YELLOW 
Dr.Vishnu Biradar 
MD, PDCC 
Fellow in Liver Transplant 
Cons. Pediatric Gastroenterologist 
DMH, Pune 
08600800123
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 
?
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 
E12n1234567890123456789d TIME 
Your Time Starts Now! UP!
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 

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
Neonatal Cholestasis 
Non-sick baby Sick Baby 
6
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 ?
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 
E12n1234567890123456789d TIME 
Your Time Starts Now! UP!
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 
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%
Stool card screening (Taiwan) 
Sensitivity : 
97% 
Chen, Pediatrics 2006;117:1147-54.
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 
?
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 
E12n1234567890123456789d TIME 
Your Time Starts Now! UP!
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) 

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
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
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 ?
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 
E12n1234567890123456789d TIME 
Your Time Starts Now! UP!
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 
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
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
Q. What will you do next to 
confirm BA? 
A. HIDA scan 
B. Liver Biopsy 
C. Per Operative Cholangiogram 
?
Q. What will you do next to 
confirm BA? 
A. HIDA scan 
B. Liver Biopsy 
C. Per Operative Cholangiogram 
E12n1234567890123456789d TIME 
Your Time Starts Now! UP!
Q. What will you do next to 
confirm BA? 
A. HIDA scan 
B. Liver Biopsy 
C. Per Operative Cholangiogram 

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
Liver Biopsy 
H & E Stain CK 7 & 19 stain
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
Q. What investigations will you do 
in sick baby except 
A. Malaria test 
B. TORCH 
C. Urine RM & CS 
D. HSV PCR 
E. GALT 
?
Q. What investigations will you do 
in sick baby except 
A. Malaria test 
B. TORCH 
C. Urine RM & CS 
D. HSV PCR 
E. GALT 
E12n1234567890123456789d TIME 
Your Time Starts Now! UP!
Q. What investigations will you do 
in sick baby except 
A. Malaria test 
B. TORCH 
C. Urine RM & CS 
D. HSV PCR 
E. GALT 

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
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 
?
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 
E12n1234567890123456789d TIME 
Your Time Starts Now! UP!
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 

Q. How frequent Vit.-K to be 
given? 
A. Once a week 
B. Twice a week 
C. Once a month 
D. Twice a month 
?
Q. How frequent Vit.-K to be 
given? 
A. Once a week 
B. Twice a week 
C. Once a month 
D. Twice a month 
E12n1234567890123456789d TIME 
Your Time Starts Now! UP!
Q. How frequent Vit.-K to be 
given? 
A. Once a week 
B. Twice a week 
C. Once a month 
D. Twice a month 

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
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
Pictures
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 
?
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 
E12n1234567890123456789d TIME 
Your Time Starts Now! UP!
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 

Normal Anatomy
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 
?
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 
E12n1234567890123456789d TIME 
Your Time Starts Now! UP!
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 

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
Radiological Investigation: 
USG Abdomen & CT Scan – 
Triphasic study 
A. All three hepatic veins showed chronic thrombosis 
with collaterals 
B. Hepatomegaly and ascites
Venoplasty Video 
A. Right Hepatic Venoplasty
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
Back Veins
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
Thank you

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. Whichof 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. Whichof 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 E12n1234567890123456789d TIME Your Time Starts Now! UP!
  • 4.
    Q 1. Whichof 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.
  • 7.
    Q. If 1month 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 1month 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 E12n1234567890123456789d TIME Your Time Starts Now! UP!
  • 9.
    Q. If 1month 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 doyou 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 doyou 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 E12n1234567890123456789d TIME Your Time Starts Now! UP!
  • 14.
    Q. What doyou 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-sickbaby 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, wedecided 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, wedecided 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 E12n1234567890123456789d TIME Your Time Starts Now! UP!
  • 19.
    Q. Now, wedecided 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-sickbaby 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 willyou do next to confirm BA? A. HIDA scan B. Liver Biopsy C. Per Operative Cholangiogram ?
  • 23.
    Q. What willyou do next to confirm BA? A. HIDA scan B. Liver Biopsy C. Per Operative Cholangiogram E12n1234567890123456789d TIME Your Time Starts Now! UP!
  • 24.
    Q. What willyou do next to confirm BA? A. HIDA scan B. Liver Biopsy C. Per Operative Cholangiogram 
  • 25.
    Neonatal Cholestasis R/oBiliary 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 sickchild, 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 investigationswill you do in sick baby except A. Malaria test B. TORCH C. Urine RM & CS D. HSV PCR E. GALT ?
  • 29.
    Q. What investigationswill you do in sick baby except A. Malaria test B. TORCH C. Urine RM & CS D. HSV PCR E. GALT E12n1234567890123456789d TIME Your Time Starts Now! UP!
  • 30.
    Q. What investigationswill you do in sick baby except A. Malaria test B. TORCH C. Urine RM & CS D. HSV PCR E. GALT 
  • 31.
    Neonatal Cholestasis Non-sickbaby 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 isthe 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 isthe dose of Vit.-K in NCS? A. IV 1 mg B. IV 1 mg/kg/day C. IV 5 mg D. IV 10 mg E12n1234567890123456789d TIME Your Time Starts Now! UP!
  • 34.
    Q. What isthe 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 frequentVit.-K to be given? A. Once a week B. Twice a week C. Once a month D. Twice a month ?
  • 36.
    Q. How frequentVit.-K to be given? A. Once a week B. Twice a week C. Once a month D. Twice a month E12n1234567890123456789d TIME Your Time Starts Now! UP!
  • 37.
    Q. How frequentVit.-K to be given? A. Once a week B. Twice a week C. Once a month D. Twice a month 
  • 38.
    Dose Route Calories125% 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 monthold 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.
  • 41.
    Q. What reallyconstitutes 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 reallyconstitutes BCS? A. Two hepatic vein block B. Three hepatic vein block C. IVC block D. All of the above E. Only B+C E12n1234567890123456789d TIME Your Time Starts Now! UP!
  • 43.
    Q. What reallyconstitutes BCS? A. Two hepatic vein block B. Three hepatic vein block C. IVC block D. All of the above E. Only B+C 
  • 44.
  • 45.
    Q. How canBCS 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 canBCS present? A. Hepatomegaly and Ascites B. Splenomegaly without ascites / Portal HTN C. Fulminant Liver Failure D. A + C E. All of the above E12n1234567890123456789d TIME Your Time Starts Now! UP!
  • 47.
    Q. How canBCS 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 • HemogramN • 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: USGAbdomen & 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.
  • 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.

Editor's Notes