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Budd-Chiari syndrome

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In medicine(gastroenterologyand hepatology), Budd-Chiari syndrome is the clinical picture caused by occlusionof the hepatic vein.

Inhaltsverzeichnis

  • 1 Signs and symptoms
  • 2 Diagnosis
  • 3 Causes
  • 4 Pathophysiology
  • 5 Treatment

Signs and symptoms

The syndrome presents with rapidly progressive abdominal pain, hepatomegaly(enlarged liver), ascites, and later the symptoms of hepatic dysfunction: elevated liver enzymes, encephalopathy.

A slower-onset form of hepatic venous occlusion is also recognised; this can be painless.

Often, the patient is known to have a tendency towards thrombosis, while Budd-Chiari syndrome can also be the first symptom of such a tendency.

Diagnosis

When Budd-Chiari syndrome is suspected, measurements are made of liver enzymelevels and other organ markers (creatinine, urea, electrolytes, LDH).

Budd-Chiari syndrome is diagnosed using ultrasoundstudies of the abdomen, although occasionally more invasive methods have to be used (retrograde angiography). Liver biopsyis sometimes necessary to differentiate between Budd-Chiari syndrome and other causes of hepatomegaly and ascites, such as galactosemiaor Reye's syndrome.

Causes

  • Primary (75%): thrombosisof the hepatic vein
  • Secondary (25%): compression of the hepatic vein by an outside structure (e.g. a tumor)

Many patients (10-40%) have Budd-Chiari syndrome as a complication of polycythemia vera(myeloproliferative diseaseof red blood cells). Patients suffering from paroxysmal nocturnal hemoglobinuria(PNH) appear to be especially at risk for Budd-Chiari syndrome, more than other forms of thrombophilia: up to 40% develops Budd-Chiari, as well as cerebrovascular accidents.

A related condition is veno-occlusive disease, which occurs in recipients of bone marrow transplantsas a complication of their medication. Although its mechanism is similar, it is not considered a form of Budd-Chiari syndrome.

Pathophysiology

Any obstruction of the venousvasculature of the liver is referred to as Budd-Chiari syndrome, from the venulesto the right atrium.

Treatment

Treatment is with anticoagulantmedication, generally unfractioned heparinand warfarin.


Health science- Medicine- Gastroenterology
Diseases of the esophagus- stomach
Halitosis- Nausea- Vomiting- GERD- Achalasia- Esophageal cancer- Esophageal varices- Peptic ulcer- Abdominal pain- Stomach cancer- Functional dyspepsia
Diseases of the liver- pancreas- gallbladder- biliary tree
Hepatitis- Cirrhosis- NASH- PBC- PSC- Budd-Chiari syndrome - Hepatocellular carcinoma- Acute pancreatitis- Chronic pancreatitis- Pancreatic cancer- Gallstones- Cholecystitis
Diseases of the small intestine
Peptic ulcer- Intussusception- Malabsorption(e.g. celiac disease, lactose intolerance, fructose malabsorption, Whipple's disease) - Lymphoma
Diseases of the colon
Diarrhea- Appendicitis- Diverticulitis- Diverticulosis- IBD(Crohn's diseaseand Ulcerative colitis) - Irritable bowel syndrome- Constipation- Colorectal cancer- Hirschsprung's disease- Pseudomembranous colitis
nl:Budd-Chiari-syndroom
Retrieved from "http://en.wikipedia.org/Budd-Chiari_syndrome"



This article is licensed under the GNU Free Documentation License.
It uses material from the http://en.wikipedia.org/wiki/Budd-Chiari+syndrome Wikipedia article Budd-Chiari syndrome.

 
  All text is available under the terms of the GNU Free Documentation License