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Paediatrics > Hepatosplenomegaly > Flashcards

Hepatosplenomegaly Flashcards

1
Q

What is the normal liver span at 2 months?

A

4cm

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2
Q

What is the normal liver span at 12 years?

A

9 cm

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3
Q

What is the normal liver span in an adult?

A

12-15 cm

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4
Q

What does the presentation of jaundice and ascites point to?

A

Portal hypertension

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5
Q

What are the functions of the liver?

A
  1. Bile production and excretion
  2. Metabolizes fats, proteins and carbohydrates
  3. Storage of nutrients( vitamins, minerals,glycogen)
  4. Excretes waste products into bile(bilirubin, cholesterol, drugs, toxins)
  5. Innate and adaptive immunity
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6
Q

What are the 8 causes of hepatosplenomegaly?

A
  1. Inflammation/infection
  2. Reticuloendothelial hyperplasia
  3. Storage/metabolic disorder
  4. Biliary obstruction
  5. Venous congestion
  6. Fat accumulation
  7. Space occupying lesions
  8. Miscellaneous
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7
Q

What are the inflammatory/infection causes of hepatosplenomegaly?

A
  1. TORCHES
  2. Viral-HIV, Hepatitis B
  3. Bacterial- TB, Typhoid
  4. Parasite-bilharzia, malaria
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8
Q

What tests would you do to test causes of inflammation?

A
  1. FBC
  2. ESR
  3. Mantoux
  4. LFT
  5. Blood culture
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9
Q

What are the causes of reticuloendothelial hyperplasia?

A
  1. Septicaemia
  2. Malignancy
    - lymphoma, leukemia, neuroblastoma
  3. Granuloma(TB)
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10
Q

What are the causes of storage/metabolic disease?

A
  1. Glycogen storage disease
  2. Lipid storage disease
  3. Galactosemia
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11
Q

What are the causes of biliary obstruction?

A
  1. Biliary atresia

2. choledochal cyst

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12
Q

What are the causes of venous congestion?

A
  1. Cardiac failure
  2. Pericardial effusion
  3. Constrictive pericarditis
  4. Budd Chiari syndrome
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13
Q

What special investigations would you do in venous congestion?

A
  1. Chest X-ray
  2. ECG
  3. Echocardiogram
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14
Q

What are the causes of fat infiltration?

A
  1. Uncontrolled DM
  2. Malnutrition
  3. Hepatotoxic drugs
  4. Reye’s syndrome
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15
Q

What tests would you do check for fat infiltrates?

A
  1. LFT

2. Serum proteins

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16
Q

What causes the space occupying lesions?

A
  1. Neoplasm

2. Absess

17
Q

What tests would you do in space occupying lesions?

A
  1. Alpha feto protein

2. Abdominal ultrasound

18
Q

What is cholestasis?

A

Stasis of bile

19
Q

When should infants with jaundice be evaluated for cholestasis?

A

After 14 days

20
Q

What is regarded as abnormal in cholestasis?

A

The conjugated bilirubin value of more than 1mg/dL If the the total bilirubin is less than 5 OR if the conjugated bilirubin is more than 20% of the total

21
Q

What are the extra-hepatic causes of cholestasis?

A
  1. Biliary atresia
  2. Choledochal cyst
  3. Neoplasm
  4. Gallstones
    5 Neonatal sclerosing cholangitis
22
Q

What are the intra-hepatic causes of cholestasis?

A
  1. idiopathic
  2. Alagille syndrome
  3. Hepatitis
23
Q

What is the presentation of cholestasis caused by bile retention or regurgitation?

A
  1. Pruritis
  2. Xanthomata
  3. Jaundice
24
Q

What is the presentation of cholestasis caused by portal hypertension?

A
  1. Hypersplenism
  2. Ascites
  3. Varices
25
Q

What is the presentation of cholestasis caused by reduced bile delivery to small intestine?

A

Malabsorption

  1. Malnutrition
  2. Steatorrhea
  3. Rickets caused by vitamin d malabsorption
26
Q

What lab tests are usually done in these children?

A
  1. FBC
  2. LFT
  3. Urine and blood culture
  4. Coagulation studies
27
Q

What would we see on sonar if biliary atresia?

A

The inability to visualize the gall bladder and triangular cord sign

28
Q

What is the most definitive test?

A

Liver biopsy

29
Q

What is biliary atresia?

A
  • an inflammatory,idiopathic disease of the bile ducts causing fibro-obliteration.
  • leads to bile obstruction and then to liver failure
30
Q

How do children with biliary atresia present?

A

They are jaundiced, with acholic stools and dark urine

31
Q

What do we find on liver function tests of children with biliary atresia?

A
  1. Elevated levels of conjugated bilirubin
  2. Elevated GGT
  3. Elevated ALP to more than 5 times the normal
32
Q

If left untreated what is the life expectancy of biliary atresia?

A

2 years

33
Q

What do you find on biopsy of the liver?

A

Biliary cirrhosis

34
Q

How can we classify biliary atresia?

A
  1. Post natal form(presents with isolated biliary atresia)

2. Fetal form(associated with other congenital malformations like meal rotation, asplenia/polysplenia)

35
Q

What surgical procedure can we do for patients with biliary atresia?

A

A kasai procedure where the small intestine is connected to the liver directly

36
Q

What is the success of the kasai procedure based on?

A
  1. The age of presentation
  2. Size of bile duct remnants
  3. Experience of the centre performing the procedure
37
Q

What is the ideal age to do a kasai procedure?

A

45-60 days

38
Q

What is a complication of doing a kasai procedure?

A

-ascending cholangitis

Paediatrics (40 decks)

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