WordNet

the physical condition of blocking or filling a passage with an obstruction (同)blockage
any structure that makes progress difficult (同)obstructor, obstructer, impediment, impedimenta
getting in someones way
the act of obstructing; "obstruction of justice"
a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"
tiny endothelium-lined passages for blood in the tissue of an organ
having a succession of waves or curves

PrepTutorEJDIC

〈U〉(…への)妨害,障害《+『to』+『名』》 / 〈C〉(…への)妨害物,障害物《+『to』+『名』》
(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/12/26 16:27:44」(JST)

wiki en

Hepatic veno-occlusive disease or veno-occlusive disease (VOD) is a condition in which some of the small veins in the liver are obstructed. It is a complication of high-dose chemotherapy given before a bone marrow transplant (BMT) and is marked by weight gain due to fluid retention, increased liver size, and raised levels of bilirubin in the blood.^[1] The name sinusoidal obstruction syndrome is now preferred if VOD happens as a result of chemotherapy or bone marrow transplantation.^[1]^[2]

Apart from chemotherapy, VOD may also occur after ingestion of certain plant alkaloids such as pyrrolizidine alkaloids (in some herbal teas),^[1] and has been described as part of a rare hereditary disease called hepatic venoocclusive disease with immunodeficiency (which results from mutations in the gene coding for a protein called SP110).^[3]

Signs and symptoms

Features of VOD include weight gain, tender hepatomegaly, ascites, and increased bilirubin. It often is associated with renal failure.

Diagnosis

Hepatic doppler ultrasound is typically utilized to confirm or suggest the diagnosis. Most common findings on liver doppler ultrasound include increased phasicity of portal veins with eventual development of portal flow reversal. The liver is usually enlarged but maintained normal echogenicity. A liver biopsy is required for a definitive diagnosis.

Pathophysiology

In the BMT setting, VOD is felt to be due to injury to the hepatic venous endothelium from the conditioning regimen.

Toxic agents causing veno-occlusive disease include plants as well as the medication cyclophosphamide.

Treatment

Treatment for VOD is primarily supportive. In the BMT setting, defibrotide is an investigational treatment that may be promising. Defibrotide is a polydeoxyribonucleotide isolated from pig intestine. Although its mechanism of action in VOD is unclear, the drug is believed to have antithrombotic properties. In August 2009, Gentium S.p.A., which sponsored the phase 3 clinical trial (pivotal) of defibrotide in hepatic VOD, announced disappointing results. Further clinical development of defibrotide for this indication is uncertain.

Prognosis

When associated with bone marrow transplant, VOD is fatal in over 30% of cases. Cases due to plant alkaloids often have a longer and more unpredictable course.

History

The first report on veno-occlusive disease, in 1920, was as a result of senecio poisoning in South Africa.^[4] Subsequent reports were mostly in Jamaicans who had consumed herbal teas.^[1] With the advent of bone marrow transplanation, most later reported cases have been in those undergoing treatment for leukemia.^[1]

References

^ ^a ^b ^c ^d ^e Helmy A (January 2006). "Review article: updates in the pathogenesis and therapy of hepatic sinusoidal obstruction syndrome". Aliment. Pharmacol. Ther. 23 (1): 11–25. doi:10.1111/j.1365-2036.2006.02742.x. PMID 16393276. http://www3.interscience.wiley.com/cgi-bin/fulltext/118572108/HTMLSTART.
^ DeLeve LD, Shulman HM, McDonald GB (February 2002). "Toxic injury to hepatic sinusoids: sinusoidal obstruction syndrome (veno-occlusive disease)". Semin. Liver Dis. 22 (1): 27–42. doi:10.1055/s-2002-23204. PMID 11928077.
^ Roscioli T, Cliffe ST, Bloch DB, (June 2006). "Mutations in the gene encoding the PML nuclear body protein Sp110 are associated with immunodeficiency and hepatic veno-occlusive disease". Nat. Genet. 38 (6): 620–2. doi:10.1038/ng1780. PMID 16648851.
^ Willmot, F; Robertson, George (1920). "Senecio disease, or cirrhosis of the liver due to senecio poisoning". The Lancet 196 (5069): 848. doi:10.1016/S0140-6736(01)00020-4.

UpToDate Contents

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1. 造血細胞移植後の肝類洞閉塞症候群（静脈閉塞性疾患）の診断 diagnosis of hepatic sinusoidal obstruction syndrome veno occlusive disease following hematopoietic cell transplantation
2. 造血細胞移植後の肝類洞閉塞症候群の治療および予防 treatment and prevention of hepatic sinusoidal obstruction syndrome following hematopoietic cell transplantation
3. 肝肥大：鑑別診断および評価 hepatomegaly differential diagnosis and evaluation
4. 肝疾患を有する患者における化学療法による肝毒性および用量調整 chemotherapy hepatotoxicity and dose modification in patients with liver disease
5. バッドキアリ症候群の病因 etiology of the budd chiari syndrome

English Journal

A randomized comparison of cyclophosphamide vs. reduced dose cyclophosphamide plus fludarabine for allogeneic hematopoietic cell transplantation in patients with aplastic anemia and hypoplastic myelodysplastic syndrome.

