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NME > Cystic Fibrosis and the GI Tract > Flashcards

Cystic Fibrosis and the GI Tract Flashcards

1
Q

CF: pathophysiology:
- what type of mutation
- what chromosome
- what protein is affected
- what is the most common mutation

A
  • autosomal recessive disease
  • abnormal CL- and HCO3-
  • due to mutation of CF transmembrane
    conductance regulator gene
  • chromosome 7
  • delta F508 is the most common (phe508del)
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2
Q

What is the physiological effect on GI of CF?

A
  • leads to abnormally viscous mucous and
    secretions in:
    • lungs
    • bile and pancreatic disease
    • intestines
  • obstructed lumens leading to clinical
    symptoms/signs
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3
Q

CF and GIT

A
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4
Q

CF (GI) History:

A
  • typical vs atypical symptoms of heartburn
  • dysphagia (difficulty swallowing)
  • weight loss
  • DHx
  • Fx
  • duration
  • affect on Quality of Life
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5
Q

CF examination:

A
  • anaemia (iron deficiency anaemia can be a GI
    cancer)
  • weight loss
  • nutritional status
  • lymphadenopathy
  • dentition
  • hoarseness
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6
Q

CF (GI) Investigations:

A
  • bloods
  • oesophagogastroduodeniscioy (OGD): upper
    GI tract endoscopy
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7
Q

Typical Symptoms of Heartburn:

A

complete later

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8
Q

Atypical Symptoms of Heartburn:

A

complete later

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9
Q

GI Tract upper ends at

A

duodenum

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10
Q

Grades of oesophageal slide

A
  • inflammation
  • stricturing
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11
Q

Gastro-oesophageal reflux disease (GORD)

A
  • common in CF
  • heartburn and regurgitation
  • dysphagia (stricture/malignancy)
  • oesphagitis
  • stricture
  • Barrett’s oesophagus: squamous cells change
    to more robust columnar which is the most
    common sign of oesophageal cancer
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12
Q

Management of Gastro-oesophageal reflux disease (GORD):

A
  • history and examination
  • OGD + Bx???
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13
Q

Management of acid reflux:

A
  • Conservative:
    • lifestyle: smaller meals, chew food well, 2-3
      hours before sleeping, lose weight
  • Medical:
    • proton pump inhibitors
    • Histamine2 antagonsits (famotidine)
  • Surgical:
    • anti-reflux procedure: create a valve
      between oesophagus and stomach
      • fundopication
      • linx
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14
Q

Pancreatic Exocrine Insufficiency:

A
  • deficiency of exocrine pancreatic enzymes
    resulting in the inability to digest food properly
    resulting in malnutrition
  • 2/3 CF patients experience this
  • predominantly fat , malabsorption
  • steatorrhea (freq, bulky, foul-smelling stools
    that are difficult to flush/float)
  • failure to thrive in young patients: short,
    skinny
  • weight loss
  • fat soluble vitamins (ADEK) not absorbed
    leading to coagulopathy (bleeding), hence
    must be replaced
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15
Q

What are the fat soluble vitamins?

A

ADEK

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16
Q

Pancreatic Exocrine Insufficiency Treatment:

A
  • Pancreatic Enzyme Replacement Therapy
    (PERT)
  • PPI
  • Fat soluble vitamins (ADEK)
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17
Q

Pancreatitis diagnosis:

A
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18
Q

Second biggest cause of death in CF patients is

A

hepatobiliary disease after pulmonary dysfunction

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19
Q

Hepatobilliary disease in CF:

A
  • cirrhosis = permanent, irreversible scarring
  • liver failure (rare for CF) = increase in toxins,
    can’t form clots
  • cirrhosis and liver failure are independent of
    each other
  • steatosis: Fatty liver (non-alcoholic) (common)
  • gallstones (common)
  • cholangiocarcinoma: cancer of biliary tree and
    bile ducts
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20
Q

Pathophysiology of Hepatobilliary disease and CF:

A
  • defective CFTR function
  • focal billiary obstruction due to mucous and
    increased secretions leading to
  • focal periportal inflammation
  • leading to multilobular cirrhosis/biliary
    cirrhosis
  • hepatosplenomegaly (big liver and big spleen)
  • ***portal hypertension leading to varicies and
    upper GI bleeding
  • hypersplenism: spleen is overactive, so
    filtering blood too efficiently hence destroying
    platelets
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21
Q

Why does liver disease and cirrhosis cause a big spleen?

