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PubMed This is a summary of 21 peer-reviewed journal articles Updated
Nephrology

Long-Term Health and Potential Complications

At a Glance

Distal renal tubular acidosis can cause kidney stones, calcium deposits in the kidneys, weak bones or poor growth, hearing loss in some genetic forms, and dangerously low potassium. Consistent alkali treatment lowers these risks, while severe weakness or palpitations require emergency care.

While distal renal tubular acidosis (dRTA) is a chemical imbalance, its effects can be felt throughout the entire body. When acid builds up and stays in your blood for a long time, it forces your organs to compensate in ways that can lead to lasting damage. However, the most important takeaway is that treatment significantly reduces the risk of these complications, and some (like bone disease) can be improved with consistent therapy [1][2].

Kidney Health and Stones

The kidneys are the primary site of damage in dRTA. Because the urine in dRTA is “too basic” (alkaline), it creates an environment for minerals to crystallize [3].

  • Nephrocalcinosis: This is a condition where calcium deposits form directly inside the kidney tissue rather than in the urine stream [3]. In long-term studies of some dRTA cohorts, nephrocalcinosis is a frequent finding, underscoring the importance of adherence to alkali therapy [4].
  • Kidney Stones: When minerals clump together in the urine, they form painful stones. This happens because dRTA causes hypocitraturia—a lack of citrate, which is the body’s natural “stone-fighter” that normally prevents calcium from sticking together [3][5].
  • Chronic Kidney Disease (CKD): Over many years, the constant presence of stones and mineral deposits can lead to scarring and reduced kidney function [4]. While severe kidney failure is rare in dRTA, long-term observational studies show some patients develop mild or moderate CKD, which requires careful monitoring [4]. Note that an eGFR dipping below 90 does not mean permanent kidney failure; true CKD requires persistent evidence of kidney damage.

Bone Health and Growth

Your bones act as a “buffer” for your blood. When there is too much acid in your blood, your body may actually “borrow” calcium and minerals from your bones to neutralize that acid [6].

  • In Children: This “borrowing” of minerals can lead to rickets (softening and weakening of bones) and stunted growth [6][7]. Fortunately, when acid levels are corrected with alkali therapy, many children experience “catch-up growth” and their bones can heal [7][8].
  • In Adults: Chronic acidosis can lead to osteomalacia (the adult version of rickets) or osteoporosis (thinning bones), making fractures and bone pain more likely [6][9].

Sensorineural Hearing Loss

For some people with genetic dRTA (specifically mutations in the ATP6V1B1, ATP6V0A4, or FOXI1 genes), the condition is linked to hearing loss [10][11]. This happens because the same “pumps” used in the kidney are also used to maintain the fluid balance in the inner ear [10].

  • Timing: Hearing loss can be present at birth or may develop later in childhood or adolescence [12][13].
  • Monitoring: Regular audiologic (hearing) tests are a standard part of care for these specific genetic subtypes, as the loss can sometimes be progressive [10][14].

🚨 Emergency Warning Signs: Severe Hypokalemia

While most symptoms of dRTA are managed over time, severe hypokalemia (dangerously low potassium) is a medical emergency. This happens when the kidneys “waste” too much potassium into the urine [15].

If you or your child experience any of the following, seek emergency medical care immediately:

  • Sudden Muscle Weakness or Paralysis: This often starts in the legs and can move up the body [16][17].
  • Trouble Breathing: Severe low potassium can weaken the muscles used for breathing [18][19].
  • Heart Palpitations or “Skipped Beats”: Low potassium can cause dangerous electrical changes in the heart (arrhythmias) [20][21].
  • Fainting or Profound Fatigue: This may be a sign that your heart or muscles are struggling to function [21].

Do NOT attempt to self-treat severe weakness by taking extra potassium at home. Emergency evaluation requires urgent ECG monitoring of your heart rhythm, repeat blood tests, and carefully supervised potassium replacement (often oral or carefully controlled IV) by medical professionals [16][18]. For more on how to prevent these emergencies, see the Standard of Care Treatment page.

