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Diabetes mellitus, ketosis-prone(KPD)

MedGen UID:
1381503
Concept ID:
C3837958
Disease or Syndrome
Synonym: KPD
SNOMED CT: Ketosis-prone diabetes mellitus (890171006)
 
Gene (location): PAX4 (7q32.1)
 
Monarch Initiative: MONDO:0100180
OMIM®: 612227

Definition

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). [from OMIM]

Clinical features

From HPO
Beta-cell dysfunction
MedGen UID:
370119
Concept ID:
C1969875
Finding
Autoimmunity
MedGen UID:
2136
Concept ID:
C0004368
Pathologic Function
The occurrence of an immune reaction against the organism's own cells or tissues.
Diabetes mellitus
MedGen UID:
8350
Concept ID:
C0011849
Disease or Syndrome
A group of abnormalities characterized by hyperglycemia and glucose intolerance.
Insulin resistance
MedGen UID:
43904
Concept ID:
C0021655
Pathologic Function
Increased resistance towards insulin, that is, diminished effectiveness of insulin in reducing blood glucose levels.
Ketoacidosis
MedGen UID:
67434
Concept ID:
C0220982
Disease or Syndrome
Acidosis resulting from accumulation of ketone bodies.

Professional guidelines

PubMed

Patil SP
Prim Care 2022 Jun;49(2):225-237. Epub 2022 Apr 22 doi: 10.1016/j.pop.2021.11.003. PMID: 35595479

Recent clinical studies

Etiology

Akil AA, Yassin E, Al-Maraghi A, Aliyev E, Al-Malki K, Fakhro KA
J Transl Med 2021 Apr 1;19(1):137. doi: 10.1186/s12967-021-02778-6. PMID: 33794915Free PMC Article
Lu X, Zhao C
Adv Exp Med Biol 2020;1228:107-121. doi: 10.1007/978-981-15-1792-1_7. PMID: 32342453
Maahs DM, West NA, Lawrence JM, Mayer-Davis EJ
Endocrinol Metab Clin North Am 2010 Sep;39(3):481-97. doi: 10.1016/j.ecl.2010.05.011. PMID: 20723815Free PMC Article
Bluestone JA, Herold K, Eisenbarth G
Nature 2010 Apr 29;464(7293):1293-300. doi: 10.1038/nature08933. PMID: 20432533Free PMC Article
Gillespie KM
CMAJ 2006 Jul 18;175(2):165-70. doi: 10.1503/cmaj.060244. PMID: 16847277Free PMC Article

Diagnosis

Calimag APP, Chlebek S, Lerma EV, Chaiban JT
Dis Mon 2023 Mar;69(3):101418. Epub 2022 May 14 doi: 10.1016/j.disamonth.2022.101418. PMID: 35577617
Akil AA, Yassin E, Al-Maraghi A, Aliyev E, Al-Malki K, Fakhro KA
J Transl Med 2021 Apr 1;19(1):137. doi: 10.1186/s12967-021-02778-6. PMID: 33794915Free PMC Article
DiMeglio LA, Evans-Molina C, Oram RA
Lancet 2018 Jun 16;391(10138):2449-2462. doi: 10.1016/S0140-6736(18)31320-5. PMID: 29916386Free PMC Article
Li W, Huang E, Gao S
J Alzheimers Dis 2017;57(1):29-36. doi: 10.3233/JAD-161250. PMID: 28222533
Atkinson MA, Eisenbarth GS, Michels AW
Lancet 2014 Jan 4;383(9911):69-82. Epub 2013 Jul 26 doi: 10.1016/S0140-6736(13)60591-7. PMID: 23890997Free PMC Article

