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


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

Approach to neonatal 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

    • 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 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

      • Liver

      • Spleen

      • Kidneys

      • Bladder

  • Masses and Tumours

    • Kidney

    • Adrenal

    • Liver

    • Lymphoma

    • Retroperitoneal


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