Transcript Approach to a child with hepatomegaly

Approach to a child with
hepatomegaly
Dr. Shreedhar Paudel
May, 2009
Approach to a child with hepatomegaly
• Considering the history and hepatomegaly
found on the exam, what are your
differentials?
• How would you evaluate this child?
• What is your diagnosis?
• What is the treatment and prognosis for this
infection?
Hepatomegaly
• Liver size may be estimated by degree of
extension of liver edge below costal margin or
by span of liver dullness to percussion
• In children liver edge may be normally
palpable up to 2 cm below right costal margin
Hepatomegaly…
• This assessment of liver size may be unreliable
if other non hepatic factors (e.g., lung disease)
push diaphragm lower and thus the liver
below the costal margin
• Liver span
– a more reliable measurement of liver size
– distance determined by percussion between
upper margin of liver dullness and its lower edge
– Span of 5–7cm is normal in infants and children,
whereas 8–10 cm is normal in adolescents
Hepatomegaly…
• Principal Causes of Hepatomegaly
– Infection/inflammation
• Hepatitis
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Viral
Bacterial
Other infections
Toxic
• Cholangitis
Hepatomegaly…
• Principal Causes of Hepatomegaly
– Hemolytic anemia
– Cardiac disorders
– Trauma
– Bile duct obstruction
• Biliary atresia
– Vascular disorders
• Budd-Chiari syndrome
• Veno-occlusive disease
Hepatomegaly…
• Principal Causes of Hepatomegaly
– Metabolic disorders
• Wilsons disease
• Disorders of carbohydrate metabolism
– Galactosemia
– Hereditary fructose intolerance
– Glycogen storage disease
• Disorders of amino acid metabolism
– Tyrosinemia
– Urea cycle defects
• Disorders of lysosomal storage
– Mucopolysaccharidoses
– Lipidoses
– Glycoprotein disorders
Hepatomegaly…
• Principal Causes of Hepatomegaly
– Systemic disorders
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Obesity
Diabetes mellitus
Cystic fibrosis
Malnutrition
Connective tissue diseases
Hepatomegaly…
• Investigations
– CBC and differential Reticulocyte count
– Liver function tests
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Serum aspartate aminotransferase (SGOT)
Alanine aminotransferase (SGPT)
Alkaline phosphatase
Total protein
Albumin
Bilirubin
– Serum alpha1-antitrypsin
– Serum ceruloplasmin
Hepatomegaly……
• Prothrombin and activated partial
thromboplastin times
• Selection of radiographic imaging
procedures(e.g., abdominal U/S and CT)
depends on suspected diagnosis
• Percutaneous liver biopsy is diagnostic of
many disorders
• Investigations should be tailored to suspected
diagnosis