18-4 Flashcards

1
Q

Cholestasis

A

Decrease in bile flow due to:

  • impaired hepatocyte secretion OR
  • obstruction
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2
Q

Cholestasis

A

Decrease in bile flow due to:

  • impaired hepatocyte secretion OR
  • obstruction
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3
Q

Decrease in bile flow?

A

Cholestasis

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4
Q

What 2 things can cause Cholestasis?

A
  • Impaired hepatocyte secretion

- obstruction

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5
Q

What is the hallmark of Cholestasis?

A

Green-brown plugs of bile pigment in hepatocytes and dilated canaliculi

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6
Q

What is the hallmark of Cholestasis?

A

Green-brown plugs of bile pigment in hepatocytes and dilated canaliculi

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7
Q

What labs will be elevated with Cholestasis?

A

Bilirubin
Alkaline phosphatase
GGT

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8
Q

Symptoms of Cholestasis?

A

Jaundice, pruritus
Xanthomas
Decreased Fat-soluble vitamins

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9
Q

2 bile functions?

A
  • Fat absorption

- Excretion of bilirubin, cholesterol, xenobiotics in stool

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10
Q

What is a toxic end product of heme degradation that is normally eliminated from the liver into bile?

A

Bilirubin

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11
Q

Jaundice will present when bilirubin is above?

A

2

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12
Q

What will help determine the cause of the hyperbilirubinemia?

A

Testing for unconjugated and conjugated bilirubin

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13
Q

Where is conjugated bilirubin eliminated?

A

In the urine

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14
Q

Where is unconjugated bilirubin eliminated?

A

INSOLUBLE - cannot be excreted in urine

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15
Q

Unconjugated bilirubin is normally bound to?

A

Albumin until levels are too high and then it diffuses into tissues
= Kernicterus

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16
Q

Increased production of bilirubin (hemolytic anemias) and decreased uptake/conjugation causes?

A

Unconjugated hyperbilirubinemia

17
Q

What are 2 syndromes that can cause conjugated hyperbilirubinemia?

A

Dubin-Johnson - black liver

Rotor syndrome

18
Q

Bile flow obstruction causes?

A

Conjugated hyperbilirubinemia

19
Q

Physiologic jaundice of a newborn is due to?

A

LOW levels of UGT1A1

- bilirubin glucuronidation enzyme

20
Q

Symptoms of Physiologic jaundice of a newborn, what makes it better and what makes it worse?

A

= Mild, transient jaundice

  • Breast milk makes it worse
  • Phototherapy makes it better
21
Q

What type of hyperbilirubinemia is Physiologic jaundice of a newborn?

A

Unconjugated

- low UGT1A1

22
Q

Breast milk includes _____ enzymes that makes jaundice worse for newborns

A

Deconjugating enzymes

23
Q

Phototherapy makes bilirubin _____

A

Soluble = excreted into urine

24
Q

If jaundice persists for 14-21 days after birth, what are 2 categories that it could be due to?

A
  1. Extrahepatic Biliary Atresia

2. Nonobstructive Neonatal Cholestasis

25
Q

If jaundice persists for 14-21 days after birth, what are 2 categories that it could be due to?

A
  1. Extrahepatic Biliary Atresia

2. Nonobstructive Neonatal Cholestasis

26
Q

Extrahepatic Biliary Atresia

A

Obstruction of extrahepatic biliary tree

– unknown cause

27
Q

Gallbladder changes with Extrahepatic Biliary Atresia?

A

Small or absent gallbladder

28
Q

With Extrahepatic Biliary Atresia, there is ____ secretion of technetium-99m in bile and the biliary tree is NOT _____

A

NO secretion of technetium-99m into bile

Biliary tree is NOT VISUALIZED

29
Q

If the biliary tree is not visualized in a neonate, they have jaundice, dark urine and acholic stools, what is the likely diagnosis?

A

Extrahepatic Biliary Atresia

30
Q

Nonobstructive Neonatal Cholestasis

A

Group of disorders that cause newborn jaundice

31
Q

Inborn errors of metabolism and Alpha1 antitrypsin deficiency fall into what category?

A

Nonobstructive Neonatal cholestasis

32
Q

Alagille Syndrome

A
  • Nonobstructive Neonatal cholestasis

= Abnormal biliary tree – altered facies

33
Q

Alagille Syndrome has mutations in?

A

JAG1 or NOTCH2

34
Q

Abnormal biliary tree with altered facies?

A

Alagille Syndrome