DISEASE SCANNER
Global Incurable Diseases Tracker
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Nephrologic Disease
Thin Basement Membrane Disease (TBMD)
LOW SEVERITY
A benign genetic condition characterized by uniformly thinned glomerular basement membranes on kidney biopsy, causing persistent microscopic hematuria. Also called benign familial hematuria. Usually non-progressive with excellent long-term prognosis.
Global Affected
1.0M
Countries
15
Symptoms
Asymptomatic microscopic hematuria
Occasional gross hematuria (during infections)
No proteinuria or minimal proteinuria
Normal kidney function
Normal blood pressure
Treatment Options
Observation and monitoring
Regular urinalysis
Blood pressure monitoring
Genetic counseling
Risk Factors
1Family history (autosomal dominant)
2COL4A3 or COL4A4 mutations (heterozygous)
3Female sex (may be underdiagnosed in males)
Diagnostic Methods
- 1Urinalysis (persistent isolated hematuria)
- 2Kidney biopsy with electron microscopy
- 3Genetic testing
- 4Family history assessment
Prognosis
Excellent; non-progressive in vast majority. Kidney function remains normal throughout life. Does not lead to kidney failure.
Prevention
- Family screening
- Genetic counseling
- Differentiation from Alport syndrome
Research Status
No specific treatment required. Regular monitoring to confirm non-progressive course. Differentiation from early Alport syndrome important. Genetic counseling for families.
Affected Countries
Sources
- https://www.kidney.org/atoz/content/thin-basement-membrane-disease
- https://www.ncbi.nlm.nih.gov/books/NBK542242
- https://www.hematology.org/education/patients
- https://www.ncbi.nlm.nih.gov/books
- https://rarediseases.org/rare-diseases
Medical Disclaimer
This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.