Neonatal Jaundice Ahmedabad: Dr SK Yachha

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Neonatal Jaundice Ahmedabad: Dr SK Yachha

  1. 1. Neonatal jaundice Surender K Yachha MD, DM Professor and Head Department of Pediatric GastroenterologySanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow
  2. 2. Neonatal Jaundice
  3. 3. Definition of conjugated jaundice Direct bilirubin level >1 mg/dL if total serum bilirubin < 5mg/dL or > 20% of the total bilirubin if > 5mg/dL
  4. 4. Consensus Report on Neonatal Cholestasis Syndrome Indian Pediatrics - August 2000, Vol. 37, Number 8
  5. 5. How to do it ? 3 wk baby, jaundice  Does the urine stain diapers:yes  What is the stool color ? ConfirmYellow Confirm Pale Not sure Confirm Worried, fast track + Nuclear scanLFT Ultrasound Liver biopsy
  6. 6. Case 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
  7. 7. PossibilityFirst Possibility Biliary atresia
  8. 8. InvestigationsTB/DB 10/6.8AST/ALT 310/125TP/Alb 5.6/3.6ALP 1435GGT 1751PT 13.1 sec
  9. 9. Liver Biopsy• Ductular proliferation• Fibrosis• Widening of portal tracts• Early cirrhosisBiliary atresia Pale stool Not sick child USG features Biopsy
  10. 10. Subtype of type 3 atresia with patent gall bladder & distal bile duct butproximal atresia. The gall bladder though good in size, is filled with mucous. Type II CBD, CHD, GB atretic; Confluence patent Type 1, CBD atretic Type III CBD, CHD, GB atretic; Confluence not patent
  11. 11. Management and follow-up Confirmed on laparotomy Kasai Portoenterostomy
  12. 12. Follow-up Successful Kasai(3mo post-surgery: TB:2.3/DB:0.7) Pigmented stools Follow up at 6 months Thriving. Wt gain present Bilirubin normal
  13. 13. Natural history Brief period of relative well being Natural course thereafter same as Unrepaired BA “Unrepaired BA” Failed Kasai KASAI Decompensation by 1 ½- 2 yr Survival beyond 3rd yr unusual Repeated hospitalisations Poor QOLBirth 6mo 1yr 1 ½ yr 2yr 3yr
  14. 14. Case II: 90 days old• Jaundice noticed at D18 of life• Pigmented stools• BW: 2.3kg , Wt gain: 30 gm/day• Uneventful pregnancy, No F/H• O/E: Not sick, Icterus +• All consecutive stools - pigmented• Liver 3cm soft , Spleen 2cm
  15. 15. InvestigationsTB/DB 7.6/4.4AST/ALT 224/86TP/Alb 6.9/3.4ALP 312GGT 54PT 10.1 sec
  16. 16. Investigations
  17. 17. Follow-up Sp le no m eg a ly Bil iru Hep b in a to me g AST aly /AL T3mo 6mo 9mo 11mo 14mo Normalized
  18. 18. Natural History: Time to normalize (n=40) 16Age in mths 12 9 9 8 6.5 4.2 5.1 4 0 aly ubin a l y G OT PT eg li r eg S SG om Bi to m le n pa Sp He SGPGIMS experience
  19. 19. Case III: 1 year old• Jaundice, pale stools• Diaper staining urine since 2 months of age• Pruritus from 5 mo age• BW: 2.2kg, Wt gain: poor• Isolated mild gross motor delay• No significant antenatal or F/H Cholestasis, PruritusPILBD,A-1ATD,PFIC,Neonatal sclerosing cholangitis
  20. 20. Jaundice Liver:3cm soft, spleen:2cmFacial dysmorphism CVS: systolic murmur in 2nd intercostal space
  21. 21. InvestigationsTB/DB 13.3/8.0AST/ALT 398/250TP/Alb 7.2/3.5ALP 1101GGT 334PT 11.4 sec
