Liver and friends Flashcards

Question

What is exudate ascites?

Answer 1

protein >25g/L

Answer 2

- Peritonitis (inflammation of peritoneum lining organs) - Pancreatitis - Peritoneal malignancy

Answer 3

- Shifting dullness (dull sounds on percussion) - Abdo distension - Signs of liver disease. - Respiratory distress (pleural effusion)

Answer 4

Diagnostic aspiration (albumin, neutrophil count) - biopsy of ascites fluid aka ascites tap

Answer 5

The serum ascitic albumin gradient (SAAG) indirectly measures portal pressure and can be used to determine if ascites is due to portal hypertension. SAAG calculation SAAG = (serum albumin) – (ascitic fluid albumin)

Answer 6

A high SAAG (>1.1g/dL) suggests the ascitic fluid is a transudate.

Answer 7

A low SAAG (<1.1g/dL) suggests the ascitic fluid is an exudate.

Answer 8

1st line - salt restriction. Diuretics - furosemide/spironolactone.

Answer 9

Spontaneous bacterial peritonitis (SBP) (8%) - an infection of ascitic fluid. The most common causes are E.coli then K.pneumoniae

Answer 10

Superior mesenteric vein (from the gut) and splenic vein (from the spleen) and transports blood into the liver through the porta hepatis (portal triad)

Answer 11

High blood pressure in the portal vein.

Answer 12

Causes: - Prehepatic - portal vein thrombosis - Intrahepatic - cirrhosis (most common) - Posthepatic - right-sided heart failure

Answer 13

Usually asymptomatic Sometimes there could be symptoms of complications: - Ascites - Bleeding varices

Answer 14

Varices = dilated (bulgy) veins They can occur in the oesophagus or proximal stomach as a complication of cirrhosis Most patients with cirrhosis develop varices, but only ⅓ bleed from them. Bleeding is often massive.

Answer 15

Upper GI endoscopy

Answer 16

Melaena Haematemesis (coffee ground vomit)

Answer 17

Active bleed : - ABCDE - Urgent gastroscopy/endoscopy - Fluid resuscitation - bleed can be massive - Terlipressin (ADH analogue) - Balloon tamponade - stops bleeding until definitive treatment - Gold standard/first-line - endoscopic variceal band ligation (EVL) Prophylaxis as the risk of recurrence is high: - BB: Propranolol (secondary prevention) - Repeat band ligation (primary prevention)

Answer 18

Alcoholic liver disease results from the effects of the long-term excessive consumption of alcohol on the liver. 3 stages: fatty liver (steatosis), alcoholic hepatitis (inflammation and necrosis), and alcoholic liver cirrhosis.

Answer 19

Caused by chronic heavy alcohol ingestion. About 40 to 80 g/day in men 20 to 40 g/day in women Over 10 to 12 years can cause liver damage without other liver diseases.

Answer 20

Non-specific: Malaise Weakness Weight loss Easy bruising

Answer 21

- Jaundice - Hepatomegaly - Spider Naevi - Palmar Erythema - Gynaecomastia - Bruising – due to abnormal clotting - Ascites - Caput Medusae – engorged superficial epigastric veins - Asterixis – “flapping tremor” in decompensated liver disease

Answer 22

CAGE: 4 questions C: Have you ever felt you needed to CUT DOWN on your drinking? A: Have people ANNOYED you by criticising your drinking? G: Have you ever felt GUILTY about drinking? E: Have you ever felt you needed a drink first thing in the morning (EYE-OPENER)? A score of 2 or more indicates dependency

Answer 23

Bloods - these are primary investigations FBC – raised MCV LFTs – AST:ALT (aspartate transaminase: alanine transaminase) ratio > 2 Clotting – elevated prothrombin time U+Es may be deranged in hepatorenal syndrome. Exclude secondary causes: e.g. viral hepatitis, autoimmune liver disease, HCC Liver Biopsy - confirm the diagnosis of alcohol-related hepatitis or cirrhosis.

Answer 24

1st line: Stop drinking alcohol permanently - Benzodiazepines used to treat alcohol withdrawal - Weight loss + smoking cessation - Nutritional support with vitamins (particularly thiamine) and a high-protein diet - Steroids help in severe alcoholic hepatitis in the short-term - Treat complications of cirrhosis (portal hypertension, varices, ascites and hepatic encephalopathy)

Answer 25

- Hepatic encephalopathy - Portal hypertension - GI bleeding - Coagulopathy - Renal failure (due to portal hypertension and increased RAAS activity) - Wernicke-Korsakoff Syndrome (WKS)

Answer 26

Wernicke-Korsakoff is a degenerative brain condition resulting from thiamine (vitamin B1) deficiency caused by excess alcohol. Poor thiamine absorption + poor diet Wernicke’s encephalopathy comes before Korsakoffs syndrome.

