2. ILUSTRASI KASUS
Bayi laki-laki umur 1 bulan dengan keluhan kuning
• Nampak kuning sejak umur 2 minggu.
• Air kencing seperti teh dan tinja kuning pucat
• Ikterus, pembesaran hati 1 cm bac
• Hasil pemeriksaan laboratorium :
Hb 12,2 g/dl, leukosit 7200/cmm.
Bilirubin total 12,6 mg/dl, direct 3.2 mg/dl, ALT
112 U/L, AST 144 U/L, GGT 865 U/L, ALP
580 U/L, bilirubin
urine dan urobilin (+)
10. • Definition of Jaundice: yellowish discoloration
of skin , mucous membranes , and sclera
• The normal TSB level is < 1 mg/dl
• Neonatal clinical jaundice is Dx. If the TSB is
> 5 mg/dL in FT NB
> 7 mg/dL in preterm NB
11. Bu Bc ± Bu
• Hemolysis
- Rh
- ABO
• Breast Milk
• Physiological
• Hypothyroidism
dark urine
acholic stools
12. Neonatal Hyperbilirubinemia:
• TSB level is > 2mg /dl
• Common condition among NB babies most of the
are benign problem
• Untreated severe unconjugated
hyperbilirubinemia potentially neurotoxic
• Conjugated hyperbilirubinemia often
signifies a serious underling illnesses
13. Bc ± Bu
• hepatosplenomegaly
• bilirubinuria
• conjugated bilirubin
• abnormal LFTs
dark urine
acholic stools
14. • Persistent (prolonged) jaundice jaundice
lasts longer than 14 to 21 days 15% cases
• Can be unconjugated or conjugated
hyperbilirubinemia difficult to differentiate
clinically
• In the beginning: no physical findings that can
differentiate between unconjugated and
conjugated hyperbilirubinemia
16. • Most causes of unconjugated hyperbilirubinemia
are relatively benign
• Conjugated hyperbilirubinemia is NEVER normal
and should always be investigated
hepatobiliary disorder
18. Neonatal cholestasis is defined as
failure of bile flow to enter duodenum in normal
amounts
conjugated hyperbilirubinemia developing
within the first 90 days of extrauterine life
Conjugated bilirubin exceeds 1.5 to 2.0 mg/dl
Conjugated bilirubin > 20% of the total bilirubin
18
CHOLESTASIS ?
19. NEONATAL CHOLESTASIS
0,04% - 0,2% live births
Neonatal Hepatitis: 1/5000
Biliary Atresia: 1/10000-1/13000 60% - 70%
transplantation
Pediatric Dept. Dr. Soetomo hospital (1999 – 2004):
243 cholestasis (from 22652 patient)
2019-2021: 139 Biliary Atresia male 82 (59%) and
57 female (41%) came >3 mounths
25. SYMPTOMS AND
FINDINGS IN
CHOLETASIS
BILE DOES
NOT REACH THE
BOWEL
BILE
REGURGITATION
IN THE BLOOD
REPERCUSSION
OF BILE ACCUMU-
LATION ON HEPATIC
CELL AND
CANALICULI
Clay stool
Bilirubinuria
No urobolinogen
Fatty stool
Maabsorption
Increase in
Pigmentation
Canalicular
pigmentation
Inflamation
Jaundice
Pruritus
Hypercholesterolemia
Xanthomas
Excretory disorder
for: Bilirubin
Alk. Phosphatse
Bronthalein
Bile acid
Cholesterol
Damage to liver cells
Anatomical Functional
Slight increase
In transaminases
25
29. TREATMENT ?
Medical management
Nutritional support
Treatment of pruritus
Choleretics and bile acid-binders
Management of portal hypertension and its
consequences
Surgical management extrahepatic
29
41. GOALS OF TIMELY EVALUATION ?
Test at 2 weeks (fractionated bilirubin levels at 2–3 weeks of age)
Immediate intervention to address the underlying condition if
cholestasis is identified
Further investigation of efficacy and feasibility of screening for
neonatal cholestasis
EARLY DIAGNOSIS & PROMPT TREATMENT
(before 1-2 months old)
41
42.
43.
44. RECOMMENDATION
Test at 2 weeks (fractionated bilirubin levels at 2–3 weeks of age)
Application of diagnostic algorithm and early referral to pediatric
gastroenterologist
Immediate intervention to address the underlying condition if
cholestasis is identified
Further investigation of efficacy and feasibility of screening for
neonatal cholestasis
