3. Neonatal cholestasis
Defined as conjugated hyperbilirubinemia occurring in the newborn as a
consequence of diminished bile flow
Conjugated hyperbilirubinemia in a as serum
Direct/conjugated bilirubin > 1.0 mg/dL if the total serum bilirubin (TSB) is
<5.0 mg/dL
greater than 20% of TSB if the TSB is >5.0 mg/d
4.
5. Clinical
presentation
Prolonged Jaundice
Acholic stool
Dark urine
Hepatomegaly
Bronze discoloration of body
after phototherapy
Other nonspecific signs
depending on the disease
18. Nutrition
Continue breast feeding
Supplement MCT based feeds
To older children: High energy diet: 200 Cal/day
Carbohydrate : Glucose polymer
Protein: 1-2 gm/day from vegetable source
2-3 % calories from free fatty acids
NG feeding in anorexic babies
Vitamin supplement continues till 3 months after resolution of jaundice
19.
20.
21. Surgical Treatment
Kasai procedure for BA
External and internal biliary diversion for PFIC without decompensated
cirrhosis
Choledochal cyst excision
Cholecystectomy
Liver transplantation
22. Kasai- Suzuki Operation
Center where min 6 portoenterostomy/year done
For BA not surgically correctible: excision of atretic segment, Roux-en-Y
portoenterostomy
Prior to 8 weeks: bile flow re-established I 80-90%
After 12 weeks; on <20%
NO CUT_OFF for late referral
Successful : Serum bilirubin Returns to normal
75-100% cases had cirrhosis at laparotomy after 16 week
23. Liver Transplantation
Bilirubin level>6mg/dl after 3 months of Kasai PE
BA with hepatic decompensation (low albumin, prolonged INR. Ascites)
Of the 355 transplants in child ,30% was for BA
Survival:
5 year{ 98%
10 years : 90%
24. Treatment
Management of portal hypertension and its consequences
Variceal bleeding:
Fluid resuscitation
Blood products
Sclerotherapy
Balloon tamponade
Porto venous shunting
Ascites:
Sodium restrictions
Diuretics{ spironolactone. Furosemides
Albumin
paracentesis
Thrombocytopenia is managed with platelet transfusion when clinically indicated
25. Key points
Promptly refer newborns with prolonged NNJ with dark urine with/without
pale stool for proper investigation
Consider EHBA as diagnosis in apparently healthy babies with pale stool
Liver biopsy is suspected ca=ses of BA is an emergency and it should be done
and reported on urgent basis
Surgery for BA and choledochal cyst should be done before 8 weeks