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Hyperchloremic acidosis

MedGen UID:
43207
Concept ID:
C0085569
Disease or Syndrome
Synonyms: Metabolic acidosis, NAG, acidifying salts; Metabolic acidosis, normal anion gap, acidifying salts
SNOMED CT: Metabolic acidosis, normal anion gap, acidifying salts (18104000); Metabolic acidosis, NAG, acidifying salts (18104000); Hyperchloremic acidosis (18104000)
 
HPO: HP:0001995

Definition

Acidosis (pH less than 7.35) that develops with an increase in ionic chloride. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHyperchloremic acidosis

Conditions with this feature

Proximal renal tubular acidosis
MedGen UID:
82804
Concept ID:
C0268435
Disease or Syndrome
A type of renal tubular acidosis characterized by a failure of the proximal tubular cells to reabsorb bicarbonate, leading to urinary bicarbonate wasting and subsequent acidemia.
Pseudohypoaldosteronism type 2A
MedGen UID:
327088
Concept ID:
C1840389
Disease or Syndrome
Pseudohypoaldosteronism type II (PHAII) is characterized by hyperkalemia despite normal glomerular filtration rate (GFR) and frequently by hypertension. Other associated findings in both children and adults include hyperchloremia, metabolic acidosis, and suppressed plasma renin levels. Aldosterone levels are variable, but are relatively low given the degree of hyperkalemia (elevated serum potassium is a potent stimulus for aldosterone secretion). Hypercalciuria is well described.
Autosomal recessive proximal renal tubular acidosis
MedGen UID:
370883
Concept ID:
C1970309
Disease or Syndrome
A rare autosomal recessive form of proximal renal tubular acidosis characterized by an isolated defect in the proximal tubule leading to the decreased reabsorption of bicarbonate and consequentially to urinary bicarbonate wastage. Presentation is typically with hyperchloremic acidosis, usually occurring in childhood. Extrarenal manifestations include ocular abnormalities (band keratopathy, glaucoma, and cataracts), intellectual disability and severe growth retardation. Other features like dental enamel defects, basal ganglia calcification and pancreatitis are sometimes present.

Professional guidelines

PubMed

Haines RW, Kirwan CJ, Prowle JR
Semin Nephrol 2019 Sep;39(5):473-483. doi: 10.1016/j.semnephrol.2019.06.007. PMID: 31514911
Chuang CL
Contrib Nephrol 2016;187:84-93. Epub 2016 Feb 8 doi: 10.1159/000442368. PMID: 26882226
Schindler AW, Marx G
Curr Opin Anaesthesiol 2016 Apr;29(2):158-65. doi: 10.1097/ACO.0000000000000303. PMID: 26784351

Recent clinical studies

Etiology

Monnet X, Lai C, Teboul JL
Crit Care 2023 Mar 24;27(1):123. doi: 10.1186/s13054-023-04363-3. PMID: 36964573Free PMC Article
Rewers A, Kuppermann N, Stoner MJ, Garro A, Bennett JE, Quayle KS, Schunk JE, Myers SR, McManemy JK, Nigrovic LE, Trainor JL, Tzimenatos L, Kwok MY, Brown KM, Olsen CS, Casper TC, Ghetti S, Glaser NS; Pediatric Emergency Care Applied Research Network (PECARN) FLUID Study Group
Diabetes Care 2021 Sep;44(9):2061-2068. Epub 2021 Jun 29 doi: 10.2337/dc20-3113. PMID: 34187840Free PMC Article
Myles PS, Andrews S, Nicholson J, Lobo DN, Mythen M
World J Surg 2017 Oct;41(10):2457-2463. doi: 10.1007/s00268-017-4055-y. PMID: 28484814
Chuang CL
Contrib Nephrol 2016;187:84-93. Epub 2016 Feb 8 doi: 10.1159/000442368. PMID: 26882226
Barker ME
J Trauma Nurs 2015 Mar-Apr;22(2):111-6. doi: 10.1097/JTN.0000000000000115. PMID: 25768968

Diagnosis

Murugesan A, Cs S
Indian J Pediatr 2023 Feb;90(2):187-189. Epub 2022 Dec 9 doi: 10.1007/s12098-022-04402-x. PMID: 36482234
Tanemoto M, Kamachi R, Kimura T, Yamada S, Yokoyama T, Okazaki Y
Clin Exp Nephrol 2020 Dec;24(12):1140-1143. Epub 2020 Aug 9 doi: 10.1007/s10157-020-01949-x. PMID: 32772208
Toyonaga Y, Kikura M
Nephrology (Carlton) 2017 Sep;22(9):720-727. doi: 10.1111/nep.12840. PMID: 27312130
Schindler AW, Marx G
Curr Opin Anaesthesiol 2016 Apr;29(2):158-65. doi: 10.1097/ACO.0000000000000303. PMID: 26784351
Wrenn K
Ann Emerg Med 1990 Nov;19(11):1310-3. doi: 10.1016/s0196-0644(05)82292-9. PMID: 2240729

