to glucose, partially reversible with the improvement of blood glucose control. Variable levels of insulin resistance are observed, especially in obese patients. Pancreatic beta-cell autoimmunity is a rare finding, and association with type 1 susceptibility HLA alleles is variable (Sobngwi et al., 2002).

Professional guidelines

PubMed

Wronka M, Krzemińska J, Młynarska E, Rysz J, Franczyk B
Int J Mol Sci 2022 Dec 12;23(24) doi: 10.3390/ijms232415743. PMID: 36555387Free PMC Article
Borse SP, Chhipa AS, Sharma V, Singh DP, Nivsarkar M
Med Princ Pract 2021;30(2):109-121. Epub 2020 Aug 20 doi: 10.1159/000511002. PMID: 32818934Free PMC Article
Kahn SE, Cooper ME, Del Prato S
Lancet 2014 Mar 22;383(9922):1068-83. Epub 2013 Dec 3 doi: 10.1016/S0140-6736(13)62154-6. PMID: 24315620Free PMC Article

Recent clinical studies

Etiology

Alejandro EU, Mamerto TP, Chung G, Villavieja A, Gaus NL, Morgan E, Pineda-Cortel MRB
Int J Mol Sci 2020 Jul 15;21(14) doi: 10.3390/ijms21145003. PMID: 32679915Free PMC Article
Hudish LI, Reusch JE, Sussel L
J Clin Invest 2019 Oct 1;129(10):4001-4008. doi: 10.1172/JCI129188. PMID: 31424428Free PMC Article
Plows JF, Stanley JL, Baker PN, Reynolds CM, Vickers MH
Int J Mol Sci 2018 Oct 26;19(11) doi: 10.3390/ijms19113342. PMID: 30373146Free PMC Article
Yazıcı D, Sezer H
Adv Exp Med Biol 2017;960:277-304. doi: 10.1007/978-3-319-48382-5_12. PMID: 28585204
Mitri J, Pittas AG
Endocrinol Metab Clin North Am 2014 Mar;43(1):205-32. Epub 2013 Dec 12 doi: 10.1016/j.ecl.2013.09.010. PMID: 24582099Free PMC Article

Diagnosis

Alejandro EU, Mamerto TP, Chung G, Villavieja A, Gaus NL, Morgan E, Pineda-Cortel MRB
Int J Mol Sci 2020 Jul 15;21(14) doi: 10.3390/ijms21145003. PMID: 32679915Free PMC Article
Plows JF, Stanley JL, Baker PN, Reynolds CM, Vickers MH
Int J Mol Sci 2018 Oct 26;19(11) doi: 10.3390/ijms19113342. PMID: 30373146Free PMC Article
Chiefari E, Arcidiacono B, Foti D, Brunetti A
J Endocrinol Invest 2017 Sep;40(9):899-909. Epub 2017 Mar 10 doi: 10.1007/s40618-016-0607-5. PMID: 28283913
Mitri J, Pittas AG
Endocrinol Metab Clin North Am 2014 Mar;43(1):205-32. Epub 2013 Dec 12 doi: 10.1016/j.ecl.2013.09.010. PMID: 24582099Free PMC Article
Kahn SE, Cooper ME, Del Prato S
Lancet 2014 Mar 22;383(9922):1068-83. Epub 2013 Dec 3 doi: 10.1016/S0140-6736(13)62154-6. PMID: 24315620Free PMC Article

Therapy

Georgakis MK, Harshfield EL, Malik R, Franceschini N, Langenberg C, Wareham NJ, Markus HS, Dichgans M
Neurology 2021 Mar 30;96(13):e1732-e1742. Epub 2021 Jan 25 doi: 10.1212/WNL.0000000000011555. PMID: 33495378Free PMC Article
Wysham C, Shubrook J
Postgrad Med 2020 Nov;132(8):676-686. Epub 2020 Jun 16 doi: 10.1080/00325481.2020.1771047. PMID: 32543261
Macut D, Bjekić-Macut J, Rahelić D, Doknić M
Diabetes Res Clin Pract 2017 Aug;130:163-170. Epub 2017 Jun 12 doi: 10.1016/j.diabres.2017.06.011. PMID: 28646699
Chiefari E, Arcidiacono B, Foti D, Brunetti A
J Endocrinol Invest 2017 Sep;40(9):899-909. Epub 2017 Mar 10 doi: 10.1007/s40618-016-0607-5. PMID: 28283913
Tabák AG, Herder C, Rathmann W, Brunner EJ, Kivimäki M
Lancet 2012 Jun 16;379(9833):2279-90. Epub 2012 Jun 9 doi: 10.1016/S0140-6736(12)60283-9. PMID: 22683128Free PMC Article

