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Reduced pancreatic beta cells

MedGen UID:
341644
Concept ID:
C1856904
Finding
HPO: HP:0006274

Definition

Reduced number of beta cells in the pancreatic islets of Langerhans. [from HPO]

Term Hierarchy

Conditions with this feature

Wolcott-Rallison dysplasia
MedGen UID:
140926
Concept ID:
C0432217
Disease or Syndrome
Wolcott-Rallison syndrome is a rare autosomal recessive disorder characterized by permanent neonatal or early infancy insulin-dependent diabetes. Epiphyseal dysplasia, osteoporosis, and growth retardation develop at a later age. Other frequent multisystem manifestations include hepatic and renal dysfunction, mental retardation, and cardiovascular abnormalities (summary by Delepine et al., 2000).

Professional guidelines

PubMed

Mikłosz A, Chabowski A
J Clin Endocrinol Metab 2023 Jul 14;108(8):1889-1897. doi: 10.1210/clinem/dgad142. PMID: 36916961Free PMC Article
Baska A, Leis K, Gałązka P
Endocr Metab Immune Disord Drug Targets 2021;21(8):1379-1386. doi: 10.2174/1568026620666201022144405. PMID: 33092516
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

Forlenza GP, McVean J, Beck RW, Bauza C, Bailey R, Buckingham B, DiMeglio LA, Sherr JL, Clements M, Neyman A, Evans-Molina C, Sims EK, Messer LH, Ekhlaspour L, McDonough R, Van Name M, Rojas D, Beasley S, DuBose S, Kollman C, Moran A; CLVer Study Group
JAMA 2023 Mar 28;329(12):990-999. doi: 10.1001/jama.2023.2064. PMID: 36826844Free PMC Article
Zhao MM, Lu J, Li S, Wang H, Cao X, Li Q, Shi TT, Matsunaga K, Chen C, Huang H, Izumi T, Yang JK
Nat Commun 2021 Sep 23;12(1):5616. doi: 10.1038/s41467-021-25952-2. PMID: 34556670Free PMC Article
Tricò D, Moriconi D, Berta R, Baldi S, Quinones-Galvan A, Guiducci L, Taddei S, Mari A, Nannipieri M
Nutrients 2021 Apr 18;13(4) doi: 10.3390/nu13041345. PMID: 33919503Free 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
Katsarou A, Gudbjörnsdottir S, Rawshani A, Dabelea D, Bonifacio E, Anderson BJ, Jacobsen LM, Schatz DA, Lernmark Å
Nat Rev Dis Primers 2017 Mar 30;3:17016. doi: 10.1038/nrdp.2017.16. PMID: 28358037

Diagnosis

Forlenza GP, McVean J, Beck RW, Bauza C, Bailey R, Buckingham B, DiMeglio LA, Sherr JL, Clements M, Neyman A, Evans-Molina C, Sims EK, Messer LH, Ekhlaspour L, McDonough R, Van Name M, Rojas D, Beasley S, DuBose S, Kollman C, Moran A; CLVer Study Group
JAMA 2023 Mar 28;329(12):990-999. doi: 10.1001/jama.2023.2064. PMID: 36826844Free PMC Article
Baska A, Leis K, Gałązka P
Endocr Metab Immune Disord Drug Targets 2021;21(8):1379-1386. doi: 10.2174/1568026620666201022144405. PMID: 33092516
Wysham C, Shubrook J
Postgrad Med 2020 Nov;132(8):676-686. Epub 2020 Jun 16 doi: 10.1080/00325481.2020.1771047. PMID: 32543261
Katsarou A, Gudbjörnsdottir S, Rawshani A, Dabelea D, Bonifacio E, Anderson BJ, Jacobsen LM, Schatz DA, Lernmark Å
Nat Rev Dis Primers 2017 Mar 30;3:17016. doi: 10.1038/nrdp.2017.16. PMID: 28358037
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

Forlenza GP, McVean J, Beck RW, Bauza C, Bailey R, Buckingham B, DiMeglio LA, Sherr JL, Clements M, Neyman A, Evans-Molina C, Sims EK, Messer LH, Ekhlaspour L, McDonough R, Van Name M, Rojas D, Beasley S, DuBose S, Kollman C, Moran A; CLVer Study Group
JAMA 2023 Mar 28;329(12):990-999. doi: 10.1001/jama.2023.2064. PMID: 36826844Free PMC Article
Yong J, Johnson JD, Arvan P, Han J, Kaufman RJ
Nat Rev Endocrinol 2021 Aug;17(8):455-467. Epub 2021 Jun 23 doi: 10.1038/s41574-021-00510-4. PMID: 34163039Free PMC Article
Powers AC
J Clin Invest 2021 Apr 15;131(8) doi: 10.1172/JCI142242. PMID: 33759815Free PMC Article
Baska A, Leis K, Gałązka P
Endocr Metab Immune Disord Drug Targets 2021;21(8):1379-1386. doi: 10.2174/1568026620666201022144405. PMID: 33092516
Katsarou A, Gudbjörnsdottir S, Rawshani A, Dabelea D, Bonifacio E, Anderson BJ, Jacobsen LM, Schatz DA, Lernmark Å
Nat Rev Dis Primers 2017 Mar 30;3:17016. doi: 10.1038/nrdp.2017.16. PMID: 28358037

