Neonatal jaundice - Dr. Vishnu Biradar

1. YELLOW
Dr.Vishnu Biradar
MD, PDCC
Fellow in Liver Transplant
Cons. Pediatric Gastroenterologist
DMH, Pune
08600800123

2. Q 1. Which of following is
neonatal cholestasis?
A. S. Bil 10 , Direct 1.8
B. S. Bil 10, Direct 2.8
C. S. Bil 3, Direct 0.9 mg
D. A + B + C
E. B + C
?

3. Q 1. Which of following is
neonatal cholestasis?
A. S. Bil 10 , Direct 1.8
B. S. Bil 10, Direct 2.8
C. S. Bil 3, Direct 0.9 mg
D. A + B + C
E. B + C
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4. Q 1. Which of following is
neonatal cholestasis?
A. S. Bil 10 , Direct 1.8
B. S. Bil 10,Direct 2.8
C. S. Bil 3, Direct 0.9 mg
D. A + B+ C
E. B + C


5. Neonatal Cholestasis
Prolonged
elevation of conjugated bilirubin
beyond 14 days of life
>20% of total bilirubin if S.bil >5mg%
>1 mg% if S.bil <5mg%
5

6. Neonatal Cholestasis
Non-sick baby Sick Baby
6

7. Q. If 1 month old baby is
not sick & conj. jaundice,
what is next appropriate step?
A. Observation
B. USG Abdomen
C. Complete LFT
D. Thyroid Function Test
E. None of the above ?

8. Q. If 1 month old baby is
not sick & conj. jaundice,
what is next appropriate step?
A. Observation
B. USG Abdomen
C. Complete LFT
D. Thyroid Function Test
E. None of the above
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9. Q. If 1 month old baby is
not sick & conj. jaundice,
what is next appropriate step?
A. Observation
B. USG Abdomen
C. Complete LFT
D. Thyroid Function Test
E. None of the above 

10. To see urine & stool
Taiwan model Pre stool card (2004) Post stool card (2005)
Sensitivity of diagnosis 72% 97%
Rate of Kasai < 60d 60% 74%

11. Stool card screening (Taiwan)
Sensitivity :
97%
Chen, Pediatrics 2006;117:1147-54.

12. Q. What do you think is
important parameter in LFT in
non-sick child?
A. Bilirubin
B. SGOT & SGPT
C. PT INR
D. Total protein and Albumin
E. Gamma Glutamyl-transpeptidase
?

13. Q. What do you think is
important parameter in LFT in
non-sick child?
A. Bilirubin
B. SGOT & SGPT
C. PT INR
D. Total protein and Albumin
E. Gamma Glutamyl-transpeptidase
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14. Q. What do you think is
important parameter in LFT in
non-sick child?
A. Bilirubin
B. SGOT & SGPT
C. PT INR
D. Total protein and Albumin
E. Gamma Glutamyl-transpeptidase (GGT)


15. Neonatal Cholestasis
Non-sick baby Sick Baby
Pale Stools Pigmented Stools
PFIC,
GGT
Bile Acid Synthetic
Defect
Moderately High
Metabolic
disease
Genetic Disease
High > 300
Normal / Low
Biliary Atresia,
PILBD, PSC,
a-antitrypsin
def. 15
Indian Pediatrics Volume 51 : March 15, 2014

16. Liver function tests
• TB: between 6-10 mg/dL
• DB: conjugated fraction raised
• Enzymes: do not predict
• Albumin: low if decompensated (cirrhosis)
• ALP,GGT: high
• INR: correctable coagulopathy (after 1-3 doses
of Vitamin K

17. Q. Now, we decided to do USG
abdomen. What to look for?
A. Liver echotexture
B. Liver surface
C. Common bile duct (CBD)
D. Gall-bladder (GB)
E. Portal Vein ?

18. Q. Now, we decided to do USG
abdomen. What to look for?
A. Liver echotexture
B. Liver surface
C. Common bile duct (CBD)
D. Gall-bladder (GB)
E. Portal Vein
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19. Q. Now, we decided to do USG
abdomen. What to look for?
A. Liver echotexture
B. Liver surface
C. Common bile duct (CBD)
D. Gall-bladder (GB)
E. Portal Vein 

20. Neonatal Cholestasis
Non-sick baby Sick Baby
Pale Stools Pigmented Stools
Fasting USG Abdomen > 4 hours
Normal
GB
Small GB
< 1.5 cm length
Absent GB Choledochal Cyst
r/o Biliary Atresia Surgery
20
Indian Pediatrics Volume 51 : March 15, 2014
Abnormal CBD
Infant : >2mm
Older children : >3.5mm

21. Importance of Gallbladder
Length of GB
• Cut off suggested: 15
mm
• Lack of
smooth/complete
echogenic mucosal lining
• Irregular/lobular
contour
Contractility index
Normal CI : 86% at 6 weeks
67% at 4 months
Farrant, Br J Radiol 2001 Kanegawa et al, AJR, 2003

22. Q. What will you do next to
confirm BA?
A. HIDA scan
B. Liver Biopsy
C. Per Operative Cholangiogram
?

23. Q. What will you do next to
confirm BA?
A. HIDA scan
B. Liver Biopsy
C. Per Operative Cholangiogram
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24. Q. What will you do next to
confirm BA?
A. HIDA scan
B. Liver Biopsy
C. Per Operative Cholangiogram


25. Neonatal Cholestasis
R/o Biliary Atresia
Age < 6 wks Age > 6 wks Age > 90 days with
ascites
Age > 120 days
Non-sick baby, Pale Stools,
Small/ Absent GB on fasting
USG
Sick Baby
Kasai Porto-enterostomy Liver Transplant
25
Indian Pediatrics Volume 51 : March 15, 2014
+
HIDA scan
Liver Biopsy

26. Liver Biopsy
H & E Stain CK 7 & 19 stain

27. LFT in sick child, what to look
for?
A. Bilirubin
B. SGOT & SGPT
C. PT INR
D. Total protein and Albumin
E. Gamma Glutamyl-transpeptidase
> 2, inspite of 3 doses of Vit-K
Neonatal Liver Failure

28. Q. What investigations will you do
in sick baby except
A. Malaria test
B. TORCH
C. Urine RM & CS
D. HSV PCR
E. GALT
?