Answer 27

NAFLD is part of the metabolic syndrome group of conditions related to processing and storing energy. Associated with obesity, dyslipidaemia, and type 2 diabetes mellitus. NAFLD = intrahepatic fat is ≥5% of liver weight

Answer 28

1. Non-alcoholic Fatty Liver Disease (steatosis - build-up of fat in liver) 2. Non-Alcoholic Steatohepatitis (NASH) 3. Fibrosis 4. Cirrhosis

Answer 29

NAFLD shares the same risk factors as cardiovascular disease and diabetes. Obesity Poor diet and low activity levels Type 2 diabetes High cholesterol Middle age onwards Smoking High blood pressure

Answer 30

Many patients asymptomatic, only symptomatic when disease enters cirrhotic stage Non- specific symptoms: - Malaise - Weakness - Weight loss - Easy bruising Signs - Palmar erythema - Dupuytren’s contracture - Jaundice - Ascites - Spider naevi - Confusion and asterixis: hepatic encephalopathy - Haematemesis: variceal bleed - Hepatosplenomegaly

Answer 31

Liver sceens to assess for possible underlying causes of liver pathology: - Ultrasound Liver - Hepatitis B and C serology - Autoantibodies (autoimmune hepatitis) - Immunoglobulins (autoimmune hepatitis) - Caeruloplasmin (Wilsons disease) - Alpha 1 Anti-trypsin levels (alpha 1 antitrypsin deficiency) - Ferritin and Transferrin Saturation (hereditary haemochromatosis)

Answer 32

Liver Ultrasound can confirm the diagnosis of hepatic steatosis (fatty liver). 1st line - Enhanced Liver Fibrosis (ELF) blood test. It measures three markers (HA, PIIINP and TIMP-1) and indicates the fibrosis of the liver: < 7.7 - none to mild fibrosis ≥ 7.7 to 9.8 - moderate fibrosis ≥ 9.8 - severe fibrosis

Answer 33

Conservative: - Weight loss - Exercise - Stop smoking - Avoid alcohol Medical - Control of diabetes, blood pressure and cholesterol - Refer patients with liver fibrosis to a liver specialist, where they may treat with vitamin E or pioglitazone. End-stage liver disease: - Liver transplant - TIPS (Transjugular intrahepatic portosystemic shunt) - for patients with complications e.g. varices, PHTN

Answer 34

Hepatitis describes inflammation in the liver. This can vary from a chronic low level inflammation to acute and severe inflammation that leads to large areas of necrosis and liver failure.

Answer 35

- Alcoholic hepatitis - Non alcoholic fatty liver disease - Viral hepatitis - Autoimmune hepatitis - Drug induced hepatitis (e.g. paracetamol overdose)

Answer 36

A, B, C, D, E

Answer 37

A is Acquired by mouth from Anus, is Always cleared Acutely and only ever Appears once B is Blood-Borne, and if not Beaten can be Bad B and D is DastarDly C is usually Chronic but Can be Cured - at a Cost. Caused by Crack (IVDU). E is Even in England and can be Eaten (found in pigs), if not always beaten.

Answer 38

Faecal-oral: contaminated food, fly vectors

Answer 39

Rare in the UK with < 1,000 cases in 2017, high prevalence in Africa, Asia, South America, Middle east.

Answer 40

It can cause cholestasis (slowing of bile flow through the biliary system) - Dark urine (excess bilirubin excreted by kidneys) - Pale stools (indicate obstructive cause) - Hepatomegaly. It resolves without treatment in around 1-3 months.

Answer 41

Non-specific symptoms: - Abdominal pain - Fatigue - Anorexia - Malaise - Pruritis (itching) - Muscle and joint aches - Nausea and vomiting - Fever (viral hepatitis) After 1-2 weeks liver symptoms/signs - jaundice (sign), hepatomegaly (sign), skin rash.

Answer 42

LFTs - Raised transaminases (AST / ALT) - enzymes released during inflammation - Raised bilirubin due to inflmmation

Answer 43

LFTs - Raised transaminases (AST / ALT) - enzymes released during inflammation - Raised bilirubin due to inflammation

Answer 44

A vaccine is available to reduce chance of developing hep A Treatment is often not required, generally with analgesia

Answer 45

Rare acute liver failure.

Answer 46

Yes, public health need to be notified of all cases

Answer 47

Registered medical professionals (doctors registered with GMC) have a legal duty to report a suspected/confirmed disease on the "notifiable disease list" to their "proper officer’ at their local council or local health protection team (HPT)

Answer 48

YES! Urgent - verbally within 24 hours via phone, encrypted email or secure fax

Answer 49

Send the form to the proper officer within 3 days, or notify them verbally within 24 hours if the case is urgent by phone, letter, encrypted email or secure fax machine.

Answer 50

DNA virus (only DNA one).

Answer 51

- Blood products (IVDU), sexually (particularly MSM). - Vertical transmission (mother to child) is most common transmission worldwide.

Answer 52

Present worldwide, prevalent in Africa, Middle and Far East.

Answer 53

Acute infection infects hepatocytes. Cellular response usually clears it within two moths. 10% of patients become chronic help B carriers, because virus DNA has integrated into their own DNA, and so they will continue to produce the viral proteins Depends on age/ immunocompetence. Inflammation can last 10 yrs -> cirrhosis.

