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3rd Year - Paediatrics > 7.4. Gastroenterology - Approach to the Yellow Baby > Flashcards

7.4. Gastroenterology - Approach to the Yellow Baby Flashcards

1
Q

What are the Different Liver Function Tests?

A
  1. Bilirubin - Total / Split Bilirubin
  2. Alanine Aminotransferase (ALT) / Aspartate Aminotransferase (AST)
  3. Alkaline Phosphatase
  4. Gamma Glutamyl Transferase (GGT)
  5. Coagulation - Prothrombin Time (PT / INR) / Activated Thromboplastin Time (APTT)
  6. Albumin
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2
Q

What Liver Function Tests are elevated in Hepatocellular Damage (Hepatitis)?

A
  1. Bilirubin

2. Alanine Aminotransferase (ALT) / Aspartate Aminotransferase (AST)

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3
Q

What Liver Function Tests are Elevated in Biliary Disease?

A
  1. Bilirubin
  2. Alkaline Phosphatase
  3. Gamma Glutamyl Transferase (GGT)
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4
Q

What is the Clinical Manifestation of Paediatric Liver Disease?

A
  1. Jaundice
  2. Incidental Finding of Abnormal Boot Test
  3. Symptoms / Signs of Chronic Liver Disease - Growth Failure
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5
Q

What is Jaundice?

A
  1. Yellow Discolouration of Skin and Tissues due to Accumulation of Bilirubin
  2. Usually most obvious in the Sclera
  3. Usually Visible when Total Bilirubin > 40-50 umol/L
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6
Q

What are the Key Stages of Bilirubin Metabolism?

A
  1. Post-Mature Erythrocytes
  2. Unconjugated Bilirubin
  3. Liver
  4. Conjugation - Conjugated Bilirubin
  5. Bile
  6. Small Intestine - Urobilinogen
  7. a) Liver
  8. b) Excreted by Kidneys
  9. c) Stercobilin (Faecal Pigment)
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7
Q

What are the 3 Types of Neonatal Jaundice?

A
  1. Early - < 24 Hours Old
  2. Intermediate - 24 Hours - 2 Weeks Old
  3. Prolonged - > 2 Weeks Old
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8
Q

What are the causes of Early (< 24 Hours Old) Neonatal Jaundice?

A
  1. Haemolysis
  2. Sepsis
    Note - ALWAYS PATHOLOGICAL
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9
Q

What are the Causes of Intermediate (24 Hours - 2 Weeks Old) Neonatal Jaundice?

A
  1. Physiological
  2. Breast Milk
  3. Sepsis
  4. Haemolysis
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10
Q

What are the Causes of Prolonged (> 2 Weeks Old) Neonatal Jaundice?

A
  1. Extrahepatic Obstruction - Conjugated
  2. Neonatal Hepatitis - Conjugated
  3. Hypothyroidism - Unconjugated
  4. Breast Milk - Unconjugated
    Note - Conjugated Jaundice in infants is ALWAYS abnormal and ALWAYS requires further investigation
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11
Q

What is Physiological Jaundice?

A

Unconjugated Jaundice, Develops after the First Days of Life, due to:

  1. Shorter Erythrocyte Life Span in Infants (80-90 days)
  2. Relative Polycythaemia
  3. Relative Immaturity of Liver Function
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12
Q

What is Breast-Milk Jaundice?

A

Unconjugated Jaundice which can persist up to 12 Weeks.
The Exact Reason for Prolongation of Jaundice in Breastfed Infants in unclear but it is suspected:
1. Inhibition of UDP by Progesterone Metabolite
2. Increased Enterohepatic Circulation

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13
Q

What are the Causes of Haemolysis in Early / Intermediate Unconjugated Infant Jaundice?
What are the Tests for these causes?

A
  1. ABO Incompatibility - Blood Group DCT
  2. Rhesus Disease - Blood Group DCT
  3. Bruising / Cephalhaematoma - Clinical Examination
  4. Red Cell Membrane Defects (e.g. Spherocytosis) - Blood Film
  5. Red Cell Enzyme Defects (e.g. G6PD) - G6PD Assay
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14
Q

What is the Investigation for Sepsis?

A
  1. Urine + Blood Cultures

2. ToRCH Screen - Toxoplasmosis / Rubella / CMV / Herpes

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15
Q

What is the Pathology of Kernicterus?

A
  1. Unconjugated Bilirubin is Fat-Soluble so can cross the Blood-Brain Barrier
  2. Neurotoxic and Deposits in the Brain
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16
Q

What are the Early Signs of Kernicterus?

A

Encephalopathy

  1. Poor Feeding
  2. Lethargy
  3. Seizures
17
Q

What are the Late Consequences of Kernicterus?

A
  1. Severe Choreoathetoid Cerebral Palsy
  2. Learning Difficulties
  3. Sensorineural Deafness
18
Q

What is the Treatment of Unconjugated Jaundice?

A

Phototherapy - Visible Light converts Bilirubin to Water Soluble Isomer (Photoisomerisation)

19
Q

What is the most Important Test in Prolonged Jaundice?

A

Split Bilirubin

Note - Conjugated Jaundice in infants is ALWAYS abnormal and ALWAYS requires further investigation

20
Q

What are the potential causes of Biliary Obstruction?

A
  1. Biliary Atresia - Conjugated Jaundice / Pale Stools
  2. Choledochal Cyst - Conjugated Jaundice / Pale Stools
  3. Alagille Syndrome - Intrahepatic Cholestasis / Dysmorphism / Congenital Cardiac Disease
21
Q

What is Biliary Atresia?

A

Congenital Fibro-Inflammatory Disease of the Bile Ducts leading to Destruction of the Extra-Hepatic Bile Ducts

22
Q

What are the Clinical Features of Biliary Atresia?

A
  1. Prolonged, Conjugated Jaundice
  2. Pale Stools
  3. Dark Urine
  4. May progress to Liver Failure (if not Identified / Treated) - Timely Diagnosis Critical as Determines Prognosis
    Note - Most common indication for Liver Transplantation in Children
23
Q

What must Always be Assessed in Infants with Prolonged Jaundice?

A

ALWAYS Assess Stool Colour in Infants with Prolonged Jaundice

24
Q

What is the Treatment of Biliary Atresia?

A

Hepatico-Jejunostomy

25
Q

What is the Aim of Assessment of Prolonged Infant Jaundice?

A

To Diagnose Patients with Biliary Atresia Early

26
Q

What are the Tests for Biliary Atresia?

A
  1. Split Bilirubin
  2. Stool Colour
  3. Ultrasound
  4. Liver Biopsy
27
Q

What are the Tests for a Choledochal Cyst?

A
  1. Split Bilirubin
  2. Stool Colour
  3. Ultrasound
28
Q

What are the Tests for Alagaille Syndrome?

A
  1. Dysmorphism

2. Genotype

29
Q

What are the Causes of Neonatal Hepatitis?

What are the Tests for these Causes?

A
  1. Alpha-1-Antitrypsin Deficiency - Phenotype / Level
  2. Galactosaemia - GAL-1-PUT
  3. Tyrosinaemia - Amino-Acid Profile
  4. Urea-Cycle Defects - Ammonia
  5. Haemochromatosis - Iron Studies / Liver Biopsy
  6. Glycogen Storage Disorders - Biopsy
  7. Hypothyroidism - Thyroid Function Tests
  8. Viral Hepatitis - Serology / PCR
  9. Parenternal Nutrition - History

3rd Year - Paediatrics (40 decks)

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