WordNet

not dangerous to health; not recurrent or progressive (especially of a tumor)
pleasant and beneficial in nature or influence; "a benign smile"; "the benign sky"; "the benign influence of pure air" (同)benignant
kindness of disposition or manner; "the benign ruler of millions"; "benign intentions"
relating to or having the characteristics of a family; "children of the same familial background"; "familial aggregation"
occurring among members of a family usually by heredity; "an inherited disease"; "familial traits"; "genetically transmitted features" (同)genetic, hereditary, inherited, transmitted, transmissible
the presence of blood in the urine; often a symptom of urinary tract disease (同)haematuria

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(心・表情などが)親切な,優しい / (事情・環境・気候などが)好適な,好都合な / 悪性でない
家族の,家族特有の / 違伝的な,血統にあらわれる

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/06/17 09:23:07」(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. 糸球体疾患の鑑別診断および評価 differential diagnosis and evaluation of glomerular disease
5. 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

English Journal

Hemangiomas In Kidneys With End-Stage Renal Disease: A Novel Clinicopathological Association.

Kryvenko ON1, Haley SL, Smith SC, Shen SS, Paluru S, Gupta NS, Jorda M, Epstein JI, Amin MB, Truong LD.
Histopathology.Histopathology.2014 Feb 18. doi: 10.1111/his.12394. [Epub ahead of print]
AIM: Study hemangiomas in end-stage renal disease (ESRD).METHODS AND RESULTS: Twenty ESRD nephrectomies from 16 patients (9 months to 68 years old) were due to hypertension (4), focal segmental glomerulosclerosis (4), lupus nephritis (3), diabetes (1), IgA nephropathy (1), hereditary nephritis (1),
PMID 24548339

A founder mutation in COL4A3 causes autosomal recessive Alport syndrome in the Ashkenazi Jewish population.

Webb B1, Brandt T, Liu L, Jalas C, Liao J, Fedick A, Linderman M, Diaz G, Kornreich R, Trachtman H, Mehta L, Edelmann L.
Clinical genetics.Clin Genet.2013 Aug 8. doi: 10.1111/cge.12247. [Epub ahead of print]
Alport syndrome is an inherited progressive nephropathy arising from mutations in the type IV collagen genes, COL4A3, COL4A4, and COL4A5. Symptoms also include sensorineural hearing loss and ocular lesions. We determined the molecular basis of Alport syndrome in a non-consanguineous Ashkenazi Jewish
PMID 23927549

How benign is hematuria? Using genetics to predict prognosis.

Gale DP.
Pediatric nephrology (Berlin, Germany).Pediatr Nephrol.2013 Aug;28(8):1183-93. doi: 10.1007/s00467-012-2399-y. Epub 2013 Jan 17.
Hematuria is a common presenting feature of glomerular disease and is sometimes associated with kidney failure later in life. Where isolated microscopic hematuria occurs in children and young adults, an underlying monogenic disorder, such as Alport syndrome or thin basement membrane nephropathy, is
PMID 23325022

Japanese Journal

Ａｌｐｏｒｔ症候群と良性家族性血尿の遺伝子変異と臨床像："ＩＶ型コラーゲン関連腎症"という概念の提唱

田中幸代,蓮井正史,野津寛大,飯島一誠,杉本圭相,竹村司,金子一成
Nihon Shoni Jinzobyo Gakkai Zasshi 23(2), 172-178, 2010
　Alport症候群 (Alport sndrome: AS) と良性家族性血尿 (bnign fmilial hmaturia: BFH) は，比較的頻度の高い遺伝性腎疾患である。前者の腎機能予後が不良であるのに対し，後者は，予後良好で，その臨床像は対照的である。近年，ASの多くはX染色体に位置する遺伝子・COL4A5の変異で，また，BFHの半数近くは2番染色体に位置する遺伝子・COL4A3やC …
NAID 130000814260

Clinical Study of 3-year-old Children with Urinary Abnormalities.

丸山健一
Ｔｈｅ　ＫＩＴＡＫＡＮＴＯ　Ｍｅｄｉｃａｌ　Ｊｏｕｒｎａｌ 52(2), 107-110, 2002
… Of these, 87 had hematuria, 6 proteinuria, 10 leukocyturia, and 1 both hematuria and proteinuria.<BR>Results : Among children with hematuria, 7 have benign familial hematuria, 1 nut-cracker phenomenon, and 1 idiopathic hypercalciuria. … The remaining 78 were diagnosed with asymptomatic hematuria. …
NAID 130000874570

千葉市の学校集団検尿についての考察

熊谷久美子,土屋尚義,村越康一,クマガヤクミコ,ツチヤタカノリ,ムラコシコウイチ,Kumagaya Kumiko,Tsuchiya Takanori,Murakoshi Koichi
千葉大学教育学部研究紀要. 第2部 27, 197-216, 1978-12-20
… These are classified IO groups: normal, benign albuminuria, micro-hematuria, a symptomatic hematuria, probable nephritis, acute nephritis, chronic nephritis, nephrosis, urogenital tract infection and others. … But we understand that many factors other than those mentioned above, such as environmental factors and familial relationship have marked influences to the findings of the pathological urine. …
NAID 110004715186

Related Pictures

★リンクテーブル★

リンク元	「菲薄基底膜病」
関連記事	「familial」「benign」

「菲薄基底膜病」

Thin basement membrane disease
Classification and external resources
ICD-10	N05.8
DiseasesDB	5363
MedlinePlus	003524
eMedicine	med/149

　　[★]

英: thin basement membrane disease
同: 家族性良性血尿良性家族性血尿 benign familial hematuria; 家族性糸球体基底膜菲薄症 familial thin glomerular basement membrane disease; 家族性基底膜菲薄症 familial thin basement membrane disease

[show details]

菲薄基底膜症候群 : 約 315 件
菲薄基底膜病 : 約 2,870 件

「familial」

　　[★]

adj.

家族性の、家系性の、家族の

関: family、family member、household、kindred

「benign」

　　[★]

adj.

良性の、穏和な

関: benignant、mild、mildly

[pmid16362391-1] 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.

[pmid12969134-2] 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.

[pmid17726307-3] 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.

[pmid11473630-4] 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.

[pmid9150478-5] 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.

[pmid15880329-6] 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.

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案内

benign familial hematuria