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PubMed This is a summary of 6 peer-reviewed journal articles Updated
Nephrology

Living with Iminoglycinuria: Monitoring & Complications

At a Glance

Iminoglycinuria is a benign condition that does not cause kidney damage or affect your lifespan. No special diet or aggressive medical treatment is needed. Your main focus should be drinking plenty of water to prevent kidney stones, which are a slightly higher risk for some patients.

Living with a diagnosis of iminoglycinuria usually requires very little change to your daily life. Because the condition is considered benign (not harmful to your overall health), aggressive medical management is not needed [1][1].

There are no known long-term side effects, no impact on your ability to work or exercise, and no reduction in lifespan [1][2].

The Main Consideration: Kidney Stones

While iminoglycinuria doesn’t damage the kidneys’ ability to filter blood, some individuals—particularly those with mutations in the SLC6A20 gene—may have a slightly higher risk of forming kidney stones (nephrolithiasis) [2][3].

Unlike other conditions where spilled substances form stones directly, proline and glycine are highly soluble and do not simply precipitate into stones. Researchers are still studying exactly why these specific genetic profiles might carry this slightly increased stone risk.

It is important to remember that not everyone with iminoglycinuria will get a kidney stone. However, being aware of the risk allows you to take simple, proactive steps.

Monitoring Your Health

Because iminoglycinuria is a benign condition, you generally do not need extensive medical testing.

  • Routine Care: Routine clinical monitoring (like 24-hour urine collections or ultrasounds) is generally not required unless you have a personal history of kidney stones [4].
  • Specialist Visits: Once your diagnosis is confirmed, you typically do not need ongoing, frequent visits with a nephrologist or metabolic geneticist. Your primary care doctor can handle your general health needs [4].
  • When to Seek Care: If you experience symptoms of a kidney stone, such as flank pain (severe pain in your side or mid-to-lower back) or notice blood in your urine, contact your doctor. Go to the emergency room if severe pain is accompanied by fever, chills, or vomiting, as this could indicate an infection.

Dietary and Lifestyle Habits

You do not need to follow a special diet that restricts protein or specific amino acids [5]. Because your body makes its own proline and glycine, eating less of them won’t stop the “spilling” into your urine.

Instead, focus on general stone prevention strategies:

  • Hydration is Key: The most effective way to prevent stones is to drink enough water to keep your urine diluted. Aim for a goal that keeps your urine very pale or clear throughout the day [6].
  • Watch Your Salt: High-salt (sodium) diets can cause the kidneys to dump more calcium into the urine, which can combine with other substances to form stones.
  • No Supplements Needed: Unless prescribed by a doctor for a different reason, you do not need amino acid supplements or special vitamins for this condition.

Reassurance for the Future

The most stabilizing fact about iminoglycinuria is that it is a transport trait, not a progressive disease. It does not lead to kidney failure or “wear out” the kidneys over time [1]. As long as you stay hydrated and keep an eye out for the symptoms of kidney stones, you can expect to lead a completely normal and healthy life [2].

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Common questions in this guide

Do I need a special diet if I have iminoglycinuria?
No, you do not need to follow a special diet that restricts protein or specific amino acids. Your body produces its own proline and glycine, so eating less of them will not stop them from spilling into your urine.
Will iminoglycinuria cause kidney failure?
No, iminoglycinuria is a benign transport trait and not a progressive disease. It does not lead to kidney failure or damage your kidneys' ability to filter blood over time.
Does iminoglycinuria increase my risk of kidney stones?
Yes, some individuals with iminoglycinuria, particularly those with specific genetic mutations like SLC6A20, may have a slightly higher risk of forming kidney stones. Staying well-hydrated is the best way to prevent them.
What are the symptoms of a kidney stone?
Symptoms of a kidney stone include severe pain in your side or mid-to-lower back, known as flank pain, as well as blood in your urine. If you experience these symptoms along with fever, chills, or vomiting, you should go to the emergency room.
Do I need to see a specialist regularly for iminoglycinuria?
Because the condition is benign, you generally do not need frequent visits with a nephrologist or geneticist once diagnosed. Your primary care doctor can usually manage your ongoing health needs.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Based on my specific genetic mutation or urine profile, how high is my actual risk for developing kidney stones?
  2. 2.Do I need a 24-hour urine collection to look for other stone-forming factors like high calcium, or is that unnecessary right now?
  3. 3.What specific symptoms of kidney stones should prompt me to go to the emergency room instead of just calling the clinic?
  4. 4.Can my primary care doctor handle my general health going forward, or do I need to see a specialist regularly?

Questions For You

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References

References (6)
  1. 1

    Amino Acid Transport Across the Mammalian Intestine.

    Bröer S, Fairweather SJ

    Comprehensive Physiology 2018; (9(1)):343-373 doi:10.1002/cphy.c170041.

    PMID: 30549024
  2. 2

    Genetic mutation of SLC6A20 (c.1072T > C) in a family with nephrolithiasis: A case report.

    Jv M, Zheng J, Yang A, et al.

    Open medicine (Warsaw, Poland) 2023; (18(1)):20230648 doi:10.1515/med-2023-0648.

    PMID: 36820062
  3. 3

    The SLC6A15-SLC6A20 Neutral Amino Acid Transporter Subfamily: Functions, Diseases, and Their Therapeutic Relevance.

    Kukułowicz J, Pietrzak-Lichwa K, Klimończyk K, et al.

    Pharmacological reviews 2023; (76(1)):142-193 doi:10.1124/pharmrev.123.000886.

    PMID: 37940347
  4. 4

    Updates in the Metabolic Management of Calcium Stones.

    Penniston KL, Nakada SY

    Current urology reports 2018; (19(6)):41 doi:10.1007/s11934-018-0791-2.

    PMID: 29663088
  5. 5

    Human intestine luminal ACE2 and amino acid transporter expression increased by ACE-inhibitors.

    Vuille-dit-Bille RN, Camargo SM, Emmenegger L, et al.

    Amino acids 2015; (47(4)):693-705 doi:10.1007/s00726-014-1889-6.

    PMID: 25534429
  6. 6

    Epidemiology of paediatric renal stone disease: a 22-year single centre experience in the UK.

    Issler N, Dufek S, Kleta R, et al.

    BMC nephrology 2017; (18(1)):136 doi:10.1186/s12882-017-0505-x.

    PMID: 28420322

This page provides educational information about living with iminoglycinuria. It is for informational purposes only and does not replace professional medical advice regarding your specific kidney stone risk or health management.

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