Answer 54

Presence of HBsAg > 6 months post acute infection

Answer 55

Acute infection - usually asymptomatic but can present with malaise, fever, fatigue, right upper quadrant pain If chronic, then signs of cirrhosis - jaundice, pruritus, ascites, Dupuytren’s contracture (fingers flex towards the palm), malaise, anorexia (loss of appetite) etc.

Answer 56

LFTs and serology

Answer 57

Antibodies are produced against pathogenic antigens. Antigen - Surface antigen (HBsAg) – active infection - E antigen (HBeAg) – marker of viral replication and implies high infectivity. - Core antigen (HBcAg) - not useful as does not circulate in blood Antibodies - Surface antibody (HBsAb) – implies vaccination or past or current infection. - E antibodies (HBeAb) - if postive but HBeAg negative, indicates previous active infection and immune response - Core antibodies (HBcAb) – implies past (high IgG) or current infection (high IgM Viral load - Hepatitis B virus DNA (HBV DNA) is a direct count of the viral load.

Answer 58

IgM is an antibody made in response to acute help B infection, so presence of IgM suggest that the patient is currently infected with hep B

Answer 59

IgG is made later on in hep B infection and can persist for months or years after the acute infection. The presence of IgG in absence of HBsAg indicates past or chronic hep B infection.

Answer 60

- HBcAb - for previous infection - HBsAg - for active infection If these are positive, then: - HBeAg - how infective? Viral replication levels? - HBV DNA - viral load

Answer 61

- Antiviral medication - Tenofovir, pegylated interferon alpha 2a - Liver transplantation for end-stage liver disease - Vaccination for people at risk - med students got it in first year

Answer 62

If chronic, there is an increased risk of liver cirrhosis, hepatocellular carcinoma

Answer 63

Bloodborne - sexually, IVDU

Answer 64

Hep D is unable to replicate on its own and requires concurrent HBV infection. It attaches itself to the HBsAg to survive and cannot survive without this protein. It makes Hep B infection more likely to progress to cirrhosis/hepatocellular carcinoma (HCC)

Answer 65

Hep D is clinically indistinguishable from acute HBV infection

Answer 66

It is the same as hep B

Answer 67

Cirrhosis and HCC

Answer 68

More common in the UK (118,000 in 2019), common in Africa

Answer 69

Transmission through percutaneous exposure - e.g. IVDU, unscreened blood products Acute infection is often asymptomatic, allowing it to become chronic. Chronic HCV infection causes slow progressive fibrosis over the years.

Answer 70

Acute = asymptomic Later = signs of chronic liver disease e.g. jaundice, pruritis, ascites, hepatomegaly

Answer 71

75% :( (3 in 4 people) Only 1 in 4 fight off the infection

Answer 72

- Screening test - Hepatitis C antibody - Hepatitis C RNA testing is used to confirm the diagnosis of hepatitis C , assess individual genotype and calculate viral load

Answer 73

Direct acting antiviral agents (DAA) (Ribavirin) is curative DAA treatment is tailored to the specific viral genotype. They successfully cure the infection in over 90% of patients. 8 - 12 weeks treatment

Answer 74

Chronic liver disease, hepatocellular carcinoma (HCC)

Answer 75

RNA virus.

Answer 76

By the faecal-oral route

Answer 77

Becoming more common in the UK. Now the most common cause of short-term (acute) hepatitis in the UK. Common in developing world, where there is poor sanitation

Answer 78

95% of cases are asymptomatic, as it is usually self-limiting.

Answer 79

Often not required, but supportive

Answer 80

Complications are rare, but hep E can progress to chronic hepatitis and liver failure in immunocompromised patients

Answer 81

Chronic hepatitis as a result of autoantibodies against hepatocytes

Answer 82

Unknown, but thought to have an environmental trigger e.g. vira infection that causes a T cell-mediated response against hepatocytes

Answer 83

They have different ages of onset and autoantibodies: Type 1: occurs in adults Type 2: occurs in children

Answer 84

- Female - Late 40s - 50s - Around/after the menopause - Fatigue and features of liver disease on examination

Answer 85

- Often in their teens/early twenties - Acute hepatitis with jaundice

Answer 86

- LFT - Raised transaminases (ALT and AST) - Raised IgG levels, and it is associated with many autoantibodies. - Diagnosis made using a liver biopsy

Answer 87

- Anti-nuclear antibodies (ANA) - not specific - Anti-smooth muscle antibodies (anti-actin) - not specific - Anti-soluble liver antigens (anti-SLA) - highly specific to AIH

Answer 88

Anti-liver kidney microsomes-1 (anti-LKM1) Anti-liver cytosol antigen type 1 (anti-LC1)

Answer 89

- High dose steroids (prednisolone) - Then taper introduce immunosuppressants e.g. azathioprine - Usually successful in inducing remission but required life-long. End-stage liver disease - liver transplant but autoimmune hepatitis can occur in transplated liver

Answer 90

Jaundice = accumulation of bilirubin in the bloodstream and subsequent deposition in the skin, sclera, and mucous membranes. Normal levels are 3.4 - 20 mmol/L Jaundice may not be clinically evident until serum levels >51 mmol/L (3 mg/dL).

Answer 91

Jaundice might result from increased bilirubin production (prehepatic), diseases that impair hepatocyte function (hepatocellular), or obstruction of the biliary system (cholestatic).

