WordNet

relatively thin in consistency or low in density; not viscous; "air is thin at high altitudes"; "a thin soup"; "skimmed milk is much thinner than whole milk"; "thin oil"
lacking excess flesh; "you cant be too rich or too thin"; "Yon Cassius has a lean and hungry look"-Shakespeare (同)lean
(of sound) lacking resonance or volume; "a thin feeble cry"
lacking spirit or sincere effort; "a thin smile"
lose thickness; become thin or thinner
make thin or thinner; "Thin the solution"
of relatively small extent from one surface to the opposite or in cross section; "thin wire"; "a thin chiffon blouse"; "a thin book"; "a thin layer of paint"
the lowermost portion of a structure partly or wholly below ground level; often used for storage (同)cellar
the ground floor facade or interior in Renaissance architecture
a thin pliable sheet of material
a pliable sheet of tissue that covers or lines or connects the organs or cells of animals or plants (同)tissue layer

PrepTutorEJDIC

『薄い』 / 細い / 『やせた』,やつれた / (液体・気体が)『薄い』,希薄な / まばらな,密集していない / 内容のない,見え透いた / (音・声が)『か細い』 / 薄く,細く,まばらに / …‘を'薄くする,細くする,まばらにする / 薄くなる,細くなる,まばらになる
地階,地下室
(生物の)膜,皮膜

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/11/03 19:41:03」(JST)

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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,^[1] 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 must be differentiated from the other two common causes of glomerular hematuria, IgA nephropathy and Alport syndrome. The history and presentation are helpful in this regard:

In Alport syndrome, there is often a family history of kidney failure, which may be associated with hearing impairment. Also, males tend to be more affected as Alport syndrome is X-linked in most cases.
In IgA nephropathy, episodes of frank hematuria are more common, and a family history is less common.

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 most 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 type IV collagen have been reported in some families.^[2]^[3]

Some individuals with TBMD are thought to be carriers for genes that cause Alport syndrome.^[4]

Treatment

Most patients with thin basement membrane disease need only reassurance. Indeed, this disease was previously referred to as "benign familial hematuria" because of its usually benign course. 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.^[5]

Thin basement membrane disease may co-exist with other kidney diseases, which may in part be explained by the high prevalence of thin basement membrane disease.^[6]

References

^ Fujinaga S, Kaneko K, Ohtomo Y, et al. (February 2006). "Thin basement membrane nephropathy associated with minimal change disease in a 15-year-old boy". Pediatr. Nephrol. 21 (2): 277–80. doi:10.1007/s00467-005-2095-2. PMID 16362391.
^ Savige J, Rana K, Tonna S, Buzza M, Dagher H, Wang YY (October 2003). "Thin basement membrane nephropathy". Kidney Int. 64 (4): 1169–78. doi:10.1046/j.1523-1755.2003.00234.x. PMID 12969134.
^ Hou P, Chen Y, Ding J, Li G, Zhang H (2007). "A novel mutation of COL4A3 presents a different contribution to Alport syndrome and thin basement membrane nephropathy". Am. J. Nephrol. 27 (5): 538–44. doi:10.1159/000107666. PMID 17726307.
^ Buzza M, Wang YY, Dagher H, et al. (August 2001). "COL4A4 mutation in thin basement membrane disease previously described in Alport syndrome". Kidney Int. 60 (2): 480–3. doi:10.1046/j.1523-1755.2001.060002480.x. PMID 11473630.
^ 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.
^ Norby SM, Cosio FG (May 2005). "Thin basement membrane nephropathy associated with other glomerular diseases". Semin. Nephrol. 25 (3): 176–9. doi:10.1016/j.semnephrol.2005.01.010. PMID 15880329.

External links

GeneReviews/NCBI/NIH/UW entry on Collagen IV-Related Nephropathies (Alport Syndrome and Thin Basement Membrane Nephropathy)

UpToDate Contents

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1. 菲薄基底膜腎症（良性家族性血尿） thin basement membrane nephropathy benign familial hematuria
2. 弧発性および持続性糸球体性血尿：IgA腎症、アルポート症候群、菲薄基底膜腎症 isolated and persistent glomerular hematuria iga alport thin basement membrane nephropathy
3. 小児における顕微鏡的血尿の評価 evaluation of microscopic hematuria in children
4. 遺伝性腎炎（アルポート症候群）の遺伝学、病因、および病理 genetics pathogenesis and pathology of hereditary nephritis alport syndrome
5. 遺伝性腎炎（アルポート症候群）の臨床症状、診断および治療 clinical manifestations diagnosis and treatment of hereditary nephritis alport syndrome

English Journal

Identification of 47 novel mutations in patients with Alport syndrome and thin basement membrane nephropathy.

