GI & Surgery 7

oelomar's version from 2016-02-27 15:25


Question Answer
What is portal hypertension?Hypertension in the portal venous system. Portal hypertension is defined as elevation of hepatic venous pressure gradient (i.e. the difference in pressure between the portal vein and the IVC) to >5mmHg, and is considered to be clinically significant when it exceeds 10mmHg.
What are the clinical features of portal hypertension?Patients are often asymptomatic and the only clinical evidence of portal hypertension is splenomegaly. Clinical features of chronic liver disease are usually present. Presenting features may include ascites, encephalopathy, oesophageal varices, haematemesis or melaena (due to rupture of gastro-oesophageal varices), and breathlessness.
What role do beta-blockers play in portal hypertension?They are used as prophylactic treatment to prevent variceal bleeding.
What is Budd-Chiari syndrome and what does it present with?A condition caused by occlusion of the hepatic veins that drain the liver (75% due to thrombosis, 25% due to compression by an outside structure e.g. tumour). It presents with a classic triad of liver enlargement, ascites, and abdominal pain.
How is Budd-Chiari syndrome treated?In the acute situation, thrombolytic therapy can be given. Ascites should be treated, as should any underlying cause. Otherwise, liver transplantation is the treatment of choice for chronic Budd–Chiari syndrome and for the fulminant form. Lifelong anticoagulation is mandatory following transplantation.
How is ascites managed?Bed rest with fluid restriction and a low salt diet are employed. Spironolactone is also given and if response is poor furosemide is added. Paracentesis can also be used to relieve symptomatic tense ascites (ascites in cirrhotic patients). A TIPS (shunt) can be used for resistant ascites (ascites that cannot be mobilised by medical treatment).
What is paracentesis and what are its main complications?The perforation of a cavity of the body or of a cyst or similar outgrowth, especially with a hollow needle, to remove fluid or gas (e.g. in ascites). Its main complications are hypovolaemia and renal dysfunction.
What is a transjugular intrahepatic shunt (TIPS) and what is it used for?An artificial channel within the liver that connects the hepatic portal vein (conducts blood from GI tract to liver) to the hepatic veins (conduct blood from the liver to the IVC). It is used in the treatment of portal hypertension.
What is a portacaval shunt and what is it used for?A connection between the hepatic portal vein (conducts blood from the GI tract and spleen to the liver) and the IVC. It is used as treatment for portal hypertension.
What is spontaneous bacterial peritonitis and in whom does it occur?Spontaneous bacterial peritonitis (SBP) is the development of peritonitis despite the absence of an obvious source for the infection. It occurs almost exclusively in people with portal hypertension, usually as a result of cirrhosis of the liver. It can also occur in patients with nephrotic syndrome.
How is spontaneous bacterial peritonitis diagnosed?Paracentesis. If the fluid contains bacteria or large numbers of neutrophil granulocytes, infection is confirmed.
What are the signs/symptoms of hepatic encephalopathy?Drowsiness, hyperreflexia and increased tone (always), foetor hepaticus (a faecal smell to the breath), flapping tremor, constructional apraxia (inability to write or draw), decreased mental function. Patient could be comatose.
What is the West Haven Criteria and how is it graded?It is a criteria used in the grading of hepatic encephalopathy. There are 4 grades which are as follows: Grade 1: trivial lack of awareness, shortened attention span, sleep disturbances. Grade 2: increasing drowsiness, confusion, slurred speech. Grade 3: Stupor, significant confusion. Grade 4: Coma.
What may an EEG show in a patient with hepatic encephalopathy?EEG shows a decrease in the frequency of the normal α-waves (8–13 Hz) to delta-waves of 1.5-3 Hz. These changes occur before coma occurs.
How is hepatic encephalopathy managed?The precipitating cause should be identified and removed. Lactulose is also given to reduce ammonia absorption and therefore empty the bowels of nitrogenous substances. Antibiotics are given for infections, and IV fluids are given as necessary.
What is the prognosis of hepatic encephalopathy?In acute encephalopathy in acute liver failure, the prognosis is very poor as the disease itself has a high mortality. In cirrhosis, chronic hepatic encephalopathy is very variable and adversely affects prognosis.
What is porto-pulmonary hypertension?The coexistence of portal and pulmonary hypertension which is a serious complication of liver disease.


