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{{Thin basement membrane disease}}
{{Thin basement membrane disease}}


{{CMG}}
{{CMG}};
{{AE}} {{MMT}}


{{SK}}: Thin membrane nephropathy; thin GBM nephropathy; thin GBM syndrome
{{SK}} [[Thin basement membrane disease]]<ref name="pmid14524583">{{cite journal |vauthors=Takemura T, Yanagida H, Yagi K, Moriwaki K, Okada M |title=Alport syndrome and benign familial hematuria (thin basement membrane disease) in two brothers of a family with hematuria |journal=Clin. Nephrol. |volume=60 |issue=3 |pages=195–200 |date=September 2003 |pmid=14524583 |doi=10.5414/cnp60195 |url=}}</ref>; [[Thin membrane nephropathy]]<ref name="pmid9150478">{{cite journal |vauthors=Nieuwhof CM, de Heer F, de Leeuw P, van Breda Vriesman PJ |title=Thin GBM nephropathy: premature glomerular obsolescence is associated with hypertension and late onset renal failure |journal=Kidney Int. |volume=51 |issue=5 |pages=1596–601 |date=May 1997 |pmid=9150478 |doi=10.1038/ki.1997.219 |url=}}</ref>; [[thin GBM nephropathy]]; [[thin GBM syndrome]]; [[benign familial hematuria]] <ref name="pmid4899625">{{cite journal |vauthors=Marks MI, Drummond KN |title=Benign familial hematuria |journal=Pediatrics |volume=44 |issue=4 |pages=590–3 |date=October 1969 |pmid=4899625 |doi= |url=}}</ref>; [[benign]] familial essential [[hematuria]]; [[congenital]] [[hereditary]] [[hematuria]]; [[hereditary]] [[hematuria]]; [[familial]] hematuric [[nephritis]]; [[benign]] [[hereditary]] hematuric [[nephritis]]


==[[Thin basement membrane disease overview|Overview]]==
==[[Thin basement membrane disease overview|Overview]]==
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==Diagnosis==
==Diagnosis==


[[Thin basement membrane disease history and symptoms|History and Symptoms]] | [[Thin basement membrane disease physical examination|Physical Examination]] | [[Thin basement membrane disease laboratory findings|Laboratory Findings]] | [[Thin basement membrane disease CT|CT]] | [[Thin basement membrane disease MRI|MRI]] | [[Thin basement membrane disease ultrasound|Ultrasound]] | [[Thin basement membrane disease other imaging findings|Other Imaging Findings]] | [[Thin basement membrane disease other diagnostic studies|Other Diagnostic Studies]]
[[Thin basement membrane disease diagnostic study of choice|Diagnostic study of choice]] | [[Thin basement membrane disease history and symptoms|History and Symptoms]] | [[Thin basement membrane disease physical examination|Physical Examination]] | [[Thin basement membrane disease laboratory findings|Laboratory findings]] | [[Thin basement membrane disease electrocardiogram|Electrocardiogram]] | [[Thin basement membrane disease x-ray findings|X-ray Findings]] | [[Thin basement membrane disease echocardiography and ultrasonographic findings|Echocardiography and Ultrasonographic Findings]] |[[Thin basement membrane disease ct scan findings|CT scan findings]] | [[Thin basement membrane disease mri findings|MRI findings]] | [[Thin basement membrane disease biopsy|Biopsy]] | [[Thin basement membrane disease other diagnostic studies|Other Diagnostic Studies]]


==Treatment==
==Treatment==


[[Thin basement membrane disease medical therapy|Medical Therapy]] | [[Thin basement membrane disease primary prevention|Primary Prevention]] | [[Thin basement membrane disease secondary prevention|Secondary Prevention]] | [[Thin basement membrane disease cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Thin basement membrane disease future or investigational therapies|Future or Investigational Therapies]]
[[Thin basement membrane disease medical therapy|Medical Therapy]] | [[Interventions]] | [[Surgery]] | [[Thin basement membrane disease primary prevention|Primary Prevention]] | [[Thin basement membrane disease secondary prevention|Secondary Prevention]] | [[Thin basement membrane disease cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Thin basement membrane disease future or investigational therapies|Future or Investigational Therapies]]


==Case Studies==
==Case Studies==
[[Thin basement membrane disease case study one|Case #1]]
[[Thin basement membrane disease case study one|Case #1]]


'''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 [[glomerulus|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.
[[Category:Disease]]
 
[[Category:Needs content]]
== 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 stone]]s 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 [[glomerulus|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 [[needle aspiration biopsy|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 [[needle aspiration biopsy|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]].{{ref|Buzza}}
 
== Treatment ==
Most patients with thin basement membrane disease need just reassurance. [[Angiotensin converting enzyme inhibitor]]s 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]].{{ref|Nieuwhof}} 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 ==
#{{note|Buzza}}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
#{{note|Nieuwhof}}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
 
 
 
[[Category:Kidney diseases]]
[[Category:Kidney diseases]]
[[Category:Nephrology]]


{{WH}}
{{WH}}
{{WikiDoc Sources}}
{{WikiDoc Sources}}

Latest revision as of 17:34, 15 December 2020

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Marufa Marium, M.B.B.S[2]

Synonyms and keywords: Thin basement membrane disease[1]; Thin membrane nephropathy[2]; thin GBM nephropathy; thin GBM syndrome; benign familial hematuria [3]; benign familial essential hematuria; congenital hereditary hematuria; hereditary hematuria; familial hematuric nephritis; benign hereditary hematuric nephritis

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Thin basement membrane disease from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Diagnostic study of choice | History and Symptoms | Physical Examination | Laboratory findings | Electrocardiogram | X-ray Findings | Echocardiography and Ultrasonographic Findings |CT scan findings | MRI findings | Biopsy | Other Diagnostic Studies

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Medical Therapy | Interventions | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

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Case #1

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  1. ↑ Takemura T, Yanagida H, Yagi K, Moriwaki K, Okada M (September 2003). "Alport syndrome and benign familial hematuria (thin basement membrane disease) in two brothers of a family with hematuria". Clin. Nephrol. 60 (3): 195–200. doi:10.5414/cnp60195. PMID 14524583.
  2. ↑ Nieuwhof CM, de Heer F, de Leeuw P, van Breda Vriesman PJ (May 1997). "Thin GBM nephropathy: premature glomerular obsolescence is associated with hypertension and late onset renal failure". Kidney Int. 51 (5): 1596–601. doi:10.1038/ki.1997.219. PMID 9150478.
  3. ↑ Marks MI, Drummond KN (October 1969). "Benign familial hematuria". Pediatrics. 44 (4): 590–3. PMID 4899625.
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