Common questions in this guide

What long-term problems can distal renal tubular acidosis cause?
Over time, distal renal tubular acidosis may cause calcium deposits in the kidney, called nephrocalcinosis, kidney stones, and sometimes reduced kidney function. It can also weaken bones, slow growth in children, or cause hearing loss in some inherited forms. Treatment and regular monitoring lower the risk of these problems.
Can treatment improve bone and growth problems caused by dRTA?
Correcting the acid imbalance with consistent alkali therapy can allow many children to have catch-up growth and can help bone disease heal. Adults may need assessment for osteomalacia or osteoporosis, especially if bone pain or fractures occur. The treating clinician should choose monitoring based on age and health.
Why are hearing tests important for some people with dRTA?
Changes in the ATP6V1B1, ATP6V0A4, or FOXI1 genes can link dRTA with inner-ear hearing loss. Hearing loss may be present at birth or appear later and can progress, so regular formal hearing tests are important for people with these genetic forms.
Which low-potassium symptoms mean I need emergency care?
Sudden muscle weakness or paralysis, trouble breathing, heart palpitations, fainting, or profound fatigue can signal dangerously low potassium and require immediate emergency evaluation. Do not take extra potassium on your own; clinicians may need an ECG and repeat blood tests while replacing potassium carefully.
Does dRTA always lead to chronic kidney disease or kidney failure?
No. Stones and calcium deposits can scar the kidneys and contribute to mild or moderate chronic kidney disease over time, but severe kidney failure is uncommon. An eGFR below 90 by itself does not prove chronic kidney disease; persistent kidney damage or reduced function must be evaluated by a clinician.
How can citrate help prevent kidney stones in dRTA?
dRTA can cause hypocitraturia, meaning too little citrate in the urine. Citrate normally helps stop calcium crystals from sticking together, so your clinician may review whether your alkali treatment provides enough citrate to reduce stone risk.

Questions For You

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Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Based on my latest imaging, what is the extent of my nephrocalcinosis, and is it affecting my overall kidney function?
  2. 2.If my child has a genetic mutation in ATP6V1B1, ATP6V0A4, or FOXI1, how often should we be doing formal hearing tests?
  3. 3.Is my current bone density or growth rate appropriate for my age, or do we need to screen for osteomalacia or rickets?
  4. 4.How can I distinguish between general fatigue and the muscle weakness that might signal a dangerous drop in potassium?
  5. 5.Does my treatment plan include enough citrate to specifically lower my risk of new kidney stones?

References

References (21)
  1. 1

    Primary Distal Renal Tubular Acidosis: Toward an Optimal Correction of Metabolic Acidosis.

    Medina E, Ariceta G, Batlle D

    Clinical journal of the American Society of Nephrology : CJASN 2024; (19(9)):1212-1222 doi:10.2215/CJN.0000000000000535.

    PMID: 38967973
  2. 2

    Distal renal tubular acidosis: ERKNet/ESPN clinical practice points.

    Trepiccione F, Walsh SB, Ariceta G, et al.

    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2021; (36(9)):1585-1596 doi:10.1093/ndt/gfab171.

    PMID: 33914889
  3. 3

    Incomplete Distal Renal Tubular Acidosis and Kidney Stones.

    Fuster DG, Moe OW

    Advances in chronic kidney disease 2018; (25(4)):366-374 doi:10.1053/j.ackd.2018.05.007.

    PMID: 30139463
  4. 4

    Treatment and long-term outcome in primary distal renal tubular acidosis.

    Lopez-Garcia SC, Emma F, Walsh SB, et al.

    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association 2019; (34(6)):981-991 doi:10.1093/ndt/gfy409.

    PMID: 30773598
  5. 5

    Renal tubular acidosis (RTA) and kidney stones: Diagnosis and management.

    Magni G, Unwin RJ, Moochhala SH

    Archivos espanoles de urologia 2021; (74(1)):123-128.

    PMID: 33459628
  6. 6

    Renal Tubular Acidosis Manifesting as Severe Metabolic Bone Disease.

    Boro H, Khatiwada S, Alam S, et al.

    TouchREVIEWS in endocrinology 2021; (17(1)):59-67 doi:10.17925/EE.2021.17.1.59.

    PMID: 35118447
  7. 7

    Distal Renal Tubular Acidosis: A Conundrum of Short Stature, Failure to Thrive, Rickets, and Nephrocalcinosis.

    Singhania P, Ponnusamy R, Dhar A, et al.

    Cureus 2025; (17(8)):e90337 doi:10.7759/cureus.90337.

    PMID: 40978892
  8. 8

    Clinical and genetic analysis of distal renal tubular acidosis in three Chinese children.

    Liu J, Shen Q, Li G, et al.

    Renal failure 2018; (40(1)):520-526 doi:10.1080/0886022X.2018.1487858.

    PMID: 30230413
  9. 9

    Renal Tubular Acidosis in Patients with Primary Sjögren's Syndrome.

