In medicine (gastroenterology and hepatology), Budd-Chiari syndrome is the clinical picture caused by occlusion of the hepatic vein.

Contents

1 Signs and symptoms 2 Analysis 3 Diagnosis 4 Treatment 5 Causes 6 Pathophysiology

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.

Analysis

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

Diagnosis

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

Treatment

With anticoagulant medication, generally unfractioned heparin and warfarin.

Causes

• Primary (75%): thrombosis of 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 disease of 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.

Pathophysiology

Any obstruction of the venous vasculature of the liver is referred to as Budd-Chiari syndrome, from the venules to the right atrium.

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 - Pancreatitis - Pancreatic cancer - Gallstones - Cholecystitis

Diseases of the small intestine

Peptic ulcer - Malabsorption (e.g. celiac disease, lactose intolerance, fructose malabsorption, Whipple's disease) - Lymphoma

Diseases of the colon

Diarrhea - Appendicitis - Diverticulitis - Diverticulosis - IBD (Crohn's disease and Ulcerative colitis) - Irritable bowel syndrome - Constipation - Colorectal cancer - Hirschsprung's disease - Pseudomembranous colitis