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Achlorhydria

MedGen UID:
1714
Concept ID:
C0001075
Pathologic Function
Synonym: Achylia Gastrica
SNOMED CT: Achylia gastrica (20754004); Achlorhydria (47481007); Absent gastric acidity (47481007); Gastric anacidity (47481007)
 
HPO: HP:0032448
Monarch Initiative: MONDO:0043465

Definition

A condition in which production of hydrochloric acid in the stomach is absent. [from HPO]

Term Hierarchy

Conditions with this feature

Mucolipidosis type IV
MedGen UID:
68663
Concept ID:
C0238286
Disease or Syndrome
Mucolipidosis IV (MLIV) is an ultra-rare lysosomal storage disorder characterized by severe psychomotor delay, progressive visual impairment, and achlorhydria. Individuals with MLIV typically present by the end of the first year of life with delayed developmental milestones (due to a developmental brain abnormality) and impaired vision (resulting from a combination of corneal clouding and retinal degeneration). By adolescence, all individuals with MLIV have severe visual impairment. A neurodegenerative component of MLIV has become more widely appreciated, with the majority of individuals demonstrating progressive spastic quadriparesis and loss of psychomotor skills starting in the second decade of life. About 5% of individuals have atypical MLIV, manifesting with less severe psychomotor impairment, but still exhibiting progressive retinal degeneration and achlorhydria.

Professional guidelines

PubMed

Lee L, Ramos-Alvarez I, Ito T, Jensen RT
Int J Mol Sci 2019 Oct 16;20(20) doi: 10.3390/ijms20205128. PMID: 31623145Free PMC Article
Babey M, Brandi ML, Shoback D
Endocrinol Metab Clin North Am 2018 Dec;47(4):889-900. doi: 10.1016/j.ecl.2018.07.012. PMID: 30390820
Neumann WL, Coss E, Rugge M, Genta RM
Nat Rev Gastroenterol Hepatol 2013 Sep;10(9):529-41. Epub 2013 Jun 18 doi: 10.1038/nrgastro.2013.101. PMID: 23774773

Recent clinical studies

Etiology

Koyyada A
Therapie 2021 Jan-Feb;76(1):13-21. Epub 2020 Jul 9 doi: 10.1016/j.therap.2020.06.019. PMID: 32718584
Rodriguez-Castro KI, Franceschi M, Noto A, Miraglia C, Nouvenne A, Leandro G, Meschi T, De' Angelis GL, Di Mario F
Acta Biomed 2018 Dec 17;89(8-S):88-92. doi: 10.23750/abm.v89i8-S.7921. PMID: 30561424Free PMC Article
Neumann WL, Coss E, Rugge M, Genta RM
Nat Rev Gastroenterol Hepatol 2013 Sep;10(9):529-41. Epub 2013 Jun 18 doi: 10.1038/nrgastro.2013.101. PMID: 23774773
O'Leary F, Samman S
Nutrients 2010 Mar;2(3):299-316. Epub 2010 Mar 5 doi: 10.3390/nu2030299. PMID: 22254022Free PMC Article
Straub DA
Nutr Clin Pract 2007 Jun;22(3):286-96. doi: 10.1177/0115426507022003286. PMID: 17507729

Diagnosis

Gluckman CR, Metz DC
Curr Gastroenterol Rep 2019 Mar 12;21(4):13. doi: 10.1007/s11894-019-0684-7. PMID: 30868284
Rodriguez-Castro KI, Franceschi M, Noto A, Miraglia C, Nouvenne A, Leandro G, Meschi T, De' Angelis GL, Di Mario F
Acta Biomed 2018 Dec 17;89(8-S):88-92. doi: 10.23750/abm.v89i8-S.7921. PMID: 30561424Free PMC Article
Li Y, Xia R, Zhang B, Li C
J Environ Pathol Toxicol Oncol 2018;37(3):241-259. doi: 10.1615/JEnvironPatholToxicolOncol.2018026839. PMID: 30317974
Neumann WL, Coss E, Rugge M, Genta RM
Nat Rev Gastroenterol Hepatol 2013 Sep;10(9):529-41. Epub 2013 Jun 18 doi: 10.1038/nrgastro.2013.101. PMID: 23774773
Bures J, Cyrany J, Kohoutova D, Förstl M, Rejchrt S, Kvetina J, Vorisek V, Kopacova M
World J Gastroenterol 2010 Jun 28;16(24):2978-90. doi: 10.3748/wjg.v16.i24.2978. PMID: 20572300Free PMC Article

Therapy

Koyyada A
Therapie 2021 Jan-Feb;76(1):13-21. Epub 2020 Jul 9 doi: 10.1016/j.therap.2020.06.019. PMID: 32718584
Lee L, Ramos-Alvarez I, Ito T, Jensen RT
Int J Mol Sci 2019 Oct 16;20(20) doi: 10.3390/ijms20205128. PMID: 31623145Free PMC Article
Babey M, Brandi ML, Shoback D
Endocrinol Metab Clin North Am 2018 Dec;47(4):889-900. doi: 10.1016/j.ecl.2018.07.012. PMID: 30390820
Krela-Kaźmierczak I, Szymczak A, Łykowska-Szuber L, Eder P, Linke K
Adv Clin Exp Med 2016 Jan-Feb;25(1):185-90. doi: 10.17219/acem/33746. PMID: 26935513
Straub DA
Nutr Clin Pract 2007 Jun;22(3):286-96. doi: 10.1177/0115426507022003286. PMID: 17507729

Prognosis

Gluckman CR, Metz DC
Curr Gastroenterol Rep 2019 Mar 12;21(4):13. doi: 10.1007/s11894-019-0684-7. PMID: 30868284
Krela-Kaźmierczak I, Szymczak A, Łykowska-Szuber L, Eder P, Linke K
Adv Clin Exp Med 2016 Jan-Feb;25(1):185-90. doi: 10.17219/acem/33746. PMID: 26935513
Basuroy R, Srirajaskanthan R, Prachalias A, Quaglia A, Ramage JK
Aliment Pharmacol Ther 2014 May;39(10):1071-84. Epub 2014 Mar 13 doi: 10.1111/apt.12698. PMID: 24628514
Bures J, Cyrany J, Kohoutova D, Förstl M, Rejchrt S, Kvetina J, Vorisek V, Kopacova M
World J Gastroenterol 2010 Jun 28;16(24):2978-90. doi: 10.3748/wjg.v16.i24.2978. PMID: 20572300Free PMC Article
Holt PR
Dig Dis 2007;25(2):144-50. doi: 10.1159/000099479. PMID: 17468550

Clinical prediction guides

Kelly P
Adv Nutr 2021 Jun 1;12(3):897-903. doi: 10.1093/advances/nmaa135. PMID: 33271592Free PMC Article
Lee L, Ramos-Alvarez I, Ito T, Jensen RT
Int J Mol Sci 2019 Oct 16;20(20) doi: 10.3390/ijms20205128. PMID: 31623145Free PMC Article
Chetty M, Johnson TN, Polak S, Salem F, Doki K, Rostami-Hodjegan A
Adv Drug Deliv Rev 2018 Oct;135:85-96. Epub 2018 Sep 4 doi: 10.1016/j.addr.2018.08.013. PMID: 30189273
Krela-Kaźmierczak I, Szymczak A, Łykowska-Szuber L, Eder P, Linke K
Adv Clin Exp Med 2016 Jan-Feb;25(1):185-90. doi: 10.17219/acem/33746. PMID: 26935513
Arnold R, Koop H
Digestion 1989;44 Suppl 1:77-86. doi: 10.1159/000200107. PMID: 2691313

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