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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?
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Approach to a child with hepatomegaly Dr. ShreedharPaudel 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 aspartateaminotransferase (SGOT) • Alanineaminotransferase (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