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Yr 4 - Paediatrics > GI: Biliary Atresia & IBD > Flashcards

GI: Biliary Atresia & IBD Flashcards

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1
Q

What is biliary atresia?

A

A rare, congenital condition that affects the biliary tree.

It is characterised by progressive inflammation and fibrosis of the extrahepatic ducts, leading to cholestasis, liver damage and failure.

It can be life-threatening if left untreated.

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2
Q

Define cholestasis

A

A decrease in bile flow from the liver to the bowel due to impaired secretion by hepatocytes or to obstruction of bile flow through intra-or extrahepatic bile ducts.

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3
Q

Who does biliary atresia present in?

A

Biliary atresia is unique to neonatal children –> the perinatal form presents in the first two weeks of life, the postnatal form presents within the first 2-8 weeks of life.

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4
Q

Does biliary atresia prevnet the excretion of conjugated or unconjugated bilirubin?

A

Conjugated bilirubin is excreted in the bile, therefore biliary atresia prevents the excretion of conjugated bilirubin.

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5
Q

What classification system is used for classifying biliary atresia?

A

The Ohi system.

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6
Q

What does the Ohi system classify biliary atresia according to?

A

The degree of anatomical damage

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7
Q

What are the 3 types of biliary atresia according to the Ohi system?

A

Type I (~10%): patent proximal ducts but atresia of the common bile duct

Type II (~2%): atresia of the common bile duct and hepatic duct

Type III (~88%): atresia of almost all extrahepatic ducts, including the porta hepatis

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8
Q

What is the most common type of biliary atresia?

A

Type III - atresia of almost all extrahepatic ducts, including the porta hepatis

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9
Q

Biliary atresia can occur on its own (‘isolated’) or with other congenital anomalies.

What is the most common congenital anomaly?

A

Biliary atresia splenic malformation syndrome –> associated with polysplenia, situs inversus, cardiac malformations, and vascular anomalies.

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10
Q

How do neonates with biliary atresia typically present?

A
  • Jaundice (beyond 14 days).
  • Typically associated with pale stools and dark urine due to biliary obstruction
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11
Q

Why can some neonates with biliary atresia present with bruising?

A

Due to vitamin K deficiency.

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12
Q

What is the most common finding on clinical examination in biliary atresia?

A

Jaundice

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13
Q

In the later stages of biliary aresia, usually seen in infants over three months old, what are some clinical findings?

A
  • Hepatosplenomegaly
  • Ascites
  • Failure to thrive: due to malabsorption of fats
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14
Q

What are sime other causes of obstructive jaundice that can occur in the neonatal period?

A

1) Choledochal cyst

2) Cholelithiasis

3) Spontaneous perforation of the bile duct

4) Allagile syndrome: a rare disorder where a baby is born with fewer bile ducts than normal

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15
Q

What are some differentials for biliary atresia (not related to the gallbladder)?

A
  • CF
  • Lipid storage disorders
  • Idiopathic neonatal hepatitis
  • Congenital infections
  • Alpha-1-antitrypsin (A1AT) deficiency
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16
Q

Biliary atresia should be suspected in neonates with jaundice lasting more than how long?

A

Term babies - >14 days
Premature babies >21 days

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17
Q

What is the 1st line investigation in biliary atresia?

A

Conjugated & unconjugated bilirubin.

Total bilirubin may be normal, whereas conjugated bilirubin is abnormally high.

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18
Q

Investigations in biliary atresia?

A

1) Serum bilirubin (conjugated and unconjugated)

2) Newborn blood spot screening: does not test for biliary atresia but includes test for CF (differential)

2) LFTs: will be abnormal with conjugated hyperbilirubinaemia and raised gamma-glutamyltransferase (GGT)

4) US

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19
Q

What will LFTs shown in biliary atresia?

A

Will be abnormal with conjugated hyperbilirubinaemia and raised gamma-glutamyltransferase (GGT).

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20
Q

When should all children with neonatal jaundice have their bilirubin levels tested?

A

Within 6 hours

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21
Q

What is the 1st line imaging in biliary atresia?

A

US

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22
Q

What are the key US features of biliary atresia?

A

1) The triangular cord sign: an echogenic sign representing the fibrous remnant of the extrahepatic bile duct

2) Hepatic artery changes, which will be mainly larger

3) Gallbladder ghost triad: small/atretic gallbladder with a length less than 19 mm, irregular or lobular contour, lack of smooth echogenic mucosal lining with an indistinct wall

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23
Q

What is the preferred diagnostic investigation in biliary atresia?

A

Percutaneous liver biopsy –> would typically show bile duct proliferation with bile plugs.2

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24
Q

Gold standard investigation for biliary atresia?

A

Operative cholangiography –> BUT it is only used if there is diagnostic uncertainty before treatment.

