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home - Liver - Vascular Problems - Veno Occlusive Disease Written by Dr Sebastian Zeki
Knowledge


Understands the risk of variceal bleeding as a complication of with
portal hypertension


Knows risk of variceal haemorrhage in cirrhotics who have not bled

Knows risk of bleeding related to variceal size endoscopic findings
and severity of liver dysfunction


Knows range of therapeutic options (both endoscopic and
pharmacological).

Skills
Recognises and can treat portal hypertension.
Behaviours
Manages patients with oesophageal varices with skill and
compassion

Able to convey the serious risks to patients and their relatives.

Also...

Knowledge


Recognises and shows understanding of vascular liver disease
including Budd-Chiari syndrome veno-occlusive disease and
portomesenteric venous thrombosis; understands the underlying
anatomy and physiology of these often complex conditions

Aware of need for investigation for associated myeloproliferative and
procoagulant conditions

Understands the role of anticoagulation and indications for further
intervention including TIPS surgery or transplantation

Skills
Can make careful clinical of these conditions and has
heightened awareness of liver vascular disease in differential
diagnosis

Able to make a potentially difficult diagnosis of less common variants
of vascular conditions

Behaviours
Shows ability to keep patient and relatives informed and to refer
appropriately for specialist management

Veno Occlusive Disease

Risk factors for disease progression and death:Serum bilirubin and % of weight gain within 1 to 2 weeks of transplantation.Poor renal function.Acute rise in the D-dimer.History of viral hepatitis, and haemochromatosis allele carrier (C282Y).Polymorphisms of the glutathione S-transferase gene.Previous treatment also has an impact of the risk of developing VOD.Prior use of Gemtuzumab ozogamicin (Myelotarg). The two major modalities that have been assessed for the treatment of VOD are human tissue-type plasminogen activator and defibrotide.AlteplaseResponse rate of 35% Rarely used as significant risk of hemorrhage especially if already have multi-organ failureDefibrotideDefibrotide (DF) is a polydeoxyribonucleotide derived from mammalian tissue with multiple antithrombotic, fibrinolytic, and angiogenic properties.Haas little systemic anticoagulant activity, suggesting that it might have a therapeutic advantage over the use of altplase and heparin.AntithrombinAntithrombin (AT) concentrate has been used in patients with VOD who have documented deficiency of AT.ProstaglandinsProstaglandin E1 Prostaglandin E1, which has both vaso-dilator and antiplatelet activity, and low-dose heparin were used to successfully treat three infants with VOD confirmed by duplex ultrasound.GlutathioneTo the extent that free radical damage is an important component of VOD, glutathione depletion may occur.Glutamine infusion in animal studies has been shown to maintain glutathione levels.Transjugular intrahepatic portosystemic stent-shuntMild disease more likely to respondLong-term survival appears to be uncommon, but has been reported.Liver transplantationHas been done successfully, but patients usually too unwell Prevention:Ursodeoxycholic acid- Reduces the incidence of VOD, and possibly reduces mortality.Heparin-unproven benefit.Defibrotide- May be useful but awaits RCT.Peripheral blood progenitor cells-Use of these as stem cell source for patients undergoing autolo-gous transplant can reduce the likelihood of devel-oping VOD.T-cell depleted donor marrow-can reduce incidence of VOD. Hepatic sinusoidal obstruction syndrome (veno-occlusive disease) following hematopoietic cell transplantation. Treatment PrognosisSurvival at day +100 was 91 % with mild VOD and 77 % with moderate disease.Recovery is usually without complications. Written by Dr Sebastian Zeki Incidence54 % of allogeneic transplants.40% of autologous transplants.30 % of cases are severe, and mortality in these patients is almost universal with fulminant acute liver failure.

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