Chronic Liver Disease(pediatrics)

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Chronic Liver Disease

Transcript of 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

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

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

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

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

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

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

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

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

KF RINGS ON SLIT LAMP

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

WHAT IS THE SPECIFIC DRUG OF CHOICE FOR WILSON DISEASE?

D-penicillamine * Zinc Trinetene Zinc + d-penicillamine

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

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

THANK YOU