29. Q. What investigations will you do
in sick baby except
A. Malaria test
B. TORCH
C. Urine RM & CS
D. HSV PCR
E. GALT
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30. Q. What investigations will you do
in sick baby except
A. Malaria test
B. TORCH
C. Urine RM & CS
D. HSV PCR
E. GALT


31. Neonatal Cholestasis
Non-sick baby Sick Baby
Sepsis Screen
Positive
Urine/
Blood
CMV PCR
HSV PCR Serum ferritin
r/o Galactosemia
Urine NGRS
By GALT Enzyme assay
HLH/
NH
31
Urine for
Succinylacetone
Tyrosinemia
Indian Pediatrics Volume 51 : March 15, 2014

32. Q. What is the dose of Vit.-K in
NCS?
A. IV 1 mg
B. IV 1 mg/kg/day
C. IV 5 mg
D. IV 10 mg
?

33. Q. What is the dose of Vit.-K in
NCS?
A. IV 1 mg
B. IV 1 mg/kg/day
C. IV 5 mg
D. IV 10 mg
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34. Q. What is the dose of Vit.-K in
NCS?
A. IV 1 mg
B. IV 1 mg/kg/day
C. IV 5 mg
D. IV 10 mg


35. Q. How frequent Vit.-K to be
given?
A. Once a week
B. Twice a week
C. Once a month
D. Twice a month
?

36. Q. How frequent Vit.-K to be
given?
A. Once a week
B. Twice a week
C. Once a month
D. Twice a month
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37. Q. How frequent Vit.-K to be
given?
A. Once a week
B. Twice a week
C. Once a month
D. Twice a month


38. Dose Route
Calories 125% Add MCT / puffed rice
powder
Vitamin A 5000-25000 IU/day
Vitamin D 400-1200 IU/ day
Vitamin E 50-400 IU/ day
Vitamin K 2.5 mg twice weekly Oral
2-5 mg once monthly sc/im/iv
Calcium 20-100 mg/kg/day Oral
Phosphorous 25-50 mg/kg/day Oral
Magnesium 1-2 mEq/kg/day Oral
0.3-0.5 mEq/ kg over 3
hours of 50 % solution
IV
Elemental iron 5-6 mg/kg/day Oral

39. Case
7 month old boy
C/o Gradually progressing abdominal distesnion with
hepatomegaly and jaundice since 15 days
No fever/ altered sensorium/ hemetemesis
O/e: Vitals N
PA: Gross ascites and tender hepatomegaly
Budd-Chiary Syndrome

40. Pictures

41. Q. What really constitutes BCS?
A. Two hepatic vein block
B. Three hepatic vein block
C. IVC block
D. All of the above
E. Only B+C
?

42. Q. What really constitutes BCS?
A. Two hepatic vein block
B. Three hepatic vein block
C. IVC block
D. All of the above
E. Only B+C
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43. Q. What really constitutes BCS?
A. Two hepatic vein block
B. Three hepatic vein block
C. IVC block
D. All of the above
E. Only B+C


44. Normal Anatomy

45. Q. How can BCS present?
A. Hepatomegaly and Ascites
B. Splenomegaly without ascites / Portal HTN
C. Fulminant Liver Failure
D. A + C
E. All of the above
?

46. Q. How can BCS present?
A. Hepatomegaly and Ascites
B. Splenomegaly without ascites / Portal HTN
C. Fulminant Liver Failure
D. A + C
E. All of the above
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47. Q. How can BCS present?
A. Hepatomegaly and Ascites (20- 30%)
B. Splenomegaly without ascites / Portal HTN (65%)
C. Fulminant Liver Failure (10%)
D. A + C
E. All of the above


48. Investigation
• Hemogram N
• Bil T/D –0.5/0.2
• SGOT -55
• SGPT-11
• ALP - 493
• GGT - 145
• Total protein- 6.58
• Albumin – 3.93
• PT INR -13.6/11
• Factor V leiden
mutation absent
• Protein C & S assay
normal
• Anti-thrombin III
levels – normal
• Homocysteine levels
normal

49. Radiological Investigation:
USG Abdomen & CT Scan –
Triphasic study
A. All three hepatic veins showed chronic thrombosis
with collaterals
B. Hepatomegaly and ascites

50. Venoplasty Video
A. Right Hepatic Venoplasty

51. Follow up – 6 months
 Child is ascites free
 Wt. gain of 1.5 kg
 Serial doppler showed patency of right hepatic vein
 No deterioration in liver parameters

52. Back Veins

53. Take Home Message
 Clinical features of BCS in children are protean.
 Absence of ascites doesn’t rule out BCS.
 Good doppler examination of hepatic veins and IVS
is important
 Endovascular management of BCS is safe and
effective treatment modality with good
intermediate to long term results along with anti-coagulation

54. Thank you