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Diabetic ketoacidosis

MedGen UID:
8351
Concept ID:
C0011880
Disease or Syndrome
Synonyms: Acidoses, Diabetic; Acidosis, Diabetic; Diabetic Acidoses; Diabetic Acidosis; Diabetic Ketoacidoses; Diabetic Ketoacidosis; Diabetic Ketoacidosis, DKA; Diabetic Ketoses; Diabetic Ketosis; DKA Diabetic Ketoacidoses; DKA Diabetic Ketoacidosis; Ketoacidoses, Diabetic; Ketoacidosis, Diabetic; Ketoacidosis, DKA Diabetic; Ketoses, Diabetic; Ketosis, Diabetic
SNOMED CT: Diabetic ketoacidosis (420422005); Ketoacidosis in diabetes mellitus (420422005); Diabetes mellitus with ketoacidosis (420422005); Diabetic acidosis (420422005); DKA - diabetic ketoacidosis (420422005); Ketoacidosis due to diabetes mellitus (420422005)
 
HPO: HP:0001953
Monarch Initiative: MONDO:0012819

Definition

A type of diabetic metabolic abnormality with an accumulation of ketone bodies. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDiabetic ketoacidosis

Conditions with this feature

Rabson-Mendenhall syndrome
MedGen UID:
78783
Concept ID:
C0271695
Disease or Syndrome
INSR-related severe syndromic insulin resistance comprises a phenotypic spectrum that is a continuum from the severe phenotype Donohue syndrome (DS) (also known as leprechaunism) to the milder phenotype Rabson-Mendenhall syndrome (RMS). DS at the severe end of the spectrum is characterized by severe insulin resistance (hyperinsulinemia with associated fasting hypoglycemia and postprandial hyperglycemia), severe prenatal growth restriction and postnatal growth failure, hypotonia and developmental delay, characteristic facies, and organomegaly involving heart, kidneys, liver, spleen, and ovaries. Death usually occurs before age one year. RMS at the milder end of the spectrum is characterized by severe insulin resistance that, although not as severe as that of DS, is nonetheless accompanied by fluctuations in blood glucose levels, diabetic ketoacidosis, and – in the second decade – microvascular complications. Findings can range from severe growth delay and intellectual disability to normal growth and development. Facial features can be milder than those of DS. Complications of longstanding hyperglycemia are the most common cause of death. While death usually occurs in the second decade, some affected individuals live longer.
Friedreich ataxia 2
MedGen UID:
356134
Concept ID:
C1865981
Disease or Syndrome
Friedreich ataxia (FRDA) is an autosomal recessive neurodegenerative disorder characterized by progressive gait and limb ataxia with associated limb muscle weakness, absent lower limb reflexes, extensor plantar responses, dysarthria, and decreased vibratory sense and proprioception. Onset is usually in the first or second decade, before the end of puberty (summary by Delatycki et al., 2000). For a general phenotypic description of Friedreich ataxia (FRDA), see FRDA1 (229300), which is caused by mutation in the FXN gene (606829) on chromosome 9q13.
Maturity-onset diabetes of the young type 10
MedGen UID:
461967
Concept ID:
C3150617
Disease or Syndrome
RCAD is associated with a combination of diabetes and kidney or urinary tract abnormalities (unrelated to the elevated blood glucose), most commonly fluid-filled sacs (cysts) in the kidneys. However, the signs and symptoms are variable, even within families, and not everyone with RCAD has both features. Affected individuals may have other features unrelated to diabetes, such as abnormalities of the pancreas or liver or a form of arthritis called gout.\n\nGCK-MODY is a very mild type of the condition. People with this type have slightly elevated blood glucose levels, particularly in the morning before eating (fasting blood glucose). However, affected individuals often have no symptoms related to the disorder, and diabetes-related complications are extremely rare.\n\nHNF1A-MODY and HNF4A-MODY have similar signs and symptoms that develop slowly over time. Early signs and symptoms in these types are caused by high blood glucose and may include frequent urination (polyuria), excessive thirst (polydipsia), fatigue, blurred vision, weight loss, and recurrent skin infections. Over time uncontrolled high blood glucose can damage small blood vessels in the eyes and kidneys. Damage to the light-sensitive tissue at the back of the eye (the retina) causes a condition known as diabetic retinopathy that can lead to vision loss and eventual blindness. Kidney damage (diabetic nephropathy) can lead to kidney failure and end-stage renal disease (ESRD). While these two types of MODY are very similar, certain features are particular to each type. For example, babies with HNF4A-MODY tend to weigh more than average or have abnormally low blood glucose at birth, even though other signs of the condition do not occur until childhood or young adulthood. People with HNF1A-MODY have a higher-than-average risk of developing noncancerous (benign) liver tumors known as hepatocellular adenomas.\n\nThe different types of MODY are distinguished by their genetic causes. The most common types are HNF1A-MODY (also known as MODY3), accounting for 50 to 70 percent of cases, and GCK-MODY (MODY2), accounting for 30 to 50 percent of cases. Less frequent types include HNF4A-MODY (MODY1) and renal cysts and diabetes (RCAD) syndrome (also known as HNF1B-MODY or MODY5), which each account for 5 to 10 percent of cases. At least ten other types have been identified, and these are very rare.\n\nMaturity-onset diabetes of the young (MODY) is a group of several conditions characterized by abnormally high levels of blood glucose, also called blood sugar. These forms of diabetes typically begin before age 30, although they can occur later in life. In MODY, elevated blood glucose arises from reduced production of insulin, which is a hormone produced in the pancreas that helps regulate blood glucose levels. Specifically, insulin controls how much glucose (a type of sugar) is passed from the blood into cells, where it is used as an energy source.
CIDEC-related familial partial lipodystrophy
MedGen UID:
815270
Concept ID:
C3808940
Disease or Syndrome
A rare genetic lipodystrophy with characteristics of abnormal subcutaneous fat distribution, resulting in preservation of visceral, neck and axillary fat and absence of lower limb and gluteofemoral subcutaneous fat. Additional clinical features are acanthosis nigricans, insulin-resistant type II diabetes mellitus, dyslipidaemia, and hypertension, leading to pancreatitis, hepatomegaly and hepatic steatosis.
Diabetes mellitus, permanent neonatal 4
MedGen UID:
1711191
Concept ID:
C5394307
Disease or Syndrome
Permanent neonatal diabetes mellitus-4 (PNDM4) is characterized by chronic hyperglycemia due to severe nonautoimmune insulin deficiency diagnosed in the first months of life (summary by Polak et al., 2008). For a discussion of genetic heterogeneity of permanent neonatal diabetes mellitus, see PNDM1 (606176).

