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Liver failure in a neonate
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Liver failure in a neonate

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    Liver failure in a neonate Liver failure in a neonate Presentation Transcript

    • Liver failure in a neonate Presenter/ Moderator Panelists: Manoj Ghoda - Gastroenterologist, Gujarat Research & Med. Inst. Ahmd Priya Kishnani – Pediatrician & Geneticist, Duke University, North Carolina, USA Seema Alam - Pediatric Hepatologist, ILBS, New Delhi S K Yachha - Pediatric Hepatologist, SGPGI, Lucknow
    • This is the story of Tanya
    • Let us go inside and find out what is going on.....
    • A case of Neonatal Liver failure Manoj K Ghoda M.D., M.R.C.P. Consultant Gastroenterologist, Ahmedabad
    • A case of Neonatal Liver Failure • Female - Term Delivery – 2.5 Kg. • Breastfed • Drowsy and jaundiced on day 5 of life – Conjugated hyperbilirubinemia • Bilirubin 7mg. – Synthetic dysfunction • PT 20s; Albumin 2.0s – Hyperammonemia 350 mMol/L Conjugated bilirubin more than 2 mg is usually suggestive of liver disease
    • If you were in Nasik as a general pediatrician, would you do any more test at this stage ?
    • Hypoglycemia RFTs were normal CBC: Mild normochromic, normocytic anemia, reticulocytes 2% WCC 21,000 with 70% polys CRP: 15 ( up to 1 mg/L) Blood culture obtained for aerobic, anerobic organisms; USG: Normal size and echo texture, collapsed GB, CBD could not be visualized. CXR: NAD Hypoglycemia soon after birth: Perinatal events, Galactosemia, GSDs, Orgamic acidemia, PHHI Hypoglycemia starting late in infancy or early childhood: FAOD, PHHI, HFI, Insulinoma, GSDs
    • What would you do now? Reassure the parents and give Udiliv and syrup? Liv-52 Refer the patient to higher centre ? Treat sepsis and reassure the parents that once the sepsis is cleared everything will be ok
    • Transferred • Appeared sick • Conservative Rx for ALF (Vit K, Sod Benzoate and Sod Phenyl Butyrate, FFP if required, ? antibiotics) • Glucose @ 7mg/kg/min Sick child means one with toxic look, FTT, tachycardia, tachypnea, vomiting, altered sensorium, edema, ascites
    • •Fluid restrict to maximum of two thirds/65% maintenance (100ml/kg/day) •Restrict sodium (hyponatremia usually reflects excess water caused by fluid retention) •Consider broad spectrum antibiotics, fluconazole and consider acyclovir •Monitor acid base status, fluid balance, blood glucose, PT, Creatinine •Monitor signs of raised intracranial pressure •Consider N-acetylcysteine •Commence Ranitidine 2mg/kg tds
    • And where metabolic disorders are a possibility: The basic principles for stabilization* •Prevent catabolism - Administration of calories is used in the treatment of acute episodes to try to slow down catabolism. •We use IV dextrose as it does no harm whatever the underlying pathology and helps most of the patients. •Limit the intake of the offending substance - if possible, through manipulation of the diet. Limit protein in urea cycle disorders, fatty food in FAOD *Hoffman and Zschocke
    • 1st lesson: if the patient is acutely ill Stabilize the patient without bothering to find out the cause of illness. 2nd lesson: When you consider sepsis, look for the focus
    • Lesson 3: When metabolic disease is suspected, don't panic! Try to stabilize patient at your place before contemplating transfer. Try to discuss pt with your metabolic specialist
    • A pediatrician’s dilemma • How do I proceed to investigate this nightmare?
