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Paediatrics > jaundice > Flashcards

jaundice Flashcards

1
Q

what is physiological jaundice

A

Jaundice that begin day 2-3 and lasts for 10 day

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2
Q

What causes physiological jaundice

A
  • more red blood cells with a shorter life span
  • immature level unable to break down bilirubin
  • immature gut means no bacteria to break down to urobillin meaning increased enterohepatic circulation
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3
Q

What is considered pathological jaundice

A
  • <24hrs old
  • rapid rise of bili >100
  • sick newborn with jaundice
  • serum bili >250 at 48hrs or >300 by 72hrs
  • Failure to respond to phototherapy
  • prolonged jaundice >14d in term and >21 in preterm
  • cnjugated bili >35
  • Pale, chalky stools and dark urine
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4
Q

What causes increase of bilirubin in the body

A
  • ABO incompatibility
  • inherited red cell membrane defects e.g. spherocytosis
  • Erythrocyte enzymatic defects (G6PD] deficiency pyruvate kinase deficiency)
  • sepsis
  • polycythemia - macrosomia in diabetic mums
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5
Q

What causes a decreased clearance of bilirubin

A

Inherited defects in the gene that encodes UGT1A1

eg. Gilberts syndrome, Crigler-Najjar syndrome

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6
Q

What is Crigler Najjar syndrome

A
  • disorder of bilirubin conjugation
  • causes severe unconjugated hyperbilirubinemia
  • can result in bilirubin-induced neurologic dysfunction
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7
Q

What inheritence pattern does Crigler Najjar syndrome have

A

rare, autosomal recessive

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8
Q

What causes Crigler-Najjar syndrome

A

absent or defective uridine diphosphate glucuronosyltransferase-1A1 (UGT1A1) enzyme

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9
Q

When do you suspect Crigler-Najjar syndrome

A

persistent unconjugated hyperbilirubinaemia
no underlying liver disease
no evidence of haemolysis

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10
Q

management of T1 Crigler Najjar syndrome

A
  • avoid dehydration
  • avoid medications that displace bilirubin
  • chronic phototherapy
  • liver transplant is curative.
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11
Q

what causes an acute exacerbation of crigler Najjar

A
  • fasting
  • infection
  • hemolysis
  • cholelithiasis
  • cholecystitis
  • general anesthesia.
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12
Q

What happens with an acute exacerbation of Crigler Najjar

A
  • increase bilirubin production (hemolysis)
  • reduce bile flow and bilirubin elimination (fasting, cholelithiasis, cholecystitis)
  • and/or reduce plasma albumin
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13
Q

Management of acute exacerbation of Crigler Najjar

A
  • intensive phototherapy
  • albumin infusions
  • plasmapheresis
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14
Q

management of T2 Crigler Najjar

A

phenobarbital

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15
Q

What is Gilberts syndrome

A

most common inherited disorder of bilirubin glucuronidation due to mutations in the UGT1A1 gene

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16
Q

What inheritence pattern is Gilberts syndrome

A

autosomal recessive

17
Q

How do you diagnose Gilberts syndrome

A

hyperbillirubinaemia in the absence of haemolysis

18
Q

What is breast milk jaundice

A

persistent benign hyperbilirubuniaemia beyond 2-3 weeks.

usually peaks within 2 weeks then resolves over 3-12 weeks

19
Q

what is lactation failure jaundice

A
  • inadequate intake of oral fluids and calorie intake resulting in weight loss + hypovolaemia
  • Usually occurs in first week of life
  • leads to hyperbilirubinaemia and sometimes hypernatraemia
20
Q

What is kinicterus

A
  • type of brain damage that can result from high levels of bilirubin
  • can cause cerebral palsy and hearing loss.
21
Q

what clinical signs would suggest a conjugated bilrubinaemia

A

dark urine

pale stools

22
Q

What is biliary atresia

A
  • extrahepatic bile ducts are obliterated by inflammation and subsequent fibrosis
  • leading to biliary obstruction and jaundice.
  • fatal if untreated
  • unknown cause
23
Q

what are the signs of biliary atresia

A

persistent jaundice (starts shortly after birth)
Pale stool
Dark urine
Failure to thrive

24
Q

What investigation findings will you find in biliary atresia

A
  • conjugated hyperbilirubinaemia

- GGT will be raised

25
Q

What is usually the diagnostic method of choice for biliary atresia

A

histology by percutaneous biopsy

26
Q

What is the medical management of biliary atresia

A
  • antibiotics to prevent cholangitis.
  • Ursodeoxycholic acid : encourage bile flow.
  • Fat-soluble vitamin supplementation and nutritional suppor
27
Q

What is the main surgical intervention for biliary atresia

A

Kasai portoenterostomy - usually performed before 8w old

If this fails, liver transplant

28
Q

What is prolonged jaundice

A

> 14 days in term infant

>21 days in pre-term infant

29
Q

What investigations should be done for prolonged jaundice

A
  • conjugated and total serum bili
  • FBC and blood film
  • Blood group and DAT
  • TFTs
  • urine microscopy and culture
  • urine for reducing substances
  • LFTs
30
Q

what is unconjugated bilirubin

A

produce of the breakdown of red cells, which circulates mostly bound to albumin.
Some is ‘free’ and able to pass to the brain

31
Q

What is conjugated bilirubin

A

unconjugated bilirubin is metabolised in the liver to produce conjugated bilirubin which then passes into the gut and excreted in stool.
Small amount is re-absorbed in the enterohepatic circulation

32
Q

Causes of prolonged unconjugated jaundice

A
  • Haemolysis due to rhesus, ABO hereditary spherocytosis, enzyme deficiency (G6PD, pyruvate kinase)
  • Brest milk jaundice
  • infections inc. UTI
  • endocrine - hypothyroid/hypopiruitarism
  • metabolic causes - glucuronyl transferase deficiency/galactosaemia.
  • sepsis
  • liver disease
33
Q

What causes breast milk jaundice

A
  • dehydration
  • poor gut motility
  • failure to pass meconium
    All increase enterohepatic circulation of bili.
34
Q

Causes of prolonged conjugated jaundice

A
  • intrauterine infection - TORCH
  • biliary atresia
  • neonatal hepatitis
  • choledochal cyst
  • parenteral nutrition
  • Metabolic: CF/a1-antitrypsin/galactosaemia
  • intrahepatic cholestasis - syndromic/familiar

Paediatrics (21 decks)

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