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Insulin autoimmune syndrome

MedGen UID:
678528
Concept ID:
C0854359
Disease or Syndrome
Synonyms: Hirata disease; insulin autoimmune hypoglycemia; insulin autoimmune syndrome
SNOMED CT: Insulin autoimmune syndrome (408539000); Hirata disease (408539000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018465
Orphanet: ORPHA411593

Definition

A rare endocrine disease characterised by hyperinsulinaemic hypoglycaemia associated with the presence of autoantibodies to endogenous insulin without previous exposure to exogenous insulin. Patients usually present in adulthood with postprandial, fasting or exercise-induced hypoglycaemia, often with pronounced neuroglycopenic symptoms. Laboratory investigations reveal markedly elevated serum insulin, as well as increased C-peptide and proinsulin. The condition may be associated with other autoimmune diseases, monoclonal gammopathy, and/or recent exposure to certain medications. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVInsulin autoimmune syndrome

Professional guidelines

PubMed

Church DS, Barker P, Burling KA, Shinwari SK, Kennedy C, Smith D, Macfarlane DP, Kernohan A, Stears A, Karamat MA, Whyte K, Narendran P, Halsall DJ, Semple RK
Diabet Med 2023 Nov;40(11):e15194. Epub 2023 Sep 1 doi: 10.1111/dme.15194. PMID: 37562398Free PMC Article
Monteiro SS, Santos TS, Dores J
Ann Endocrinol (Paris) 2023 Feb;84(1):83-85. Epub 2022 Oct 15 doi: 10.1016/j.ando.2022.10.001. PMID: 36252848
Patel M, Shah R, Ramteke-Jadhav S, Patil V, Patel SK, Lila A, Shah N, Bandgar T
Clin Endocrinol (Oxf) 2020 May;92(5):409-420. Epub 2020 Feb 26 doi: 10.1111/cen.14174. PMID: 32064669

Recent clinical studies

Etiology

Capece U, Moffa S, Improta I, Di Giuseppe G, Nista EC, Cefalo CMA, Cinti F, Pontecorvi A, Gasbarrini A, Giaccari A, Mezza T
Nutrients 2022 Dec 21;15(1) doi: 10.3390/nu15010018. PMID: 36615676Free PMC Article
Della Pepa G, Vetrani C, Lupoli R, Massimino E, Lembo E, Riccardi G, Capaldo B
Crit Rev Food Sci Nutr 2022;62(12):3250-3263. Epub 2021 Jan 18 doi: 10.1080/10408398.2020.1864617. PMID: 33455416
Calder GL, Ward GM, Sachithanandan N, MacIsaac RJ
J Clin Endocrinol Metab 2020 Apr 1;105(4) doi: 10.1210/clinem/dgz301. PMID: 32182368
Yuan T, Li J, Li M, Li N, Duan L, Ping F, Li W, Wang L, Zhao W
Clin Ther 2019 May;41(5):920-928. Epub 2019 Apr 13 doi: 10.1016/j.clinthera.2019.03.009. PMID: 30992145
Zeng XX, Tang YL, Hu KX, Wang J, Zhu LY, Liu JY, Xu J
Medicine (Baltimore) 2017 Dec;96(51):e9213. doi: 10.1097/MD.0000000000009213. PMID: 29390469Free PMC Article

Diagnosis

Church DS, Barker P, Burling KA, Shinwari SK, Kennedy C, Smith D, Macfarlane DP, Kernohan A, Stears A, Karamat MA, Whyte K, Narendran P, Halsall DJ, Semple RK
Diabet Med 2023 Nov;40(11):e15194. Epub 2023 Sep 1 doi: 10.1111/dme.15194. PMID: 37562398Free PMC Article
Capece U, Moffa S, Improta I, Di Giuseppe G, Nista EC, Cefalo CMA, Cinti F, Pontecorvi A, Gasbarrini A, Giaccari A, Mezza T
Nutrients 2022 Dec 21;15(1) doi: 10.3390/nu15010018. PMID: 36615676Free PMC Article
Della Pepa G, Vetrani C, Lupoli R, Massimino E, Lembo E, Riccardi G, Capaldo B
Crit Rev Food Sci Nutr 2022;62(12):3250-3263. Epub 2021 Jan 18 doi: 10.1080/10408398.2020.1864617. PMID: 33455416
Boro H, Gupta U, Singh C, Malhotra R, Khadgawat R
Diabetes Metab Syndr 2021 Nov-Dec;15(6):102294. Epub 2021 Sep 20 doi: 10.1016/j.dsx.2021.102294. PMID: 34562871
Yuan T, Li J, Li M, Li N, Duan L, Ping F, Li W, Wang L, Zhao W
Clin Ther 2019 May;41(5):920-928. Epub 2019 Apr 13 doi: 10.1016/j.clinthera.2019.03.009. PMID: 30992145

