4 neonatal cholestasis


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liver failure

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  • One thing in history is consistent that the treating pediatrician kept reassuring. This is very dangerous unless you are absolutely certain that you are dealing with a benign variety.
  • It is very important that you see the stool colour your self. Urine in the diaper could confuse so obtain stool sample not contaminated with urine.
  • There was conjugated hyperbilirubinemia with mild transaminitis and correctable coagulopathy. USG showed normal CBD and IHBR thus making EHBA unlikely; viral markers were negative
  • Different etiologies and different tempo of progression separates this case from previous one.
  • So at the outset we differentiate if there is decompensation or no decompensation. Of course there will be overlap and also the time at which you see the patient is very important. We focus on non decompensated cases here.
  • First and foremost, pediatricians need to separate unconjugated from conjugated variety as the two have totally different etiologies and natural history. In this talk we will deal with conjugated variety only which has various underlying causes as mentioned here.
  • In our experience, conjugated hyperbilirubinemia can be looked at from different angles to get a complete picture.
  • There are various angles of looking at neonatal jaundice. One of them is as shown above
  • Some metabolic disorders present themselves at birth. Others present sometimes after birth. Yet other appear late in infancy; the examples are PFIC or AIH in early childhood. Around five years, you start seeing Wilson’s disease, AIH, Drug induced and viral diseases and so on
  • Armed with information thus obtained, your mind must work like a dual core processor. You have to synthesize all information simultaneously to come to a conclusion.
  • This another approach by Indian Pediatric Academy. Problem we find here is that it starts at 14 days, by that time many diseases may be far advanced or the patient may already be dead especially in the “sick child category”.
    Second concern is stool colour. Now we all now that this could keep changing over a course of few weeks or days.
    Third concern is that certain milder variety of metabolic or viral disease could be missed going by this protocol and we feel they should be testes as well (green box). The list in green box is surely going to increase over a period of time
  • If you rely too much on HIDA scan, you will keep sending patients for “Kasai”.

    Surgeon, if equally unaware will operate and mess it up.
    For us HIDA scan is no no.
  • Although metabolic diseases are an important causes, they differ in their presentation and speed of progression.
  • Based on information gathered using previous strategy We mainly use tests mentioned above. Not all are done at time; more depends upon history, state of liver functions and results of initial investigations.
  • Many cases will come out from this category like many have come out already.
  • 4 neonatal cholestasis

