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EFIM Winter School of Internal MedicineCase Presentation

Johan Van Laethem, January 2015

(Belgium)

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Interesting numbers

3

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Official languages

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3

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Interesting numbers

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8 0

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Interesting statistics% of billiart players use Belgian (made) balls

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2,3

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Tallest European man: Alain Delaunois

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1

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Using discount coupons (2nd USA)

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540

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540 days without government in 2011

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22

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22 kg of chocolate ingested/y

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68

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68 y: life expectancy anaesthesiologist

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92 y: life expectancy radiologist

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1. Introduction

Systemic interrogation:

- Referral GP: cytolysis, cholestasis since 1 month, asthenia

- Fatigue, muscle waisting

- No history of alcohol intake

Physical examination:

- Lean

- Icterus

- Asterixis after few days

- Parameters stable

Medication intake:

-Iron supplementation

-Tamsulosin (alfa-blocker)

-Aspirin 80 mg/d

-Protein powder (Nutricia)

Medical history:

-Partial gastrectomy 1983, gastric carcinoma

-2012 intestinal operation

-Benign prostate hypertrophy

-Iron deficiency

Male, 68 yr

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2. Diagnostic work-up: lab results and imaging

ANCA’s, ANF, LKM Ab, smooth muscle Ab, anti-mitochondrial Ab: negativeAbdominal CT en echography: normal liver morphology, no biliary stones nor bile duct dilatation

01/1207/1212/12

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3. What would you do?

A. Treat the patient with corticosteroidsB. Perform a liver biopsyC. Watchful waitingD.Refine the interrogationE. Put the patient on the liver transplantation list

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4. Refined interrogation and clinical evolution

12/12:

Hepatic encephalopathy

01/12:

Rise of liver tests

Patient still responsive

24/11:

Cytolysis, bili 1,5 g/dl

09/10:

Start protein powder

3/10:

Normal Liver tests

Refined interrogation and clinical evolution

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Low ceruloplasmin

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Wilson’s disease?

Low ceruloplasmin (PPV 6%):• Wilson’s• Acute viral hepatitis • Chronic hepatitis • Drug-induced liver injury (DILI)• Alcohol-induced liver disease• Malabsorption

Split lamp examination: no Kaiser Fleischer rings (sensitivity 60%)

Nonceruloplasmin-bound copper (mcg/L) = serum copper (mcg/L) – (3.15 mcg/g x serum ceruloplasmin [mg/L])Wilson’s: free copper typically > 20-25 mcg/dl

HES staining and picture: prof. A. Hoorens

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6. Management: what would you do?

A. Discontinuation of the medicationB. Discontinuation of the protein powderC. Start corticosteroidsD. Lactulose for hepatic encephalopathyE. Something else?

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7. Cause of the medicamentous/ toxic hepatitis?

1. Iron supplementation/ Aspirin/ Tamsulosin ??

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8. Protein powder?

“Thank you for the information…but it seems unlikely the liver failure was due to our product because the patient ingested 4,4-8,8 g of proteins a day, which would be equal to the protein amount of 1 glass of milk.”

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Clinical and biochemical evolution

• Slow decrease cytolysis and bilirubin over time, correction of PT• Decrease of icterus• Patient still weak • PET-CT: no particularities

Based on the chronology of liver failure our first hypothesis is liver failure due to protein powder

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Unanswered questions

Place of corticoids?(no proof of beneficial effects, only hypersensitivity reactions)

Causative drug/ supplement?

Cause of progressive hypoalbuminemia?

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Conclusions

1. Drug induced liver injury (DILI) is frequent (15-30% acute liver failures)2. Liver biopsy is useful in diagnosis3. Identification of causative drug is difficult, no routine test. Re-challenge is

difficult.4. Cornerstones of treatment: discontinuation of the drug, sometimes liver

transplant5. Corticotherapy has no proven effect but can be tried in evident

hypersensitivity

Thank you for listening

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Thank you for listening

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