Paediatric liver conditions Present with combinations of the following symptoms and signs Jaundice Encephalopathy (altered consciousness or behaviour)

Paediatric liver conditionsPresent 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 hepatitisHepatic schistosomiasisVeno-occlusive diseaseCirrhosis

• 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 diseaseIntrauterine infectionPrematurity

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 jaundiceHaemolysis

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)

EvaluationExamination: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 infectionsTORCHES, 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

• HistoryFeeding StoolingAbdominal painPrevious illnesses of all relevant organ

systems and progress• Examination

Growth and nutritional stateCareful inspection, palpation, percussionStool 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• Food

• Air/gas

• Unabsorbed fluid contents

• Omentum• Fat

• Lymph nodes

• Ascites fluid

Abdominal Distension

• Liver • 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 bowelIleusGut 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• Liver• Spleen• Kidneys• Bladder

• Masses and Tumours• Kidney• Adrenal• Liver• Lymphoma• Retroperitoneal