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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 – – – – 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 • • • • • Obesity Diabetes mellitus Cystic fibrosis Malnutrition Connective tissue diseases Hepatomegaly… • Investigations – CBC and differential Reticulocyte count – Liver function tests • • • • • • 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