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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
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
Investigation Patient value Normal values
Hb 7.1 >12
TC 4800 4000-12000
Platelet count 1,00,000 1.5-4.5 lakh
Serum Bilirubin :
Total
3 <1
Serum bilirubin:
Direct
1.5 0-0.3
Serum Albumin 2.6 3.5-5
Serum globulin 4.5 2-3.5
INR 2.2 0.9-1.1
AST 210 0-35
ALT 140 0-35
ALP 380 Upto 130
GGT 110 Upto 85
Nodular liver with coarse echotexture
Liver span 15 cm
Splenomegaly 15 cm
Leinorenal collaterals
Moderate ascites
Ascites tapped
Protein 2 g/dl, albumin 0.8, cell count 200, N
40, L 60, ADA -15
Supportive – fluids, blood, antibiotics
Endoscopic measures
Pharmacotherapy
Prophylaxis of variceal bleed
Upper GI scopy
Large esophageal varices with red colour
signs
Band ligation done, mild portal hypertensive
gastropathy
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, BCS
Cardiac
High cell count
Predom lymphocytes
ADA > 33
High LDH
Malignant ascites
High cell count
Malignant cells +ve
High LDH
Bile ascites
Fluid Bil>
serum Bil
Nephrotic ascites
Protein < 2.5
Secondary
Bacterial
Peritonitis
Multiple organisms
Total protein > 1 gm
Low LDH U/L
Glucose < 50 mg/dl Pancreatic
Ascites
Amylase >1000
Ascitic fluid protein > 2.5 g/dl
Transudate/exudate
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
Serum ceruloplasmin
Serum copper
24 hr urinary copper
Post Pencillamine challenge 24 hr urinary
copper
Liver copper stain and quantification
MRI brain
NO SINGLE TEST CAN BE CONSIDERED A GOLD
STANDARD FOR DIAGNOSIS
D-penicillamine *
Zinc
Trinetene
Zinc + d-penicillamine
24 hour Urine copper
Complete blood count
24 hour Urine protein
Free copper?
 High Protein Diet
 Vaccination –
Hepatitits A,B
High Salt Diet
NSAIDs
Benzodiazepines
Aminoglycosides
ACE inhibitors
Hepatotoxic drugs
with caution
Contrast agents
with caution
Chronic Liver Disease(pediatrics)

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Chronic Liver Disease(pediatrics)

  • 1. 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
  • 2. 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
  • 3. Investigation Patient value Normal values Hb 7.1 >12 TC 4800 4000-12000 Platelet count 1,00,000 1.5-4.5 lakh Serum Bilirubin : Total 3 <1 Serum bilirubin: Direct 1.5 0-0.3 Serum Albumin 2.6 3.5-5 Serum globulin 4.5 2-3.5 INR 2.2 0.9-1.1 AST 210 0-35 ALT 140 0-35 ALP 380 Upto 130 GGT 110 Upto 85
  • 4. Nodular liver with coarse echotexture Liver span 15 cm Splenomegaly 15 cm Leinorenal collaterals Moderate ascites Ascites tapped Protein 2 g/dl, albumin 0.8, cell count 200, N 40, L 60, ADA -15
  • 5. Supportive – fluids, blood, antibiotics Endoscopic measures Pharmacotherapy Prophylaxis of variceal bleed
  • 6. Upper GI scopy Large esophageal varices with red colour signs Band ligation done, mild portal hypertensive gastropathy
  • 7. Chronic liver disease (cirrhosis) with portal hypertension Likely etiology: Viral hepatitis (Hep B & C) Wilson’s disease Autoimmune hepatitis Non-alcoholic fatty liver disease
  • 8. Approach to ascites SAAG (serum ascitic albumin gradient) > 1.1 < 1.1 Peritoneal TB Ascites in cirrhosis, BCS Cardiac High cell count Predom lymphocytes ADA > 33 High LDH Malignant ascites High cell count Malignant cells +ve High LDH Bile ascites Fluid Bil> serum Bil Nephrotic ascites Protein < 2.5 Secondary Bacterial Peritonitis Multiple organisms Total protein > 1 gm Low LDH U/L Glucose < 50 mg/dl Pancreatic Ascites Amylase >1000 Ascitic fluid protein > 2.5 g/dl Transudate/exudate
  • 9. 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
  • 10.
  • 11. KF rings Serum ceruloplasmin Serum copper 24 hr urinary copper Post Pencillamine challenge 24 hr urinary copper Liver copper stain and quantification MRI brain NO SINGLE TEST CAN BE CONSIDERED A GOLD STANDARD FOR DIAGNOSIS
  • 13. 24 hour Urine copper Complete blood count 24 hour Urine protein Free copper?
  • 14.  High Protein Diet  Vaccination – Hepatitits A,B High Salt Diet NSAIDs Benzodiazepines Aminoglycosides ACE inhibitors Hepatotoxic drugs with caution Contrast agents with caution