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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
What is the presentation of cholestasis caused by reduced bile delivery to small intestine?
Malabsorption 1. Malnutrition 2. Steatorrhea 3. Rickets caused by vitamin d malabsorption
26
What lab tests are usually done in these children?
1. FBC 2. LFT 3. Urine and blood culture 4. Coagulation studies
27
What would we see on sonar if biliary atresia?
The inability to visualize the gall bladder and triangular cord sign
28
What is the most definitive test?
Liver biopsy
29
What is biliary atresia?
- an inflammatory,idiopathic disease of the bile ducts causing fibro-obliteration. - leads to bile obstruction and then to liver failure
30
How do children with biliary atresia present?
They are jaundiced, with acholic stools and dark urine
31
What do we find on liver function tests of children with biliary atresia?
1. Elevated levels of conjugated bilirubin 2. Elevated GGT 3. Elevated ALP to more than 5 times the normal
32
If left untreated what is the life expectancy of biliary atresia?
2 years
33
What do you find on biopsy of the liver?
Biliary cirrhosis
34
How can we classify biliary atresia?
1. Post natal form(presents with isolated biliary atresia) | 2. Fetal form(associated with other congenital malformations like meal rotation, asplenia/polysplenia)
35
What surgical procedure can we do for patients with biliary atresia?
A kasai procedure where the small intestine is connected to the liver directly
36
What is the success of the kasai procedure based on?
1. The age of presentation 2. Size of bile duct remnants 3. Experience of the centre performing the procedure
37
What is the ideal age to do a kasai procedure?
45-60 days
38
What is a complication of doing a kasai procedure?
-ascending cholangitis

Paediatrics (40 decks)

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