Professional guidelines

PubMed

Glaser N, Fritsch M, Priyambada L, Rewers A, Cherubini V, Estrada S, Wolfsdorf JI, Codner E
Pediatr Diabetes 2022 Nov;23(7):835-856. doi: 10.1111/pedi.13406. PMID: 36250645
Dhatariya KK; Joint British Diabetes Societies for Inpatient Care
Diabet Med 2022 Jun;39(6):e14788. Epub 2022 Feb 27 doi: 10.1111/dme.14788. PMID: 35224769
Fayfman M, Pasquel FJ, Umpierrez GE
Med Clin North Am 2017 May;101(3):587-606. doi: 10.1016/j.mcna.2016.12.011. PMID: 28372715Free PMC Article

Recent clinical studies

Etiology

Syed FZ
Ann Intern Med 2022 Mar;175(3):ITC33-ITC48. Epub 2022 Mar 8 doi: 10.7326/AITC202203150. PMID: 35254878
Dhatariya KK; Joint British Diabetes Societies for Inpatient Care
Diabet Med 2022 Jun;39(6):e14788. Epub 2022 Feb 27 doi: 10.1111/dme.14788. PMID: 35224769
Musso G, Saba F, Cassader M, Gambino R
BMJ 2020 Nov 12;371:m4147. doi: 10.1136/bmj.m4147. PMID: 33184044
Dhatariya KK, Glaser NS, Codner E, Umpierrez GE
Nat Rev Dis Primers 2020 May 14;6(1):40. doi: 10.1038/s41572-020-0165-1. PMID: 32409703
Karslioglu French E, Donihi AC, Korytkowski MT
BMJ 2019 May 29;365:l1114. doi: 10.1136/bmj.l1114. PMID: 31142480

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
Glaser N, Fritsch M, Priyambada L, Rewers A, Cherubini V, Estrada S, Wolfsdorf JI, Codner E
Pediatr Diabetes 2022 Nov;23(7):835-856. doi: 10.1111/pedi.13406. PMID: 36250645
Long B, Lentz S, Koyfman A, Gottlieb M
Am J Emerg Med 2021 Jun;44:157-160. Epub 2021 Feb 16 doi: 10.1016/j.ajem.2021.02.015. PMID: 33626481
Evans K
Clin Med (Lond) 2019 Sep;19(5):396-398. doi: 10.7861/clinmed.2019-0284. PMID: 31530688Free PMC Article
Modi A, Agrawal A, Morgan F
Curr Diabetes Rev 2017;13(3):315-321. doi: 10.2174/1573399812666160421121307. PMID: 27097605