Answer 92

Haem from old RBC broken down into unconjugated bilirubin > travels to liver bound to albumin > conjugated bilirubin > water-soluble and excreted in bile > gut bacteria convert conjugated bilirubin into urobilinogen and stercobilinogen, which are excreted in the urine and stool.

Answer 93

Increased RBC destruction or dysfunction in bilirubin conjugation causes a rise in unconjugated (indirect) serum bilirubin Referred to as prehepatic because the pathological process occurs before the liver metabolises the bilirubin.

Answer 94

Damage to the structure and/or function of hepatocytes causes elevated conjugated (direct) bilirubin levels, which result in hepatocellular jaundice. Conditions include infections, toxin, cancer, autoimmune conditions, and genetic conditions.

Answer 95

Obstruction of bile drainage causes elevated conjugated (direct) bilirubin levels. Causes may be benign or malignant.

Answer 96

Haemolytic anaemia Hereditary - Sickle cell anaemia - Thalassaemia Acquired: - Cytomegalovirus, infectious mononucleosis (Epstein-Barr Virus), and toxoplasmosis infection Autoimmune: - Rheumatic arthritis - Systemic lupus erythematosus

Answer 97

- Viral hepatitis A - E - HIV infection - ALD/NAFLD - Alcohol - HCC, lymphomas, liver mets - Gilbert's syndrome

Answer 98

Sometimes classified as pre-hepatic An inherited condition caused by defect in the UGT1A1 gene > encodes hepatic conjugation enzyme uridine-diphosphoglucuronate glucuronosyltransferase (UGT enzyme) > underactive UGT enzyme

Answer 99

- Choledocholithiasis (gallstones in common bile duct) - Postoperative stricture - Ascending cholangitis - an infection of the biliary tree, most commonly caused by obstruction - Pancreatic cancer - Metastases - liver mets can compress biliary tree - Lymphoma

Answer 100

Two separate conditions that are closely associated. They result from thiamine (B1) deficiency caused by excessive alcohol use Wernicke's encephalopathy comes before Kosarkoff's syndrome (psychosis)

Answer 101

A neurological emergency resulting from thiamine deficiency with varied neurocognitive manifestations, typically involving mental status changes, gait and oculomotor dysfunction.

Answer 102

A late complication of Wernicke's encephalopathy, characterised by anterograde and retrograde memory loss, disorientation and confabulation (false memories). 85% of patients with untreated WE develop Kosarkoff's psychosis

Answer 103

- Confusion - Oculomotor disturbances (disturbances of eye movements) - Ataxia (difficulties with coordinated movements)

Answer 104

- Evidence of long-term alcohol abuse: e.g. macrocytic anaemia, deranged LFTs and clotting - Therapeutic trial of parenteral thiamine - clinical response to treatment - Finger-prick glucose - normal - FBC - normal - Serum electrolytes - normal but may be abnormal if not treated or in late-presenting disease

Answer 105

Acute: - Stabilisation/resuscitation - Thiamine - Magnesium - Multivitamins - Folic acid (B9) For patients with chronic alcoholism and poor dietary intake - dietary thiamine supplement

Answer 106

A brain dysfunction caused by advanced liver dysfunction and portosystemic shunt (abnormal connection between the portal vascular system and systemic circulation, bypasses liver). Liver dysfunction and portosystemic shunt cause a build-up of toxins (especially ammonia) in the blood.

Answer 107

Ammonia is produced by intestinal bacteria when they break down proteins and is absorbed in the gut. 1. Functional impairment of the liver cells in cirrhosis prevents the metabolism of ammonia into harmless waste products 2. Collateral vessels between the portal and systemic circulation allow ammonia bypasses the liver altogether and enters the systemic system directly.

Answer 108

Acutely, it presents with reduced consciousness and confusion. It can present chronically with changes to personality, memory and mood.

Answer 109

Precipitating Factors - factors that trigger the current problem and maintain the problem once established - Constipation - Electrolyte disturbance - particularly hypokalaemia - Infection - GI bleed - High protein diet - Medications (particularly sedative medications)

Answer 110

- LFTs- abnormal - Serum glucose - normal to rule out hyper-and hypoglycemia - Coagulation profile - increased prothrombin time - Serum electrolytes - hyponatremia and hypokalemia (which can precipitate HE)

Answer 111

- Laxatives (i.e. lactulose) promote the excretion of ammonia before its absorbed by the gut. They may require enemas initially. - Antibiotics (i.e. rifaximin) reduces the number of intestinal bacteria producing ammonia. - Nutritional support. They may need nasogastric feeding.