Therapy

Monnet X, Lai C, Teboul JL
Crit Care 2023 Mar 24;27(1):123. doi: 10.1186/s13054-023-04363-3. PMID: 36964573Free PMC Article
Rewers A, Kuppermann N, Stoner MJ, Garro A, Bennett JE, Quayle KS, Schunk JE, Myers SR, McManemy JK, Nigrovic LE, Trainor JL, Tzimenatos L, Kwok MY, Brown KM, Olsen CS, Casper TC, Ghetti S, Glaser NS; Pediatric Emergency Care Applied Research Network (PECARN) FLUID Study Group
Diabetes Care 2021 Sep;44(9):2061-2068. Epub 2021 Jun 29 doi: 10.2337/dc20-3113. PMID: 34187840Free PMC Article
Hoorn EJ
J Nephrol 2017 Aug;30(4):485-492. Epub 2016 Nov 29 doi: 10.1007/s40620-016-0363-9. PMID: 27900717Free PMC Article
Chuang CL
Contrib Nephrol 2016;187:84-93. Epub 2016 Feb 8 doi: 10.1159/000442368. PMID: 26882226
Schindler AW, Marx G
Curr Opin Anaesthesiol 2016 Apr;29(2):158-65. doi: 10.1097/ACO.0000000000000303. PMID: 26784351

Prognosis

Loftus TJ, Efron PA, Bala TM, Rosenthal MD, Croft CA, Smith RS, Moore FA, Mohr AM, Brakenridge SC
J Trauma Acute Care Surg 2018 Feb;84(2):350-357. doi: 10.1097/TA.0000000000001730. PMID: 29140948Free PMC Article
Silva JM Jr, Ribas Rosa de Oliveira AM, Mendes Nogueira FA, Vianna PM, Amendola CP, Carvalho Carmona MJ, Sá Malbouisson LM
Anesth Analg 2016 Nov;123(5):1163-1171. doi: 10.1213/ANE.0000000000001575. PMID: 27644062
Barker ME
J Trauma Nurs 2015 Mar-Apr;22(2):111-6. doi: 10.1097/JTN.0000000000000115. PMID: 25768968
Ichai C, Orban JC, Fontaine E
Crit Care 2014 Jul 7;18(4):163. doi: 10.1186/cc13973. PMID: 25043707Free PMC Article
Fichtner J
Urol Int 1999;63(1):40-5. doi: 10.1159/000030417. PMID: 10592489

Clinical prediction guides

Gottfriedová H, Lánská V, Jabor A, Čáslavská M, Špičák J, Horáčková M, Schück O
Int J Clin Pharmacol Ther 2020 May;58(5):261-267. doi: 10.5414/CP203624. PMID: 32213284
Silva JM Jr, Ribas Rosa de Oliveira AM, Mendes Nogueira FA, Vianna PM, Amendola CP, Carvalho Carmona MJ, Sá Malbouisson LM
Anesth Analg 2016 Nov;123(5):1163-1171. doi: 10.1213/ANE.0000000000001575. PMID: 27644062
Brunner R, Drolz A, Scherzer TM, Staufer K, Fuhrmann V, Zauner C, Holzinger U, Schneeweiß B
Crit Care 2015 Apr 6;19(1):148. doi: 10.1186/s13054-015-0890-0. PMID: 25888397Free PMC Article
Brill SA, Stewart TR, Brundage SI, Schreiber MA
Shock 2002 Jun;17(6):459-62. doi: 10.1097/00024382-200206000-00003. PMID: 12069180
Fichtner J
Urol Int 1999;63(1):40-5. doi: 10.1159/000030417. PMID: 10592489

Recent systematic reviews

Habbous S, Przech S, Acedillo R, Sarma S, Garg AX, Martin J
Nephrol Dial Transplant 2017 Jan 1;32(1):111-125. doi: 10.1093/ndt/gfw312. PMID: 27651467
Guellec I, Gascoin G, Beuchee A, Boubred F, Tourneux P, Ramful D, Zana-Taieb E, Baud O
J Pediatr Gastroenterol Nutr 2015 Dec;61(6):605-9. doi: 10.1097/MPG.0000000000000898. PMID: 26147627

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