basics about chronic liver disease for a pediatrician. fast and easy guide to common causes of chronic liver diseases in children
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2. No standard definition
Progressive destruction + regeneration of
liver parenchyma
Hepatocellular necrosis, inflammation and
fibrosis
For hepatitis B and C = >6 month
Often misleading in children for other diseases
Autoimmune hepatitis and metabolic
disorders = CLD @ 1st contact
3. Pathological:
Protracted necroinflammatory liver disease,
irrespective of etiology that may be complicated
by cirrhosis.
*Digestive and Liver Disease 43S (2011) S331–S343
11. Severity of
necroinflammatory
activity (severity of
underlying disease
process)
Acceleration
Longitudinal data
Measure of fibrosis and
architectural alteration
(level of scarring)
Distance
Point data
12. Supportive:
Condition Drug Dose
Cholestasis Phenobarbitone 3-10mg/kg/day
Cholestyramine 250-500 mg/kg/day
Steatorrhoea Formula containing medium chain TG
Pruritus Ursodeoxycholic acid 10-15mg/kg/day
Vitamin Deficiency Vitamin A 10,000-25,000
I.U./day
Vitamin D 3-5 µg/kg/day
Vitamin E 0.05-2 µg/kg/day
Vitamin K 25-200 I.U./kg/day
Micronutrient Calcium 25-100mg/kg/day
Phosphorus 25-50 mg/kg/day
Zinc 1 mg/kg/day
14. Surgical:
Recurrent variceal bleed
Hypersplenism
Extrahepatic or intrahepatic presinusoidal type of
portal hypertension
Radiological:
PTE (Percutaneous transhepatic embolisation)
TIPSS (Transjugular intrahepatic portosystemic shunt)
Treatment of causative factors
15. Hepadnavirideae
Ongoing hepatic injury >6m
Risk of developing chronic infection is
inversely related to age of acquisition
90% in<1yr of age
30% in 1-5 yrs of age
2% for older
20. Phases:-
Treatment:-
Phases HBeAg HBV DNA ALT Symptoms
Immunotolerant Positive High Normal No
Immunoactive Positive (wild)
Negative (mutant)
High elevated +/-
Non replicative Negative Low Normal -
HBeAg HBV DNA
(10^5 copies/ml)
ALT Management
+ + ≤ 2 x normal Observe
+ + >2 x normal Observe 6m.
Treat If no
seroconversion
- + >2 x normal Treat
- - ≤2 x normal Observe
+/- + Cirrhosis Treat
+/- - cirrhosis Observe
*AASLD Practice Guidelines 2009
22. Antivirals:-
Peginterferon-α2 and nucleotide/nucleoside
analogs (telbivudine, tenofevir, entecavir) used
in adults
Agent Advantages Disadvantages
Interferon HBsAg loss
Short duration (24 wks)
No resistance
Subcutaneous
Poor tolerance
Lamivudine Oral
Excellent tolerance
Used in ESLD
Use in ADV failure
Drug resistance
common
Adefovir Oral
Excellent tolerance
Use in ESLD
Use in LAM failure
Approved in >12 yrs
Drug resistance rare
25. Diagnosis:
ANTI HCV HCV RNA INTERPRETATION
Positive Positive Acute on chronic
Positive Negative Resolution of HCV, acute HCV during
period of low viremia
Negative Positive Early acute HCV infection, chronic
HCV in immunosuppresed state, false
positive HCV RNA test
Negative Negative No infection
Treatment: in >3 yrs age
IFN-α2B
Ribavirin
Interferon and ribavirin for 48 wks
Peginterferon
Telaprevir
Studies are pending in pediatrics
*Nelson Textbook of Pediatrics 19th Edition
26. Non resolving inflammation of unknown
origin
Characterstics:
Histology:
interface hepatitis
portal plasma cell infiltration
piecemeal necrosis of hepatocytes
Hypergammaglobulinemia
Circulating organ non specific antibodies
Females > males
4 – 6% of chronic liver disease in childhood
31. AIH Type 1 Type 2
Autoantibodies ANA, Anti actin
antibodies, auto
antibodies against liver
antigen and liver
pancreas antigen,
atypical pANCA, anti
smooth-muscle antibody
Against liver-kidney
microsome 1, antibody
against liver cytosol
Age at presentation Any age Childhood and young
adulthood
Sex Female in 75% cases Female in 95% cases
Other autoimmune
disorders
Common Common, autoimmune
polyendocrinopathy-
candidiasis-ectodermal
dystrophy
Severity Broad range Severe
Treatment failure Infrequent Frequent
Relapse Variable common
* Nelson Textbook of Pediatrics 19th Edition
33. Inborn error of metabolism
Toxic accumulation of copper in liver, brain,
cornea and other tissue.
Abnormal gene ATP7B (metal transporting
ATPase)
36. Avoid food and water with high concentration of copper
Definitive treatment- liver transplant
*AASLD Practice Guidelines 2008
37. Increased inappropriate absorption of oral
iron (in genetically predisposed and those
with ineffective erythropoiesis)
*AASLD Practice guidelines 2011
41. Obstruction of hepatic veins
Efferent hepatic veins to entry of IVC into the
right atrium
No specific cause found
Thrombosis can result from hypercoagulable
states
Clinical Features:
Progressive abdominal distension
Hepatomegaly and ascitis
Portal hypertension
Progressive derangement of liver function
*Liver International ISSN 1478-3223