1 / 15

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.

jennis
Télécharger la présentation

INFANT WITH ACUTE LIVER FAILURE

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  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

More Related