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Work up of chronic liver disease(CLD) in children summarized


                        Work up of chronic liver disease(CLD)
         
                                      Dr Sara Malik

Basic aim:
1.    To know etiology of CLD
2.    To know age of onset
3.    To know difference between compensated & decompensated CLD
History:
1.age of onset (neonate,infant,or child)
2.yellowish discoloration of skin or sclera duration
3.colour of stool and urine
4.history of blood transfusion or any injection /other pricks
5.medications usage
6.any behavioural disturbance or altered state of consciousness
7.any mucocutaneous bleed
8 .history of cld in family
9.decompensated CLD symptoms like hematemesis,malena,abdominal distension,altered sensorium.
10.any surgery

Examination:
General inspection:
·         GCS
·         Jaundice
·         Pallor
·         Facial dysmorphism
·         Abnormal involuntary movements
·         Romberg sign
Peripheral stigmata of CLD
·         Spider naevi
·         Bruising
·         Xanthomas
·         Leuconychia
·         Palmar erythema
·         Wrist widening
Abdominal exam
Prominent abdominal wall veins (portal HTN)
Splenomegaly (portal HTN)
Right and/or left lobe of liver palpable
Fliud shift or thrill



Investigations:

LIVER function test
Synthetic function
Serum albumin will b decreased
PT/INR increased
Serum glucose level
Biliary excretion
Direct & indirect bilirubin
cholestasis
GGT & ALP increased
Hepatocellular damage
AST & ALT increased

Abdominal ultrasound:
liver texture,spleenomegaly, free fluid in peritoneal cavity
specific labs related to etiology:
                                                 
                                               infants
Urine for reducing substances
urine culture
Galactosemia
UTI
TORCH titre
TORCH infection
Thyroid function test
hypothyroidism
Radiology
Butterfly vertebrae
Alagille syndrome
Alpha 1 antitrypsin assay & phenotype deficiency
Alpha 1 antitrypsin deficiency
Abdominal ultrasound for bile duct diameter & gall bladder & choledochal cyst
To rule out  extrahepatic bileduct atresia or choledochal cyst
Sweat inophoresis
Cystic fibrosis
echocardiography
TORCH INFECTION
Pulmonary stenosis in Alagile syndrome
If above labs normal
Go for percutaneous liver biopsy
                                                     Older child
K-F rings ,serum cerruloplasmin level <20 mg /dl
Increase urinary copper excretion >100 ug/day

Wilson disease
HBsAg,HCV titre
Chronic active hepatitis
ANA
Anti smooth muscle antibodies
Celiac workup due to associated autoimmune status so anti TTG Ig A& IgG
Autoimmune hepatitis





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