I Hate Dialysis Message Board
Dialysis Discussion => Dialysis: General Discussion => Topic started by: Clancy1600 on March 01, 2008, 10:09:15 AM
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A diagnosis of "Thin Basement Membrane" runs in the female side of my family. Does anyone here have this diagnosis? (I think there is a more modern name for "Thin Basement Membrane", but I don't know what that term is. Anyone happen to know?)
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Without Google..I'd be nothing. You're looking for "Thin Glomerular Basement Membrane Disease".
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
Thin basement membrane disease is inherited in an autosomal dominant fashion. The mutations have been shown to occur in the alpha-3 and -4 chains of type 4 collagen, which is an important part of the framework for the basement membrane. (Online 'Mendelian Inheritance in Man' (OMIM) 141200).
Some individuals with TBMD are thought to be carries for genes that cause Alport syndrome.
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.
Prognosis
Overall, most people with thin basement membrane disease have an excellent prognosis. Some reports, however, suggest that a minority might develop hypertension.[2] 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.
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And there you have it. Try kidneyoogle next time. Probabely get the same result but it helps IHD family of sites.
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WOW - everything you wanted to know about thin membrane but never dared ask!!! Thanks for the info.
Clancy - is that our answer? time will tell.
sandyb
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Livecam and Sluff, thank you for this information! Slywalker, I'm beginning to wonder if you and I are related. We seem to share so many similarities!