Therapy

Barski L, Golbets E, Jotkowitz A, Schwarzfuchs D
Eur J Intern Med 2023 Nov;117:38-44. Epub 2023 Jul 5 doi: 10.1016/j.ejim.2023.07.005. PMID: 37419787
Pollack R, Cahn A
Heart Fail Clin 2022 Oct;18(4):635-643. doi: 10.1016/j.hfc.2022.03.002. PMID: 36216492
Qiu M, Ding LL, Zhang M, Zhou HR
Diab Vasc Dis Res 2021 Mar-Apr;18(2):14791641211011016. doi: 10.1177/14791641211011016. PMID: 33887983Free PMC Article
Self WH, Evans CS, Jenkins CA, Brown RM, Casey JD, Collins SP, Coston TD, Felbinger M, Flemmons LN, Hellervik SM, Lindsell CJ, Liu D, McCoin NS, Niswender KD, Slovis CM, Stollings JL, Wang L, Rice TW, Semler MW; Pragmatic Critical Care Research Group
JAMA Netw Open 2020 Nov 2;3(11):e2024596. doi: 10.1001/jamanetworkopen.2020.24596. PMID: 33196806Free PMC Article
Long B, Willis GC, Lentz S, Koyfman A, Gottlieb M
J Emerg Med 2020 Sep;59(3):371-383. Epub 2020 Aug 4 doi: 10.1016/j.jemermed.2020.06.059. PMID: 32763063

Prognosis

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
Fayfman M, Pasquel FJ, Umpierrez GE
Med Clin North Am 2017 May;101(3):587-606. doi: 10.1016/j.mcna.2016.12.011. PMID: 28372715Free PMC Article
Goldenberg RM, Berard LD, Cheng AYY, Gilbert JD, Verma S, Woo VC, Yale JF
Clin Ther 2016 Dec;38(12):2654-2664.e1. doi: 10.1016/j.clinthera.2016.11.002. PMID: 28003053
Nyenwe EA, Kitabchi AE
Metabolism 2016 Apr;65(4):507-21. Epub 2015 Dec 19 doi: 10.1016/j.metabol.2015.12.007. PMID: 26975543
Pasquel FJ, Umpierrez GE
Diabetes Care 2014 Nov;37(11):3124-31. doi: 10.2337/dc14-0984. PMID: 25342831Free PMC Article

Clinical prediction guides

Griffey RT, Schneider RM, Girardi M, Yeary J, McCammon C, Frawley L, Ancona R, Cruz-Bravo P
Acad Emerg Med 2023 Aug;30(8):800-808. Epub 2023 Feb 27 doi: 10.1111/acem.14685. PMID: 36775281
Khunti K, Del Prato S, Mathieu C, Kahn SE, Gabbay RA, Buse JB
Diabetes Care 2021 Dec;44(12):2645-2655. Epub 2021 Oct 8 doi: 10.2337/dc21-1318. PMID: 34625431Free PMC Article
Kuppermann N, Ghetti S, Schunk JE, Stoner MJ, Rewers A, McManemy JK, Myers SR, Nigrovic LE, Garro A, Brown KM, Quayle KS, Trainor JL, Tzimenatos L, Bennett JE, DePiero AD, Kwok MY, Perry CS 3rd, Olsen CS, Casper TC, Dean JM, Glaser NS; PECARN DKA FLUID Study Group
N Engl J Med 2018 Jun 14;378(24):2275-2287. doi: 10.1056/NEJMoa1716816. PMID: 29897851Free PMC Article
Fazeli Farsani S, Brodovicz K, Soleymanlou N, Marquard J, Wissinger E, Maiese BA
BMJ Open 2017 Aug 1;7(7):e016587. doi: 10.1136/bmjopen-2017-016587. PMID: 28765134Free PMC Article
Qiu H, Novikov A, Vallon V
Diabetes Metab Res Rev 2017 Jul;33(5) Epub 2017 Feb 23 doi: 10.1002/dmrr.2886. PMID: 28099783

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
Hamud AA, Mudawi K, Shamekh A, Kadri A, Powell C, Abdelgadir I
Arch Dis Child 2022 Nov;107(11):1023-1028. Epub 2022 Jun 23 doi: 10.1136/archdischild-2022-324042. PMID: 35738870
Fazeli Farsani S, Brodovicz K, Soleymanlou N, Marquard J, Wissinger E, Maiese BA
BMJ Open 2017 Aug 1;7(7):e016587. doi: 10.1136/bmjopen-2017-016587. PMID: 28765134Free PMC Article
Li W, Huang E, Gao S
J Alzheimers Dis 2017;57(1):29-36. doi: 10.3233/JAD-161250. PMID: 28222533
Burke KR, Schumacher CA, Harpe SE
Pharmacotherapy 2017 Feb;37(2):187-194. Epub 2017 Jan 16 doi: 10.1002/phar.1881. PMID: 27931088

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