Liver failure in a neonate
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
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.
A case of Neonatal Liver Failure
• Female - Term Delivery – 2.5 Kg.
• 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 ?
RFTs were normal
CBC: Mild normochromic, normocytic anemia, reticulocytes
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.
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
Refer the patient to higher centre ?
Treat sepsis and reassure the parents that once the
sepsis is cleared everything will be ok
• 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
•Restrict sodium (hyponatremia usually reflects excess water caused
by fluid retention)
•Consider broad spectrum antibiotics, fluconazole and consider
•Monitor acid base status, fluid balance, blood glucose, PT,
•Monitor signs of raised intracranial pressure
•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
if the patient is acutely ill
Stabilize the patient without bothering to find out
the cause of illness.
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
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
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?
Neonatal Liver Dysfunction
Liver failure/ Decompensation
Jaundice without decompensation
• Non sick looking child
• No gross metabolic
• Coagulopathy, if present, is
• Ascites/edema is a late
• Sick child
• Gross metabolic
Could liver failure be.....
Metabolic Diseases: When should the alarm
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
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
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
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
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
•Total Galactose, Galactose phosphate/ Gal-1-T, withdrawal of Galactose
•Ferritin and transferrin saturation
•Urinary succinyl acetone
•Urinary organic acids
•HSV PCR/Viral load
Coming back to our case.....
• Female - Term Delivery
– 2.5 Kg.
• Drowsy and jaundiced
on day 5 of life
• Bilirubin 17mg.
– Synthetic dysfunction
• PT 20sAlbumin 2.0s
• Managed for sepsis
Penicillin / Gentamicin
Blood cultures negative
Take home message
•Neonatal liver failure is an emergency
•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
•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