Thin basement membrane disease

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Thin basement membrane disease Classification and external resources
DiseasesDB	5363
MedlinePlus	003524
eMedicine	med/149

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Thin basement membrane disease (TBMD, also known as benign familial hematuria and thin basement membrane nephropathy) is, along with IgA nephropathy, the most common cause of asymptomatic hematuria. The only abnormal finding in this disease is a thinning of the basement membrane of the glomeruli in the kidneys. Its importance lies in the fact that it has a benign prognosis, with patients maintaining a normal kidney function throughout their lives.

Signs and symptoms

Most patients with thin basement membrane disease are incidentally discovered to have microscopic hematuria on urinalysis. The blood pressure, kidney function and the urinary protein excretion are usually normal. Mild proteinuria (less than 1.5 g/day) and hypertension are seen in a small minority of patients. Frank hematuria and loin pain should prompt a search for another cause, such as kidney stones or loin pain-hematuria syndrome. Also, there are no systemic manifestations, so presence of hearing impairment or visual impairment should prompt a search for hereditary nephritis such as Alport syndrome.

Diagnosis

Thin basement membrane disease has to be differentiated from the other two common causes of isolated glomerular hematuria, IgA nephropathy and Alport syndrome. The history and presentation are helpful in this regard:

• There is usually a family history of kidney failure, which may be associated with hearing impairment in Alport syndrome. Also, more males tend to be affected since it is more often X-linked.
• In IgA nephropathy, episodes of frank hematuria are more common, and a family history is rare.

A kidney biopsy is the only way to diagnose thin basement membrane disease. It reveals thinning of the glomerular basement membrane from the normal 300 to 400 nanometers (nm) to 150 to 250 nm. However, a biopsy is rarely done in cases where the patient has isolated microscopic hematuria, normal kidney function and no proteinuria. The prognosis is excellent in this setting unless the clinical manifestations progress, as occurs in all males and some females with Alport syndrome and many patients with IgA nephropathy.

Genetics

The molecular basis for thin basement membrane disease has yet to be elucidated fully; however, defects in the gene encoding the a4 chain of type IV collagen have been reported in some families.

Some individuals with TBMD are thought to be carries for genes that cause Alport syndrome.^[2]

Treatment

Most patients with thin basement membrane disease need just reassurance. Angiotensin converting enzyme inhibitors have been suggested to reduce the episodes of hematuria, though controlled studies are lacking. Treating co-existing hypercalciuria and hyperuricosuria will also be helpful in reducing hematuria. The molecular basis for thin basement membrane disease has yet to be elucidated fully; however, defects in the gene encoding the a4 chain of type IV collagen have been reported in some families.

Prognosis

Overall, most people with thin basement membrane disease have an excellent prognosis. Some reports, however, suggest that a minority might develop hypertension.^[3] The high incidence of thin basement disease also means that it may be co-existing with other kidney diseases, such as diabetic nephropathy, which may have a not-so-benign prognosis.

References

1. ^ Buzza M, Wang Y, Dagher H, Babon J, Cotton R, Powell H, Dowling J, Savige J. COL4A4 mutation in thin basement membrane disease previously described in Alport syndrome. Kidney International (2001) 60, 480–483. PMID 11473630
2. ^ Nieuwhof, CM, de Heer, F, de Leeuw, P, van Breda Vriesman, PJ. Thin GBM nephropathy. Premature glomerular obsolescence is associated with hypertension and late onset renal failure. Kidney Int 1997;51:1596. PMID 9150478

v • d • e WikiDoc Research Resources for Thin basement membrane disease
Articles on Thin basement membrane disease	Most recent articles on Thin basement membrane disease • Most cited articles on Thin basement membrane disease • Review articles on Thin basement membrane disease • Articles on Thin basement membrane disease in N Eng J Med, Lancet, BMJ
Media (Slides, Video, Images, MP3) on Thin basement membrane disease	Powerpoint slides on Thin basement membrane disease • Images of Thin basement membrane disease • Photos of Thin basement membrane disease • Podcasts & MP3s on Thin basement membrane disease • Videos on Thin basement membrane disease
Evidence Based Medicine Regarding Thin basement membrane disease	Cochrane Collaboration on Thin basement membrane disease • Bandolier on Thin basement membrane disease • TRIP on Thin basement membrane disease
Cost Effectiveness of Thin basement membrane disease	Cost Effectiveness of Thin basement membrane disease
Clinical Trials Involving Thin basement membrane disease	Ongoing Trials on Thin basement membrane disease at Clinical Trials.gov • Trial results on Thin basement membrane disease • Clinical Trials on Thin basement membrane disease at Google
Guidelines / Policies / Government Resources (FDA/CDC) Regarding Thin basement membrane disease	US National Guidelines Clearinghouse on Thin basement membrane disease • NICE Guidance on Thin basement membrane disease • NHS PRODIGY Guidance • FDA on Thin basement membrane disease • CDC on Thin basement membrane disease
Textbook Information on Thin basement membrane disease	Books and Textbook Information on Thin basement membrane disease
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Genetics, Pharmacogenomics, and Proteinomics of Thin basement membrane disease	Genetics of Thin basement membrane disease • Pharmacogenomics of Thin basement membrane disease • Proteomics of Thin basement membrane disease
Newstories on Thin basement membrane disease	Thin basement membrane disease in the news • Be alerted to news on Thin basement membrane disease • News trends on Thin basement membrane disease
Commentary on Thin basement membrane disease	Blogs on Thin basement membrane disease
Patient Resources on Thin basement membrane disease	Patient resources on Thin basement membrane disease • Discussion groups on Thin basement membrane disease • Patient Handouts on Thin basement membrane disease • Directions to Hospitals Treating Thin basement membrane disease • Risk calculators and risk factors for Thin basement membrane disease
Healthcare Provider Resources on Thin basement membrane disease	Symptoms of Thin basement membrane disease • Causes & Risk Factors for Thin basement membrane disease • Diagnostic studies for Thin basement membrane disease • Treatment of Thin basement membrane disease
Continuing Medical Education (CME) Programs on Thin basement membrane disease	CME Programs on Thin basement membrane disease
International Resources on Thin basement membrane disease	Thin basement membrane disease en Espanol • Thin basement membrane disease en Francais
Business Resources on Thin basement membrane disease	Thin basement membrane disease in the Marketplace • Patents on Thin basement membrane disease
Informatics Resources on Thin basement membrane disease	List of terms related to Thin basement membrane disease

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