Answer 112

Intermittent right upper quadrant pain caused by gallstones irritating bile ducts

Answer 113

Cholestasis: blockage to the flow of bile

Answer 114

Cholelithiasis: presence of gallstone(s) Gallstone disease (cholelithiasis) is highly prevalent, but most cases are asymptomatic

Answer 115

Choledocholithiasis: gallstone(s) in the common bile duct

Answer 116

Cholecystitis: inflammation of the gallbladder

Answer 117

Cholangitis: inflammation of the bile ducts

Answer 118

Cholecystectomy: surgical removal of the gallbladder

Answer 119

Cholecystostomy: inserting a drain into the gallbladder

Answer 120

Most cases are asymptomatic but can cause symptoms if a stone obstructs the cystic, bile, or pancreatic duct, preventing gallbladder drainage. Typical symptom = biliary colic Other symptoms include: - Severe, colicky epigastric or right upper quadrant pain - Pain triggered by meals (particularly high-fat meals) - Lasting between 30 minutes and 8 hours - Nausea and vomiting

Answer 121

- Acute cholecystitis (inflammation of the gallbladder caused by stones blocking the cystic duct) - Acute cholangitis (inflammation of the bile duct caused by stones blocking bile drainage) - Obstructive jaundice - resulting from the stones blocking the ducts - Pancreatitis

Answer 122

Fat entering the digestive system causes cholecystokinin (CCK) secretion from the duodenum. CCK triggers contraction of the gallbladder, which leads to biliary colic in the presence of gallstones Patients with gallstones and biliary colic are advised to avoid fatty foods to prevent CCK release and gallbladder contraction.

Answer 123

Symptoms - RUQ pain - Nausea - Right shoulder pain Signs - RUQ tenderness with or w/o Murphy's sign (inspiratory arrest upon applying pressure to the RUQ) - Signs and symptoms of inflammation (fever, elevated WBC, ESR, CRP) - Palpable mass - distended, tender gallbladder

Answer 124

- 1st line: abdominal ultrasound - if the patient is not septic, confirms diagnosis - CT or MRI of the abdomen - if the patient septic - FBC - elevated WBC due to inflammation - CRP - elevated due to inflammation - Bilirubin - elevated - LFTs - elevated alkaline phosphatase (ALP), Alanine transaminase (ALT) and aspartate transaminase (AST)

Answer 125

Magnetic resonance cholangiopancreatography (MRCP) - MRI scan hat produces a detailed image of the biliary system. It is very sensitive and specific for biliary tree diseases, such as stones in the bile duct and malignancy.

Answer 126

Severe acute cholecystitis: - ICU management, analgesia + fluid resuscitation - Antibiotics therapy - Percutaneous cholecystostomy for patients unsuitable for surgery (draining of fluid from the gallbladder) Mild/moderate: - Analgesia - Fluid resuscitation - Antibiotic therapy (if patients are at risk of infection and sepsis) - Early laparoscopic cholecystectomy (i.e. keyhole surgery or percutaneous cholecystostomy

Answer 127

Bile duct injury during surgery Cholecystoenteric fistulas - can lead to small bowel obstruction if gallstone falls through into GI tract

Answer 128

Acute cholangitis, previously known as ascending cholangitis, is an infection of the biliary tree, most commonly caused by obstruction.

Answer 129

Most common: - Cholelithiasis leading to choledocholithiasis and biliary obstruction. - Iatrogenic biliary duct injury - via surgical injury during cholecystectomy > benign strictures > obstruction

Answer 130

Obstruction of the common bile duct causes inflammation, bacterial accumulation, and growth in the biliary tree, which initially leads to a less severe form of acute cholangitis. Over time, as the obstruction progresses, the pressure in the bile duct continues to increase. This can cause the extravasation of bacteria into the bloodstream. If untreated, then it can lead to sepsis - this is known as toxic cholangitis or cholangitis with sepsis - LIFE-THREATENING!

Answer 131

- Charcot's triad: fever (most common), RUQ abdominal pain, Jaundice - If more severe - Reynold’s pentad = Charcot’s triad with confusion and hypotension (evidence of sepsis)

Answer 132

First line: transabdominal ultrasound detects common bile duct dilatation and the presence of gallstones. FBC: leukocytosis with neutrophilia LFTs: obstructive jaundice with raised ALP > ALT, and bilirubin U&Es: pre-renal acute kidney injury in sepsis CRP raised BLood culture - check for sepsis

Answer 133

- 1st line: ERCP (Endoscopic retrograde cholangiopancreatography) to help see where to insert stent + drainage stent - Aggressive fluid resuscitation - IV Abx → penicillins and aminoglycosides - Analgesia: opioids + paracetamol - Cholecystectomy when better

Answer 134

Progressive autoimmune destruction of the liver and biliary tree leading to fibrosis and eventually cirrhosis

Answer 135

An autoimmune disease, autoantibodies against mitochondrial antigens (antimitochondrial antibodies - present in 95% of patients) Mainly directed against pyruvate dehydrogenase complex E2 subunit (PDC-E2)

Answer 136

Autoantibodies cause inflammation and the progressive destruction of the epithelial cells lining the small intrahepatic bile ducts Progressive damage > liver fibrosis > eventually cirrhosis End-stage disease = complete loss of small intrahepatic bile ducts.

