acute liver failure with hemolysis

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Presenter : Ravi Bhardwaj Moderator: Anupam SibalPanelists : BR Thapa, Harshad Devarbhavi, RK Dhiman, Srinivas Sankaranarayanan

Case Discussion Acute liver failure with hemolysis needing a transplant


Dr Ravi BharadwajFNB Pediatric Gastroenterology

Apollo Center For Advanced PediatricsIndraprastha Apollo Hospital

Presenting complaints10 year old FemalePresented in June 2016 with c/o:Poor appetite with nauseaFatigabilityProgressive abdominal distension

Symptoms for 4 weeksTook medicines from nearby practitioner

5th week of illness Two episode of cola colored urinepainlessA day later parents noticed yellowish discolorationof eyesDecreased urine output

No documented feverNo diarrhea/abdominal pain/vomitingNo skin lesions/joint pain/joint swelling/chest painNo dysuria/edema/pustulesNo significant drug historyNo bleeding from any site (skin, GI)No seizures/alteration in sensorium/abnormalmovements/behavioural changes

Question? Differential diagnosis?


Past HistoryNo history of blood transfusionNo similar history in past No history of prior admission for any illness

Developmentally normalVaccinated for ageFamily history One younger male sibling 7 years, wellno history of similar illness in familyNo h/o consanguinityDietary history: Calories 90 Cal/kg/day Protein 1.5 g/kg/day


Admitted in nearby hospitalEvaluated and referred for further evaluation

On examinationRR: 26/minPulse: 96/minTemp: 98.3 FBP: 116/70mm HgSPO2: 96%Weight: 25 kg (-1 to -2 SD)Height: 128 cms ( -1 to -2 SD )


Pallor +, Icterus +No clubbing/spider nevi/palmar erythemaPeriorbital puffiness +P/A: distended, soft Liver 2 cms BCM/span 10 cms, firm with sharpmargins Spleen 2 cms BCM, firm FF+CVS WNLChest no added soundsCNS WNL

Question? Differential diagnosis?

Question?How should this child be investigated further?

InvestigationsInvestigationsHb8.3Bil T/D19.6/10.2TLC13100 P32L63AST/ALT670/214Platelet count113000GGT42Peripheral smearnormocytes, few schistocytes Fragmented RBCsALP47ESR21 mm/hrProt/Albumin 7.3/3.5Retic count 7% (corrected)PT/INR3.7DCTnegativeBU/Cr54/1.1Urine R/MPositive for HbProtein 1 +Uric acid 1.1LDH886CulturessterileUSG abdomenCoarse liver, spleen enlarged, mild ascites

InvestigationsAnti-HAV IgM and totalNRHBsAgNRAnti-HCVNRCeruloplasmin 8 mg/dl24 Hr Urinary Copper (without challenge)413 mcg/dayKF ringpositive ANAnegative

Score 7 on WD criteria (Leipzig score) by Ferenci et al Serum Cp 2 ULN +2KF ring+2Coombs negative hemolytic anemia +1

Wilson's disease ( score > 4)

Acute Liver Failure in WDModest rises in serum aminotransferases (2.2 and ALP/Bil 451101-1501.3-1.6101-1506.8-8.334-442151-2001.7-1.9151-3008.4-10.325-333201-3002.0-2.4301-40010.4-15.321-244>301>2.5>401>15.4


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