Paediatric liver conditions Present with combinations of the following symptoms and signs Jaundice Encephalopathy (altered consciousness or behaviour) Bleeding tendency Abdominal distension and ascites Hepatomegaly and/or hepatosplenomegaly

Paediatric Liver Conditions Persistent Neonatal Jaundice Hepatomegaly and hepatosplenomegaly Acute onset jaundice Acute liver failure Ascites Chronic liver disease Chronic hepatitis Hepatic schistosomiasis Veno-occlusive disease Cirrhosis Portal hypertension

Neonatal jaundice Failure to clear bilirubin Excess production of bilirubin Liver immaturity Liver disease/involvement in disease Obstruction to bile flow Persistence beyond 10 days to 2 weeks

Approach to neonatal jaundice Onset day 1 Haemolytic disease Intrauterine infection Prematurity Evaluation Examine : Pale? splenomegaly? Heart failure? Other signs of illness Tests : FBC, Coombs, Blood groups, TSB, Cultures, TORCHeS

Approach to neonatal jaundice Onset after day 2 Physiological jaundice Haemolysis Jaundice of immaturity Infection Evaluation Examine:GA?, well?, pallor?, splenomegaly? Tests: FBC, TSB, Blood groups, Urine dipstix, possible cultures for infection

Approach to neonatal jaundice Onset after day 3 – 5 Infection (UTI, Sepsis) Neonatal hepatitis Metabolic disease Biliary atresia Breast milk jaundice Evaluation Examination: careful search for infection, liver? Stool colour? Tests: FBC, CRP, TSB/conjugated, Urine, B/C, TORCHeS, Urine Clinitest, TFT, Stool bile pigments, LFT’s

Approach to neonatal jaundice Jaundice persisting beyond 10 days Neonatal hepatitis Biliary obstruction/atresia Metabolic disease Breast milk jaundice Infection (congenital/acquired) Evaluation Examination:Well?, liver and spleen?, Stool appearance? Tests: TSB/conjugated, FBC, CRP, LFT’s, TFT, Urine mcs and clinitest, TORCHeS and Hepatitis viruses, Stool for bile pigment Early referral of obstructive jaundice

Persistent neonatal jaundice Failure to conjugate : Unconjugated Haemolysis Genetic Hypothyroidism Conjugated hyperbilirubinaemia (20%) Liver disorder Bile obstruction

Conjugated Hyperbilirubinaemia Identifiable infections TORCHES, sepsis, UTI Metabolic conditions Galactosaemia, 1Antitrypsin def. Neonatal hepatitis syndrome Idiopathic neonatal cholestasis Intrahepatic biliary obstruction Extrahepatic biliary obstruction Biliary atresia, choledochus cyst

Hepatomegaly Inflammation Infection, Auto-immune, Toxic and drug reactions Reticulo-endothelial hyperplasia Septicaemia, HIV, granulomata Venous congestion CCF, Constrictive pericarditis, Hepatic vein/IVC obstruction

Hepatomegaly Infiltrations and neoplasia Extramedullary haemopoiesis Leukaemia, lymphoma, hepatoma Fat accumulation Malnutrition, toxic damage Storage disorders Glycogen, lipid, mucopolysaccharides

Hepatosplenomegaly Same cause for both organs to be enlarged Reticulo-endothelial hyperplasia, sepsis Spleen enlarged secondary to liver Portal hypertension Spleen enlargement more significant than liver Parasitic disease Haematological and RES disorders

Investigation of liver disease History Feeding Stooling Abdominal pain Previous illnesses of all relevant organ systems and progress Examination Growth and nutritional state Careful inspection, palpation, percussion Stool examination

Investigation II Ultrasonography Organ sizes and appearance Free fluid Masses Specialized investigations for specific indications

Abdominal distension Definition Abdominal wall on a higher plane than the xiphisternum in patients lying on their back on a straight firm surface Normal lordotic posture giving appearance of pot-belly when standing up

Abdominal Distension Fluid Gaseous distension Faeces retention Organ enlargement including bladder Inflammatory masses Tumours Pregnancy

Abdominal Distension Gut distensibility Omentum Food Air/gas Unabsorbed fluid contents Omentum Fat Lymph nodes Ascites fluid

Abdominal Distension Liver Span at different ages Normal size measurements Edge below the costal margin Span of dullness to percussion in midclavicular line Normal span varies with age Relatively bigger liver in young children relative to body size Span at different ages 4.5 – 5 cm at 1 week of age 7 – 8 cm at 12 years (boys) 6 – 6.5 cm at 12 years (girls)

Fluid in the abdomen Fluid in the bowel Ileus Gut disease with secretion/absorption abnormalities (May show shifting dullness, but not fluid thrill) Fluid in the peritoneal cavity (ascites)

Ascites Exudate High protein content usually > 30g/l Inflammatory cells Transudate Protein : serum protein ratio <0.5 Blood Chyle Milky fluid, lymphocytes

Ascites Lymphatic obstruction TB, congenital, neoplastic Raised intravascular hydrostatic pressure portal hypertension Decreased intravascular oncotic pressure hypoalbuminaemia Inflammation and increased permeability peritonitis

Abdominal Distension Organomegaly Masses and Tumours Liver Spleen Kidneys Bladder Masses and Tumours Kidney Adrenal Lymphoma Retroperitoneal