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Neonatal Jaundice Ahmedabad: Dr SK Yachha
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    Neonatal Jaundice Ahmedabad: Dr SK Yachha Neonatal Jaundice Ahmedabad: Dr SK Yachha Presentation Transcript

    • Neonatal jaundice Surender K Yachha MD, DM Professor and Head Department of Pediatric GastroenterologySanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow
    • Neonatal Jaundice
    • 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
    • Consensus Report on Neonatal Cholestasis Syndrome Indian Pediatrics - August 2000, Vol. 37, Number 8
    • 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
    • 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
    • PossibilityFirst Possibility Biliary atresia
    • InvestigationsTB/DB 10/6.8AST/ALT 310/125TP/Alb 5.6/3.6ALP 1435GGT 1751PT 13.1 sec
    • Liver Biopsy• Ductular proliferation• Fibrosis• Widening of portal tracts• Early cirrhosisBiliary atresia Pale stool Not sick child USG features Biopsy
    • 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
    • Management and follow-up Confirmed on laparotomy Kasai Portoenterostomy
    • 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
    • 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
    • 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
    • InvestigationsTB/DB 7.6/4.4AST/ALT 224/86TP/Alb 6.9/3.4ALP 312GGT 54PT 10.1 sec
    • Investigations
    • 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
    • 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
    • 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
    • Jaundice Liver:3cm soft, spleen:2cmFacial dysmorphism CVS: systolic murmur in 2nd intercostal space
    • InvestigationsTB/DB 13.3/8.0AST/ALT 398/250TP/Alb 7.2/3.5ALP 1101GGT 334PT 11.4 sec
    • InvestigationsLiver Biopsy: 1 bile duct out of 9 portal tracts Paucity of interlobular bile ducts..
    • Final diagnosisSyndromic PILBD(Alagille Syndrome)
    • 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
    • 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
    • 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
    • PFIC Progressive Familial Intrahepatic cholestasisIntracanalicular and intracellular cholestasis + Pruritus Type 1 or 2: Low GGT
    • 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
    • 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
    • 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
    • Case VI: 37daysUneventful pregnancy No F/H Jaundice with pigmented stools from D10 SeizuresBW:2.9kg
    • 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
    • Investigations
    • 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
    • Case VII: 3 moUneventful pregnancy No F/H Jaundice with pigmented stools BW: 3 kg Breast fed 1mo 3mo
    • 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
    • Management
    • Case VII… Further course GAL-1-PUT “normal” Lactose restarted by lab!! Ra pid dowSoya feed 2 w nhill eek cou s rse3mo 4mo
    • 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