Prognosis

Walker JT, Saunders DC, Rai V, Chen HH, Orchard P, Dai C, Pettway YD, Hopkirk AL, Reihsmann CV, Tao Y, Fan S, Shrestha S, Varshney A, Petty LE, Wright JJ, Ventresca C, Agarwala S, Aramandla R, Poffenberger G, Jenkins R, Mei S, Hart NJ, Phillips S, Kang H, Greiner DL, Shultz LD, Bottino R, Liu J, Below JE; HPAP Consortium, Parker SCJ, Powers AC, Brissova M
Nature 2023 Dec;624(7992):621-629. Epub 2023 Dec 4 doi: 10.1038/s41586-023-06693-2. PMID: 38049589
Su L, Chen Y, Huang C, Wu S, Wang X, Zhao X, Xu Q, Sun R, Kong X, Jiang X, Qiu X, Huang X, Wang M, Wong PP
Sci Transl Med 2023 Jan 11;15(678):eabl7895. doi: 10.1126/scitranslmed.abl7895. PMID: 36630483
Zhao X, Wang M, Wen Z, Lu Z, Cui L, Fu C, Xue H, Liu Y, Zhang Y
Front Endocrinol (Lausanne) 2021;12:721135. Epub 2021 Aug 23 doi: 10.3389/fendo.2021.721135. PMID: 34497589Free PMC Article
Powers AC
J Clin Invest 2021 Apr 15;131(8) doi: 10.1172/JCI142242. PMID: 33759815Free PMC Article
Katsarou A, Gudbjörnsdottir S, Rawshani A, Dabelea D, Bonifacio E, Anderson BJ, Jacobsen LM, Schatz DA, Lernmark Å
Nat Rev Dis Primers 2017 Mar 30;3:17016. doi: 10.1038/nrdp.2017.16. PMID: 28358037

Clinical prediction guides

Puri S, Maachi H, Nair G, Russ HA, Chen R, Pulimeno P, Cutts Z, Ntranos V, Hebrok M
Nat Commun 2024 Jan 18;15(1):588. doi: 10.1038/s41467-023-44384-8. PMID: 38238288Free PMC Article
Murakami T, Inagaki N, Kondoh H
Front Endocrinol (Lausanne) 2022;13:869414. Epub 2022 Mar 31 doi: 10.3389/fendo.2022.869414. PMID: 35432205Free PMC Article
Zhao MM, Lu J, Li S, Wang H, Cao X, Li Q, Shi TT, Matsunaga K, Chen C, Huang H, Izumi T, Yang JK
Nat Commun 2021 Sep 23;12(1):5616. doi: 10.1038/s41467-021-25952-2. PMID: 34556670Free PMC Article
Hallakou-Bozec S, Vial G, Kergoat M, Fouqueray P, Bolze S, Borel AL, Fontaine E, Moller DE
Diabetes Obes Metab 2021 Mar;23(3):664-673. Epub 2020 Dec 29 doi: 10.1111/dom.14277. PMID: 33269554Free PMC Article
Cheng CW, Villani V, Buono R, Wei M, Kumar S, Yilmaz OH, Cohen P, Sneddon JB, Perin L, Longo VD
Cell 2017 Feb 23;168(5):775-788.e12. doi: 10.1016/j.cell.2017.01.040. PMID: 28235195Free PMC Article

Recent systematic reviews

Gandhi GR, Hillary VE, Antony PJ, Zhong LLD, Yogesh D, Krishnakumar NM, Ceasar SA, Gan RY
Crit Rev Food Sci Nutr 2024;64(19):6526-6545. Epub 2023 Jan 28 doi: 10.1080/10408398.2023.2170320. PMID: 36708221
Mori Y, Matsui T, Hirano T, Yamagishi SI
Int J Mol Sci 2020 Feb 22;21(4) doi: 10.3390/ijms21041509. PMID: 32098413Free PMC Article
Loureiro LMR, Reis CEG, da Costa THM
Int J Sport Nutr Exerc Metab 2018 May 1;28(3):284-293. Epub 2018 Jun 12 doi: 10.1123/ijsnem.2017-0342. PMID: 29345166

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