Prognosis

Abel ED, Gloyn AL, Evans-Molina C, Joseph JJ, Misra S, Pajvani UB, Simcox J, Susztak K, Drucker DJ
Cell 2024 Jul 25;187(15):3789-3820. doi: 10.1016/j.cell.2024.06.029. PMID: 39059357Free PMC Article
Xourafa G, Korbmacher M, Roden M
Nat Rev Endocrinol 2024 Jan;20(1):27-49. Epub 2023 Oct 16 doi: 10.1038/s41574-023-00898-1. PMID: 37845351
Georgakis MK, Harshfield EL, Malik R, Franceschini N, Langenberg C, Wareham NJ, Markus HS, Dichgans M
Neurology 2021 Mar 30;96(13):e1732-e1742. Epub 2021 Jan 25 doi: 10.1212/WNL.0000000000011555. PMID: 33495378Free PMC Article
Redondo MJ, Hagopian WA, Oram R, Steck AK, Vehik K, Weedon M, Balasubramanyam A, Dabelea D
Diabetologia 2020 Oct;63(10):2040-2048. Epub 2020 Sep 7 doi: 10.1007/s00125-020-05211-7. PMID: 32894314Free PMC Article
Wang T, Lu J, Shi L, Chen G, Xu M, Xu Y, Su Q, Mu Y, Chen L, Hu R, Tang X, Yu X, Li M, Zhao Z, Chen Y, Yan L, Qin G, Wan Q, Dai M, Zhang D, Gao Z, Wang G, Shen F, Luo Z, Qin Y, Chen L, Huo Y, Li Q, Ye Z, Zhang Y, Liu C, Wang Y, Wu S, Yang T, Deng H, Zhao J, Lai S, Bi Y, DeFronzo RA, Wang W, Ning G; China Cardiometabolic Disease and Cancer Cohort Study Group
Lancet Diabetes Endocrinol 2020 Feb;8(2):115-124. Epub 2019 Dec 23 doi: 10.1016/S2213-8587(19)30425-5. PMID: 31879247

Clinical prediction guides

Georgakis MK, Harshfield EL, Malik R, Franceschini N, Langenberg C, Wareham NJ, Markus HS, Dichgans M
Neurology 2021 Mar 30;96(13):e1732-e1742. Epub 2021 Jan 25 doi: 10.1212/WNL.0000000000011555. PMID: 33495378Free PMC Article
Hudish LI, Reusch JE, Sussel L
J Clin Invest 2019 Oct 1;129(10):4001-4008. doi: 10.1172/JCI129188. PMID: 31424428Free PMC Article
Christensen AA, Gannon M
Curr Diab Rep 2019 Aug 9;19(9):81. doi: 10.1007/s11892-019-1196-4. PMID: 31399863
Esser N, Legrand-Poels S, Piette J, Scheen AJ, Paquot N
Diabetes Res Clin Pract 2014 Aug;105(2):141-50. Epub 2014 Apr 13 doi: 10.1016/j.diabres.2014.04.006. PMID: 24798950
Tabák AG, Herder C, Rathmann W, Brunner EJ, Kivimäki M
Lancet 2012 Jun 16;379(9833):2279-90. Epub 2012 Jun 9 doi: 10.1016/S0140-6736(12)60283-9. PMID: 22683128Free PMC Article

Recent systematic reviews

Hjort A, Iggman D, Rosqvist F
Clin Nutr 2024 Apr;43(4):915-925. Epub 2024 Feb 22 doi: 10.1016/j.clnu.2024.02.014. PMID: 38401227
Yao X, Geng S, Zhu L, Jiang H, Wen J
Chemosphere 2023 Aug;332:138866. Epub 2023 May 8 doi: 10.1016/j.chemosphere.2023.138866. PMID: 37164202
Semnani-Azad Z, Blanco Mejia S, Connelly PW, Bazinet RP, Retnakaran R, Jenkins DJA, Harris SB, Hanley AJ
Obes Rev 2021 Sep;22(9):e13257. Epub 2021 Apr 28 doi: 10.1111/obr.13257. PMID: 33913230
Valaiyapathi B, Gower B, Ashraf AP
Curr Diabetes Rev 2020;16(3):220-229. doi: 10.2174/1573399814666180608074510. PMID: 29879890Free PMC Article
Kramer CK, Zinman B, Retnakaran R
Lancet Diabetes Endocrinol 2013 Sep;1(1):28-34. Epub 2013 Feb 4 doi: 10.1016/S2213-8587(13)70006-8. PMID: 24622264

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