Answer 137

- Female sex (F:M = 10:1) - Age between 45-60 years - Family history of PBC/autoimmune disease - Smoking

Answer 138

Classic presentation: middle-aged female with a significant itch, might have hx of hypercholesterolemia and fx of autoimmune disease Symptoms - Pruritus - Fatigue and weight loss - Dry eyes and dry mouth: Sjögren's syndrome - Obstructive jaundice (late sign) - Pale stool and dark urine Signs - Clubbing - Skin hyperpigmentation: due to increased melanin - Mild hepatosplenomegaly - Xanthelasma (yellow fat deposits under skin, commonly eyelid) (late sign) - Scleral icterus (late sign)

Answer 139

Primary investigations: - Antimitochondrial antibodies (AMA): 95% of patients (highly specific) - Antinuclear antibodies (ANA): 50% of patients - LFTs: obstructive picture = raised ALP, gamma-glutamyl transferase (GGT) and bilirubin. AST and ALT may be mildly deranged - Coagulation profile: assess the synthetic function of the liver. Deranged in advanced disease - Transabdominal ultrasound: excludes other causes of cholestasis e.g. gallstones.

Answer 140

Early-stage disease: First-line - Bile acid therapy using ursodeoxycholic acid to reduce cholestasis Fat-soluble vitamin supplements - ADEK Cholestyramine: bile acid sequestrant for symptomatic relief of pruritus End-disease disease: liver transplant

Answer 141

- Hypercholesterolaemia - Osteoporosis - Portal hypertension secondary to cirrhosis long term - Hepatoma (or hepatocellular carcinoma)

Answer 142

A condition where the intrahepatic or extrahepatic ducts become strictured (narrowed) and fibrotic (hardened). This obstructs bile flow from the liver and into the intestines. Sclerosis = stiffening and hardening of the bile ducts Cholangitis = inflammation of the bile ducts Chronic bile obstruction eventually leads to liver inflammation (hepatitis), fibrosis and cirrhosis (end-stage liver disease)

Answer 143

Aetiology is not established yet, thought to be a combination between genetic, autoimmune, intestinal microbiome and environmental factors. There is an established association with ulcerative colitis - around 70% patients with primary sclerosing cholangitis have UC

Answer 144

- Male - Aged 30-40 - Ulcerative Colitis - Family History

Answer 145

- Jaundice - Chronic right upper quadrant pain - Pruritus - Fatigue - Hepatomegaly

Answer 146

GOLD STANDARD and diagnostic = magnetic resonance cholangiopancreatography (MRCP) - MRI scan of the liver, bile ducts and pancreas. > shows bile duct lesions or strictures (beaded appearance) Autoantibodies - elevated: - Antineutrophil cytoplasmic antibody (p-ANCA) (~94%) - Antinuclear antibodies (ANA) (~77%) LFTs - cholestatic picture (slowing of flow through bile ducts) - Alkaline phosphatase - deranged Bilirubin levels - elevated

Answer 147

Patients with inflammatory bowel disorders Heavily associated with ulcerative colitis - 70% patients with PSC has UC.

Answer 148

Liver transplant (around 80% survival at five years). ERCP (Endoscopic Retrograde Cholangio-Pancreatography) can be used to dilate and stent any strictures Cholestyramine - helps with pruritus

Answer 149

ERCP - a camera is inserted through the patient's oesophagus to the duodenum (sphincter of Oddi and into the ampulla of Vater) > bile ducts X-rays and injected contrasts can then identify any strictures and doctors can treat them

Answer 150

Patients are monitored for complications such as cholangiocarcinoma, cirrhosis and oesophageal varices

Answer 151

Predisposed to developing hepatocellular carcinoma, cholangiocarcinoma and colorectal cancer, fat-soluble vitamin deficency

Answer 152

Pancreatitis = inflammation of the pancreas Two types: Acute pancreatitis- after an episode, normal function usually returns. Chronic pancreatitis - longer-term inflammation and symptoms with a progressive and permanent deterioration in pancreatic function.

Answer 153

Most common causes: - Gallstones - Alcohol - Idiopathic I GET SMASHED: I – Idiopathic (~20%) G – Gallstones (~40%) E – Ethanol (alcohol consumption) (~30%) T – Trauma S – Steroids M – Mumps A – Autoimmune S – Scorpion sting (the one everyone remembers) H – Hyperlipidaemia E – ERCP D – Drugs (furosemide, thiazide diuretics and azathioprine)

Answer 154

Most common: - Excess alcohol ~70 - 80% cases - Idiopathic

Answer 155

Alcohol is directly toxic to pancreatic cells, resulting in inflammation. Alcohol-induced pancreatitis is more common in men and younger patients.

Answer 156

- Pancreatitis can occur when gallstones become trapped at the ampulla of Vater (biliary-duodenal junction), blocking the flow of bile and pancreatic juice into the duodenum and causing reflux of bile into the pancreatic duct > inflammation of the pancreas - More common in women and older patients.

Answer 157

Symptoms - Severe epigastric pain - classically radiating through to the back - Associated vomiting - Systemically unwell (e.g., low-grade fever and tachycardia) - Poor urinary output Signs - Abdominal tenderness - Abdominal distension - Tachycardic and/or hypotensive: patients may be in shock

Answer 158

- Severe epigastric pain (dull, radiating to back, 15-30 mins after eating, improves by leaning forward) - Steatorrhoea (increased fat in stool) - Weight loss and malnutrition - Symptoms of DMTII e.g. polydipsia, polyuria - Nausea + vomitting Signs - Epigastric tenderness - Signs of liver disease (e.g. jaundice and ascites) - Diabetes mellitus/glucose intolerance

Answer 159

The Glasgow Score is used to assess the severity of pancreatitis. Each criterion below adds 1 point P - PAO2 on room air <8 kPa A -age >55 years N - neutrophils, WCC >15 x 10⁹/L C - calcium < 2mmol/L R - renal, urea > 16.1 mmol/L E - enzymes, lactate dehydrogenase A - albumin <32 g/L S - sugar, blood glucose >10.0 mmol/L

Answer 160

0 or 1 – mild pancreatitis 2 – moderate pancreatitis 3 or more – severe pancreatitis > 3 point = ITU

Answer 161

2 out of 3 can diagnose acute pancreatitis: - Amylase (x3 normal) or lipase elevated - more sensitive and specific than amylase (first line) - Clinical features are consistent with pancreatitis (e.g. epigastric pain radiating to back) - CT abdomen shows signs of acute pancreatitis (e.g. inflammation), not needed for diagnosis Tests for the Glasgow score: - FBC (for white cell count) - U&E (for urea) - LFT (for transaminases and albumin) - Calcium - ABG (for PaO2 and blood glucose)

Answer 162

CT or MRI abdomen is recommended first-line for the diagnosis - demonstrate pancreatic calcification and enlargement

Answer 163

Patients can rapidly deteriorate so immediate supportive management is needed Moderate/severe cases = ITU/HDU - First-line: Initial resuscitation (ABCDE approach + IV fluids) - to combat insensible fluid loss, third-spacing - Nil by mouth (pancreas rest) - Analgesia with opates - Early nutritional support - e.g. oral/enteral feeding - Treatment of gallstones in gallstone pancreatitis (ERCP / cholecystectomy) - Treatment of complications (e.g., endoscopic or percutaneous drainage of large collections) Most patients will improve within 3-7 days.

Answer 164

- Pancreatic enzymes replacement (Creon) plus PPI - Alcohol and smoking cessation - Analgesia - OTC and weak opioid e.g. tramadol - Low-fat diet - 5 -6 small meals a day A lack of enzymes leads to fat malabsorption, greasy stools (steatorrhoea), and deficiency in fat-soluble vitamins.

Answer 165

If untreated, a lack of pancreatic enzymes leads to fat malabsorption, greasy stools (steatorrhoea), and deficiency in fat-soluble vitamins.

Answer 166

- Necrosis of the pancreas - Infection in a necrotic area - Abscess formation - Pseudocysts (collections of pancreatic juice) - Chronic pancreatitis

Answer 167

- Chronic epigastric pain - Loss of exocrine function > a lack of pancreatic enzymes (particularly lipase) secreted into the GI tract - Loss of endocrine function > lack of insulin > diabetes - Damage and strictures to the duct system > obstructs the excretion of pancreatic juice and bile into the duodenum - Formation of pseudocysts or abscesses

Answer 168

Inflammation of the peritoneal cavity

Answer 169

Primary: - Spontaneous bacterial peritonitis (SBP) - Ascites (secondary to liver cirrhosis) Secondary - underlying case - Malignancy - Ruptured ectopic pregnancy - Perforated bowel

Answer 170

10% patient with ascites secondary to liver cirrhosis When a bacterial infection develops in the ascitic fluid and peritoneal lining without any clear cause (e.g. not secondary to an ascitic drain or bowel perforation).

Answer 171

Gram-negative: Escherichia coli, Klebsiella pnuemoniae Gram-positive cocci: staphylococcus and enterococcus

Answer 172

Symptoms - Can be asymptomatic - Fever - Abdominal pain Signs - Ileus (failure of peristalsis in GI tract) - Hypotension - Deranged bloods (raised WBC, CRP, creatinine or metabolic acidosis)

Answer 173

Ascitic tap - extracting ascitic fluid for culture showing causative microorganism Deranged bloods (raised WBC, CRP, creatinine or metabolic acidosis) Rule out as cause: - Pregnancy (hGH (human chorionic gonadotropin) test) - Bowel perforation (Abdo XR)

Answer 174

- IV resuscitation + ABCDE - IV Abx e.g. metronidazole, cefotaxime) - Surgery - peritoneal lavage which is surgical clean-out of the peritoneum

Answer 175

- Septicemia if not treated early - Pelvic abscesses - Ileus (lack of bowel contraction)

Answer 176

Haemochromatosis is an autosomal recessive disorder where there is iron storage dysfunction that results in excessive total body iron and the deposition of iron in tissues.

Answer 177

Haemochromatosis is most commonly caused by mutations in the human haemochromatosis protein (HFE) gene located on chromosome 6. Genetic mutations are autosomal recessive. This gene is essential in regulating iron metabolism.

Answer 178

- Middle age - Male sex (presents later in females as menstrual cycle removes iron each month) - White ancestry - Family history

Answer 179

- Middle age - Male sex (presents later in females as mensural cycle removes iron each month) - White ancestry - Family history

Answer 180

Low levels of iron regulatory hormone, hepcidin leads to unregulated duodenal absorption of iron > iron overload

Answer 181

Usually presents after 40 when iron overload becomes symptomatic, presents later in women because menstruation removes iron: - Chronic tiredness - Joint pain - Skin hyperpigmentation (bronze/slate-grey discolouration) - Hair loss - Erectile dysfunction - Amenorrhoea (absence of menstruation) - Cognitive symptoms (memory and mood disturbance)

Answer 182

First-line - Serum transferrin saturation (>50% male, 40% female) - Ferritin > 300 mg/L (male) or > 200 mg/L (female) - Serum iron raised

Answer 183

- Venesection (a weekly protocol of removing blood to decrease total iron) - Monitoring serum ferritin - Avoid alcohol - Genetic counselling - Monitoring and treatment of complications

Answer 184

- Type 1 Diabetes (iron affects the functioning of the pancreas) - Liver Cirrhosis - Iron deposits in the pituitary and gonads lead to endocrine and sexual problems (hypogonadism, impotence, amenorrhea, infertility) - Cardiomyopathy (iron deposits in the heart) - Hepatocellular Carcinoma - Hypothyroidism (iron deposits in the thyroid) - Chondrocalcinosis/pseudogout (calcium deposits in joints) causing arthritis

Answer 185

Wilson’s disease is a rare, autosomal recessive disorder of copper accumulation and toxicity.

Answer 186

It is caused by mutations in the ATP7B gene on chromosome 13, resulting in dysfunction in ATP-mediated hepatocyte copper transport.

Answer 187

The mutation in the ATP7B gene causes dysfunction in ATP-mediated hepatocyte copper transport. Copper is not transported into bile and combined with ceruloplasmin (copper carrier protein) > reduced biliary excretion Therefore, copper accumulation in hepatocytes and leakage into the serum Increased free serum copper > toxic accumulation tissues including basal ganglia, kidney, and cornea, causing oxidative damage.

Answer 188

Patients are usually young and will present with a range of neuropsychiatric and liver disease signs and symptoms: Neurological - Behavioural and psychiatric issues: such as depression and delusions, often the first presentation - Parkinsonism: half of patients present with a tremor - Asterixis (abnormal movement) Hepatic - Hepatosplenomegaly - Hepatitis and cirrhosis - Jaundice - Ascites - Asterixis and encephalopathy Also, Kayser-Fleischer rings present in the cornea - a a hallmark of Wilson’s disease

Answer 189

Usually diagnosed from clinical presentation. 1st line Copper studies: - Reduced ceruloplasmin levels AND Increased 24-hour urinary copper excretion highly suggestive of WD - Increased free serum copper but reduced total serum copper. - LFTs: deranged with increased AST and ALT due to hepatitis

Answer 190

1st line: copper chelation, chelators bind copper and facilitate renal excretion. E.g. D-penicillamine Lifestyle advice: high copper-content foods such as shellfish should be avoided. Liver transplantation: indicated in acute liver failure or decompensated cirrhosis

Answer 191

Liver failure - may need transplantation. Renal failure: often due to D-penicillamine therapy rather than the disease itself

Answer 192

Alpha-1-antitrypsin (A1AT) deficiency is a condition caused by an abnormality in the gene for a protease inhibitor called alpha-1-antitrypsin.

Answer 193

A1AT is coded for on chromosome 14. In A1AT deficiency, there is an autosomal recessive mutation in the gene for A1AT. A1AT is produced by the liver and is a protease inhibitor of elastase. Elastase is an enzyme that digests connective tissue, secreted by neutrophils. A lack of A1AT = elastase-mediated damage, particularly in the lungs

Answer 194

The liver and the lungs are mainly affected by A1AT deficiency. Liver: - Normal A1AT made in the liver - In A1AT deficiency, mutated version produced and accumulates in the liver > liver damage > cirrhosis after 50 > can lead to HCC Lungs: Absence of functioning alpha-1-antitrypsin protein > an excess of elastase that attacks connective tissue in the lungs > bronchiectasis (bronchial widening causing excess mucus accumulation) and emphysema after 30 years old

Answer 195

COPD-like symptoms: - Chronic productive cough (productive of spetum) - Shortness of breath on exertion - Current cigarette smoker (common for smokers aged 32 -41 to present with symptoms) - Wheezing - Chest hyperinflation

Answer 196

- Gold standard: serum-alpha 1-antitrypsin levels - low - Liver biopsy - cirrhosis and staining shows mutant A1AT proteins in hepatocytes - Genetic testing for the A1AT gene - High-resolution CT thorax diagnoses bronchiectasis and emphysema.

Answer 197

Not curable - Stop smoking (smoking dramatically accelerates emphysema) - Treat COPD with short and long-acting bronchodilators, and inhaled corticosteroids - Patients with very low A1AT levels and respiratory disease start on IV A1AT therapy - Organ transplant for end-stage liver or lung disease - Monitoring for complications (e.g. hepatocellular carcinoma)

Answer 198

The AST/ALT ratio can be used to determine the likely cause of LFT derangement: ALT > AST is associated with chronic liver disease AST > ALT is associated with cirrhosis and acute alcoholic hepatitis

Liver and friends Flashcards

(226 cards)