U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Renal tubular acidosis, distal, 3, with or without sensorineural hearing loss(DRTA3)

MedGen UID:
1732975
Concept ID:
C5399980
Disease or Syndrome
Synonym: DRTA3
 
Gene (location): ATP6V0A4 (7q34)
 
Monarch Initiative: MONDO:0011268
OMIM®: 602722

Disease characteristics

Excerpted from the GeneReview: Hereditary Distal Renal Tubular Acidosis
Individuals with hereditary distal renal tubular acidosis (dRTA) typically present in infancy with failure to thrive, although later presentations can occur, especially in individuals with autosomal dominant SLC4A1-dRTA. Initial clinical manifestations can also include emesis, polyuria, polydipsia, constipation, diarrhea, decreased appetite, and episodes of dehydration. Electrolyte manifestations include hyperchloremic non-anion gap metabolic acidosis and hypokalemia. Renal complications of dRTA include nephrocalcinosis, nephrolithiasis, medullary cysts, and impaired renal function. Additional manifestations include bone demineralization (rickets, osteomalacia), growth deficiency, sensorineural hearing loss (in ATP6V0A4-, ATP6V1B1-, and FOXI1-dRTA), and hereditary hemolytic anemia (in some individuals with SLC4A1-dRTA). [from GeneReviews]
Authors:
R Todd Alexander  |  Linda Law  |  Helena Gil-Peña, et. al.   view full author information

Clinical features

From HPO
Hypercalciuria
MedGen UID:
43775
Concept ID:
C0020438
Finding
Abnormally high level of calcium in the urine.
Nephrocalcinosis
MedGen UID:
10222
Concept ID:
C0027709
Disease or Syndrome
Nephrocalcinosis is the deposition of calcium salts in renal parenchyma.
Growth delay
MedGen UID:
99124
Concept ID:
C0456070
Pathologic Function
A deficiency or slowing down of growth pre- and postnatally.
Failure to thrive
MedGen UID:
746019
Concept ID:
C2315100
Disease or Syndrome
Failure to thrive (FTT) refers to a child whose physical growth is substantially below the norm.
Vomiting
MedGen UID:
12124
Concept ID:
C0042963
Sign or Symptom
Forceful ejection of the contents of the stomach through the mouth by means of a series of involuntary spasmic contractions.
Bilateral sensorineural hearing impairment
MedGen UID:
96788
Concept ID:
C0452138
Disease or Syndrome
A bilateral form of sensorineural hearing impairment.
Rickets
MedGen UID:
48470
Concept ID:
C0035579
Disease or Syndrome
Rickets is divided into two major categories including calcipenic and phosphopenic. Hypophosphatemia is described as a common manifestation of both categories. Hypophosphatemic rickets is the most common type of rickets that is characterized by low levels of serum phosphate, resistance to ultraviolet radiation or vitamin D intake. There are several issues involved in hypophosphatemic rickets such as calcium, vitamin D, phosphorus deficiencies. Moreover, other disorder can be associated with its occurrence such as absorption defects due to pancreatic, intestinal, gastric, and renal disorders and hepatobiliary disease. Symptoms are usually seen in childhood and can be varied in severity. Severe forms may be linked to bowing of the legs, poor bone growth, and short stature as well as joint and bone pain. Hypophosphatemic rickets are associated with renal excretion of phosphate, hypophosphatemia, and mineral defects in bones. The familial type of the disease is the most common type of rickets.
Dehydration
MedGen UID:
8273
Concept ID:
C0011175
Disease or Syndrome
A condition resulting from the excessive loss of water from the body. It is usually caused by severe diarrhea, vomiting or diaphoresis.
Hypokalemia
MedGen UID:
5712
Concept ID:
C0020621
Finding
An abnormally decreased potassium concentration in the blood.
Metabolic acidosis
MedGen UID:
65117
Concept ID:
C0220981
Pathologic Function
Metabolic acidosis (MA) is characterized by a fall in blood pH due to a reduction of serum bicarbonate concentration. This can occur as a result of either the accumulation of acids (high anion gap MA) or the loss of bicarbonate from the gastrointestinal tract or the kidney (hyperchloremic MA). By definition, MA is not due to a respirary cause.
Distal renal tubular acidosis
MedGen UID:
853429
Concept ID:
C1704380
Disease or Syndrome
A type of renal tubular acidosis characterized by a failure of acid secretion by the alpha intercalated cells of the cortical collecting duct of the distal nephron. The urine cannot be acidified below a pH of 5.3, associated with acidemia and hypokalemia.