    • Approach to a case of neonatal and pediatric jaundice/Liver failure One thing a pediatrician should never do Is to say......that… Everything will be Ok in 3 months, .....just keep the child in sunlight Schedule a follow up visit or admit the child Make sure that the things are going well and the child is thriving If not send the patient to ME
    • Consensus report on Neonatal Cholestasis Syndrome Indian Pediatrics - August 2000, Vol. 37, Number 8 Sick child means toxic look, FTT, tachycardia, tachypnea,, vomiting, altered sensorium, edema, ascites
    • Armature's Approach to a case of neonatal and pediatric jaundice/Liver failure • Is it unconjugated? • Is it conjugated? 1
    • Neonatal Liver Dysfunction Liver failure/ Decompensation Jaundice without decompensation • Non sick looking child • No gross metabolic disturbances • Coagulopathy, if present, is reversible • Ascites/edema is a late event • Sick child • Gross metabolic disturbances • Coagulopathy • Ascites/edema 2
    • Could liver failure be..... • • • • • Pregnancy related Structural defects Metabolic Diseases Viral diseases Unknown 3
    • Metabolic Diseases: When should the alarm bell ring? if you see a child with….. • • • • • Acute illness following a period of normalcy. Intermittent illness for no obvious reason. Recurrent unexplained vomiting. Failure to thrive. Aversion to certain food or the illness starting with particular food. • Jaundice • Organomegaly
    • Age at onset of jaundice and likely etiology • • Appearing sometime after birth and progressively increasing • Appearing in infancy or early childhood • Appearing in late childhood Present at birth 4
    • Associated system involvement • • • • Renal involvement CNS involvement Cardio-respiratory involvement Hematological involvement (DIC, Familial haemophagocytic syndrome) 5
    • So when you see a patient with jaundice, you have to process information simultaneously ..Consanguinity in parents….. Jaundice soon after birth Progressively increasing…… coagulopathy.. Ascites…. Edema Jaundice in a child with mother having herpes simplex infection.. Cutaneous lesions….. DIC…..Encephalitis.. Jaundice noticed at about 3 weeks, progressively increasing, no significant FTT, no liver failure, stool pale all throughout Jaundice…..developed at 5 months… no failure to thrive….severe itching….coagulopathy reversible with vitamin K
    • How do we proceed from here?
    • Neonatal 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 Metabolic Liver Diseases Herpes Simplex hepatitis Familial hemophagocytic syndrome
    • Metabolic causes of neonatal liver failure
    • Herpes simplex hepatitis •The incubation period is 4 to 21 days after delivery. •Symptomatic between 6 and 21 days, •Resembles bacterial sepsis •Vesicular skin lesions are the most predominant symptom and, when lesions are present, herpes infection must be presumed until proven otherwise. •Associate CNS involvement and DIC leaves you with no other diagnosis
    • Familial hemophagocytic syndrome Multiorgan failure with jaundice, hepatosplenomegaly, fever, skin rash, and pancytopenia. The diagnosis is confirmed by finding erythrophagocytosis in bone marrow, liver, and occasionally cerebrospinal fluid. Confirmatory investigations include elevated plasma triglycerides and increased serum ferritin.
    • Which of the following tests you would like to do in this patient based on our discussion? •Urinary reducing substances •Urinary ketones •Urinary GAG/Oligo •TORCH titre •Total Galactose, Galactose phosphate/ Gal-1-T, withdrawal of Galactose •Urea •Lactate •Ferritin and transferrin saturation •Alpha- fetoprotein •Urinary succinyl acetone •Plasma aminoacids •Urinary organic acids •HSV PCR/Viral load •Bone marrow
    • Coming back to our case..... • Female - Term Delivery – 2.5 Kg. • Breastfed • Drowsy and jaundiced on day 5 of life – Conjugated hyperbilirubinaemia • Bilirubin 17mg. – Synthetic dysfunction • PT 20sAlbumin 2.0s • Managed for sepsis – – – – Penicillin / Gentamicin IV dextrose Blood cultures negative Felt better
    • Is this the end of story?
    • Would you believe this????. • Female - Term Delivery – 2.5 Kg • Breastfed • Drowsy and jaundiced on day 5 of life – Conjugated hyperbilirubinaemia • Bilirubin 17mg. – Synthetic dysfunction • PT 20sAlbumin 2.0s • Managed for sepsis – Penicillin / Gentamicin – IV dextrose – Blood cultures negative • Reintroduced feeds – Worsening liver failure • Eye r/v – Oil droplet cataract
    • Coming back to our case.... • Urine reducing sugars – POSITIVE • Galactosaemia screen – POSITIVE – Gal-1-phosphate >2000 – GALT Q188R homozygous • DIAGNOSIS – CLASSICAL GALACTOSAEMIA • Treatment – Soya formula – Resolution of jaundice within 4-5 days – Resolution of cataracts over 3 months – Calcium supplements from age 3 – Galactose-free solids
    • Summary:
    • Take home message •Neonatal liver failure is an emergency •Assess quickly •Obtain blood and urine samples in duplicate and freeze them at -20 if processing is delayed •Stabilize as per situation; consider an opinion •Withdraw Galactose as soon as the blood sample is taken •Consider blind antibiotics if sepsis is a possibility •Use IVIG if neonatal hemochromatosis is likely •Use Acyclovir if Herpes simplex is a real possibility •Make a short list and proceed stepwise