  22. 22. InvestigationsLiver Biopsy: 1 bile duct out of 9 portal tracts Paucity of interlobular bile ducts..
  23. 23. Final diagnosisSyndromic PILBD(Alagille Syndrome)
  24. 24. Natural History of Alagille SyndromeOverall 95% Progression 15% CirrhosisCholestasis and liver failure LTx Overall Mortality: 10% Liver disease: 25% Cardiac, renal, vascular: 75% Emerick. Hepatology. 1999; 29: 822–9 Lykaveiris Gut 2001;49:431–435
  25. 25. Case 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• O/E: Icterus +• Liver 3cm soft, Spleen: NP
  26. 26. Investigations USG abdomen TB/DB 7.6/5.3 Liver heterogenous echotextureAST/ALT 45/29 Good sized contractile GB TP/Alb 6.6/3.7 NGRS negative, TORCH/VDRL negative ALP 630 Normal AFP, Ferritin GGT 08 Normal Eye exam. X-ray dorsal spine PT 10.8 sec Normal Lipid profile, ECHO Liver Biopsy : 2 bile duct out of 20 portal tracts Paucity of interlobular bile ducts. Intracanalicular and intracellular cholestasis
  27. 27. PFIC Progressive Familial Intrahepatic cholestasisIntracanalicular and intracellular cholestasis + Pruritus Type 1 or 2: Low GGT
  28. 28. Management and follow-upOn UDCA and Rifampicin, Cholestyramine At 2 yr follow-up Persistently low GGT Pruritus difficult to control LFT no improvement Wt gain present but not satisfactory
  29. 29. Comparison Clinical Parameters PFIC 1 PFIC 2 PFIC 3Age of onset Infancy Neonatal age AdolescenceCourse Relapsing Persistent DelayedRate of progression Moderate Fast SlowCirrhosis First By 1 year of life Young adults decadePruritus Severe Severe ModerateGrowth retardation ++ +++ -Extrahepatic manifestations Diarrhea, Pancreatitis, Present None None Deafness, wheeze
  30. 30. Natural History of PFIC 4yr 1.5yr n=1 (1.5-7.5yr)100% 70% 7.5% 0% 50%Pruritus UDCA response Biliary diversion HCC LTx 100% 58% 30% 26% 47% 5yr 7yr (1.5-10.5yr (0.4-13yr) Davit-Spraul A. Hepatology 2010;51:1645-1655
  31. 31. Case VI: 37daysUneventful pregnancy No F/H Jaundice with pigmented stools from D10 SeizuresBW:2.9kg
  32. 32. Case VI: 37days TLC , DLC 7900, N70 L30 Platelets 80000 CRP PositiveTB/DB 24/8AST/ALT 89/79TP/Alb 3.8/2.8ALP 434GGT 78PT 20.8 sec not correctable
  33. 33. Investigations
  34. 34. Management and follow-upGalactose-free diet (Off breast milk)At 1 yr follow-up• LFT normalized• Coagulopathy corrected• Organomegaly regressed• No major intercurrent illnesses• Steady weight gain• Normal development
  35. 35. Case VII: 3 moUneventful pregnancy No F/H Jaundice with pigmented stools BW: 3 kg Breast fed 1mo 3mo
  36. 36. Case VII: 3 moTLC , DLC 16700, N79 L20 Platelets 160,000 CRP Positive TB/DB 15/9AST/ALT 913/525 TP/Alb 3.2/1.7 ALP 632 GGT 67 INR 4.2 not correctable
  37. 37. Management
  38. 38. Case VII… Further course GAL-1-PUT “normal” Lactose restarted by lab!! Ra pid dowSoya feed 2 w nhill eek cou s rse3mo 4mo
  39. 39. Long term complications• Verbal dyspraxia and lower IQ despite strict diet• Progressive neurological disease: ataxia, tremor• Hypergonadotrophic hypogonadism• Infertility in women Elsas LJ Galactosemia Gene Reviews 1993

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