Neonatal Neonatal jaundice jaundice Surender K Yachha MD, DM Surender K Yachha MD, DM Professor and Head Professor and Head Department of Pediatric Gastroenterology Department of Pediatric Gastroenterology Sanjay Gandhi Postgraduate Institute of Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow Medical Sciences, Lucknow
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Surender K Yachha MD, DMSurender K Yachha MD, DMProfessor and Head Professor and Head
Department of Pediatric GastroenterologyDepartment of Pediatric GastroenterologySanjay Gandhi Postgraduate Institute of Medical Sciences, LucknowSanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow
Neonatal JaundiceNeonatal Jaundice
Definition of conjugated Definition of conjugated jaundicejaundice
Direct bilirubin level
>1 mg/dL if total serum bilirubin < 5mg/dL
or
> 20% of the total bilirubin if > 5mg/dL
Indian Pediatrics - August 2000, Vol. 37, Number 8
Consensus Report on Neonatal Cholestasis Syndrome
How to do it ?How to do it ?3 wk baby, jaundice3 wk baby, jaundice
Does the urine stain diapers:yes
What is the stool color ?
Yellow PaleConfirmConfirm Confirm
Not sure
UltrasoundLFT
Worried, fast track
Liver biopsy
Nuclear scan
+
Case I: 64 days old babyCase I: 64 days old baby
• Jaundice noticed from day 3 life..• Diaper staining urine..• Persistently pale stools from birth• Not sick• BW: 2.2kg, No weight gain despite adequate
feeding• No abnormal facies, dysmorphism, Cataract• Liver 3cm firm, Spleen:1cm• CVS: Normal
PossibilityPossibility
First Possibility First Possibility Biliary atresia Biliary atresia
InvestigationsInvestigations
TB/DB 10/6.8
AST/ALT 310/125
TP/Alb 5.6/3.6
ALP 1435
GGT 1751
PT 13.1 sec
Liver BiopsyLiver Biopsy• Ductular proliferation • Fibrosis • Widening of portal tracts• Early cirrhosis
Not sick childNot sick childUSG featuresUSG features
BiopsyBiopsy
Subtype of type 3 atresia with patent gall bladder & distal bile duct but Subtype of type 3 atresia with patent gall bladder & distal bile duct but proximal atresia. The gall bladder though good in size, is filled with mucous. proximal atresia. The gall bladder though good in size, is filled with mucous.
Type 1, Type 1, CBD atreticCBD atretic
Type IIType IICBD, CHD, GB atretic; CBD, CHD, GB atretic;
Confluence patentConfluence patent
Type IIIType IIICBD, CHD, GB atretic; CBD, CHD, GB atretic; Confluence not patentConfluence not patent
Liver Biopsy: 1 bile duct out of 9 portal tracts Liver Biopsy: 1 bile duct out of 9 portal tracts Paucity of interlobular bile ducts..Paucity of interlobular bile ducts..
Liver Biopsy: 1 bile duct out of 9 portal tracts Liver Biopsy: 1 bile duct out of 9 portal tracts Paucity of interlobular bile ducts..Paucity of interlobular bile ducts..
InvestigationsInvestigations
Final diagnosis
Syndromic PILBD(Alagille Syndrome)
Final diagnosis
Syndromic PILBD(Alagille Syndrome)
Overall 95% Overall 95% CholestasisCholestasis
ProgressionProgression 15% Cirrhosis 15% Cirrhosis and liver failureand liver failure
Emerick. Hepatology. 1999; 29: 822–9Emerick. Hepatology. 1999; 29: 822–9Lykaveiris Gut 2001;49:431–435Lykaveiris Gut 2001;49:431–435
Natural History of Alagille Natural History of Alagille SyndromeSyndrome
LTxLTx
Case IV: 4 moCase IV: 4 mo
• Jaundice from 3rd month of life• Intermittently pale and pigmented stools• Pruritus 15 days; PILBD, PFIC, A-1 AT def. ?• Poor wt gain, Normal development
Family history• H/O Sib death – jaundice, pruritus and
diarrhea at 6mo age, death at 5yrs• H/O Cholestasis of pregnancy in mother
Good sized contractile GBGood sized contractile GB
NGRS negative, TORCH/VDRL negativeNGRS negative, TORCH/VDRL negativeNormal AFP, Ferritin Normal AFP, Ferritin Normal Eye exam. X-ray dorsal spine Normal Eye exam. X-ray dorsal spine Normal Lipid profile, ECHONormal Lipid profile, ECHO
NGRS negative, TORCH/VDRL negativeNGRS negative, TORCH/VDRL negativeNormal AFP, Ferritin Normal AFP, Ferritin Normal Eye exam. X-ray dorsal spine Normal Eye exam. X-ray dorsal spine Normal Lipid profile, ECHONormal Lipid profile, ECHO
Liver Biopsy : 2 bile duct out of 20 portal tracts Liver Biopsy : 2 bile duct out of 20 portal tracts Paucity of interlobular bile ducts. Paucity of interlobular bile ducts.
Intracanalicular and intracellular Intracanalicular and intracellular cholestasischolestasis
Liver Biopsy : 2 bile duct out of 20 portal tracts Liver Biopsy : 2 bile duct out of 20 portal tracts Paucity of interlobular bile ducts. Paucity of interlobular bile ducts.
Intracanalicular and intracellular Intracanalicular and intracellular cholestasischolestasis
At 1 yr follow-up• LFT normalized• Coagulopathy corrected• Organomegaly regressed• No major intercurrent illnesses• Steady weight gain • Normal development
1mo 3mo
BW: 3 kg BW: 3 kg
Breast fedBreast fed
Jaundice with pigmented stoolsJaundice with pigmented stools
GAL-1-PUT “normal”GAL-1-PUT “normal”Lactose restarted by labLactose restarted by lab!!!!
Case VII… Further courseCase VII… Further course
Long term complicationsLong term complications
• Verbal dyspraxia and lower IQ despite strict diet• Progressive neurological disease: ataxia, tremor• Hypergonadotrophic hypogonadism • Infertility in women