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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