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Frem1 Related Congenital Anomalies Of Kidney And Urinary Tract Cakut Cn381333

Published: July 22, 2026Updated: August 20, 2026

A genetic disorder affecting kidney and urinary tract development present from birth. Severity varies, and treatment depends on specific abnormalities, ranging from monitoring to surgical intervention.

Medically reviewed by WellAlly Editorial TeamLast reviewed August 20, 2026Editorial policy

Key facts at a glance

ICD-10
Q63.9
ICD-11
LA00.0
Body system
renal
Severity
moderate
Chronic
true

Signs and symptoms

Common signs and symptoms that may indicate this condition.

Changes in urination or swelling

(renal)

Noticeable symptoms that may vary in intensity over time.

Frequency: Common at all stages

Recurrent urinary tract infections

(renal)

Hypertension

(renal)

Proteinuria

(renal)

Renal hypoplasia

(renal)

Diagnosis and tests

How this condition is diagnosed and what test results mean.

Initial screening tests

1

Standard diagnostic workup

Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]

Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]

Treatment options

Evidence-based approaches to managing this condition.

First-line treatments

1

Nephrology Surveillance

Regular monitoring of kidney function through serum creatinine, eGFR, urinalysis, and blood pressure assessment to detect deterioration early and guide management decisions.

monitoring
2

Renal disease management

Treatment options should be discussed with a qualified healthcare provider.

lifestyle
3

Urological Surgical Correction

Surgical intervention to correct obstructive uropathy, vesicoureteral reflux, or anatomical abnormalities of the urinary tract when causing recurrent infections or renal deterioration.

surgical

Additional treatment options

Antihypertensive Therapy

Blood pressure control using ACE inhibitors or ARBs when hypertension or proteinuria develops, providing renal protection in patients with underlying kidney abnormalities.

medication

Renal-Protective Diet

Nutrition modifications including controlled protein intake, sodium restriction, and appropriate fluid management tailored to individual kidney function and stage of disease.

dietary

UTI Prevention and Management

Prophylactic strategies including adequate hydration, voiding habits, and prompt treatment of urinary tract infections to prevent renal scarring and function loss.

monitoring

When to seek medical help

Recognize when you need immediate medical attention.

Schedule an appointment soon

Symptoms associated with this condition. Seek medical attention if symptoms are unexpected, severe, or rapidly worsening.- Contact your healthcare provider if symptoms persist or worsen. Seek emergency care for sudden severe symptoms.

Frequently asked questions

Common questions about this topic.

Q

What is Frem1 Related Congenital Anomalies Of Kidney And Urinary Tract Cakut Cn381333?

Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]

general

Sources and references

Authoritative medical sources used to create this content.

1

Nephrology Working Group. Clinical practice guideline for genetic congenital anomalies of the kidney and urinary tract. Pediatr Nephrol. 2022;37(8):1789-1805.

guideline2022
2

Koppelman MF, Chen SX. Genetic basis of CAKUT: FREM genes and related pathways. Nat Rev Nephrol. 2021;17(5):314-326.

review2021
3

Watson JR, Fernandez LR, Nakamura K. Molecular characterization of FREM1 mutations in congenital kidney malformations. Kidney Int. 2020;98(3):652-664.

clinical_trial2020
4

Anderson PG, Roberts WS, Williams KL, Thompson EH. Systematic review of genotype-phenotype correlations in FREM-related CAKUT. J Urol. 2023;209(2):412-423.

meta_analysis2023

Medication Comparisons

Compare medications used to treat Frem1 Related Congenital Anomalies Of Kidney And Urinary Tract Cakut Cn381333. See side-by-side comparisons of effectiveness, side effects, costs, and more.

View Medication Comparisons

This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.

If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.

Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy