GI Diseases 2 Flashcards

Question

What is Hepatorenal syndrome?

Answer 1

Life-threatening medical condition that consists of rapid deterioration of kidney function in individuals with cirrhosis or fulminant liver failure Occurs due to portal hypertension

Answer 2

Malignant tumour of the liver which occurs primarily in patients with pre-existing liver cirrhosis or chronic hepatitis Most common type of primary liver cancer Malignant tumour of the liver which occurs primarily in patients with pre-existing liver cirrhosis or chronic hepatitis Most common type of primary liver cancer

Answer 3

A stepwise pattern; of mutational activation of oncogenes ( ie Kras) and inactivation of tumour suppressor genes (eg p53) that results in cancer

Answer 4

* Smoking * Alcohol * Diet; processed meat ( especially red meat), increased obesity risk, green vegetables and diet high in fibre protective against colorectal cancer * Chronic inflammation ie patients with ulcerative colitis have increased risk of developing colorectal cancer * Upper GI cancer; H pylori infection?

Answer 5

APC and P53

Answer 6

1. Inactiviation of APC 2. Activation of Kras 3. Inactivation of p53

Answer 7

Same genes different order 1. P53 2. Kras 3. APC

Answer 8

Oesophageal cancers Gastric cancers

Answer 9

colorectal cancers

Answer 10

4th rectum 2nd

Answer 11

family history, those that have FAP or HNPCC, diet with lack of fibre, inflammatory bowel disease (ie ulcerative colitis)

Answer 12

Classic familial adenomatous polyposis, called FAP or classic FAP, is a genetic condition. It is diagnosed when a person develops more than 100 adenomatous colon polyps. An adenomatous polyp is an area where normal cells that line the inside of a person's colon form a mass on the inside of the intestinal trac

Answer 13

Autosomal dominant inheritance

Answer 14

Autosomal dominant inheritance

Answer 15

Mutations in the DNA mismatch repair genes (MSH2 (60%)) and MLH1 (30%)), causing microsatellite instability. These genes normally maintain stability of DNA during replication

Answer 16

* Unintentional / unexplained weight loss * Rectal bleeding * Change in bowel habit * Abdominal masses * Iron deficiency anaemia * In history; ask about family history- family members with ovarian and bowel cancer can be significan

Answer 17

Most common : adenocarcinoma ( a malignant tumour formed from glandular structures of the epithelial tissue) Less common; mucinous adenocarcinoma, signet ring cell carcinoma, small cell carcinoma Glandular dysplasia is the premalignant lesion, when it forms a poly it is commonly referred to as an adenoma Adenomas classified as tubular, tubulovillous and villous (the is the worst one) Adenomas are also graded in terms of dysplasia (low or high)

Answer 18

Epigastric pain; may be relieved by food and antacids Dysphagia especially if tumour involves stomach fundus Anaemia Nausea, vomiting and weight loss Can have Liver, bone , brain and lung involvement

Answer 19

Zollinger–Ellison syndrome is a disease in which tumours cause the stomach to produce too much acid, resulting in peptic ulcers. A gastrin secreting tumour (gastrinoma) - Excess gastric acid secretion leads to (unusual/severe peptic ulceration, oesophagitis, diarrhoea and

Answer 20

* Neuroendocrine tumours * Gastrin secreting * Cause severe peptic ulcer disease and diarrhoea (referred to as the Zollinger Ellison Syndrome) * Can either be sporadic (75-80% of cases) or associated with Multiple endocrine neoplasia type 1 (20-30% of cases)

Answer 21

Sudden inflammation, haemorrhage of pancreas due to autodigestion from inappropriate activation of zymogen (trypsinogen → trypsin) Reversible

Answer 22

**Gallstones** - Obstruct sphincter of Oddi causing reflux . Bile salts irritates pancreatic duct **Ethanol** - Increases acinar cell secretion, decreases ductal cell secretion → thickened pancreatic juice . ROS damages the cells **Trauma** - Usually ruptured duct in the body of the pancreas from direct trauma (vertebral column) **Drugs** - sodium valporate, furosemide, thiazides, Azathioprine

Answer 23

Elevated serum amylase \>1000 IU/L **Elevated serum lipase (2-5x normal value)** LFTs: gallstones aetiology (jaundice) Imaging CT: swollen gland with inflammation, necrosis, pseudocyst USS: gallstones

Answer 24

- Chronic inflammation causing irreversible structural damage Atrophy of acinar cells Fibrosis Calcification Dilation of ducts/ narrowing - Formation of chronic pseudocysts, intrapancreatic cysts

Answer 25

Obstruction in the pancreatic ducts Ductal dilatation and damage to tissue (atrophy of acinar cells) Stellate cells lay down fibrotic tissue Further resulting in atrophy of acinar cells, intraluminal calcification, narrowing of ducts

Answer 26

- Recurrent episodes of acute pancreatitis (alcohol) - Duct obstruction: tumour, gallstones, structural abnormalities (annular pancreas, pancreas divisum), traumatic strictures - Cystic fibrosis

Answer 27

Serum lipase and amylase may or may not be elevated Glucose tolerance test (endocrine function) Imaging CT : calcification, intrapancreatic cysts or chronic pseudocysts ERCP: dilated and strictured ducts (chain of lakes pattern) To differentiate from carcinoma Pancreatic imaging + EUS + targeted needle biopsy

Answer 28

Increased risk of pancreatic carcinoma Pancreatic pseudocysts, fistula, ascites Biliary obstruction Malnutrition Diabetes mellitus

Answer 29

Western lifestyle Cigarette smoking High-fat diet Liver cirrhosis Chronic pancreatitis Obesity Family history/genetic factors (BRCA1/BRCA2/FAP/HNPCC)

Answer 30

Hirschprung’s Disease Meckel’s Diverticulum Omphalocele Gastroschisis

Answer 31

Agangliosis of myenteric and submucosal plexus in the distal colon and rectum which causes lack of peristalsis Functional obstruction from spasms in denervated colon Severe constipation within first 2 months Investigations X ray: colon distension Biopsy to identify transition zone Management: surgical removal of affected segment

Answer 32

**Persistence of vitellointestinal duct** **_Rule of 2s_** 2% of population Present in first 2 years 2 inches in length Approximately 2 ft from ileocaecal valve

Answer 33

Herniation of intestines (sometimes liver and other organs) out of umbilicus at birth due to abdominal wall defects Failure to return to abdomen after natural protrusion during development Covered by peritoneal membrane and amnion Investigations: USS antenatal scan shows herniated loop contained within peritoneum Associated with severe malformations, 25% mortality

Answer 34

Herniation of bowel loops (sometimes stomach and liver) through para-umbilical wall defect (lateral to the umbilicus) Not covered by surrounding membrane Investigations: Antenatal USS show herniation to lateral of umbilicus, free-floating

Answer 35

Alcoholic liver disease results from the effects of the long term excessive consumption of alcohol on the liver. The onset and progression of alcoholic liver disease varies between people, suggesting that there may be a **genetic predisposition** to having harmful effects of alcohol on the liver.

Answer 36

**1. Alcohol related fatty liver** Drinking leads to a build-up of fat in the liver. If drinking stops this process reverses in around 2 weeks. **2. Alcoholic hepatitis** Drinking alcohol over a long period causes inflammation in the liver sites. Binge drinking is associated with the same effect. Mild alcoholic hepatitis is usually reversible with permanent abstinence. **3. Cirrhosis** This is where the liver is made up of scar tissue rather than healthy liver tissue. This is irreversible. Stopping drinking can prevent further damage. Continued drinking has a very poor prognosis.

Answer 37

That latest recommendations (Department of Health, 2016) are to **not regularly drink more than 14 units per week** for both men and women. If drinking 14 units in a week, this should be spread evenly over 3 or more days and not more than 5 units in a day. The government guidelines also state that any level of alcohol consumption increases the risk of cancers, particularly breast, mouth and throat. Pregnant women should avoid alcohol completely.

Answer 38

The Alcohol Use Disorders Identification Test (AUDIT) was developed by the World Health Organisation to screen people for harmful alcohol use. It involves 10 questions with multiple choice answers and gives a score. A score of 8 or more gives an indication of harmful use.

Answer 39

* Alcoholic Liver Disease * Cirrhosis and the complications of cirrhosis including hepatocellular carcinoma * Alcohol Dependence and Withdrawal * Wernicke-Korsakoff Syndrome (WKS) * Pancreatitis * Alcoholic Cardiomyopathy

Answer 40

* 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 41

**Bloods** FBC – raised MCV LFTs – elevated ALT and AST (transaminases) and particularly raised gamma-GT. ALP will be elevated later in the disease. Low albumin due to reduced “synthetic function” of the liver. Elevated bilirubin in cirrhosis. Clotting – elevated prothrombin time due to reduced “synthetic function” of the liver U+Es may be deranged in hepatorenal syndrome. **Ultrasound** An ultrasound of the liver may show fatty changes early on described as **“increased echogenicity”**. It can also demonstrate changes related to cirrhosis if present. _“FibroScan”_ can be used to check the elasticity of the liver by sending high frequency sound waves into the liver. It helps assess the degree of cirrhosis. **Endoscopy** Endoscopy can be used to assess for and treat oesophageal varices when portal hypertension is suspected. **CT and MRI scans** CT and MRI can be used to look for fatty infiltration of the liver, hepatocellular carcinoma, hepatosplenomegaly, abnormal blood vessel changes and ascites. **Liver Biopsy** Liver biopsy can be used to confirm the diagnosis of alcohol-related hepatitis or cirrhosis. NICE recommend considering a liver biopsy in patients where steroid treatment is being considered.

Answer 42

* Stop drinking alcohol permanently * Consider a **detoxication regime** * Nutritional support with vitamins (particularly **thiamine**) and a high protein diet * **Steroids** improve short term outcomes (over 1 month) in severe alcoholic hepatitis but infection and GI bleeding need to be treated first and do not improve outcomes over the long term * Treat complications of cirrhosis **(portal hypertension, varices, ascites and hepatic encephalopathy)** * **Referral for liver transplant** in severe disease however they must abstain from alcohol for 3 months prior to referral

Answer 43

* 6-12 hours: tremor, sweating, headache, craving and anxiety * 12-24 hours: hallucinations * 24-48 hours: seizures * 24-72 hours: “delirium tremens”

Answer 44

Delirium tremens is a medical emergency associated with **alcohol withdrawal** with a mortality of 35% if left untreated. Alcohol stimulates **GABA receptors** in the brain. **GABA receptors** have a “relaxing” effect on the rest of the brain. Alcohol also inhibits **glutamate receptors** (also known as **NMDA receptors**) having a further inhibitory effect on the electrical activity of the brain.

Answer 45

* Acute confusion * Severe agitation * Delusions and hallucinations * Tremor * Tachycardia * Hypertension * Hyperthermia * Ataxia (difficulties with coordinated movements) * Arrhythmias

Answer 46

(Clinical Institute Withdrawal Assessment – Alcohol revised) tool can be used to score the patient on their withdrawal symptoms and guide treatment.

Answer 47

Chlordiazepoxide (“Librium”) Diazepam

Answer 48

Alcohol excess leads to **thiamine (vitamin B1)** deficiency. Thiamine is poorly absorbed in the presence of alcohol and alcoholics tend to have poor diets and rely on the alcohol for their calories. **Wernicke’s encephalopathy** comes before **Korsakoffs syndrome**. These result from thiamine deficiency.

Answer 49

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

Answer 50

* Memory impairment (retrograde and anterograde) * Behavioural changes *

Answer 51

Non alcholic fatty liver disease (NAFLD) forms part of the **“metabolic syndrome”** group of chronic health conditions relating to processing and storing energy that increase risk of heart disease, stroke and diabetes. It is estimated that up to 30% of adults have NAFLD. It is characterised by fat deposited in liver cells. These fat deposits can interfered with the functioning of the liver cells. NAFLD does not cause problems initially, however it can progress to **hepatitis** and **cirrhosis.** Non-alcoholic steatohepatitis (NASH) is most extremeform and→cirrhosis in 10%

Answer 52

1. Non-alcoholic Fatty Liver Disease 2. Non-Alcoholic Steatohepatitis (NASH) 3. Fibrosis 4. Cirrhosis

Answer 53

* **Obesity** * Poor diet and low activity levels * **Type 2 diabetes** * **High cholesterol** * Middle age onwards * Smoking * **High blood pressure**

Answer 54

**Liver Ultrasound** can confirm the diagnosis of hepatic steatosis (fatty liver). It does not indicate the severity, the function of the liver or whether there is liver fibrosis. **Enhanced Liver Fibrosis (ELF)** blood test. This is the first line recommended investigation for assessing fibrosis but it is not currently available in many areas. It measures three markers (HA, PIIINP and TIMP-1) and uses an algorithm to provide a result that indicates the fibrosis of the liver: * \< 7.7 indicates _none to mild fibrosis_ * ≥ 7.7 to 9.8 indicates _moderate fibrosis_ * ≥ 9.8 indicates _severe fibrosis_ **NAFLD fibrosis** score is the **second line** recommended assessment for liver fibrosis where the ELF test is not available. It is based on an algorithm of age, BMI, liver enzymes, platelets, albumin and diabetes and is helpful in ruling out fibrosis but not assessing the severity when present. **Fibroscan** is the third line investigation. It involves a particular ultrasound that measures the stiffness of the liver and gives an indication of fibrosis. This is performed if the ELF blood test or NAFLD fibrosis score indicates fibrosis. * **BMI** * **Glucose, fasting lipids** * **↑transaminases: AST:ALT \<** * **Liver biopsy**

Answer 55

* Weight loss * Exercise * Stop smoking * Control of diabetes, blood pressure and cholesterol * Avoid alcohol Refer patients with **liver fibrosis** to a liver specialist where they may treat with **vitamin E or pioglitazone.**

Answer 56

Liver cirrhosis is the result of chronic inflammation and damage to liver cells. When the liver cells are damaged they are replaced with scar tissue (fibrosis) and nodules of scar tissue form within the liver.

Answer 57

portal hypertension

Answer 58

* Alcoholic liver disease * Non Alcoholic Fatty Liver Disease * Hepatitis B * Hepatitis C

Answer 59

* **Genetic**:Wilson’s,α1ATD, HH, CF * **AI**:AH, PBC, PSC * **Drugs:**Methotrexate, amiodarone, methyldopa,INH * **Neoplasm:**HCC, mets * **Vasc**:Budd-Chiari, RHF,constrict. pericarditis

Answer 60

Hands * Clubbing (± periostitis) * Leuconychia (↓albumin) * Terry’s nails (white proximally, red distally) * Palmer erythema- caused by hyperdynamic cirulation * Dupuytron’s contracture * **Asterixis** – “flapping tremor” in decompensated liver diseas Face * Pallor: ACD * Xanthelasma: PBC * Parotid enlargement (esp. c̄EtOH) * **Jaundice** – caused by raised bilirubin Trunk * Spider naevi (\>5, fill from centre)- these are telangiectasia with a central arteriole and small vessels radiating away * Gynaecomastia- in males due to endocrine dysfunction * Loss of sexual hair * Brusing- due to abnormal clotting Abdo * Striae * Hepatomegaly (may be small in late disease) * Splenomegaly- due to portal hypertension * **Caput Medusa**e – distended paraumbilical veins due to portal hypertension * Testicular atrophy- in males due to endocrine dysfunction

Answer 61

**Bloods** * FBC:↓WCC and↓plats indicate hypersplenism * ↑LFTs * ↑INR * ↓Albumin **Find Cause** * EtOH:↑MCV,↑GGTNASH:hyperlipidaemia,↑glucose * Infection:Hep, CMV, EBV serology * Genetic:Ferritin,α1AT, caeruloplasmin (↓in Wilson’s) * Autoimmune:Abs (there is lots of cross-over) * AIH:SMA, SLA, LKM, ANA * PBC:AMA * PSC:ANCA, ANA * Ig:↑IgG – AIH,↑IgM – PBC * Ca:α-fetoprotein **Abdo US + PV Duplex** * Small / large liver * Focal lesions * Reversed portal vein flow * Ascites **Ascitic Tap + MCS** PMN \>250mm3indicates SBP **Liver biopsy**

Answer 62

adenocarcinomas

Answer 63

Post Hepatic Obstructive jaundice

Answer 64

liver, then to the peritoneum, lungs and bones.

Answer 65

* **Painless Obstructive jaundice** * **Yellow skin and sclera** * **Pale stools** * **Dark urine** * Generalised itching * Non-specific upper abdominal or back pain * Unintentional weight loss * Palpable mass in the epigastric region * Change in bowel habit * Nausea or vomiting * New‑onset diabetes or worsening of type 2 diabetes

Answer 66

Pancreatic cancer

Answer 67

* Over 40 with jaundice – referred on a **2 week wait referral** * Over 60 with weight loss plus an additional symptom (see below) – referred for a **direct access CT abdomen**

Answer 68

if a patient has **weight loss** plus any of: * Diarrhoea * Back pain * Abdominal pain * Nausea * Vomiting * Constipation * New‑onset diabetes

Answer 69

Courvoisier’s law suggests cholangiocarcinoma or pancreatic cancer

Answer 70

refers to **migratory thrombophlebitis** as a sign of malignancy, particularly **pancreatic adenocarcinoma**. **Thrombophlebitis** is where blood vessels become inflamed with an associated blood clot (thrombus) in that area. **Migratory** refers to the thrombophlebitis reoccurring in different locations over time.

Answer 71

Diagnosis is based on **imaging** (usually CT scan) plus **histology** from a biopsy. **Staging CT scan** involves a full **CT thorax, abdomen and pelvis (CT TAP)**. This is used to look for metastasis and other cancers. **CA 19-9 (carbohydrate antigen)** is a tumour marker that may be raised in pancreatic cancer. **Magnetic resonance cholangio-pancreatography (MRCP**) may be used to assess the biliary system in detail to assess the obstruction. **Endoscopic retrograde cholangio-pancreatography (ERCP)** can be used to put a stent in and relieve the obstruction, and also obtain a biopsy from the tumour. **Biopsy** may be taken through the skin (percutaneous) under ultrasound or CT guidance, or during an endoscopy under ultrasound guidance.

Answer 72

Surgery * Total pancreatectomy * Distal pancreatectomy * Pylorus-preserving pancreaticoduodenectomy (PPPD) (modified Whipple procedure) * Radical pancreaticoduodenectomy (Whipple procedure) In most cases, curative surgery is not possible. Palliative treatment may involve: * Stents inserted to relieve the biliary obstruction * Surgery to improve symptoms (e.g., bypassing the biliary obstruction) * Palliative chemotherapy (to improve symptoms and extend life) * Palliative radiotherapy (to improve symptoms and extend life) * End of life care with symptom control

Answer 73

A Whipple procedure (pancreaticoduodenectomy) is a surgical operation to remove a tumour of the head of the pancreas that has not spread. A Whipple procedure is a massive operation so patients need to be in good baseline health.

Answer 74

* Head of the pancreas * Pylorus of the stomach * Duodenum * Gallbladder * Bile duct * Relevant lymph nodes

Answer 75

pylorus-preserving pancreaticoduodenectomy (PPPD). leave pylorus in place

Answer 76

Symptoms * GI bleeding (May contain gastric mucosa → gastric ulcerations) * Abdominal pain and cramping * Tenderness near the belly button * Obstruction of bowels

Answer 77

Excess (billirubin) BR production * Haemolytic anaemia * Ineffective erythropoiesise.g. thalassaemia

Answer 78

**↓BR Uptake** * Drugs: contrast, RMP * CCF **↓BR Conjugation** * Hypothyroidism * Gilbert’s (AD) * Crigler-Najjar (AR) Neonatal jaundice is both ↑production +↓conju

Answer 79

**Hepatocellular Dysfunction** * **Congen:**HH, Wilson’s,α1ATD * **Infection**:Hep A/B/C, CMV, EBV * **Toxin:**EtOH, drugs * **AI**:AIH * **Neoplasia**:HCC, mets * **Vasc:**Budd-Chiari **↓Hepatic BR Excretion** * Dubin-Johnson * Rotor’

Answer 80

Obstruction * **Stones** * **Ca pancreas** * Drugs * PBC * PSC * Biliary atresia * Choledochal cyst * Cholangio Ca

Answer 81

BR-UDP-glucuronyl transferase in live

Answer 82

* Normal BR = 3-17uM * Jaundice visible @ 50uM (3 x ULN

Answer 83

In Gilbert's syndrome, slightly higher than normal levels of a substance called bilirubin build up in the blood. * Auto dom partial UDP-GT deficiency * 2% of the population * Jaundice occurs during intercurrent illness * Dx:↑uBR on fasting, normal LFTs

Answer 84

* Rare auto rec total UDP-GT deficiency * Severe neonatal jaundice and kernicterus * Rx:liver Transplant

Answer 85

**Child-Pugh Grading of Cirrhosis** * Predicts risk of bleeding, mortality and need for Tx * Graded A-C using severity of 5 factors * Albumin * Bilirubin * Clotting * Distension: Ascites * Encephalopathy * Score \>8 = significant risk of variceal bleeding

Answer 86

* Inflammatory disease of unknown cause characterisedby Abs directed vs. hepatocyte surface antigens * Predominantly young and middle-aged women

Answer 87

Classified according to Abs * T1:Adult, SMA+ (80%), ANA+ (10%),↑IgG * T2:Young, LKM+ * T3:Adult, SLA+

Answer 88

Autoimmune thyroiditis DM Pernicious anaemia PSC UC GN AIHA (Coombs +ve)

Answer 89

* ↑LFTs * ↑IgG * Auto Abs: SMA, LKM, SLA, ANA * ↓WCC and↓plats = hypersplenism * Liver biopsy

Answer 90

Immunosuppression * Prednisolone * Azathioprine as steroid-sparer Liver transplant (disease may recur)