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Gastrointestinal > Pediatric Cholestasis > Flashcards

Pediatric Cholestasis Flashcards

1
Q

What type of bilirubin is elevated in cholestasis?

A

Direct

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2
Q

What are the usual etiologies of cholestasis?

A

Obstructive
Metabolic/genetic
Infx

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3
Q

What is the enzyme that takes Hene and converts it to biliverdin? Bilirubin?

A

heme oxygenase, then biliverdin reductase

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4
Q

Unconjugated bili binds to what?

A

Albumin

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5
Q

What is the signal for the bladder to contract?

A

CCK

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6
Q

What happens to conjugated bili in the intestines?

A

Converted into urobilinogen by intestinal flora, and then secreted by kidneys

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7
Q

What is the structure through which bile and pancreatic secretions pas through prior to passing through to the duodenum?

A

Ampulla of Vater

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8
Q

What are the components of bile?

A

Bili
Cholesterol/phospholipids
Bile pigments/acids

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9
Q

What is the function of bile?

A

Excreting toxins

Modulate cholesterol

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10
Q

What are the ssx of cholestasis in peds?

A

poor feeding/weight gain

Irritability

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11
Q

Acholic bile suggests what?

A

Obstruction

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12
Q

What are the congenital ssx?

A

Microcephaly
Growth restriction
Low platelets

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13
Q

What is the normal range of direct bilirubin?

A

Greaters than 2 mg/dL

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14
Q

What are the labs like in cholestasis/obstructive pattern of cholestasis? (3)

A

Elevated alk phos
Elevated direct bili
Elevated GGT

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15
Q

What are the labs like in non-cholestatic/hepatocellular pattern of cholestasis? (2)

A

AST/ALT elevation

Direct/indirect bili

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16
Q

What fraction of newborn jaundice is physiologic?

A

2/3

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17
Q

In newborn jaundice, what type of bili is elevated?

A

Indirect

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18
Q

How long should newborn jaundice last? If it lasts longer than this, what should you suspect?

A

Two weeks

Suspect cholestasis

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19
Q

What prolongs neonatal jaundice? (2)

A

Breast milk
Prematurity
Poor oral intake

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20
Q

What are the characteristics of premature neonatal jaundice?

A

Peaks later, lasts longer

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21
Q

What are the ssx of biliary atresia? (3)

A

Cholestatic jaundice
Hepatomegaly
Acholic stools

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22
Q

What is the cause of biliary atresia?

A

Progressive inflammatory destruction of bile ducts

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23
Q

When does biliary atresia usually present?

A

50% in the first few weeks

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24
Q

What are the two types of biliary atresia? Describe each.

A

Perinatal/acquired (normal birth o/w, then recurs)

Embryonic (cholestatic jaundice at birth, w/o jaundice free interval)

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25
Q

What is the single, most useful test to diagnose biliary atresia?

A

Bx

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26
Q

How do you confirm the diagnosis of biliary atresia?

A

Laparotomy and intraoperative cholangiography

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27
Q

What is the treatment for biliary atresia?

A

Kasai procedure (hook up small intestines to atretic gallbladder

28
Q

What is the prognosis for biliary atresia?

A

72% survive 10 years.

29
Q

What is the difference between cholelithiasis, cholecystitis, and choledocolithiasis?

A 
Lithiasis = gallstones
Cholecystitis = Inflamed gallbladder
Choledocolithaiasis = gallstones in the duct
30
Q

What are ssx of cholelithiasis?

A

n/v
Jaundice
RUQ pain

31
Q

What is the prognosis for cholelithiasis in infants?

A

Most pass spontaneously

32
Q

What are the complications with cholelithiasis?

A

Pancreatitis
Ascending cholangitis
Cholecystitis

33
Q

What is the treatment for cholelithaisis?

A

ERCP and cholecystectomy

34
Q

What are choledochal cysts?

A

Congenital cysts that cause dilation of the biliary tree and CBD

35
Q

What are the ssx of choledochal cysts?

A

Usual cholestatic lab pattern, + palpable mass in a newborn

36
Q

What are the complications of choledochal cysts?

A

Premalignant (cholangiocarcinoma)

37
Q

What is the treatment for choledochal cysts?

A

surgical -roux-en-y choledochojejunostomy

38
Q

What is Alagille syndrome?

A

AD familial intrahepatic cholestasis, caused by a mutation in jagged 1 gene

39
Q

What causes the cholestasis in Alagille syndrome?

A

Paucity of interlobular bile ducts

40
Q

What is the usual presentation of Alagille syndrome?

A

Cholestasis
Pruritis by 6 mo
Xanthomas

41
Q

What are the face findings with Alagille syndrome?

A

Prominent forehead, deep set eyes, small pointed chin

42
Q

What are the CV findings with Alagille syndrome?

A

Peripheral pulmonic stenosis

43
Q

What are the vertebral findings with Alagille syndrome?

A

Butterfly vertebrae

44
Q

What are the eye findings with Alagille syndrome?

A

Posterior embryotoxon

45
Q

What percent of kid with Alagille syndrome have growth retardation?

A

50%

46
Q

What is the prognosis for Alagille syndrome?

A

Usually die around 20s d/t cardiac diseae

47
Q

What is the treatment for Alagille syndrome?

A

Liver transplant

48
Q

What is the genetic mutation in A1ATD?

A

PiZ and PiS

49
Q

What is the stain that is used to diagnose A1ATD?

A

PAS positive

50
Q

What are the ssx of A1ATD?

A

Liver and lung issues. May have cirrhosis in the first few months of life

51
Q

What is the treatment for A1ATD?

A

Liver transplant

52
Q

Which phenotype of A1ATD most commonly causes COPD?

A

ZZ phenotype

53
Q

What is PFIC 1-3?

A 
1 = ATP issue
2 = bili transporter
3 = MDR3
54
Q

What is benign recurrent intrahepatic cholestasis?

A

Mutations in FIC1 or BSEP,

55
Q

What is Dubin-Johnson syndrome?

A

Defect in MDR2

56
Q

What is the treatment for benign recurrent intrahepatic cholestasis?

A

urso

57
Q

How does TPN cause cholestasis?

A

IV lipids/phytosterols via regulation of BSEP

58
Q

What is association of congenital hypothyroidism with gallbladder issues?

A

Prolonged physiologic jaundice,

59
Q

What are the effects of congenital hypothyroidism?

A

Permanent MR

60
Q

What is congenital panhypopituitarism?

A

Cortisol, CH, thyroid hormone promote bile formation and secretion

61
Q

What is the GGT like in congenital panhypopituitarism?

A

normal

62
Q

What are the ssx of congential panhypopituitarism?

A

UTI and sepsis

63
Q

What is the cause of galactosemia? Ssx? Treatment?

A

AR deficiency in galactose-1-phosphate Uridyl transferase.

Liver injury, HSM, hypoglycemia

Stopping lactose

64
Q

What type of fats do not need the biliary system?

A

MCFAs

65
Q

What is the treatment for chronic cholestasis?

A

ADEK supplementation

Gastrointestinal (42 decks)

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