Jaundice in the neonatal period Flashcards

1
Q

Causes of unconjugated hyperbilirubinemia

A

1) Hemolytic
a) Intrinsic
- Membrane: spherocytosis, elliptocytsis
- Enzyme:G6PD, PK deficiency
- Hb: thalassemia
b) Extrinsic
- ABO, Rh incompatibility
- Splenomegaly
- Sepsis
- AV malformation

2) Non-hemolytic
- Hematoma
- Polycythemia
- Sepsis
- Hypothyroidism
- Gilbert
- Crigler-Najjar

3) Physiologic

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2
Q

Causes of conjugated hyperbilirubinemia

A

1) Hepatic
a) Infectious- Hep A, B, C, Sepsis, TORCH
b) Metabolic- Galactosemia, tyrosinemia, A1AT, Hypothyroid, CF
c) Drugs
d) TPN
e) Idiopathic neonatal hepatitis

2) Post hepatic
- Biliary atresia
- Choledochal cyst

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3
Q

Important history

A
Baby unwell- sepsis? bowel obstruction?
Dehydration, poor weight gain- can exacerbate
serology, viral
FHx of hemolysis
Dark urine, pale stools
Plethora
Hepatosplenomegaly->metabolic, viral
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4
Q

Investigations in unwell child/febrile

A
Septic screen
FBE/CRP/BC
UEC/LFT/VBG/Glucose
IV antibiotics
Bilirubin
Blood film/Reticulocytes
Blood group/Coombs
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5
Q

Investigations in well, afebrile

A

FBE
Bilirubin
Blood film/Reticulocytes
Blood group/Coombs

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6
Q

Investigations if conjugated bilirubin >15%

A
LFTs
Clotting
TFTs
Septic screen
Viral serology
A1AT levels
Abdominal USS
DW gastroenterology
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7
Q

Investigations when evidence of hemolysis

A

DW haematologist

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8
Q

Investigations when total in treatable range

A
NNU consultations
Urine MCS/reducible
UEC
G6PD
TFTs
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9
Q

Investigations when prolonged, and what is considered prolonged w/o an obvious cause

A
Prolonged is >2 weeks in full term and >3 weeks in preterm
DW paediatric senior
Urine MCS/reducing substrates
TFTs
G6PD
Arrange follow up
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10
Q

What are reducing substances

A

Galactose
Fructose
Glucose

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11
Q

Glycosuria -ve, but + urine for reducing substances, management

A

Further testing required

Remove lactose immediately from diet, pending results of further investigations

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12
Q

Discharge instructions for unconjugated hyperbilirubinemia

A

Sunlight is not treatment
Early F/U with GP
Ensure adequate oral intakes, especially

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13
Q

Phototherapy- hydration, monitoring, rechecking bilirubin

A

++Attention to fluid intake and hydration
Correct hydration over at least 24 hours
Check bilirubin after 6 hours starting therapy

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14
Q

What level of bilirubin rise should warrant call to NNU

A

If rate of rise >10micromol/L/hour

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15
Q

Final option for treatment if supportive and phototherapy no longer appropriate

A

Exchange transfusion

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16
Q

Important examination

A
Extent of the jaundice
Congenital viral infection->petechiae, anemia, hepatosplenomegaly
Dehydration
Well
Evidence of infection
Examine the stool
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17
Q

Why is checking clotting and vitamin K levels important

A

Prolonged jaundice can +risk of bleeding disorders associated with vitamin K deficiency

18
Q

Wavelength of phototherapy

A

450nm

19
Q

Commonest cause of jaundice in an older child

A

Hepatitis A

20
Q

Other causes of jaundice in older children

A
Chronic hemolysis->spherocytosis, G6PD
Autoimmune chronic hepatitis
Reyes syndrome
Paracetamol overdose
Wilson's
Gilberts
Cigglar-Najar
21
Q

When does physiological jaundice occur, and when does it resolve

A

Occurs on day 2-3, usually resolves in 7 days

22
Q

Pathophysiology of physiological jaundice (3)

A

1) +Hct and reduced RBC lifespan
2) Immature conjugating system
3) Increased enterohepatic circulation

23
Q

Risk factors for jaundice: maternal, perinatal, neonatal

A

Maternal:
Ethnic group
Complications->diabetes
Rh/ABO incompatibility

Perinatal:
Birth trauma
Prematurity

Neonatal factors:
Difficulty establishing breathing
Infection
Genetics
Polycythemia
Drugs
24
Q

Causes of jaundice

A
25
Q

Causes of jaundice 24-72

A
24-72 h
Physiologic, polycythemia
Dehydration
(breastfeeding jaundice)
Hemolysis
G6PD deficiency
Pyruvate kinase deficiency
Spherocytosis
Bruising, hemorrhage,
hematoma
Sepsis/congenital infection
26
Q

Causes of jaundice 72-96

A
72-96 h 
Physiologic ± breastfeeding
Sepsis
Prolonged
Breast milk jaundice
27
Q

Causes of prolonged

A
Breast milk jaundice
Prolonged physiologic
jaundice in preterm
Hypothyroidism
Neonatal hepatitis
Conjugation dysfunction
e.g. Gilbert syndrome,
Crigler-Najjar syndrome
Inborn errors of metabolism
e.g. galactosemia
Biliary tract obstruction
e.g. biliary atresia
28
Q

Differentiate breastfeeding jaundice vs breast milk jaundice

A

Breastfeeding jaundice if when reduced breast milk production->dehydration->exagerated physiologic jaundice

Breast milk jaundice: glucuronyl transferase inhibitor found in breast milK, onset day 7, persists for several weeks and resolves by 6 weeks. If not reason to cease breastfeeding

29
Q

Red flags for pathalogical

A

1-2 weeks of age

30
Q

Hemolytic work up

A
CBC
Reticulocyte
Blood group
Blood smear
Coombs test
Bilirubin
31
Q

How does phototherapy work

A

Insoluble unconjugated bilirubin is converted to excretable form via photoisomerisation

32
Q

Can you perform phototherapy in conjugated hyperbilirubinemia, why

A

No

Results in bronzed baby

33
Q

Etiology of kernicterus

A

Unconjugated bilirubin levels ++ than binding capacity of albumin->deposit in brain stem and basal ganglia

34
Q

What level of bilirubin= risk of kernicterus

A

When levels >340umol/L

35
Q

When can kernicterus occur at lower levels than

A

sepsis, meningitis, hemolysis, hypoxia, acidosis,

hypothermia, hypoglycemia and prematurity

36
Q

Clinical presentations: early (4), mid (6), late stage (6)

A

Early: hypotonia, lethargic, poor feeding, emesis
Mid: hypertonic, high pitched cry, opisthotonic posturing (back archign), bulging fontanelles, seizures and pulmonary hemorrhage.
Late (beyond a year): hypotonia, EPS (choreoathetoid cerebral palsy), gaze palsy, mitral regurgitation, sensorineural hearing loss.

37
Q

Long term complications of kernicterus

A

Sensorineural deafness
Choreoathetoid cerebral palsy
Gaze palsy
Mental retardation

38
Q

What is biliary atresia and how does it present

A
Atresia of extrahepatic bile ducts->cholestasis, +conjugated bilirubin
Dark urine, pale stool
Jaundice persisting for >2 weeks
Abdominal distension
hepatomegaly
39
Q

Diagnosis of biliary atresia- investigations

A

Conjugated hyperbilirubinemia, abdominal USS
HIDA san
Liver biopsy

40
Q

Treatment of biliary atresia

A
  • surgical drainage procedure

* hepatoportoenterostomy (Kasai procedure; most successful if