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Thin Basement Membrane Nephropathy

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What is Thin Basement Membrane Nephropathy

Thin basement membrane nephropathy, also known as benign familial hematuria (benign familial hematuria, BFH), more common in young and middle-aged, but also reported that 1-year-old and 86-year-old patients. More common in women, the male to female ratio of 1:2.5. As the main clinical manifestations of persistent microscopic hematuria, positive family history, diffuse thinning of the glomerular basement membrane of the electron microscope.

Symptoms of Thin Basement Membrane Nephropathy

Clinical manifestations:

The majority TBMN patients presented with persistent microscopic hematuria, upper respiratory tract infection or strenuous exercise can present with gross hematuria.

2 children with asymptomatic hematuria common, 45% to 60% of the combined adult patients with mild proteinuria,, also part TBMN patients with heavy proteinuria or nephrotic syndrome, but better response to hormone.

The majority of patients with normal blood pressure, some adult patients (<20%) may have mildly elevated.

4. Vast majority of patients with renal function may be maintained in the normal range for a long time, a small number of patients may produce renal failure.

What Causes Thin Basement Membrane Nephropathy

Genetics and pathogenesis:

A higher incidence of the disease in patients with a positive family history, genetic important factors, past studies have shown that the disease is autosomal dominant.

The pathogenesis of the disease have not yet been clarified, some people considered GBM of immature likely to is the direct cause of the disease.

Pathological manifestations:

Light microscopy no diagnostic significance pathological changes, the majority of patients with glomerular and tubular quality were normal.

2 The majority of patients immunofluorescence immunofluorescence examination was negative, even visible IgM and (or) C3 deposition in the mesangial area, but weak.

Electron microscopy the check on TBMN diagnosis is essential. Diffuse GBM thinning is the disease is the only or the most important pathological changes.

Tests and Diagnosis for Thin Basement Membrane Nephropathy

Diagnostic tests:

The diagnosis of the disease depends on renal ultrastructure observation.

(1) renal biopsy immunofluorescence negative or C3, IgM mesangial area showed a weak positive;

(2) light microscopy examination was normal or mildly abnormal;

(3) electron microscope showed diffuse GBM thinning without electron dense deposits.

Differential diagnosis:

(1) of the disease and Alport syndrome identification, according to the clinical features and pathological changes (especially ultrastructural changes) easier to identify.

(2) need mesangial IgA nephropathy identify mainly hematuria, former renal biopsy immunofluorescence mainly IgA deposition, electron microscope, a chunk of electron-dense material, data can be identified.

(3) In addition, with the identification of surgical hematuria, and a number of primary and secondary glomerular diseases.

Treatments of Thin Basement Membrane Nephropathy

Treatment:

TBMN is a benign disease, without the need for special treatment. Avoid the cold and fatigue, avoid unnecessary treatment and application of nephrotoxic drugs, strengthen a small number of existing hypertension in patients with blood pressure control, attention to the protection of renal function, and disease management.

Note:

The only clinical manifestations. Few thin basement membrane nephropathy in adult patients with mild proteinuria.

Regardless of the degree of variation and the light microscope, the severity of the pathological changes of electron microscope, the GBM.

3 research GBM damaged hematuria reasons.

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active 7 March 2013 at 15:33

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