Kim H, Lee JH, Joo YD, Bae SH, Hyun MS, Lee JH, Kim DY, Lee WS, Ryoo HM, Kim MK, Park JH, Lee KH; Cooperative Study Group A for Hematology (COSAH).SourceDivision of Hematology-Oncology, Ulsan University Hospital University of Ulsan College of Medicine, Ulsan, Republic of Korea.
Annals of hematology.Ann Hematol.2012 Sep;91(9):1459-69. Epub 2012 Apr 18.
Recently, a less toxic regimen comprising reduced cyclophosphamide (Cy), fludarabine, and anti-thymocyte globulin (ATG) (Cy-Flu-ATG) was used to condition high-risk patients scheduled for allogeneic hematopoietic cell transplantation (alloHSCT) instead of standard Cy-ATG in patients with severe apla
PMID 22526363

Successful cord blood transplantation in a patient with malignant infantile osteopetrosis and hemophilia.

Buchbinder D, Steward CG, Puthenveetil G, Nugent D, Hsieh L, Kirov I, Neudorf S, Soni A.SourceDivision of Hematology, CHOC Children's Hospital, Orange, CA, USA Division of Hematology, Royal Hospital for Children, Bristol, UK Division of Oncology, CHOC Children's Hospital, Orange, CA, USA.
Pediatric transplantation.Pediatr Transplant.2012 Aug 23. doi: 10.1111/j.1399-3046.2012.01758.x. [Epub ahead of print]
Buchbinder D, Steward CG, Puthenveetil G, Nugent D, Hsieh L, Kirov I, Neudorf S, Soni A. Successful cord blood transplantation in a patient with malignant infantile osteopetrosis and hemophilia. Abstract: MIOP is a congenital disorder of osteoclast differentiation or dysfunction. Inadequate bone
PMID 22913475

Japanese Journal

Comparison of direct costs for allogeneic bone marrow transplantation from unrelated donors and umbilical cord blood transplantation for childhood acute lymphoblastic leukemia in Japan

羽賀洋一,松本邦愛,小原明,佐地勉,長谷川友紀
東邦医学会雑誌 60(2), 76-84, 2013-03-00
… Seven adverse events associated with transplantation were analyzed: severe bacterial infection, severe fungal infection, cytomegalovirus infection, herpes virus infection, graft failure, sinusoidal obstruction syndrome, and acute graft-versus-host disease. …
NAID 120005306377

Sorafenib attenuates monocrotaline-induced sinusoidal obstruction syndrome in rats through suppression of JNK and MMP-9.

Nakamura Kojiro,Hatano Etsuro,Narita Masato,Miyagawa-Hayashino Aya,Koyama Yukinori,Nagata Hiromitsu,Iwaisako Keiko,Taura Kojiro,Uemoto Shinji
Journal of hepatology 57(5), 1037-1043, 2012-11-00
… [Background & Aims]: Sinusoidal obstruction syndrome (SOS) is a drug-induced liver injury that occurs with oxaliplatin treatment and is associated with postoperative morbidity after hepatectomy. … Survival after hepatectomy was examined and immunohistochemistry and electron microscopy were performed to assess sinusoidal injury. …
NAID 120004920432

Fatal hepatic sinusoidal obstruction syndrome in a child with primary CNS lymphoma during induction therapy

YAMAMOTO Shohei,AKIYAMA Kousuke,OYAMA Nobuo,HAYASHI Mayumi,FUJIMOTO Yoko,IKEDA Hirokazu,ISOYAMA Keiichi
International journal of hematology 96(2), 284-286, 2012-08-01
NAID 10031043160

Related Pictures

★リンクテーブル★

リンク元	「類洞閉塞症候群」「hepatic veno-occlusive disease」
関連記事	「obstruction」「sinusoid」「sinusoidal」「syndrome」

「類洞閉塞症候群」

Hepatic veno-occlusive disease
	Classification and external resources
ICD-10	K76.5
OMIM	235550
DiseasesDB	34365
eMedicine	ped/2396
MeSH	D006504
GeneReviews	Venous Occlusive Disease with Immunodeficiency;

　　[★]

英: sinusoidal obstruction syndrome
関: 肝中心静脈閉塞症

「hepatic veno-occlusive disease」

　　[★]

肝中心静脈閉塞症

関: sinusoidal obstruction syndrome

「obstruction」

　　[★]

n.

妨害、障害、支障、差し障り。妨害物、
閉塞、閉塞症。

関: blockage、deterrent、obliteration、occlude、occlusion、stasis

「sinusoid」

　　[★]

n.

シヌソイド、類洞、ジヌソイド、洞様血管、洞様毛細血管

「sinusoidal」

　　[★]

類洞の、正弦の

関: sinusoid

「syndrome」

　　[★]

n.

症候群

[Helmy-1] Helmy A (January 2006). "Review article: updates in the pathogenesis and therapy of hepatic sinusoidal obstruction syndrome". Aliment. Pharmacol. Ther. 23 (1): 11–25. doi:10.1111/j.1365-2036.2006.02742.x. PMID 16393276. http://www3.interscience.wiley.com/cgi-bin/fulltext/118572108/HTMLSTART.

[2] DeLeve LD, Shulman HM, McDonald GB (February 2002). "Toxic injury to hepatic sinusoids: sinusoidal obstruction syndrome (veno-occlusive disease)". Semin. Liver Dis. 22 (1): 27–42. doi:10.1055/s-2002-23204. PMID 11928077.

[3] Roscioli T, Cliffe ST, Bloch DB, (June 2006). "Mutations in the gene encoding the PML nuclear body protein Sp110 are associated with immunodeficiency and hepatic veno-occlusive disease". Nat. Genet. 38 (6): 620–2. doi:10.1038/ng1780. PMID 16648851.

[4] Willmot, F; Robertson, George (1920). "Senecio disease, or cirrhosis of the liver due to senecio poisoning". The Lancet 196 (5069): 848. doi:10.1016/S0140-6736(01)00020-4.

匿名

検索

案内

案内

sinusoidal obstruction syndrome