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25
What is the only definitive treatment for biliary atresia?
Surgical intervention.
26
Ideally, when should surgery be performed in biliary atresia?
<45 days of life --> provides the best success rate and helps avoid liver transplantation.
27
What is the surgical intervention of choice in biliary atresia?
Kasai portoenterostomy.
28
What does a Kasai portoenterostomy involve?
This procedure involves removing the damaged bile ducts and replacing them with a loop of intestine to allow bile to flow from the liver to the intestine. The porta hepatis is anastomosed (in a Roux-en-Y fashion) to a part of the jejunum at the level of the hepatic hilum. The jejunum is then attached on the other end to the rest of the small intestine forming the Y-shaped connection.
29
If a Kasai portoenterostomy fails in biliary atresia, what is next step?
Liver transplant (sometimes a liver transplant may be required even after a successful Kasai procedure due to the progression of liver damage).
30
What are the indications for liver transplant in biliary atresia?
1) Failure of the Kasai procedure and reappearance of symptoms 2) Growth retardation that is non-responsive to intensive nutritional support 3) Development of portal hypertension and its complications (variceal bleeding, ascites) 4) Progressive liver dysfunction marked by pruritus and coagulopathy
31
What will all patients with biliary atresia require for the first year of life post-surgery?
Antibiotic prophylaxis to prevent the development of cholangitis.
32
What are the most common complications of biliary atresia?
Related to surgical intervention: 1) Ascending cholangitis: would present with a recurrence of the initial symptoms (e.g. jaundice and pale stools) 2) Obstruction of the Roux-en-Y loop: causing recurrent or delayed ascending cholangitis 3) Cirrhosis --> can eventually lead to HCC 4) Portal hypertension
33
Clinical features of ulcerative colitis (UC)?
- Diarrhoea (often bloody ± mucus) - Faecal urgency/incontinence - Tenesmus - Abdo pain (often LLQ) - Bloating - Fatigue/malaise - Anorexia - Fever - Weight loss
34
What are some extra-intestinal manifestations of UC?
MSK: - tenosynovitis - dactylitis - osteopenia, osteomalacia & osteoporosis Skin: - erythema nodosum - pyoderma gangrenosum - aphthous mouth ulcers Eyes: - episcleritis - scleritis - uveitis Haem: - anaemia - increased VTE risk Hepatobiliary: - primary sclerosing cholangitis - gallstones - autoimmune hepatitis
35
What is erythema nodosum?
A form of panniculitis. Presents as ender, erythematous subcutaneous nodules predominantly affecting the anterior shin region.
36
What conditions is erythema nodosum associated with?
- Strep infections - sarcoidosis - TB - IBD
37
Key stool test in UC?
Faecal calprotectin (raised in IBD)
38
Investigations in UC?
1) Flexible sigmoidoscopy 2) Colonoscopy 3) AXR 3) CT with contrast
39
Flexible sigmoidoscopy vs colonscopy in UC?
Flexible sigmoidoscopy --> Imaging is limited to the distal colon but requires less bowel preparation than a colonoscopy. Colonoscopy --> May be required if disease extends more proximally
40
What are the histopathological changes seen in UC?
- Continuous inflammation that does not extend beyond the colonic submucosa - Erythema +/- ulceration - Crypt abscesses and neutrophil infiltration - Depleted colonic goblet cells - Inflammatory polyps
41
What are crypt abscesses?
The accumulation of inflammatory cells within the crypts of the GI tract.
42
Crypt abscesses can be found in both UC and Crohn's. Which type of IBD are they more commonly found in?
UC
43
What are goblet cells?
Goblet cells are intestinal mucosal epithelial cells that serve as the primary site for nutrient digestion and mucosal absorption.
44
UC is classified based on the extent and severity of colonic involvement. What is: a) ulcerative proctitis b) proctosigmoiditis c) left-sided colitis d) pancolitis
a) Inflammation is limited to the rectum. b) Involves the rectum and sigmoid colon. c) Extends from the rectum to the splenic flexure. d) Affects the entire colon.
45
Is rectal bleeding more common in Crohn's or UC?
UC
46
The severity of UC is usually classified as being mild, moderate or severe. Define: a) mild b) moderate c) severe
a) <4 stools/day, only a small amount of blood b) 4-6 stools/day, varying amounts of blood, no systemic upset c) >6 stools/day + features of systemic upset (pyrexia, tachycardia, anaemia, raised inflammatory markers)
47
What medications are often the first line of therapy for mild-to-moderate UC?
Aminosalicylates (5-ASAs) e.g. mesalazine, sulfasalazine
48
What types of medications may be involved in the management of UC?
1) Aminosalicylates (5-ASAs) 2) Corticosteroids 3) Immunomodulators e.g. Azathioprine, Methotrexate 4) Biologics 5) Janus kinase (JAK) inhibitors
49
What is the 1st line management of a severe UC flare?
IV steroids usually given first line (IV ciclosporin may be used if steroids are contraindicated).
50
In a severe UC flare, if there has been no improvement after 24h treatment with IV steroids, what should be considered?
