APPROACH TO A CHILD WITH HEPATOSPLENOMEGALY

Summary of pathophysiological mechanisms

1. Inflammation ( is often present) 2. Kupffer cell hyperplasia with TB or other generalized 3. Congestions – exclude cardiac causes 4. Storage products – usually abnormal appearance, neurological signs and marked firm enlargement of and . 5. Space occupying lesions 6. Fat Infiltration – typical of Kwashiokor and malnutrition 7. Metabolic Disorders

1. INFLAMMATION Congenital – CMV, Herpes Simplex, Toxoplasmosis, , Viral - EBV, HIV, Hep B Parasites - Hydatid, Amoebiasis, Bilharzia Fungal - , Coccidomycosis

Autoimmune Hepatitis Toxins and Drugs Biliary Tract Obstruction

2. RETICULOENDOTHELIAL HYPERPLASIA Septicaemia Malignancy , Leukaemia, Neuroblastoma Granulomatous Response TB

3. VENOUS CONGESTION Congestive Pericardial effusion, constrictive pericarditis Budd Chiari IVC valves

4. STORAGE DISORDERS Carbohydrate Storage Disease Glycogen Storage Disease, Galactossaemia Lipidosis Gauchers, Niemann Pick, Tay Sachs Tyrosinaemia

5. SPACE OCCUPYING LESIONS Abscess Primary and secondary neoplasms

6. FAT INFILTRATION Malnutrition Hyperalimentation Uncontrolled Diabetes Mellitus Mauriac Syndrome Hepatotoxic drugs TB treatment Reye’s Syndrome

7. METABOLIC DISORDERS Wilson’s disease Cystic Fibrosis Galactossaemia

Approach to Hepatosplenomegaly

Inflammatory Reticuloendothelial Venous Storage Space Occupying Fat Metabolic Hyperplasia Congestion disorders lesion infiltration

Infection Septicaemia Cardiac failure Glycogen storage Abscess Malnutrition Cystic Fibrosis -congenital Malignancy Pericardial Galactossaemia Primary Neoplasm TPN Wilson’s disease - TB effusion Mucopolysaccharidosis Secondary Neoplasm Uncontrolled -parasites Constrictive Lipidosis diabetes mellitus -fungal pericarditis Tyrosinaemia Hepatotoxic drugs Autoimmune Budd Chiari Reye’s Syndrome Toxins/Drugs IVC valves Biliary Tract Obstruction