Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pediatrics > Cholestasis > Flashcards

Cholestasis Flashcards

1
Q

What is cholestasis

A

Reduced bile formation\flow = retention of biliary substance in liver

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Cholestasis can be classified as

A

1- billary: affecting the ducts (most common)

2- hepatocellular: affecting the parenchyma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How does patient with biliary atresia present to the hospital

A

Thriving and normal patient

Only the pale stool and jaundice

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Pale stool is always associated with

A

Blockage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What are the lab findings in biliary atresia?

A
  • Conjugated hyper-billirubinemia
    (>20%) of totaly.
  • high AST\ALP, GGT, alkaline phosphatase
  • normal albumin\PT
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the US finding in biliary atresia?

A

Triangular cord sign

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

how to preform HIDA scan

A
  • Phenobarb 5 days before it

- showing discontinuation at the CBD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the role of HIDA in biliary atresia

A

Not specific

It can rule out biliary atresia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the gold standard for biliary atresia

A

Intraoperative cholangiogram

Diagnostic and theraputic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the golden period for biliary atresia

A

Within 60 days

After 90 days > high risk for hepatitis and transplant.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the first treatment for biliary atresia?

A

Kasai hepatopotrostomy.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

When does jaundice resolve after kasai?

A

By 3 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the post-operative care after kasai

A
  • UDCA “Ursodeoxycholic acid”
  • Antibiotic (to avoid ascending cholangitis)
  • supplements of (ADEK)
  • increase calories
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What are the prophylaxis for ascending cholangitis post kasai?

A

Bacterim + neomycin

“More intensive if the patient develops cholangitis (4-6wks)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the indications of liver transplant?

A
  • failure of Kasai
  • persistent cholestasis after 6em
  • growth failure
  • liver disease (cirrhosis, varicceal, hepatopulmonary, ascitis, Portal HTN)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is the alagille syndrome?

A

Autosomal dominant characterised by paucity of intralobular ducts

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is the most common form of familal intrahepatic cholestasis

A

Alagille syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What is the genetic mutation in ALGS

A

JAG1 (95%)

NOTCH (2%)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What are the clinical features of ALGS?

A
  • broad forehead
  • deep seated eye
  • small pointy skin
  • hypertolersim
  • straigh nose w\bulbous tip
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is the most common developmental cardiac defect in allagile syndrome

A

Peripheral Pulmonary stenosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What are the associated conditions with ALGS?

A
  • eye: posterior emberyotoxin
  • X-ray: butterfly vertebra
  • kidney: renal tubular acidosis\ tubulointerstital disease
  • cardiac: Pulmonary stenosis\ TOF
22
Q

How does infant with CF + jaundice usually present

A
  • failure to thrive
  • meconium ileus
  • obstructive cholangiophathy (cholestasis)
23
Q

How to diagnose baby with CF?

A

screening: immunoreactive trypsonogin

Gold standard: CFTR - positive sweat chloride

24
Q

What is the most common cause of inhereted neonatal cholestasis

A

Alpha-1-antitrypsin

25
Q

What is the gene involved in alpha antitrypsin deficency?

A

SIRPINA 1

26
Q

What is the picture seen in alpha-1antitrypsin def?

A

Mixed picture

Hepatitis\hepatocellular + billary

27
Q

Differentiate between using alpha-1-antitrypsin serum concentration and using phenotype to diagnose

A
  • serum: insufficient as it’s an acute phase reactant

- phenotype: more accurate (ZZ, SZ) - heterozygos (MZ, MS)

28
Q

What is the type of progressive familial intrahepatic cholestasis that has high GGT?

A

Type 3

29
Q

What is the abnormality in PFIC 1?

A

P-type ATPase

30
Q

What is the presentation of PFIC1?

A

infnancy

Short strature - deafness - pancreatitis - diarrhea

31
Q

What is the abnormality in PFIC 2?

A

Bile salt export pump

32
Q

What is the presentation of PFIC1?

A

Infnacy

Aggressive and

  • hepatic failure in the first few years of life
  • risk of hepatocellular carcinoma
33
Q

What is the presentation of PFIC3?

A

Multidrug resistance protein 3

ABCB4

34
Q

Alongside with high GGT, what else is is special about PFIC 3

A
  • Only 1\3rd of patients are infnant

- concorrent gallstones

35
Q

What is PFIC treatment

A

1- nutritional: vitamins + calories
2- Pruritus treatment
3- refractory: surgical\ if failed liver transplantation

36
Q

What is the clinical presentation in bile acid biosynthesis disorders

A
  • neonatal jaundice
  • Failure thrive
  • Hepatosplenomegaly
  • Rickets
  • bleeding
37
Q

What in these disorders has low or normal GGT

A

PFIC 1-2-4-5 And BASD

38
Q

What are the finding in BASD?

A
  • low bile acid
  • normal\low GGT
  • fast atom bombardment mass spectrometry urine
  • molecular screening
39
Q

What is the treamtent BASD?

A

Cholic acid and chenodeoxycholic acid

40
Q

What is the diagnostic gold standard in choledochal cyst

A

Abdominal US

41
Q

What is the definitive treatment of choledochal cyst

A

Surgical resection

42
Q

What happens if there’s a residual cyst from the choledochal cyst that the surgical left behind

A

Risk of cholangiocarcinoma or squamous cell carcinoma

43
Q

How does infant have hemolytic disease?

A
  • congenital biliary abnormalities
  • hemolysis
  • Sepsis
  • TPN prolong
  • prematurity
44
Q

When does patient getting TPN requires surgery if he has gallstoneS?

A

If the stone is getting larger

45
Q

What is the risk of TPN?

A

If taken over 7 days might cause liver disease PNALD “parentral nutrition associated”

(>2 weeks for sure)

46
Q

How to avoid PNALD due to TPN

A
  • early initiation of feeding
  • reduce intralipid dose
  • replace the lipid (avoid polysaturated & give omega3)
  • ursodiol to clear the bile Flow
47
Q

What is the enzyme abnormality cause of galactosemia?

A

GAL-1-PUT

48
Q

What are the symptoms of galactosemia

A

Failure to thrive - jaundive - vomitting - diarrhea - gram negative sepsis

49
Q

Gram negative sepsis differentia should be

A

Galactosemia due to breast feeding or milk based formula

50
Q

How to diagnose galactosemia

A

1- urine reducing substance before starting the soy-based formula
2- RBC assay: complete absense of Gal-1-put

51
Q

What is the definitive diagnosis for galactosemia?

A

RBC assay showing Gal-1-Put deficiency

Pediatrics (41 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions