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Jaundice in Children. Abdulwahab Telmesani FRCPC,FFAP Faculty of Medicine and Medical Science Umm Al-Qura University. An Approach to a Child With Direct Hyperbilirubinemia. Classic Approach. Proper detailed history Proper physical examination Formalize an impression of prioritized DDx
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Jaundice in Children Abdulwahab Telmesani FRCPC,FFAP Faculty of Medicine and Medical Science Umm Al-Qura University
Classic Approach • Proper detailed history • Proper physical examination • Formalize an impression of prioritized DDx • Appropriate investigations
Identify • Acute • Chronic (more than 6 months)
In Children • Acute • Chronic (more than 6 months)
Identify • Hepatocellular • Chlestatic
In Children • Hepatocellular(ALT/AST more than twice of ALP) • Cholestatic (ALT/AST less than twice of ALP)
Remember The prognostic value of • Albumin • Coagulation profile
Etiology • Infection • Drugs • Specific Entities • Vascular
Etiology • Infection • Drugs • Specific Entities • Vascular
Infections • Viral • Bacterial • Parasitic
Viral Hepatitis • Hepatotropic Virus’s (replicate in the liver and causes hepatitis) • Others
Hepatotropic Viruses • HBV (10-20% Chronic active hepatitis) • HCV (70-80% Chronic active hepatitis)
Hepatotropic Viruses Non B / C Viral Hepatitis • HAV • HEV • HFV • HGV • TTV • SEN
Others • EBV • CMV • Herpes • Other
Hepatitis A Virus Most common cause of community acquired hepatitis through out the world
Hepatitis A Virus • RNA Picorna Virus (Rhinovirus, Enterovirus, Cocxackievirus) • Feco - oral transmission (Food – borne +/- Water – borne) • Day care centers account for 10% of cases
Hepatitis A Virus Transmission in 50% of contacts
Hepatitis A Virus Liver injury in HAV is secondary to immune response not to cytopathy
Hepatitis A Virus Presentation • Incubation period 4 weeks • Prodrome 1 week • Jaundice 1 – 3 weeks • Hepatomegaly • Liver enzymes 20 – 100 time upper normal • Spontaneous resolution
Hepatitis A Virus Presentation • Sporadic • Epidemic • Endemic
Hepatitis A Virus Clinical Presentation in Endemic areas • 10 % of children below 6 years • 40 % of children 6 – 14 years • 70 % of subjects older than 14 years • 70 – 100 % of children have been infected
Hepatitis A Virus Epidemic • Tend to seasonal • Symptoms as in sporadic cases
Hepatitis A Virus No Chronic Sequelae
Hepatitis A Virus Variants • Relapsing course up to 1 year • Cholestatic up to 2 years • Immune-complex features ( vasculitis, arthritis…)
Hepatitis A Virus Fatalities • Secondary to acute hepatic failure • Less than 2 % • More in older children and adults • When on top of chronic hepatitis
Hepatitis A Virus In Shanghais HVA epidemic, mortality was 5 times higher among patients with chronic hepatitis B
Hepatitis A Virus Prevention • Immunoglobulin • Vaccination ( 2 doses 6 months apart above 1 year of age)
Hepatitis A Virus ? Atopy protect against enteric infection including HAV P N Black Allergy 2005
Hepatitis B Virus Vaccination decreased the incidence of hepatic carcinoma in children (in adults in future)
Hepatitis C Virus • Perinatal transmission about 6% • Elective C/S might lower the risk • No evidence of risk of breast feeding
Hepatitis E Virus • Single Strand RNA • Feco – oral transmission • Endemic in Tropical and Subtropical countries • Mortalities 0.2 % but as high as 4 % in pregnant women
Hepatitis E Virus • Incubation period 2 – 9 weeks • Presentation similar to Hepatitis A • Diagnosed by Anti HEV IGM serology • No chronic sequelae reported • It worsens chronic hepatitis • No vaccine available yet
Hepatitis G Virus • Enveloped RNA virus • Parental transmission • Detected by PCR • 2-39% of non A-E hepatitis • 16-43% of Fulminant hepatitis • ? Hepatotropic • No established serology
TTV • Single strand DNA • Isolated from patients post transfusion (100 %) • Isolated from patients with non A-E Hepatitis • Presents in health individuals 1 – 13% (89 %) • ? Feco – oral transmission • ? Normal human viral flora
SEN Virus • Single strand DNA virus • Most recent cause of non A- E Hepatitis • Found in Blood donors 1- 13% • In 70% of transfused patients • ? Hepatotropic • ? Feco – oral transmission.
Etiology • Infection • Drugs • Specific Entities • Vascular
Paracetamol • Commonest cause of acute liver failure in USA • We all have it at home • Toxic dose is more than 150 mg /Kg
Paracetamol • Need repeated serum drug level • Follow Rumack-Matthew nomogram • A point of irreversible liver damage (end stage liver disease) • N-cetylcysteine is the anti-dote (oral/intravenous) • Liver transplant when end stage liver disease
Etiology • Infection • Drugs • Specific Entities • Vascular
Specific Entities • Wilson’s Disease • A1 Antitrypsin deficiency • IBD Hepatitis • Auto-immune Hepatitis • Syndromatic Diseases • Metabolic • Progressive Familial Intrahepatic Cholestasis
Wilson’s Disease • Autosomal Recessive Disease • Low cerulplasmin • Copper deposition in; liver, brain, kidneys, eyes, heart, Hemolysis
Wilson’s Disease Presents in any of the following; • Acute liver disease • Chronic liver disease • Minimal neurological manifestations • Sever neurological manifestations • Psychiatric symptoms • Renal tubular acidosis • Bony deformities • Hemolytic anemia
Wilson’s Disease An 18 years old male and 19 years female reportedwithSchizophrenic symptoms; • No Kayser -Fleischer ring • Normal physical examination • Low cerulplasmin, high serum copper and high 24 HR urine copper • Symptoms improved on D – Penicillamine Patrick Stiller J Psych. Neurosci 2002
Wilson’s Disease Liver biopsy and determination of hepatic copper is the golden standard for diagnosis of Wilson’s Disease
Wilson’s Disease Diagnosis can be made based on at least two of the following; • Low serum Cerulplasmin • High 24 HR urine copper • K.F Ring Ashish Bavdekar J Gastr & Hepat 2004
Wilson’s Disease Treatment; • D- Penicillamine • Trientine • Zinc
Etiology • Infection • Drugs • Specific Entities • Vascular