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