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INFANT WITH ACUTE LIVER FAILURE

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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INFANT WITH ACUTE LIVER FAILURE

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  1. INFANT WITH ACUTE LIVER FAILURE

  2. 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

  3. ON ENQUIRY: Child apparently alright 3 days back when he developed; • Yellowish discoloration of skin and eyes with high colored urine • No clay colored stools. • Abdominal distension progressively increasing leading to respiratory distress. • Stool frequency 9-12 episodes of green stools/day

  4. One episode of malena. • H/o fever, low to moderate grade for one day • No h/o prolonged neonatal jaundice, • No h/o seizures, • No h/o refusal to feed or decreased urinary output. Birth h/o: Uneventful Development h/o : Normal Family & Past history: Not significant

  5. GENERAL EXAMINATION Drowsy, afebrile; 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. Icterus+++,Pallor+, No dysmorphic features. No cataracts. No skin changes.

  6. SYSTEMIC EXAMINATION P/A: • Distended, dilated abdominal veins, umbilical hernia+, • Liver 2cm, span 8 cm, firm, sharp margins, nontender. • Spleen 3 cm,firm. • Fluid thrill + RS: Air entry b/l equal CVS: S1S2 normal. CNS : Drowsy, tone, reflexes normal.

  7. IMPRESSION Hyperacute liver failure unlikely due to infection alone; D/D: • In born error of metabolism precipitated by an infection. • TORCH Infection – but no h/o antenatal illness or prematurity or IUGR, Normal at birth, until 3 days before admission

  8. INITIAL MANAGEMENT O2 by hood I.V Fluids to maintain Euglycemia Blood Cultures collected 1st dose antibiotics given InjVit K i.v

  9. FURTHER MANAGEMENT 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

  10. INVESTIGATIONS FOR ETIOLOGICAL DIAGNOSIS SEPSIS CRP Negative,Blood Cultures negative. TORCH Titres Negative HLH(HemophagocyticLymphoHistiocytosis ) normalferritin, bone marrow- no hemophagocytes.

  11. Ctd.. • IEM: TYROSINEMIA  AFP 400ng/ml (normal) GALACTOSEMIA  Urine Thin Layer Chromatography galactose+, Total Galactose High, GALT Enzyme level Low.

  12. On 9th day— Child developed increasing respiratory distress, Persistent hypoglycemia on GDR of 14, Intubated & ventilated.. Child succumbed to his disease.

  13. CONCLUSION 1.5 mnths old infant with • Hyperacute liver failure • Direct hyperbilirubinemia • Persistent hypoglycemia inspite of high GDR. • Metabolic Acidosis • Urine Thin Layer Chromatography - Galactose+, • Total Galactose High, • GALT Enzyme level Low. • Diagnosed as GALACTOSEMIA

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