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Gastrointestinal System > EXAM #3: PEDIATRIC CHOLESTASIS > Flashcards

EXAM #3: PEDIATRIC CHOLESTASIS Flashcards

1
Q

What lab is characteristic for pediatric cholestasis?

A

Elevated direct bilirubin

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2
Q

What are the etiologies of pediatric cholestasis?

A

1) Obstructive
2) Metabolic/ genetic
3) Infectious

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3
Q

What are the functions of bile?

A

1) Excretion of toxins
2) Modulation of cholesterol metabolism
3) Absorption of lipids and fat soluble vitamins

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4
Q

How does cholestasis present?

A

1) Jaundice/icterus (4-5 mg/dL)
2) Dark urine
3) Pruritus
4) RUQ pain (older kids)

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5
Q

What sign is suggestive for obstructive jaundice?

A

Acholic stool (gray-colored)

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6
Q

How should you work-up a child with jaundice?

A

Order:

1) Bilirubin
2) ALP
3) GGT

If older than 2 weeks, get a direct bilirubin in addition to total

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7
Q

What is the definition of a direct hyperbilirubinemia?

A
  • Greater than 2mg/dL

- More than 20% of total

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8
Q

What is the obstructive pattern of cholestasis?

A

1) Elevated direct bilirubin

2) ALP, GGT

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9
Q

What type of hyperbilirubinemia is associated with physiologic/ neonatal jaundice?

A

Excess unconjugated bilirubin

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10
Q

What is breast-feeding jaundice?

A
  • Mom is not producing much milk initially

- Caloric deficiency leads to jaundice

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11
Q

What is breast-milk jaundice?

A
  • 2nd week of life

-

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12
Q

What should you do if a child is jaundiced for more than 2-3 weeks?

A

Order a direct bilirubin

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13
Q

What is Biliary Atresia?

A

Progressive inflammation of bile ducts in the first few weeks of life

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14
Q

How serious is Biliary Atresia?

A

V. serious, requires liver transplant

Primary cause of pediatric death from liver disease

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15
Q

What are the two types of Biliary Atresia?

A

1) Perinatal/ acquired
2) Embryonic

If a child is jaundiced AT BIRTH it is a red flag

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16
Q

What is the sequence of testing to diagnose Biliary Atresia?

A

1) Elevated direct bilirubin
2) Ultrasound
3) Biopsy

TO OR

4) Laparotomy
5) Intraoperative cholangiography

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17
Q

How is Biliary Atresia initially managed in the OR?

A

Kasai procedure
- Connects the small intestine to liver

Note that this is more successful the quicker it is performed

18
Q

What is typically required following the Kasai procedure for Biliary Atresia?

A

Liver transplant

19
Q

What is the definition of choledocolithiasis?

A

Stone in common bile ducts

20
Q

What is acalculous cholecystitis?

A

Cholecystitis (inflammation) without stone

21
Q

How do gallstones and cholecystitis present in kids?

A
  • Nausea and vomiting
  • Jaundice
  • RUQ pain
22
Q

What is the treatment for gallstones/ cholecystitis in kids?

A

1) ERCP

2) Cholecystectomy

23
Q

What is a choledochal cyst?

A

Cyst in the common bile duct

24
Q

What is unique about the presentation of a choledochal cyst?

A

Palpable mass in the newborn

25
Q

What is an important complication of a choledochal cyst?

A

Premalignant for cholangiocarcinoma

26
Q

How is a choledochal cyst treated?

A

Roux-en Y choledochochojejunostomy

27
Q

What is Alagille Syndrome?

A
  • Autosomal dominant mutation in Jagged 1 gene/ Notch receptor
  • Leads to progressive loss of interlobular bile ducts
28
Q

How does Alagille Syndrome present?

A

Typically by 3 years of age:

  • Cholestasis
  • Pruritus
  • Xanthomas
  • Hepatomegaly
29
Q

What anomalies are associated with Alagille Syndrome?

A

1) Facial changes
2) Peripheral pulmonic stenosis
3) Butterfly vertebrae
4) Posterior embryotoxon
5) Growth retardation

30
Q

What is the normal function of Alpha 1 antitrypsin (A1AT)?

A

Inhibition of proteolytic/ protease enzymes

31
Q

What is the normal phenotype of A1AT?

A

PiM

32
Q

What is the most common phenotype of A1AT with lung and liver disease?

A

PiZZ

33
Q

What is the definitive treatment for A1AT deficiency?

A

Liver transplant

34
Q

What is TPN induced cholestasis?

A

Cholestasis caused by Total Parenteral Nutrition in the NICU

35
Q

How is TPN cholestasis treated?

A

1) STOP TPN

2) Reduce copper and manganese in TPN

36
Q

What is a cause of prolonged physiologic jaundice?

A

Congenital hypothyroidism

37
Q

What can congenital hypothyroidism that goes undetected cause?

A

Permanent physical and mental retardation

Thus, it is on the newborn screen

38
Q

What lab can tip you off to congenital panhypopiruitarism?

A

Normal GGT

39
Q

What enzyme mutation is seen in Galactosemia?

A

Autosomal defect in Galactose-1-phoshpate UDP

40
Q

What is important to remember about Galactosemia?

A

1) STOP giving Galactose i.e. Lactose
2) Remember this is can be FATAL in days
3) Is on the newborn screen

41
Q

What type of fats do NOT need the biliary system for metabolism?

A

Medium chain triglycerides

Gastrointestinal System (42 decks)

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