Chronic Liver Disease(pediatrics)

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CHRONIC LIVER DISEASE Presented by : Dr Sonita Trivedi Pg teacher : Dr Aasheeta S Shah HOD Paediatrics V S General Hospital Moderator : Dr Aabha Nagral Jaslok Hospital & Research centre

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Chronic Liver Disease

Transcript of Chronic Liver Disease(pediatrics)

Page 1: Chronic Liver Disease(pediatrics)

CHRONIC LIVER DISEASE

Presented by : Dr Sonita TrivediPg teacher : Dr Aasheeta S Shah HOD Paediatrics V S General HospitalModerator : Dr Aabha Nagral Jaslok Hospital & Research centre

Page 2: Chronic Liver Disease(pediatrics)

11 year old boy, presented with large volume hematemesis

Oedema feet and abdominal distension for 2 months

Born of a non consanguineous marriage

BMI of 28 On examination, pulse rate

120/min, blood pressure 84/50 mm Hg

Pallor+, oedema feet++, mild icterus and moderate ascites present, liver just palpable, spleen +4 cm

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INVESTIGATIONSInvestigation Patient value Normal valuesHb 7.1 >12TC 4800 4000-12000Platelet count 1,00,000 1.5-4.5 lakhSerum Bilirubin : Total

3 <1

Serum bilirubin: Direct

1.5 0-0.3

Serum Albumin 2.6 3.5-5Serum globulin 4.5 2-3.5INR 2.2 0.9-1.1AST 210 0-35ALT 140 0-35ALP 380 Upto 130GGT 110 Upto 85

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USG ABDOMEN Nodular liver with coarse echotexture Liver span 15 cm Splenomegaly 15 cm Leinorenal collaterals Moderate ascites

Ascites tappedProtein 2 g/dl, albumin 0.8, cell count 200, N 40, L 60, ADA -15

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HOW TO MANAGE VARICEAL BLEED? Supportive – fluids, blood, antibiotics Endoscopic measures Pharmacotherapy Prophylaxis of variceal bleed

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Upper GI scopyLarge esophageal varices with red colour signsBand ligation done, mild portal hypertensive gastropathy

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DIFFERENTIAL DIAGNOSIS Chronic liver disease (cirrhosis) with

portal hypertension

Likely etiology:

Viral hepatitis (Hep B & C) Wilson’s disease Autoimmune hepatitis Non-alcoholic fatty liver disease

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Approach to ascites SAAG

(serum ascitic albumin gradient)

> 1.1 < 1.1

Peritoneal TB

Ascites in cirrhosis, BCSCardiac

High cell countPredom lymphocytesADA > 33High LDH

Malignant ascitesHigh cell countMalignant cells +veHigh LDH

Bile ascitesFluid Bil> serum Bil

Nephrotic ascites Protein < 2.5

SecondaryBacterialPeritonitisMultiple organismsTotal protein > 1 gmLow LDH U/LGlucose < 50 mg/dl Pancreatic

AscitesAmylase >1000

Ascitic fluid protein > 2.5 g/dl

Transudate/exudate

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INVESTIGATIONS

HBsAg -ve AntiHCV -ve ANA -ve Other autoimmune markers LKM1 and

Antismooth muscle antibody negative Serum ceruloplasmin 15 mg/dl (20-60) 24 hr urine copper 75 mcg in 24 hrs post d-penicillamine challenge, 24 hr

urine copper: 340 mcg in 24 hrs Lipid profile –normal and blood sugars

normal

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KF RINGS ON SLIT LAMP

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DIAGNOSIS OF WILSON DISEASEKF ringsSerum ceruloplasminSerum copper24 hr urinary copperPost Pencillamine challenge 24 hr urinary

copperLiver copper stain and quantificationMRI brain

NO SINGLE TEST CAN BE CONSIDERED A GOLDSTANDARD FOR DIAGNOSIS

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WHAT IS THE SPECIFIC DRUG OF CHOICE FOR WILSON DISEASE?

D-penicillamine * Zinc Trinetene Zinc + d-penicillamine

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HOW DOES ONE MONITOR ON TREATMENT? 24 hour Urine copper Complete blood count 24 hour Urine protein Free copper?

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DO’S AND DON’TS OF CIRRHOSIS High Protein Diet Vaccination –

Hepatitits A,B

High Salt Diet NSAIDs Benzodiazepines Aminoglycosides ACE inhibitors

Hepatotoxic drugs with caution

Contrast agents with caution

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THANK YOU