    Jung SW, Park EJ, Kim JS, et al.

    Electrolyte & blood pressure : E & BP 2017; (15(1)):17-22 doi:10.5049/EBP.2017.15.1.17.

    PMID: 29042903
  10. 10

    Hearing Loss Related to Gene Mutations in Distal Renal Tubular Acidosis.

    Ay E, Gurses E, Aslan F, et al.

    Audiology & neuro-otology 2023; (28(5)):350-359 doi:10.1159/000529486.

    PMID: 37121229
  11. 11

    Acidosis and Deafness in Patients with Recessive Mutations in FOXI1.

    Enerbäck S, Nilsson D, Edwards N, et al.

    Journal of the American Society of Nephrology : JASN 2018; (29(3)):1041-1048 doi:10.1681/ASN.2017080840.

    PMID: 29242249
  12. 12

    Mutations in ATP6V1B1 and ATP6V0A4 genes cause recessive distal renal tubular acidosis in Mexican families.

    Escobar LI, Simian C, Treard C, et al.

    Molecular genetics & genomic medicine 2016; (4(3)):303-11 doi:10.1002/mgg3.205.

    PMID: 27247958
  13. 13

    A rare case of autosomal recessive ATP6V0A4 variant of distal renal tubular acidosis in a young female with recurrent nephrolithiasis.

    Anupama PH, Abraham G, Shanmugasundaram L, Anupama SH

    Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia 2019; (30(6)):1442-1446 doi:10.4103/1319-2442.275490.

    PMID: 31929293
  14. 14

    Novel compound heterozygous ATP6V1B1 mutations in a Chinese child patient with primary distal renal tubular acidosis: a case report.

    Zhao X, Lu J, Gao Y, et al.

    BMC nephrology 2018; (19(1)):364 doi:10.1186/s12882-018-1173-1.

    PMID: 30558562
  15. 15

    Hypokalemic Distal Renal Tubular Acidosis.

    Vallés PG, Batlle D

    Advances in chronic kidney disease 2018; (25(4)):303-320 doi:10.1053/j.ackd.2018.05.003.

    PMID: 30139458
  16. 16

    Hypokalemic Periodic Paralysis in a Patient With Primary Sjögren's Syndrome and Distal Renal Tubular Acidosis: A Case Report.

    Varma V, Patel AK, Pathak N, et al.

    Clinical medicine insights. Case reports 2025; (18()):11795476251372407 doi:10.1177/11795476251372407.

    PMID: 40894112
  17. 17

    Recurrent Attacks of Hypokalemic Quadriparesis: An Unusual Presentation of Primary Sjögren Syndrome.

    Seirafian S, Shafie M, Abedini A, et al.

    Internal medicine (Tokyo, Japan) 2016; (55(13)):1797-800 doi:10.2169/internalmedicine.55.6453.

    PMID: 27374687
  18. 18

    Hypokalemic Quadriparesis as the Initial Presentation of Sjögren's Syndrome With Distal Renal Tubular Acidosis: A Case Report.

    Sunkari SHB, Nikhare MU, Baig S, Bhoyar JJ

    Cureus 2025; (17(11)):e96825 doi:10.7759/cureus.96825.

    PMID: 41404221
  19. 19

    Severe Hypokalemia and Respiratory Muscle Paralysis: An Atypical Manifestation of Primary Sjögren's Syndrome.

    Sobrosa P, Ferreira Â, Vilar da Mota R, et al.

    Cureus 2024; (16(12)):e76240 doi:10.7759/cureus.76240.

    PMID: 39845259
  20. 20

    Hypokalaemic paralysis as the initial clinical presentation of Sjogren's syndrome induced distal renal tubular acidosis.

    Kielty JM, Ryan P, Sexton D, Kelly YP

    BMJ case reports 2021; (14(5)) doi:10.1136/bcr-2020-241300.

    PMID: 33958361
  21. 21

    Quadriparesis and Broad Complex Tachycardia Secondary to Severe Hypokalaemia Induced by Distal Renal Tubular Acidosis as the Initial Manifestation of Sjogren's Syndrome.

    Kaeley N, Gangdev AM, Datta SS, Singh UP

    Cureus 2023; (15(5)):e38984 doi:10.7759/cureus.38984.

    PMID: 37378130

This page is for informational purposes only and does not constitute medical advice. Your nephrology team can interpret your kidney, bone, hearing, and potassium results and advise when urgent care is needed.