Weber S1, Strasser K1, Rath S2, Kittke A2, Beicht S2, Alberer M3, Lange-Sperandio B4, Hoyer PF1, Benz MR5, Ponsel S4, Weber LT5, Klein HG2, Hoefele J6,7.
Pediatric nephrology (Berlin, Germany).Pediatr Nephrol.2016 Jun;31(6):941-55. doi: 10.1007/s00467-015-3302-4. Epub 2016 Jan 25.
BACKGROUND: Alport syndrome (ATS) is a progressive hereditary nephropathy characterized by hematuria and proteinuria. It can be associated with extrarenal manifestations. In contrast, thin basement membrane nephropathy (TBMN) is characterized by microscopic hematuria, is largely asymptomatic, and is
PMID 26809805

RAAS inhibition and the course of Alport syndrome.

Savva I1, Pierides A2, Deltas C3.
Pharmacological research.Pharmacol Res.2016 Mar 16;107:205-210. doi: 10.1016/j.phrs.2016.03.017. [Epub ahead of print]
Alport syndrome (AS) is a hereditary progressive glomerulonephritis with a high life-time risk for end-stage renal disease (ESRD). Most patients will reach ESRD before the age of 30 years, while a subset of them with milder mutations will do so at older ages, even after 50 years. Frequent extrarenal
PMID 26995302

A novel FN1 variant associated with familial hematuria: TBMN?

Yuan L1, Xu H1, Yuan J2, Deng X1, Xiong W3, Yang Z1, Huang Y1, Deng H4.
Clinical biochemistry.Clin Biochem.2016 Mar 9. pii: S0009-9120(16)00088-6. doi: 10.1016/j.clinbiochem.2016.01.026. [Epub ahead of print]
OBJECTIVE: Thin basement membrane nephropathy (TBMN), an autosomal dominant inherited condition in general, is characterized clinically by persistent hematuria and pathologically by thinning of glomerular basement membrane. TBMN is occasionally accompanied with proteinuria, hypertension and renal im
PMID 26968105

Japanese Journal

Morphological diagnosis of Alport syndrome and thin basement membrane nephropathy by low vacuum scanning electron microscopy

OKADA Shinichi,INAGA Sumire,KITAMOTO Koichi,KAWABA Yasuo,NAKANE Hironobu,NAGURO Tomonori,KAIDOH Toshiyuki,KANZAKI Susumu
Biomedical Research 35(5), 345-350, 2014
… Alport syndrome (AS) and thin basement membrane nephropathy (TBMN) are genetic disorders caused by mutations of the type IV collagen genes COL4A3, COL4A4, and/or COL4A5. … We here aimed to investigate the three-dimensional ultrastructure of the glomerular basement membrane (GBM) in order to introduce a novel method of diagnosing AS and TBMN. …
NAID 130004701685

常染色体性 Alport 症候群

岡政史,野津寛大,飯島一誠,松尾雅文
日本小児腎臓病学会雑誌 = Japanese journal of pediatric nephrology 23(1), 8-12, 2010-04-15
　Alport症候群 (AS) は感音性難聴を伴う遺伝性進行性腎疾患である。高頻度に末期腎不全に至るとされ臨床的に重要な疾患であるが，その臨床像，自然予後は十分には解明されていない。今回，分子遺伝学的特徴および近年報告された家族性良性血尿症候群との関連について，常染色体性Alport症候群を中心に概要をまとめる。
NAID 10026412082

ＣＯＬ４Ａ３／ＣＯＬ４Ａ４のヘテロ接合体変異を有する菲薄基底膜腎症のドナーから生体腎移植を実施した常染色体劣性アルポート症候群の２例

梶保祐子,上田博章,水谷誠,谷口貴実子,古山政幸,石塚喜世伸,藤井寛,近本裕子,秋岡祐子,岡政史,野津寛大,飯島一誠,服部元史
Nihon Shoni Jinzobyo Gakkai Zasshi 23(2), 113-118, 2010
… 　血尿を特徴とする菲薄基底膜腎症 (thin basement membrane nephropathy: TBMN) は，従来は腎不全に至る率の低い予後良好な疾患と考えられてきた。 …
NAID 130000814249

Related Pictures

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