2. • Medical management
• Nutritional support
• Treatment of pruritus
• Choleretics and bile acid-binders
• Management of portal hypertension and its
consequences
• Specific treatment depends on aetiology
3. • The initial step is rapid diagnosis and early initiation of therapy of
treatable disorders to avoid significant progression of the illness as sepsis
,urinary tract infection hypothyroidism,inborn errors of metabolism as
galactosemia.,. , biliary atresia
• Early surgical intervention for biliary atresia( Before 2 months of age)
results in improved outcome.
Phototherapy (Nomogram , charts ) standard or Intensive phototherapy ,
IVIG immunoglobulin (Rh and ABO hemolytic disease )
Exchange transfusion
4. Nutritional Treatment
Goals of early nutritional intervention
- prevent or correct deficiencies,
- improve growth ultimately,
- reduce morbidity and mortality
• Adequate calories and protein
• Supplement calories with medium chain triglycerides
• Maintain levels of essential long-chain fatty acids
• Treatment and/or prophylaxis for fat-soluble vitamin
deficiencies
5. CARBOHYDRATES
• Monitor blood glucose levels during fasting or intercurrent illness
• Give 50-60% of calories as carbohydrates.
PROTEINS
• Children with prolonged jaundice or CLD should not be protein restricted
unless encephalopathic.
• Protein supplementation up to 2-4 g/kg/d .
FATS
• Targeted to offer 30% of your caloric intake
• Around 30% to 50% of total fat should be provided as MCTs, although
compliance can be problematic because of its poor palatability.
• The rest should be given as LCTs to provide essential fatty acids and enable
absorption of fat soluble vitamins
6. MICRONUTRIENTS
VITAMIN A
5000-250000 UNITS/DAY
VITAMIN D
400 IU/D preferably as 25OHD3. Monitor calcium and phosphorus levels
VITAMIN E
15-25iu/kg/d
VITAMIN K
2.5-5 mg/d
WATER SOLUBLE VITAMINS
Multivitamin preparation providing at least 100%of recommended daily
allowance
TRACE ELEMENTS
7. Hydration
There is no evidence that excessive fluid administration affects the serum
bilirubin concentration. Some infants who are admitted with high bilirubin
levels are also mildly dehydrated and may need supplemental fluid intake to
correct their dehydration. Because these infants are almost always breastfed,
the best fluid to use in these circumstances is a milk-based formula, because
it inhibits the enterohepatic circulation of bilirubin and should help to lower
the serum bilirubin level .
8. PHARMACOLOGIC THERAPY
Phenobarbital
• UGT activity can be increased or induced with administration of
phenobarbital,
• After the demonstration that phenobarbital administration to a child with
Crigler-Najjar syndrome type II disease reduced TB concentrations,
• phenobarbital administration to pregnant mothers and their offspring was
shown to reduce by about 50%, peak serum TB concentrations caused by
physiologic jaundice.
• The drug is much less effective in premature neonates.
• Even then, the premature neonates most susceptible to the toxic effects of
hyperbilirubinemia would receive little or no beneficial effect.
• Phenobarbital is potentially addictive, may lead to excessive sedation of the
newborn.
9. Metal (tin [Sn] and zinc [Zn]) metalloporphyrins.
• Zinc sulfate supplementation is a controversial potential approach for treating
neonatal jaundice. A systematic review and meta-analysis comprising data
from 645 neonates over 5 randomized controlled trials did not show any
significant reductions in levels of total serum bilirubin on days 3 and 7, nor
reductions in the incidence of bilirubinemia and phototherapy requirements,
but zinc supplementation did result in a significantly shorter duration of
phototherapy ( Medscape ) .
• They work by decreasing the production of bilirubin by competitive inhibition
of heme oxygenase
• SnMP has been extensively studied and found to effectively reduce the need
for phototherapy and exchange transfusion
10. Albumin- There is a paucity of evidence surrounding the
use of albumin as adjunctive therapy in neonates
requiring exchange transfusion and it is used only in the
NICU. A single RCT showed the use of albumin priming
prior to exchange transfusion (1 mg/kg, 1 hour before
exchange transfusion) resulted in lower TBS values at 12
hours and shorter length of stay for neonates receiving
albumin If used .
11. the effect of clofibrate on the newborns with hyperbilirubinemia
• Clofibrate is a glucuronosyl transferase inducer that has been proposed
to increase the elimination of bilirubin in neonates with
hyperbilirubinemia.
• Dosage : 100 mg/kg
• Clofibrate effect in decreasing bilirubin level is more prominent than
Phenobarbital
• clofibrate is effective in reducing neonatal jaundice and its effect
appeared 24 hours after treatment
The Effect of Clofibrate on Neonatal Hyperbilirubinemia in Uncomplicated
Jaundice Hamid Reza Badeli's
12. • Supplementation of probiotics appears to show promise for newborns with
pathologic neonatal jaundice. A systematic review and meta-analysis of 13
randomized controlled trials involving 1,067 neonates with jaundice who
received probiotics showed a reduction in total serum bilirubin levels after 3,
5, and 7 days, as well as a decrease in the time of jaundice fading, the
duration of phototherapy, and length of hospitalization relative to neonates
in the control group.
https://www.ncbi.nlm.nih.gov/pubmed/?term Zhe chen etal
13. Ursodeoxycholic acid (ursodiol)
• Ursodeoxycholic acid (ursodiol) is commonly used for intrahepatic
cholestasis, but it is contraindicated for extrahepatic biliary obstruction .
• It promotes bile flow and decrease the pruritus of cholestasis.
• The usual dose is 10-20mg/kg /day
15. References
• The Effect of Clofibrate on Neonatal Hyperbilirubinemia in Uncomplicated
Jaundice Hamid Reza Badeli's
• https://www.ncbi.nlm.nih.gov/pubmed/?term Zhe chen etal
• Medscape Neonatal Jaundice
• Prolonged Neonatal Jaundice by DR Ahmed Laving
Editor's Notes
The ‘little and often’ approach
PROTEIN RESTRICTION INDICATIONS: Hepatic encephalopathy and hepatorenal disease.
Vit k dependent factors deficiency
exclude galactose from the diet until galactosaemia is excluded .
Give fat –soluble vitamins ADEK twice recommended daily allowance – ketovite liquid syrup –multivitamin syrup 5ml, vit k inj 2mg , or Abidec syruo –multivitamin syrup
Vit D : 3-10 times RDA
5 mg/kg per day for neonatal treatment.
Clofibrate has been used for several years as a hypolipidemic drug , can decrease the time needed for phototherapy and hospitalization,
Shahrivar Children's Hospital of Guilan University of Medical Sciences
(1) protection of cholangiocytes against cytotoxicity of hydrophobic bile acids
2- stimulation of hepatobiliary secretion, putatively via Ca(2+)- and protein kinase C-alpha-dependent mechanisms
3- protection of hepatocytes against bile acid-induced apoptosis
Further treatment of pruritus due to cholestasis may include cholestyramine to bind intestinal bile acids and prevent reabsorption, or rifampin
(5 mg/kg/day to 10 mg/kg/day