Question Answer
What is primary biliary cirrhosis?Also known as primary biliary cholangitis, this is a chronic autoimmune disorder of the liver in which there is progressive destruction of the small bile ducts in the liver. Bile and other toxins build up in the liver as a result of the damage, and this can lead to cirrhosis over time.
What is the female:male ratio in primary biliary cirrhosis?9:1.
What are the signs/symptoms of primary biliary cirrhosis?May be asymptomatic. Pruritus is classically the earliest symptom, preceding jaundice by a few years. Fatigue may accompany pruritus. When jaundice appears, hepatomegaly is usually found. Xanthelasma (yellowish fat deposits underneath the skin on/around eyelids) may also be found.
What is the association between primary biliary cirrhosis and autoimmune disorders?Primary biliary cirrhosis is an autoimmune disorder. Also, in primary biliary cirrhosis, other autoimmune disorders occur with increased frequency.
What is the association between primary biliary cirrhosis and keratoconjunctivitis sicca?Keratoconjunctivitis sicca (dry eye syndrome) is seen in 70% of cases of primary biliary cirrhosis.
What are anti-mitochondrial antibodies and what is their presence indicative of?These are autoantibodies formed against mitochondria – primarily those in the cells of the liver. The presence of these in the serum may be indicative of the presence of, or the potential to develop, the autoimmune condition primary biliary cirrhosis.
What investigations can be carried out in a patient with primary biliary cirrhosis?Anti-mitochondrial antibodies can be found in the serum (specifically the “M2” antibody is found in 98% of patients). ALP levels may also be high, as well as serum IgM.
How is primary biliary cirrhosis treated?The most commonly used treatment is ursodeoxycholic acid which helps reduce the cholestasis (inability of the bile to flow from the liver to the duodenum). Pruritus is treated with cholestyramine (used for preventing bile acids from re-entering bloodstream and being excreted instead). Otherwise, primary biliary cirrhosis is a major indication for liver transplantation.
What complications can be seen in primary biliary cirrhosis?The complications seen are those of cirrhosis (i.e. ascites, splenomegaly, oesophageal varcies, hepatic encephalopathy). Otherwise, osteoporosis, and rarely osteomalacia and polyneuropathy can be seen.
What is primary sclerosing cholangitis?PSC is a chronic disease of bile ducts that is characterised by fibrosing inflammatory destruction of both the intra- and extrahepatic bile ducts. The cause is unknown.
What is the association between primary sclerosing cholangitis and inflammatory bowel disease?75% of PSC patients have IBD (usually ulcerative colitis).
What are the clinical features of primary sclerosing cholangitis?Patients may be asymptomatic, but may have intense pruritus, jaundice, cirrhosis, hepatomegaly, right upper quadrant pain, and some other symptoms.
How is primary sclerosing cholangitis usually detected?Incidentally as a raised ALP during screening of IBD patients.


