Chronic Liver Disease(pediatrics)
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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