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Insulin resistance - type A

MedGen UID:
472057
•Concept ID:
C0342336
•
Disease or Syndrome
Synonyms: Insulin Resistant Diabetes Mellitus with Acanthosis Nigricans and Hyperandrogenism; Type A insulin resistance
SNOMED CT: Type A insulin resistance (237651005); Insulin resistance - type A (237651005)

Definition

A syndrome of insulin resistance caused by mutation(s) in the INSR gene, encoding the insulin receptor. This condition is characterized by a clinical triad of hyperinsulinemia, acanthosis nigricans, and hyperandrogenism without lipodystrophy. This is the least severe of a spectrum of disorders; the other two conditions are Rabson-Mendenhall Syndrome and Donohoe Syndrome. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVInsulin resistance - type A
Follow this link to review classifications for Insulin resistance - type A in Orphanet.

Professional guidelines

PubMed

New classification and diagnostic criteria for insulin resistance syndrome.
Ogawa W, Araki E, Ishigaki Y, Hirota Y, Maegawa H, Yamauchi T, Yorifuji T, Katagiri H
Endocr J 2022 Feb 28;69(2):107-113. Epub 2022 Feb 1 doi: 10.1507/endocrj.EJ21-0725. PMID: 35110500
Structural Basis and Genotype-Phenotype Correlations of INSR Mutations Causing Severe Insulin Resistance.
Hosoe J, Kadowaki H, Miya F, Aizu K, Kawamura T, Miyata I, Satomura K, Ito T, Hara K, Tanaka M, Ishiura H, Tsuji S, Suzuki K, Takakura M, Boroevich KA, Tsunoda T, Yamauchi T, Shojima N, Kadowaki T
Diabetes 2017 Oct;66(10):2713-2723. Epub 2017 Aug 1 doi: 10.2337/db17-0301. PMID: 28765322
IGF-I treatment of insulin resistance.
McDonald A, Williams RM, Regan FM, Semple RK, Dunger DB
Eur J Endocrinol 2007 Aug;157 Suppl 1:S51-6. doi: 10.1530/EJE-07-0271. PMID: 17785698

Recent clinical studies

Diagnosis

Syndrome of Congenital Insulin Resistance Caused by a Novel INSR Gene Mutation.
Rojek A, Wikiera B, Noczynska A, Niedziela M
J Clin Res Pediatr Endocrinol 2023 Aug 23;15(3):312-317. Epub 2021 Dec 30 doi: 10.4274/jcrpe.galenos.2021.2021.0256. PMID: 34965699Free PMC Article
Clinical and Functional Characterization of Novel INSR Variants in Two Families With Severe Insulin Resistance Syndrome.
Zhou Q, Yu J, Yuan X, Wang C, Zhu Z, Zhang A, Gu W
Front Endocrinol (Lausanne) 2021;12:606964. Epub 2021 Apr 29 doi: 10.3389/fendo.2021.606964. PMID: 33995269Free PMC Article
Mecasermin in Insulin Receptor-Related Severe Insulin Resistance Syndromes: Case Report and Review of the Literature.
Plamper M, Gohlke B, Schreiner F, Woelfle J
Int J Mol Sci 2018 Apr 24;19(5) doi: 10.3390/ijms19051268. PMID: 29695048Free PMC Article
Insulin resistance Type A and short 5th metacarpals.
Patel VK, Davies HA
Diabet Med 2003 Jun;20(6):500-4. doi: 10.1046/j.1464-5491.2003.00982.x. PMID: 12786688
Receptor binding studies and clinical effects of human insulin (recombinant DNA): studies in patients with newly diagnosed type i diabetes, type II diabetes, insulin resistance (type A and type B), insulin antibodies, insulin allergy, and "brittle" diabetes.
Schlüter KJ, Kerp L
Diabetes Care 1982 Nov-Dec;5 Suppl 2:152-60. doi: 10.2337/diacare.5.2.s152. PMID: 6765528

Therapy

Mecasermin in Insulin Receptor-Related Severe Insulin Resistance Syndromes: Case Report and Review of the Literature.
Plamper M, Gohlke B, Schreiner F, Woelfle J
Int J Mol Sci 2018 Apr 24;19(5) doi: 10.3390/ijms19051268. PMID: 29695048Free PMC Article
Beneficial metabolic effects of insulin-like growth factor I in patients with severe insulin-resistant diabetes type A.
Zenobi PD, Glatz Y, Keller A, Graf S, Jaeggi-Groisman SE, Riesen WF, Schoenle EJ, Froesch ER
Eur J Endocrinol 1994 Sep;131(3):251-7. doi: 10.1530/eje.0.1310251. PMID: 7921209
Receptor binding studies and clinical effects of human insulin (recombinant DNA): studies in patients with newly diagnosed type i diabetes, type II diabetes, insulin resistance (type A and type B), insulin antibodies, insulin allergy, and "brittle" diabetes.
Schlüter KJ, Kerp L
Diabetes Care 1982 Nov-Dec;5 Suppl 2:152-60. doi: 10.2337/diacare.5.2.s152. PMID: 6765528

Prognosis

Clinical and Functional Characterization of Novel INSR Variants in Two Families With Severe Insulin Resistance Syndrome.
Zhou Q, Yu J, Yuan X, Wang C, Zhu Z, Zhang A, Gu W
Front Endocrinol (Lausanne) 2021;12:606964. Epub 2021 Apr 29 doi: 10.3389/fendo.2021.606964. PMID: 33995269Free PMC Article
Mecasermin in Insulin Receptor-Related Severe Insulin Resistance Syndromes: Case Report and Review of the Literature.
Plamper M, Gohlke B, Schreiner F, Woelfle J
Int J Mol Sci 2018 Apr 24;19(5) doi: 10.3390/ijms19051268. PMID: 29695048Free PMC Article

Clinical prediction guides

Syndrome of Congenital Insulin Resistance Caused by a Novel INSR Gene Mutation.
Rojek A, Wikiera B, Noczynska A, Niedziela M
J Clin Res Pediatr Endocrinol 2023 Aug 23;15(3):312-317. Epub 2021 Dec 30 doi: 10.4274/jcrpe.galenos.2021.2021.0256. PMID: 34965699Free PMC Article
Clinical and Functional Characterization of Novel INSR Variants in Two Families With Severe Insulin Resistance Syndrome.
Zhou Q, Yu J, Yuan X, Wang C, Zhu Z, Zhang A, Gu W
Front Endocrinol (Lausanne) 2021;12:606964. Epub 2021 Apr 29 doi: 10.3389/fendo.2021.606964. PMID: 33995269Free PMC Article
Molecular genetics of severe insulin resistance.
Elsas LJ 2nd, Longo N, Langley S, Griffin LD, Shuster RC
Yale J Biol Med 1989 Sep-Oct;62(5):533-47. PMID: 2697987Free PMC Article
Acromegaloid patients with type A insulin resistance: parallel defects in insulin and insulin-like growth factor-I receptors and biological responses in cultured fibroblasts.
Low L, Chernausek SD, Sperling MA
J Clin Endocrinol Metab 1989 Aug;69(2):329-37. doi: 10.1210/jcem-69-2-329. PMID: 2546962
Receptor binding studies and clinical effects of human insulin (recombinant DNA): studies in patients with newly diagnosed type i diabetes, type II diabetes, insulin resistance (type A and type B), insulin antibodies, insulin allergy, and "brittle" diabetes.
Schlüter KJ, Kerp L
Diabetes Care 1982 Nov-Dec;5 Suppl 2:152-60. doi: 10.2337/diacare.5.2.s152. PMID: 6765528

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