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Diagnosis: Thin Basement Glomerular Membrane Condition
Thin basement membrane condition (TBM, also called "thin basement membrane disease", "thin basement membrane nephropathy", "thin basement membrane lesion."or "thin basement membrane syndrome") is a relatively common condition. The only abnormal finding on renal biopsy is diffuse thinning of the glomerular basement membranes (GBM) requiring electron microscopy for the diagnosis. Historically, these patients were often described as having benign familial hematuria; however, this terminology is no longer favored. TBM is often familial, with a family history of hematuria being noted in 30 to 50 percent of cases. Numerous mutations of the type IV collagen genes COL4A3 and COL4A4 have been identified in patients with TBMN, but such mutations are not present in all families. The diagnosis of TBMis usually inferred from the benign presentation and course, the positive family history of hematuria, and the negative family history of renal failure. Renal biopsy is generally not performed in patients who present with familial isolated glomerular hematuria, no or minimal proteinuria, normal renal function, and no family history of renal failure. The prognosis is excellent in such patients unless the clinical manifestations progress, as occurs in all males and some females with Alport syndrome, as well as many patients with IgA nephropathy (Kashtan CE. Thin basement membrane nephropathy (benign familial hematuria). In: eMedicine, consulted on October 27th, 2017 [Link to the website]).
No universal agreement exists concerning the normal GBM thickness—distance between the endothelial and epithelial cell membranes—and consequently the definition of thin GBM. During infancy and early childhood, the GBM thickness increases progressively from 100 to 150 nm at birth to 200 nm at 1 year of age, until reaching adult values by 11 years of age. GBM thickness less than 250 nm has been the criterion accepted in most pediatric and adult series (Jennette JC, et al (editors). Heptinstall’s pathology of the kidney, 7° ed. Wolters Kluwer, Philadelphia, 2015. pp. 542-3) .
See the chapter: Hereditary diseases of our Tutorial (only Spanish version).
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References
- Kashtan CE. Thin basement membrane nephropathy (benign familial hematuria). In: eMedicine, consulted on October 27th, 2017 [Link to the website]
- Zurawski J, Burchardt P, Moczko J, Seget M, Iwanik K, Sikora J, Woźniak A, Salwa-Zurawska W. Morphological assessment of thin basement membrane disease. Pol J Pathol. 2016;67(2):114-21. [PubMed link].
- Zouvani I, Aristodemou S, Hadjisavvas A, Michael T, Vassiliou M, Patsias C, Pierides A, Ioannou K, Kyriacou K. Incidence of thin basement membrane nephropathy in 990 consecutive renal biopsies examined with electron microscopy. Ultrastruct Pathol. 2008;32(6):221-6. [PubMed link].
- Jennette JC, Olson JL, Silva FG, D'Agati VD (editors). Heptinstall’s pathology of the kidney, 7° ed. Wolters Kluwer, Philadelphia, 2015. pp. 542-3.