Professional guidelines

PubMed

Palmer BF, Kelepouris E, Clegg DJ
Adv Ther 2021 Feb;38(2):949-968. Epub 2020 Dec 26 doi: 10.1007/s12325-020-01587-5. PMID: 33367987Free PMC Article
Chandrasekhar SS, Tsai Do BS, Schwartz SR, Bontempo LJ, Faucett EA, Finestone SA, Hollingsworth DB, Kelley DM, Kmucha ST, Moonis G, Poling GL, Roberts JK, Stachler RJ, Zeitler DM, Corrigan MD, Nnacheta LC, Satterfield L
Otolaryngol Head Neck Surg 2019 Aug;161(1_suppl):S1-S45. doi: 10.1177/0194599819859885. PMID: 31369359
Michels TC, Duffy MT, Rogers DJ
Am Fam Physician 2019 Jul 15;100(2):98-108. PMID: 31305044

Recent clinical studies

Etiology

Samocha-Bonet D, Wu B, Ryugo DK
Ageing Res Rev 2021 Nov;71:101423. Epub 2021 Aug 9 doi: 10.1016/j.arr.2021.101423. PMID: 34384902
Giglio S, Montini G, Trepiccione F, Gambaro G, Emma F
J Nephrol 2021 Dec;34(6):2073-2083. Epub 2021 Mar 26 doi: 10.1007/s40620-021-01032-y. PMID: 33770395Free PMC Article
LeGros TL, Murphy-Lavoie H
Undersea Hyperb Med 2020 Second Quarter;47(2):271-295. doi: 10.22462/04.06.2020.14. PMID: 32574445
Young YH
Int J Audiol 2020 Apr;59(4):243-253. Epub 2019 Nov 12 doi: 10.1080/14992027.2019.1689432. PMID: 31714154
Kuhn M, Heman-Ackah SE, Shaikh JA, Roehm PC
Trends Amplif 2011 Sep;15(3):91-105. Epub 2011 May 22 doi: 10.1177/1084713811408349. PMID: 21606048Free PMC Article

Diagnosis

Shave S, Botti C, Kwong K
Pediatr Clin North Am 2022 Apr;69(2):221-234. doi: 10.1016/j.pcl.2021.12.006. PMID: 35337535
Giglio S, Montini G, Trepiccione F, Gambaro G, Emma F
J Nephrol 2021 Dec;34(6):2073-2083. Epub 2021 Mar 26 doi: 10.1007/s40620-021-01032-y. PMID: 33770395Free PMC Article
Prince ADP, Stucken EZ
J Am Board Fam Med 2021 Jan-Feb;34(1):216-223. doi: 10.3122/jabfm.2021.01.200199. PMID: 33452100
Palmer BF, Kelepouris E, Clegg DJ
Adv Ther 2021 Feb;38(2):949-968. Epub 2020 Dec 26 doi: 10.1007/s12325-020-01587-5. PMID: 33367987Free PMC Article
Kuhn M, Heman-Ackah SE, Shaikh JA, Roehm PC
Trends Amplif 2011 Sep;15(3):91-105. Epub 2011 May 22 doi: 10.1177/1084713811408349. PMID: 21606048Free PMC Article

Therapy

Tan WJT, Song L
Hear Res 2023 Jul;434:108783. Epub 2023 Apr 29 doi: 10.1016/j.heares.2023.108783. PMID: 37167889
Joshua TG, Ayub A, Wijesinghe P, Nunez DA
JAMA Otolaryngol Head Neck Surg 2022 Jan 1;148(1):5-11. doi: 10.1001/jamaoto.2021.2685. PMID: 34709348Free PMC Article
Herrera M, García Berrocal JR, García Arumí A, Lavilla MJ, Plaza G; Grupo de Trabajo de la Comisión de Audiología de la SEORL
Acta Otorrinolaringol Esp (Engl Ed) 2019 Sep-Oct;70(5):290-300. Epub 2018 Aug 6 doi: 10.1016/j.otorri.2018.04.010. PMID: 30093087
Marx M, Younes E, Chandrasekhar SS, Ito J, Plontke S, O'Leary S, Sterkers O
Eur Ann Otorhinolaryngol Head Neck Dis 2018 Feb;135(1S):S23-S28. Epub 2018 Feb 1 doi: 10.1016/j.anorl.2017.12.011. PMID: 29396226
Kuhn M, Heman-Ackah SE, Shaikh JA, Roehm PC
Trends Amplif 2011 Sep;15(3):91-105. Epub 2011 May 22 doi: 10.1177/1084713811408349. PMID: 21606048Free PMC Article

