chronic liver disease(pediatrics)
DESCRIPTION
Chronic Liver DiseaseTRANSCRIPT
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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
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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