A
  • main blood vessel to liver is the portal vein
    (30%)
  • portal vein is formed by splenic and inferior
    mesenteric vein
  • backpressure into spleen which is dangerous
    because mild trauma will cause burst and
    bleed?
22
Q

***What are varicies and why are they important

A
  • blood capillaries ???
  • most common cause of alcholics death
    outside of liver failure
23
Q

CF related liver disease:

A
  • 2.5% of overall mortality in Cf patients
  • single most important non-pulmonary cause
    of death
  • 90% diagnosed before 20yrs old
  • bloods: WBC high because inflammation,
    platelets low if hypersplenism, billirubin high
    (jaundice if 35)
  • elevated liver enzymes: 1-2.5x higher than
    normal
  • cirrhosis more common in males
24
Q

Management of CF related liver disease:

A
  • history
  • examination: signs of chronic liver diease
  • blood test: WBC, plateltets, billirubin
  • USS: is liver fibrosed?
  • Fibroscan: severity of liver fibrosis
  • Liver biopsy
  • liver transplant: immunosuppression leading
    to cancer
25
Q

32 year old female with CF presents with right upper quadrant pain.

How would you assess?

A

differentials: pancreatitis, gallstones, hepatitis,
liver disease, Pardo nephritis,
kidney stones, pneumonia, tumour
in hepatic flexture of colon,
inflammation of ribs/muscles

  • history
  • examination: anaemia, clubbing, asterixis,
    dupuytrens contracture, jaundice, massesm
    hernias, peritonism, Murphy’s sign
26
Q

RUQ pain

A
  • tender, fever, tachycardia
  • Murphy’s positive
  • Ni else
27
Q

What is the only cause of peritonitis that does not require surgery?

A

pancreatitis

28
Q

GI causes of clubbing:

A

Coeliac
CF
asterixis

29
Q

Asterixis:

A

liver flap: hands out, few seconds start flapping

30
Q

Dupuytrens contracture is

A

tendons in hands remain contracted….

31
Q

Murphy’s signs shows

A

sign of liver failure

32
Q

What is Murphy’s signs?

A

complete later

33
Q

Investigations for CF patients cholelithiasis (gallstones):

A
  • Bloods: WCC, CRP, Amylase, LFTs, clotting
    screen
  • Urinalysis: pregnancy test (women!)
  • Imaging: Ultrasounds, CT: no radiation with
    ultrasound, gallstones easier to see on an
    ultrasound
34
Q

Best imaging modality for gallstones (cholelithiasis)?

A

ultrasound

35
Q

Image of gallstone

A
36
Q

Complications of gallstones:

A

V good for yr 5
let liver lobe drains via left hepatic duct right liver lobe drains via right hepatic duct
under 8 is cystic duct which joins with left and right ducts
joins to form common bile duct into duodenum???

37
Q

Treatment of Gallstones:

A
  • Laparoscopic Cholecystectomy
  • ***Endoscopic retrograde
    cholangiopancreatography (ERCP) to treat
    gallstones in bile duct
  • or lifestyle and deal with pain ideally take out
    gallbladder
38
Q

Patients with gallstone in bile duct presents with = choledocholithiasis

A
  • blocks bile
  • so no bile traveeling to duodenum for
    digestion
  • jaundice when bile duct is blocked!!!
39
Q

Intestinal Complications in CF:

A
  • Meconium ileus
  • Distal intestinal obstruction syndrome
  • bowel obstruction
  • cancer
40
Q

Meconium ileus:

A
  • first 1-2 days of life
  • failure to pass meconium
  • 15-20% of patients with CF
  • family history
  • AXR: obstruction, ground glass appearance
  • Treatment:
    - hypertonic enema (diagnostic and
    therapeutic)
    - rarely surgery, perforation leads to
    meconium peritonitis
41
Q

Cardinal signs of bowel obstruction (ileus):

A
  • absolute constipation: no faeces of flatus
  • distended abdomen
  • nausea and vomiting
  • colicky abdominal pain (comes and goes in
    waves)
42
Q

Distal intestinal obstruction syndrome (DIOS):

A
43
Q

Normal diameter of small bowel, large bowel and caecum?

A

3,6,9 cm

44
Q

GI Malignancy:

A
  • upregulation of oncogenic genes
  • inflammatory state in GIT promotes oncogenesis
  • increased risk of GI cancers in CF:
    - colon cancer commonest
    - M>F
    - from age of 40, 5 fold risk increase
    - small bowel
    - biliary tract
    - pancreas
45
Q

Difference between screening and surveillence

A

screening = picking disease in an asymptomatic
population
surveillance = know they have it and monitoring

46
Q

What is hematochezia?

A

blood in stool

47
Q

Colon Cancer Management:

A
  • colonoscopic screening begins at 40 every 5
    years
  • if polyps detected then screened every 3 years
  • anaemia, change in bowel habit, weight loss,
    hematochezia
  • Faecal Immune Test (FIT)
  • Colonoscopy
  • CT
  • Capsule Endoscopy
48
Q

Nutrition for CF patients:

A
  • Pancreatic enzymes (Creon)
  • Fat-soluble vitamins
  • High-calorie diet to maintain weight
  • Oral feeding best
  • Enteral:
    - PEG/jejunostomy
  • Parenteral
    - Increased risk of sepsis
49
Q

CF patients who have undergone transplantation are at an increased risk of cancer.

True or False?

A

True

50
Q

Dysphagia in a CF patient is usually a sign of oesophageal cancer.

true or False?

A

False
usually secondary to reflux/benign stricture

NME (52 decks)

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