APPROACH TO A CHILD WITH HEPATOSPLENOMEGALY
Summary of pathophysiological mechanisms
1. Inflammation (jaundice is often present) 2. Kupffer cell hyperplasia with TB or other generalized infections 3. Congestions – exclude cardiac causes 4. Storage products – usually abnormal appearance, neurological signs and marked firm enlargement of liver and spleen. 5. Space occupying lesions 6. Fat Infiltration – typical of Kwashiokor and malnutrition 7. Metabolic Disorders
1. INFLAMMATION Infection Congenital – CMV, Herpes Simplex, Toxoplasmosis, Rubella, Syphilis Viral hepatitis - EBV, HIV, Hep B Parasites - Hydatid, Amoebiasis, Bilharzia Fungal - Histoplasmosis, Coccidomycosis
Autoimmune Hepatitis Toxins and Drugs Biliary Tract Obstruction
2. RETICULOENDOTHELIAL HYPERPLASIA Septicaemia Malignancy Lymphoma, Leukaemia, Neuroblastoma Granulomatous Response TB
3. VENOUS CONGESTION Congestive heart failure Pericardial effusion, constrictive pericarditis Budd Chiari IVC valves
4. STORAGE DISORDERS Carbohydrate Storage Disease Glycogen Storage Disease, Galactossaemia Mucopolysaccharidosis 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 -viral hepatitis 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