INFANT WITH ACUTE LIVER FAILURE. 1.5 months, male, born of non consanguineous marriage, 1 st by birth order, birth weight 2.9kg, with h/o: Yellowish discoloration of eyes and skin since 3days Abdominal distension with increased frequency of stools since 2days Fever since 1day.
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ACUTE LIVER FAILURE
1.5 months, male, born of non consanguineous marriage, 1st by birth order, birth weight 2.9kg, with h/o:
Yellowish discoloration of eyes and skin since 3days
Abdominal distension with increased frequency of stools since 2days
Fever since 1day
Child apparently alright 3 days back when he developed;
Birth h/o: Uneventful
Development h/o : Normal
Family & Past history: Not significant
HR=124/min, pulses well felt;
RR=58/min, subcostal retractions+;
SPO2=98 on room air
BP= 74/46 mmHg, HGT -30 mg/dl
Anthropometry: Weight -4.2 kg,10thcentile,
Length -52cms, 5thcentile.
No dysmorphic features.
No skin changes.
RS: Air entry b/l equal
CVS: S1S2 normal.
CNS : Drowsy,
tone, reflexes normal.
Hyperacute liver failure unlikely due to infection alone;
O2 by hood
I.V Fluids to maintain Euglycemia
Blood Cultures collected
1st dose antibiotics given
InjVit K i.v
For Fulminant hepatic failure
Started i.v NAC, i.v L-ornithine, L-aspartate, GDR(glucose delivery rate) increased,
PRBC Transfusion, FFP Transfusion.
Sensorium deteriorated with worsening LFTs,
Hypoglycemia inspite of increasing GDR.
Shifted to IPCU
SEPSIS CRP Negative,Blood Cultures negative.
TORCH Titres Negative
normalferritin, bone marrow- no hemophagocytes.
TYROSINEMIA AFP 400ng/ml (normal)
Urine Thin Layer Chromatography galactose+,
Total Galactose High,
GALT Enzyme level Low.
Child developed increasing respiratory distress,
Persistent hypoglycemia on GDR of 14,
Intubated & ventilated..
Child succumbed to his disease.
1.5 mnths old infant with