Hepatomegaly Flashcards
Causes of Hepatomegaly?
SHIRTS
Structural Storage Haematological Heart Infection Inflammatory Infiltrative Reticuloendothelial Rheumatological Tumour Trauma
Structural causes of hepatomegaly?
Extrahepatic biliary atresia Choledochola cyst intrahepatic biliary hypoplasia polycystic disease congenital hepatic fibrosis
Storage/metabolic causes of hepatomegaly?
Defective lipid metabolism-Gaucher, Neimman Pick, hyperlipoproteinomas, cholesteryl ester storage, carnitine deficiency, mucolipidoses
Defective CHO metabolism- DM, GSD (Types 1,3,4, 6), hereditary fructose intolerance, galactosaemia, cushings, syndrome, mucopolysaccharidoses
Defective AA metabolism- tyrosinaemia type 1, urea cycle enzyme disorders
Defective mineral metabolism- Wilsons
Defective electrolyte transport - CF
Defective nutrition- PCM, TPN
Deficiency of protease- Apha-1 antitrypsin deficiency
Haematological causes of hepatomegaly?
Thallasaemia
Sickle cell (Chronic haemolysis and transfusion haemosiderosis)
Acute leukaemia
CML
Heart causes of hepatomegaly
CCF
Constrictive pericarditis
Obstructed IVC
Infective causes of hepatomegaly?
Viral infections- Congenital rubella, CMV, Coxsackie, echo, Hepatitis, EBV
Bacterial- neonatal septicaemia, E.Coli UTI, TB, congenital syphilis
Parasitic- hydatid disease, malaria, schistosomiasis, toxoplasmosis, visceral larva migrans
Inflammatory causes of hepatomegaly?
CAH
Chronic persistent hepatitis
IBD associated
Infiltrative causes of hepatomegaly?
Histiocytosis X
Sarcoidosis
Reticuloendothelial causes of hepatomegaly?
Non-hodgkins lymphoma
Hodgkin’s disease
Rheumatological causes of hepatomegaly?
Systemic onset juvenile idiopathic arthritis (SOJIA)
SLE
Tumour
Primary hepatic neoplasms
Secondary deposits- Wilms, neuroblastoma, gonadal tumours
Vascular malformations-infantile haemangioendothelioma, cavernous haemangioma
Jaundiced infants -ddx hepatomegaly?
EHBA A1AT deficiency Arteriohepatic dysplasia Metabolic- HF1, galactosaemia, tyrosinosis, CF Choledocal cyst Infection Hypopituatarism
Non jaundiced infants - ddx hepatomegaly?
Tumours Choledochal cyst Haemangioma GSD 1, 3, 6 LSD Congenital hepatic fibrosis CCF Metabolic
Jaundiced preschoolers - ddx hepatomegaly?
Metabolic
Chronic active hepatits
Choledochal cyst
Non jaundiced preschooler
Infection- Hep, IMN, CMV, toxo
Metabolic- HF1, GSD, Cholesteryl ester storage disease
Structural- choledochal cyst, tumours, congenital hepatic fibrosis