Question Answer
What is endoscopic retrograde cholangiopancreatography?A technique that combines the use of endoscopy and fluoroscopy to diagnose and treat certain problems of the biliary or pancreatic ductal systems.
What is magnetic resonance cholangiopancreatography?A medical imaging technique that uses magnetic resonance imaging to visualise the biliary and pancreatic ducts in a non-invasive manner. This procedure can be used to determine if gallstones are lodged in any of the ducts surrounding the gallbladder.
How is primary sclerosing cholangitis diagnosed?ERCP is the standard diagnostic tool, showing multiple biliary strictures. P-ANCA (a type of antibody) is also positive in 60% of cases.
How is primary sclerosing cholangitis treated?The only treatment is liver transplantation.
What is hereditary haemochromatosis (HH)?A hereditary disease characterised by excessive intestinal absorption of dietary iron, leading to excess iron accumulation in the liver, pancreas, heart, skin, joints, adrenals, pituitary, gonads, and kidneys, eventually leading to fibrosis and functional organ failure.
What are the clinical features of hereditary haemochromatosis?The classic triad is of bronze skin pigmentation (due to melanin deposition), cirrhosis, and diabetes mellitus (only present in cases of gross iron overload). Hypogonadism secondary to pituitary dysfunction is the most common endocrine feature. Cardiac manifestations, particularly heart failure and arrhythmias, are common. Polyarthropathy also occurs as a result of deposition of calcium pyrophosphate in both large and small joints.
What is the association between hereditary haemochromatosis and hepatocellular carcinoma?30% of patients with cirrhosis will develop HCC.
What investigation can be used to help diagnose a patient with hereditary haemochromatosis?Transferrin saturation >45% is the most sensitive diagnostic test. Serum ferritin can also be measured, where levels in excess of 1000ng/ml of blood is almost always attributable to haemochromatosis. MRI-based testing is a non-invasive and accurate alternative to measure liver iron concentrations.
How is hereditary haemochromatosis managed?Venesection (phlebotomy) is the treatment of choice. In patients who cannot tolerate venesection, desferrioxamine is used as iron-chelation therapy (removal of iron from the body via injection of desferrioxamine which can “bind to” or “grab hold” of the iron).


Question Answer
What is Wilson’s disease?An autosomal recessive genetic disorder in which there are decreased levels of ceruloplasmin (copper-carrying protein) and excessive deposition of copper in the liver and brain.
What are the clinical features of Wilson’s disease?Neurological or psychiatric problems, and liver disease, including hepatitis, cirrhosis, and speech and behavioural problems. Other clinical features include Kayser-Fleischer rings (brown ring on the edge of the iris), renal tubular acidosis (especially Fanconi syndrome), haemolysis, and blue nails. Children usually present with hepatic problems. Young adults have more neurological problems.
At what age do symptoms of Wilson’s disease usually begin to show?10-25 years.
How is Wilson’s disease diagnosed?Reduced serum ceruloplasmin. Increased 24hr urinary copper excretion.
How is Wilson’s disease treated?Lifetime treatment with penicillamine. Penicillamine binds copper (chelation) and leads to excretion of copper in the urine. Once all results have returned to normal, zinc (usually in the form of zinc acetate) may be used instead of chelators to maintain stable copper levels in the body.
What are the side effects of penicillamine?Serious side effects of the drug occur in 10% and include skin rashes, leukopenia (reduced WBCs), skin changes, and renal damage. Ask the patient to report a sore throat.


Question Answer
What is α1-antitrypsin deficiency associated with?Liver disease and pulmonary emphysema.
What are the clinical features of α1-antitrypsin deficiency?10–15% of adult patients will develop cirrhosis, usually over the age of 50 years, and 75% will have respiratory problems. Approximately 5% of patients die of their liver disease.
What investigations can be undertaken in a patient with α1-antitrypsin deficiency?Measurement of serum α1-antitrypsin levels (they will be low). Histologically, periportal hepatocytes stain positive for PAS (periodic-acid Schiff).
What are Mallory bodies and what are they associated with?Mallory bodies are intracytoplasmic bodies seen in hepatocytes and are classically found in the livers of people suffering from alcoholic liver disease, but are also a recognised feature of Wilson’s disease, primary biliary cirrhosis, and hepatocellular carcinoma among other conditions.
What is delirium tremens, what causes it, and how is it treated?Delirium tremens is a state of confusion of rapid onset that is usually caused by withdrawal from alcohol. It can be treated with benzodiazepines such as diazepam.
Wernicke-Korsakoff syndrome is usually secondary to alcohol abuse. How is it treated?Intravenous thiamine (vitamin B1).