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

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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?

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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

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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 )

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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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