Consider adding IV ciclosporin or surgery.
51
What is the stepwise management of mild-mod UC flare (proctitis)?
1) Topical (rectal) aminosalicylate 2) If remission is not achieved within 4 weeks, add an oral aminosalicylate 3) If remission still not achieved add topical or oral corticosteroid
52
What is the stepwise management of mild-mod UC flare (proctosigmoiditis and left-sided ulcerative colitis)?
1) Topical (rectal) aminosalicylate 2) If remission not achieved within 4 weeks, add a high-dose oral aminosalicylate OR switch to a high-dose oral aminosalicylate and a topical corticosteroid 3) If remission still not achieved stop topical treatments and offer an oral aminosalicylate and an oral corticosteroid
53
What is the stepwise management of mild-mod UC flare (extensive disease)?
1) topical (rectal) aminosalicylate and a high-dose oral aminosalicylate 2) if remission is not achieved within 4 weeks, stop topical treatments and offer a high-dose oral aminosalicylate and an oral corticosteroid
54
What is 1st line for maintaining remission after a mild-mod UC flare?
For proctitis and proctosigmoiditis --> Topical aminosalicylate (+/- oral aminosalicylate). For left-sided and extensive UC --> low maintenance dose of an oral aminosalicylate
55
What is the maintenance for UC following a severe relapse or >=2 exacerbations in the past year
Oral azathioprine or oral mercaptopurine
56
Is the risk of colorectal cancer higher in UC or Crohn's?
UC
57
what are some complications of UC?
1) colorectal cancer 2) toxic megacolon 3) primary sclerosing cholangitis
58
Clinical features of Crohn's disease?
- Persistent diarrhoea (may be bloody ± mucus ± pus) - Abdominal pain (RLQ pain/mass may be reported if terminal ileum affected) - Tenesmus - Fever - Malaise/fatigue - Anorexia - Aphthous ulcers - Perianal lesions (fissures, abscesses, fistulas) - Weight loss/faltering growth
59
Location of abdo pain in UC vs Crohn's?
UC --> typically LLQ Crohn's --> typically RLQ
60
What are some extra-intestinal manifestations of Crohn’s disease?
MSK: - pauci-articular arthritis - enthesitis - tenosynovitis - dactylitis - osteopenia, osteomalacia and osteoporosis Skin: - erythema nodosum - aphthous mouth ulcers - psoriasis, pyoderma gangrenosum Eyes: - episcleritis - uveitis
61
What is the most common extra-intestinal symptom of Crohn's?
pauci-articular arthritis
62
Histology findings in Crohn's?
- Transmural inflammation (i.e. full thickness) - Cobblestone appearance of the mucosa caused by fissures and deep ulcers - Non-caseating granulomas - Skip lesions (due to patchy distribution of inflammation)
63
Are granulomas seen in Crohn's or UC?
Crohn's (non-ceseating)
64
Which IBD gives a 'cobblestone appearance'?
Crohn's
65
Are continuous lesions seen in Crohn's or UC?
UC
66
Complications of Crohn's vs UC?
Crohn's: - Strictures - Bowel obstruction - Fistulas - Perianal abscesses UC: - GI bleeding - Toxic megacolon - Peritonitis - Adenocarcinoma
67
Give some GI complications seen in Crohn's
1) Strictures 2) Fistulas 3) Abscesses 4) Perianal disease 5) Malabsorption and nutritional deficiencies 6) Colorectal cancer (risk less than UC though)
68
What causes strictures in Crohn's? How may it present?
Chronic inflammation and fibrosis may lead to narrowing of the bowel lumen. Obstructive symptoms such as abdominal pain, nausea, vomiting, and constipation.
69
Management of strictures in Crohn's ?
Endoscopic balloon dilation, stricturoplasty, or bowel resection, depending on the severity and location.
70
What causes fistulas in Crohn's? Symptoms?
Abnormal connections between different bowel segments or between the bowel and other organs (e.g., bladder, vagina) may develop due to transmural inflammation. Fistulas can cause abscess formation, recurrent infections, and malabsorption.
71
How are abscesses caused by Crohn's disease typically managed?
Abx and percutaneous or surgical drainage.
72
What are some perianal complications seen in Crohn's?
- fissures - abscesses - fistulas
73
What lifestyle advice should be given to all patients with Crohn's?
patients should be strongly advised to stop smoking
74
1st line for maintaining remission in Crohn's?
azathioprine or mercaptopurine
75
1st line for inducing remission in Crohn's flare?
1st line --> Steroids (oral, topical or IV) 2nd line --> 5-ASA drugs (e.g. mesalazine) but not as effective
76
What is a perianal fistula?
an inflammatory tract or connection between the anal canal and the perianal skin
77
What is the investigation of choice for a suspected perianal fistula?
MRI --> can be used to determine if there is an abscess and if the fistula is simple (low fistula) or complex (high fistula that passes through or above muscle layers).
78
Management of a perianal fistula?
If symptomatic --> oral metronidazole May require surgery.
79
What is the management of a perianal abscess in Crohn's?
incision and drainage combined with antibiotic therapy
80

Yr 4 - Paediatrics (78 decks)

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