Prognosis

Simani L, Oron Y, Shapira U, Handzel O, Abu Eta R, Warshavsky A, Horowitz G, Muhanna N, Shilo S, Ungar OJ
Otol Neurotol 2022 Oct 1;43(9):1016-1021. Epub 2022 Aug 30 doi: 10.1097/MAO.0000000000003661. PMID: 36040043
Fidan V, Akin O, Koyuncu H
Am J Otolaryngol 2021 Sep-Oct;42(5):102996. Epub 2021 Mar 31 doi: 10.1016/j.amjoto.2021.102996. PMID: 33831819Free PMC Article
Dedhia K, Graham E, Park A
Clin Perinatol 2018 Dec;45(4):629-643. Epub 2018 Sep 24 doi: 10.1016/j.clp.2018.07.004. PMID: 30396409
Stew BT, Fishpool SJ, Williams H
Br J Hosp Med (Lond) 2012 Feb;73(2):86-9. doi: 10.12968/hmed.2012.73.2.86. PMID: 22504750
Schreiber BE, Agrup C, Haskard DO, Luxon LM
Lancet 2010 Apr 3;375(9721):1203-11. doi: 10.1016/S0140-6736(09)62071-7. PMID: 20362815

Clinical prediction guides

Bodh V, Kumar R, Sharma R, Sharma B, Sachdeva A, Azad R
Indian J Gastroenterol 2022 Apr;41(2):143-148. Epub 2022 Mar 5 doi: 10.1007/s12664-021-01216-1. PMID: 35247189
McDermott JH, Wolf J, Hoshitsuki K, Huddart R, Caudle KE, Whirl-Carrillo M, Steyger PS, Smith RJH, Cody N, Rodriguez-Antona C, Klein TE, Newman WG
Clin Pharmacol Ther 2022 Feb;111(2):366-372. Epub 2021 Jun 20 doi: 10.1002/cpt.2309. PMID: 34032273Free PMC Article
Palmer BF, Kelepouris E, Clegg DJ
Adv Ther 2021 Feb;38(2):949-968. Epub 2020 Dec 26 doi: 10.1007/s12325-020-01587-5. PMID: 33367987Free PMC Article
Kuhn M, Heman-Ackah SE, Shaikh JA, Roehm PC
Trends Amplif 2011 Sep;15(3):91-105. Epub 2011 May 22 doi: 10.1177/1084713811408349. PMID: 21606048Free PMC Article
Jerger J
J Am Acad Audiol 2011 Mar;22(3):128. doi: 10.3766/jaaa.22.3.1. PMID: 21545765

Recent systematic reviews

Plontke SK, Meisner C, Agrawal S, Cayé-Thomasen P, Galbraith K, Mikulec AA, Parnes L, Premakumar Y, Reiber J, Schilder AG, Liebau A
Cochrane Database Syst Rev 2022 Jul 22;7(7):CD008080. doi: 10.1002/14651858.CD008080.pub2. PMID: 35867413Free PMC Article
Joshua TG, Ayub A, Wijesinghe P, Nunez DA
JAMA Otolaryngol Head Neck Surg 2022 Jan 1;148(1):5-11. doi: 10.1001/jamaoto.2021.2685. PMID: 34709348Free PMC Article
Loughrey DG, Kelly ME, Kelley GA, Brennan S, Lawlor BA
JAMA Otolaryngol Head Neck Surg 2018 Feb 1;144(2):115-126. doi: 10.1001/jamaoto.2017.2513. PMID: 29222544Free PMC Article
Tikka C, Verbeek JH, Kateman E, Morata TC, Dreschler WA, Ferrite S
Cochrane Database Syst Rev 2017 Jul 7;7(7):CD006396. doi: 10.1002/14651858.CD006396.pub4. PMID: 28685503Free PMC Article
Lie A, Skogstad M, Johannessen HA, Tynes T, Mehlum IS, Nordby KC, Engdahl B, Tambs K
Int Arch Occup Environ Health 2016 Apr;89(3):351-72. Epub 2015 Aug 7 doi: 10.1007/s00420-015-1083-5. PMID: 26249711Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...