Therapy

Capece U, Moffa S, Improta I, Di Giuseppe G, Nista EC, Cefalo CMA, Cinti F, Pontecorvi A, Gasbarrini A, Giaccari A, Mezza T
Nutrients 2022 Dec 21;15(1) doi: 10.3390/nu15010018. PMID: 36615676Free PMC Article
Della Pepa G, Vetrani C, Lupoli R, Massimino E, Lembo E, Riccardi G, Capaldo B
Crit Rev Food Sci Nutr 2022;62(12):3250-3263. Epub 2021 Jan 18 doi: 10.1080/10408398.2020.1864617. PMID: 33455416
Boro H, Gupta U, Singh C, Malhotra R, Khadgawat R
Diabetes Metab Syndr 2021 Nov-Dec;15(6):102294. Epub 2021 Sep 20 doi: 10.1016/j.dsx.2021.102294. PMID: 34562871
Calder GL, Ward GM, Sachithanandan N, MacIsaac RJ
J Clin Endocrinol Metab 2020 Apr 1;105(4) doi: 10.1210/clinem/dgz301. PMID: 32182368
Yuan T, Li J, Li M, Li N, Duan L, Ping F, Li W, Wang L, Zhao W
Clin Ther 2019 May;41(5):920-928. Epub 2019 Apr 13 doi: 10.1016/j.clinthera.2019.03.009. PMID: 30992145

Prognosis

Zhang C, Zhang H, Huang W
Endocrine 2023 Apr;80(1):40-46. Epub 2022 Dec 2 doi: 10.1007/s12020-022-03268-5. PMID: 36459334Free PMC Article
Calder GL, Ward GM, Sachithanandan N, MacIsaac RJ
J Clin Endocrinol Metab 2020 Apr 1;105(4) doi: 10.1210/clinem/dgz301. PMID: 32182368
Yuan T, Li J, Li M, Li N, Duan L, Ping F, Li W, Wang L, Zhao W
Clin Ther 2019 May;41(5):920-928. Epub 2019 Apr 13 doi: 10.1016/j.clinthera.2019.03.009. PMID: 30992145
Zeng XX, Tang YL, Hu KX, Wang J, Zhu LY, Liu JY, Xu J
Medicine (Baltimore) 2017 Dec;96(51):e9213. doi: 10.1097/MD.0000000000009213. PMID: 29390469Free PMC Article
Lupsa BC, Chong AY, Cochran EK, Soos MA, Semple RK, Gorden P
Medicine (Baltimore) 2009 May;88(3):141-153. doi: 10.1097/MD.0b013e3181a5b42e. PMID: 19440117

Clinical prediction guides

Oest L, Roden M, Müssig K
Clin Endocrinol (Oxf) 2022 Mar;96(3):328-338. Epub 2021 Nov 14 doi: 10.1111/cen.14634. PMID: 34778997
Calder GL, Ward GM, Sachithanandan N, MacIsaac RJ
J Clin Endocrinol Metab 2020 Apr 1;105(4) doi: 10.1210/clinem/dgz301. PMID: 32182368
Reis MZR, Fernandes VO, Fontenele EGP, Sales APAM, Montenegro Junior RM, Quidute ARP
Arch Endocrinol Metab 2018 Oct;62(5):566-570. doi: 10.20945/2359-3997000000078. PMID: 30462811Free PMC Article
Gullo D, Evans JL, Sortino G, Goldfine ID, Vigneri R
Clin Endocrinol (Oxf) 2014 Aug;81(2):204-9. Epub 2013 Oct 23 doi: 10.1111/cen.12334. PMID: 24111525
Thomas JW
Immunol Res 2001;23(2-3):235-44. doi: 10.1385/IR:23:2-3:235. PMID: 11444388

Recent systematic reviews

Chen S, Qiang J, Zhang Y, Zhao B, Tian R, Yuan T, Li M, Li M, Li Y, Zhu H, Pan H
Front Endocrinol (Lausanne) 2023;14:1091933. Epub 2023 Feb 28 doi: 10.3389/fendo.2023.1091933. PMID: 36926026Free PMC Article
Patel M, Shah R, Ramteke-Jadhav S, Patil V, Patel SK, Lila A, Shah N, Bandgar T
Clin Endocrinol (Oxf) 2020 May;92(5):409-420. Epub 2020 Feb 26 doi: 10.1111/cen.14174. PMID: 32064669

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