15 months male born of non consanguineous marriage 1 st by birth order birth weight 29kg with ho Yellowish discoloration of eyes and skin since 3days Abdominal distension with increased frequency of stools since 2days ID: 999104
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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 3daysAbdominal distension with increased frequency of stools since 2daysFever 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,10th centile, Length -52cms, 5th centile. Icterus+++,Pallor+,No dysmorphic features. No cataracts.No skin changes.
6. SYSTEMIC EXAMINATIONP/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 equalCVS: 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 Inj Vit K i.v
9. Day1Day 2Day 3Day 4Day 5Day 6Hb(g/dL)6.85.57.65.17.37.0TLC(/cumm)22,20026,50014,8003,6006,5005,900Plt(/cumm)2.4L2.18 L1.3 L40,00038,00025,000s.Bili T/D(mg/dl)38/1432/2324/6.123/4.517.2/5.717.6/3.6SGOT(IU/L)3062181709491105SGPT(IU/L)94144100444240s.Alb(g/dl)3.22.42.62.7PT/PTT78.7/>2 mins52.5/>2minsRBS30131281426062ABG(pH/Hco3/pCO2)7.46/13.5/18.6Met Acidosis+Resp Alk7.5/14.5/18.1NH3(micromol/l)108188134LDH(IU/L)2263U.Red subsTrace2+3+CRPNRNRNR
10. FURTHER MANAGEMENTFor Fulminant hepatic failureStarted 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
11. Day1Day 2Day 3Day 4Day 5Day 6Hb(g/dL)6.85.57.65.17.37.0TLC(/cumm)22,20026,50014,8003,6006,5005,900Plt(/cumm)2.4L2.18 L1.3 L40,00038,00025,000s.Bili T/D(mg/dl)38/1432/2324/6.123/4.517.2/5.717.6/3.6SGOT(IU/L)3062181709491105SGPT(IU/L)94144100444240s.Alb(g/dl)3.22.42.62.7PT/PTT78.7/>2 mins52.5/>2minsRBS30131281426062ABG(pH/Hco3/pCO2)7.46/13.5/18.6Met Acidosis+Resp Alk7.5/14.5/18.1NH3(micromol/l)108188134LDH(IU/L)2263U.Red subsTrace2+3+CRPNRNRNR
12. INVESTIGATIONS FOR ETIOLOGICAL DIAGNOSIS SEPSIS CRP Negative,Blood Cultures negative.TORCH Titres NegativeHLH(Hemophagocytic Lympho Histiocytosis )normal ferritin, bone marrow- no hemophagocytes.
13. Ctd..IEM:TYROSINEMIA AFP 400ng/ml (normal) GALACTOSEMIA Urine Thin Layer Chromatography galactose+,Total Galactose High,GALT Enzyme level Low.
14. On 9th day—Child developed increasing respiratory distress,Persistent hypoglycemia on GDR of 14,Intubated & ventilated..Child succumbed to his disease.
15. CONCLUSION1.5 mnths old infant withHyperacute liver failureDirect hyperbilirubinemiaPersistent hypoglycemia inspite of high GDR.Metabolic AcidosisUrine Thin Layer Chromatography - Galactose+,Total Galactose High,GALT Enzyme level Low.Diagnosed as GALACTOSEMIA