Therapy

Antar SA, Ashour NA, Sharaky M, Khattab M, Ashour NA, Zaid RT, Roh EJ, Elkamhawy A, Al-Karmalawy AA
Biomed Pharmacother 2023 Dec;168:115734. Epub 2023 Oct 17 doi: 10.1016/j.biopha.2023.115734. PMID: 37857245
van der Feltz-Cornelis C, Allen SF, Holt RIG, Roberts R, Nouwen A, Sartorius N
Brain Behav 2021 Feb;11(2):e01981. Epub 2020 Dec 4 doi: 10.1002/brb3.1981. PMID: 33274609Free PMC Article
Chellappan DK, Sivam NS, Teoh KX, Leong WP, Fui TZ, Chooi K, Khoo N, Yi FJ, Chellian J, Cheng LL, Dahiya R, Gupta G, Singhvi G, Nammi S, Hansbro PM, Dua K
Biomed Pharmacother 2018 Dec;108:1188-1200. Epub 2018 Oct 2 doi: 10.1016/j.biopha.2018.09.138. PMID: 30372820
Shapiro AM, Pokrywczynska M, Ricordi C
Nat Rev Endocrinol 2017 May;13(5):268-277. Epub 2016 Nov 11 doi: 10.1038/nrendo.2016.178. PMID: 27834384
van Belle TL, Coppieters KT, von Herrath MG
Physiol Rev 2011 Jan;91(1):79-118. doi: 10.1152/physrev.00003.2010. PMID: 21248163

Prognosis

Lambrinoudaki I, Paschou SA, Armeni E, Goulis DG
Nat Rev Endocrinol 2022 Oct;18(10):608-622. Epub 2022 Jul 7 doi: 10.1038/s41574-022-00708-0. PMID: 35798847
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
Desai S, Deshmukh A
Curr Diabetes Rev 2020;16(5):438-441. doi: 10.2174/1573399815666191004112647. PMID: 31584373
Maahs DM, West NA, Lawrence JM, Mayer-Davis EJ
Endocrinol Metab Clin North Am 2010 Sep;39(3):481-97. doi: 10.1016/j.ecl.2010.05.011. PMID: 20723815Free PMC Article
Atkinson MA, Eisenbarth GS
Lancet 2001 Jul 21;358(9277):221-9. doi: 10.1016/S0140-6736(01)05415-0. PMID: 11476858

Clinical prediction guides

Redondo MJ, Gignoux CR, Dabelea D, Hagopian WA, Onengut-Gumuscu S, Oram RA, Rich SS
Lancet Diabetes Endocrinol 2022 Aug;10(8):597-608. Epub 2022 Jun 17 doi: 10.1016/S2213-8587(22)00159-0. PMID: 35724677Free 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
Balasubramanyam A
Trans Am Clin Climatol Assoc 2019;130:145-155. PMID: 31516178Free PMC Article
Agashe S, Petak S
Methodist Debakey Cardiovasc J 2018 Oct-Dec;14(4):251-256. doi: 10.14797/mdcj-14-4-251. PMID: 30788010Free PMC Article
Khoury J, Zohar Y, Shehadeh N, Saadi T
Hepatobiliary Pancreat Dis Int 2018 Apr;17(2):113-118. Epub 2018 Feb 19 doi: 10.1016/j.hbpd.2018.02.006. PMID: 29709217

Recent systematic reviews

Rahmati M, Keshvari M, Mirnasuri S, Yon DK, Lee SW, Il Shin J, Smith L
J Med Virol 2022 Nov;94(11):5112-5127. Epub 2022 Jul 22 doi: 10.1002/jmv.27996. PMID: 35831242Free PMC Article
Winkley K, Upsher R, Stahl D, Pollard D, Kasera A, Brennan A, Heller S, Ismail K
Health Technol Assess 2020 Jun;24(28):1-232. doi: 10.3310/hta24280. PMID: 32568666Free PMC Article
Kelkar A, Kelkar J, Mehta H, Amoaku W
Indian J Ophthalmol 2018 Oct;66(10):1401-1410. doi: 10.4103/ijo.IJO_1158_17. PMID: 30249823Free PMC Article
Turton JL, Raab R, Rooney KB
PLoS One 2018;13(3):e0194987. Epub 2018 Mar 29 doi: 10.1371/journal.pone.0194987. PMID: 29596460Free PMC Article
Li W, Huang E, Gao S
J Alzheimers Dis 2017;57(1):29-36. doi: 10.3233/JAD-161250. PMID: 28222533

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