| 词条 | Hepatic veno-occlusive disease |
| 释义 |
| name = Hepatic veno-occlusive disease | synonyms = | image = | caption = | pronounce = | field = | symptoms = | complications = | onset = | duration = | types = | causes = | risks = | diagnosis = | differential = | prevention = | treatment = | medication = | prognosis = | frequency = | deaths = }}Hepatic veno-occlusive disease or veno-occlusive disease with immunodeficiency (VODI) 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 preferred if VODI happens as a result of chemotherapy or bone marrow transplantation.[1][2] Apart from chemotherapy, VODI 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 symptomsFeatures of VODI include weight gain, tender enlargement of the liver, ascites, and yellow discoloration of the skin; it often is associated with acute kidney failure.[4] PathophysiologyIn the BMT setting, VODI 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. DiagnosisDoppler ultrasound of the liver is typically utilized to confirm or suggest the diagnosis. 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.{{citation needed|date=December 2016}} TreatmentTreatment generally includes supportive care including pain management and possibly diuretics.[5] In those with severe disease due to a bone marrow transplant, defibrotide is a proposed treatment.[8] It has been approved for use in severe cases in Europe and the United States.[6][7] A placebo controlled trial, however, has not been done as of 2016.[5] PrognosisMild disease has a risk of death of about 10% while moderate disease has a risk of death of 20%.[5] When it occurs as a result of bone marrow transplant and multiorgan failure is present, the risk of death is greater than 80%.[8] HistoryThe first report on veno-occlusive disease, in 1920, was as a result of senecio poisoning in South Africa.[9] 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] See also
References1. ^1 2 3 4 {{cite journal |author=Helmy A |title=Review article: updates in the pathogenesis and therapy of hepatic sinusoidal obstruction syndrome (SOS) |journal=Aliment. Pharmacol. Ther. |volume=23 |issue=1 |pages=11–25 |date=January 2006 |pmid=16393276 |doi=10.1111/j.1365-2036.2006.02742.x |url=http://www3.interscience.wiley.com/cgi-bin/fulltext/118572108/HTMLSTART|archive-url=https://archive.today/20121209144102/http://www3.interscience.wiley.com/cgi-bin/fulltext/118572108/HTMLSTART|dead-url=yes|archive-date=2012-12-09}} 2. ^{{cite journal |vauthors=DeLeve LD, Shulman HM, McDonald GB |title=Toxic injury to hepatic sinusoids: sinusoidal obstruction syndrome (veno-occlusive disease) |journal=Semin. Liver Dis. |volume=22 |issue=1 |pages=27–42 |date=February 2002 |pmid=11928077 |doi=10.1055/s-2002-23204}} 3. ^{{cite journal |vauthors=Roscioli T, Cliffe ST, Bloch DB |title=Mutations in the gene encoding the PML nuclear body protein Sp110 are associated with immunodeficiency and hepatic veno-occlusive disease |journal=Nat. Genet. |volume=38 |issue=6 |pages=620–2 |date=June 2006 |pmid=16648851 |doi=10.1038/ng1780 |url=https://eprints.soton.ac.uk/400997/1/roscioli_ng_06_sp110.pdf }} 4. ^{{Cite web|url=http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=890|title=Orphanet: Hepatic veno occlusive disease|last=RESERVED|first=INSERM US14 -- ALL RIGHTS|website=www.orpha.net|access-date=2016-12-26}} 5. ^1 2 {{cite journal|last1=Dalle|first1=JH|last2=Giralt|first2=SA|title=Hepatic Veno-Occlusive Disease after Hematopoietic Stem Cell Transplantation: Risk Factors and Stratification, Prophylaxis, and Treatment.|journal=Biology of Blood and Marrow Transplantation : Journal of the American Society for Blood and Marrow Transplantation|date=March 2016|volume=22|issue=3|pages=400–9|doi=10.1016/j.bbmt.2015.09.024|pmid=26431626}} 6. ^{{cite journal|last1=Keating|first1=GM|title=Defibrotide: a review of its use in severe hepatic veno-occlusive disease following haematopoietic stem cell transplantation.|journal=Clinical Drug Investigation|date=December 2014|volume=34|issue=12|pages=895–904|doi=10.1007/s40261-014-0242-x|pmid=25351934}} 7. ^{{cite web|title=Press Announcements - FDA approves first treatment for rare disease in patients who receive stem cell transplant from blood or bone marrow|url=https://www.fda.gov/newsevents/newsroom/pressannouncements/ucm493225.htm|website=www.fda.gov|accessdate=18 July 2017|language=en}} 8. ^1 {{cite journal|last1=Fulgenzi|first1=A|last2=Ferrero|first2=ME|title=Defibrotide in the treatment of hepatic veno-occlusive disease.|journal=Hepatic Medicine: Evidence and Research|date=2016|volume=8|pages=105–113|doi=10.2147/HMER.S79243|pmid=27843363|pmc=5098529}} 9. ^{{cite journal | doi=10.1016/S0140-6736(01)00020-4 | title=Senecio disease, or cirrhosis of the liver due to senecio poisoning | year=1920 | author=Willmot, F | journal=The Lancet | volume=196 | pages=848–849 | last2=Robertson | first2=George | issue=5069}} Further reading
External links{{Medical resources| DiseasesDB = 34365 | ICD10 = {{ICD10|K|76|5|k|70}} | ICD9 = | ICDO = | OMIM = 235550 | MedlinePlus = | eMedicineSubj = ped | eMedicineTopic = 2396 | MeshID = D006504 | GeneReviewsNBK = NBK1271 | GeneReviewsName = Venous Occlusive Disease with Immunodeficiency }}{{Vascular diseases}}{{Gastroenterology}}{{DEFAULTSORT:Hepatic Veno-Occlusive Disease}} 3 : Hepatology|Blood disorders|Rare diseases |
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