    1. 1. Approach to a case of neonatal Cholestasis Dr Arif Vohra 3rd year resident, B J Medical college Dr Manoj K Ghoda
    2. 2. 2 months old female Referred for persistent jaundice. 1st by order of birth FTNVD No consanguinity Noted to have jaundice by parents @ 15 days or so Reassured by treating pediatrician Progressive deepening of jaundice FTT
    3. 3. O/E: Deeply jaundice, No edema or ascites Liver ++ 2 fingers bellow costal margin Splenic tip palpable No visible veins Diapers were stained yellow, Stool color on personal inspection was yellow
    4. 4. S. Bil: 10.2, 70% conjugated ALT: 376 i.u. GGT: 240 i.u. ALP: 357 i.u. S. Alb: 2.81 S. Glob: 2.45 INR: 1.9, corrected to 1.2 after vit K Hb: 9.8, normochromic normocytic picture WCC: 10,200 with 65% polys Plt: 150,000 RFT: urea 32, creat: 0.9 HBsAg: Non reactive HAV: Non reactive HEV Non reactive USG: Mild hepatomegaly, slightly altered parenchymal pattern, PV and SV were normal in size, Spleen mildly enlarged GB distended CBD and IHBR seen and not dilated
    5. 5. How doe this case differ from the previous one ?
    6. 6. Neonatal/ Infantile Liver Dysfunction Liver failure/ Decompensation • Sick child • Gross metabolic disturbances • Coagulopathy • Ascites/edema Jaundice without decompensation • Non sick looking child • No gross metabolic disturbances • Coagulopathy, if present, is reversible • Ascites/edema is a late event
    7. 7. How does a pediatrician approach a case of neonatal jaundice?
    8. 8. A pediatrician’s dilemma • Is it unconjugated? • Is it conjugated?
    9. 9. Making sense of conjugated hyperbilirubinemia
    10. 10. Conjugated hyperbilirubinemia in Neonates and infants: Etiology based approach • Pregnancy related • Structural defects • Metabolic Diseases • Viral diseases other than those related to pregnancy • Unknown
    11. 11. Jaundice in Neonates, infants and children: Approach based on age of onset • Present at birth • Appearing sometime after birth and progressively increasing with or without decompensation, • Appearing in infancy or early childhood • Appearing in late childhood Each category has certain diseases which are peculiar to that category
    12. 12. Based on stool colour •Creamy white from birth •Normal yellow at birth and then creamy white •Intermittently yellow and white •Yellow all the time Based on Liver status •Early decompensation •Late decompensation
    13. 13. So when you see a patient with jaundice, you may have to process information simultaneously Jaundice soon after birth….. Progressively increasing…… coagulopathy.. Ascites…. Edema…stool yellow Jaundice detected after a few days, progressively increasing…pale stool..no edema, no ascites…no FTT Jaundice detected after a few days, progressively increasing…initially yellow but now pale stool..no edema, no ascites…some FTT Jaundice…..developed in late infancy … no failure to thrive….severe itching….coagulopathy reversible with vitamin K stool intermittently yellow and white
    14. 14. Indian Pediatrics - August 2000, Vol. 37, Number 8 Consensus report on Neonatal Cholestasis Syndrome Sick child means toxic look, FTT, tachycardia, tachypnea,, vomiting, altered sensorium, edema, ascites Galactosemia screen Sugar TORCH Plasma aminoacids Urinary organic acids Ammonia Urea Lactate
    15. 15. What is your experience with HIDA scan, which I am sure is our audience is using quite often? In next 24 hours it could rain or it may not rain. To be sure look at the sky and decide for your self.
    16. 16. HIDA scan consistently fails to differentiate between neonatal cholestasis and biliary atresia. This doesn’t help if there is failure to excrete dye; you will invariably need second investigation to confirm the diagnosis before surgery which is not a minor undertaking. Liver biopsy with USG and clinical history is what we use. Laparoscopic operative cholangiogram where you can also take liver biopsy is also available to us. Our main use is therefore before surgery that a wrong indication is not subjected to surgery
    17. 17. Do all metabolic diseases present identically with jaundice ? •They differ in their presentation •Galactosemia and chronic variety of tyrosinemia primarily could present with neonatal hepatitis and soon develop features of decompensation which is progressive if not treated •FAOD and UCD may present with other symptoms and during work-up are found to have liver dysfunction
    18. 18. Is Liver biopsy safe in this age group? Does it tell everything •Safe •Needs good interpreter •Not good for a metabolic diseases
    19. 19. Could you summarize your approach for the benefit of the audience….
    20. 20. •USG: Structural defects, biliary Atresia, spontaneous perforation of bile duct •TORCH+ Viral markers: For viral etiology •Septic screen: For Cholestasis of inflammation •Galactosemia and Tyrosinemia screen •TMS and Acyl carnitine profile: FAOD, UCD, Tyrosinemia •ECHO: PPH •Fundus examination, X-ray spine for butterfly vertebrae •Liver biopsy •Lap/op cholangiogram
    21. 21. Coming back to our case…. Cytomegalo IgM : Positive GALIPUT: Within normal range Alpha feto protein was not significantly elevated Fundus: Normal ECHO: Normal
    22. 22. Should a pediatrician accept this as evidence of Cytomegalo virus infection as the cause of hepatitis in this patient? No. •CMV is ubiquitous …and not everybody develops manifest infection. •False-positive results are common; •PCR ...to confirm the activity and to ascertain the magnitude of the viral load ... to initiate therapy,
    23. 23. If PCR was high in this case would you treat this patient? Treatment criteria • Immunocompromised are at risk of developing life-threatening and sight-threatening CMV disease.
    24. 24. Or if the patient has Cytomegalo Inclusion Disease (CID) • IUGR, • hepatosplenomegaly, • hematological abnormalities particularly severe thrombocytopenia, and • cutaneous manifestations, including petechiae and purpura The most significant manifestations of CID involve the CNS. • Microcephaly, • ventriculomegaly, • cerebral atrophy, • chorioretinitis, and • sensorineural hearing loss
    25. 25. •CMV PCR was negative. •Liver biopsy was carried out which showed giant cell hepatitis. •The child was treated with supportive treatment and eventually recovered •Is Idiopathic Neonatal Hepatitis a homogenous entity?
    26. 26. •Heterogenous •It may be a form of hepatitis where there is yet unidentified group of disorders •Familial or sporadic •Outcome varies •25-30% could have adverse outcome including death
    27. 27. Summary: Neonatal cholestasis differs from neonatal liver failure; tempo is much slower Overlap is possible Good history, examination of stool color and judicious use of tests are cornerstone for diagnosis In our centre, following causes are seen; •Viral •Galactosemia •Tyrosinemia •Fructosemia •Allagille But most of the time we have Idiopathic neonatal hepatitis as the final diagnosis More alertness is required