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Paediatric liver conditions Present with combinations of the following symptoms and signs

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.

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Paediatric liver conditions Present with combinations of the following symptoms and signs

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  1. 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

  2. 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

  3. 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

  4. 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

  5. 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

  6. 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

  7. 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 Approach to neonatal jaundice

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

  9. 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

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

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

  12. 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

  13. 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

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

  15. 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

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

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

  18. 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)

  19. 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)

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

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

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

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