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    Abdullah Shiddiq
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    This document discusses various types of encephalopathy seen in pediatric patients, including hepatic encephalopathy, kernicterus, and Reye's syndrome. It provides details on the causes, clinical presentations, diagnostic criteria, management strategies, and outcomes of each condition. Key factors in management include controlling intracranial pressure, water and electrolyte balance, oxygenation, hypoglycemia, coagulopathy, and interventions like exchange transfusion or liver transplantation.

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    This document discusses various types of encephalopathy seen in pediatric patients, including hepatic encephalopathy, kernicterus, and Reye's syndrome. It provides details on the causes, clinical presentations, diagnostic criteria, management strategies, and outcomes of each condition. Key factors in management include controlling intracranial pressure, water and electrolyte balance, oxygenation, hypoglycemia, coagulopathy, and interventions like exchange transfusion or liver transplantation.

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    © All Rights Reserved
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    42 views18 pages

    10 Encephalopathy

    Uploaded by

    Abdullah Shiddiq
    This document discusses various types of encephalopathy seen in pediatric patients, including hepatic encephalopathy, kernicterus, and Reye's syndrome. It provides details on the causes, clinical presentations, diagnostic criteria, management strategies, and outcomes of each condition. Key factors in management include controlling intracranial pressure, water and electrolyte balance, oxygenation, hypoglycemia, coagulopathy, and interventions like exchange transfusion or liver transplantation.

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    of 18

    Atan Baas Sinuhaji

    Sub Division of Pediatrics Gastroentero-Hepatology


    Department of Childhealth,School of Medicine
    University of Sumatera Utara/ Adam Malik Hospital
    Medan
    ENCEPHALOPATHY

    DISORDER OF CEREBRAL
    FUNCTION ==> Inflammation (-)

    DIGESTIVE TRACT

    1. Hepatic Encephalopathy
    2. Kernicterus
    3. Reye Syndrome
    4. Dehydration +disorder of consciousness
    5. Hypo/hypernatremia
    severe hypophosphatemia
    GE + SEVERE DEHYDRATION

    CIRCULATORY DISTURBANCES

    OXYGENATION

    CONSCIOUSNESS

    GE+SEVERE DEHYDRATION+ENCEPHALOPATHY

    COMA ≠ SHOCK
    P

    < 1 mg %

    Energy 

    Dysfunction of RBC Rhabdomyolisis


    leucocytes &
    thrombocytes
    O2 delivery  

    Cerebral Anoxia
    KERNICTERUS
    = BILIRUBIN ENCEPHALOPATHY
    • Unconsciousness
    • Spasme
    • Convulsion
    • Mortality  75%
    • Recovery - Blind
    - Deafness
    - Neuromusc. Incoord.
    (chorea athetosis)
    Th 1. EXCHANGE TRANSFUSION ==> UNCONJUG.
    BILIRUBIN > 20 mg %
    2. PHOTOTHERAPY

    3. PHENOBARBITAL

    4. INHIBITION OF ABSORPTION

    5. TIN PROTOPORFIRIN

    INHIBITION OF BILIVERDIN TO BILIRUBIN

    6. SUPPORTIVE
    HEPATIC ENCEPHALOPATHY

    = LIVER FAILURE
    = HEPATOCELLULER FAILURE
    = HEPATOCYTIC FAILURE
    = “GAGAL HATI”
    ≠ HEPATIC COMA
    ≠ PORTO SYSTEMIC ENCEPH
    LIVER FAILURE

    SEVERE HEPATIC DYSFUNCTION

    PT ≥ 15 SECONDS or INR ≥ 1,5 + ENCEPHALOPATHY


    OR
    PT ≥ 20 SECONDS OR INR ≥ 2 WITHOUT ENCEPHALOPATHY
    ACUTE
    ( WITHIN 8 WEEKS OF ONSET OF ILLNES/JAUNDICE

    FULMINANT SUBFULMINANT
    ( 2 WEEKS) ( 2-8 WEEKS )
    CLINICAL GRADING OF HEPATIC ENCEPHALOPATHY

    I. CONFUSED

    II. DROWSY

    III. STUPOROUS

    IV. - COMA
    HEPATIC COMA
    - DEEP COMA
    Systemic

    Inferior v. cava

    liver

    portal v.

    Porto systemic encephelopathy


    ETIOLOGIES

    1.Infection

    2.Drugs and toxin

    3.Autoimmune

    4.Metabolic

    5.Vascular

    6.Miscellanous : -Malignant infiltration

    7.Indeterminate :-Graft non-function


    LIVER FAILURE

    ENCEPHALOPATHY DYSFUNCTION
    INTRAHEPATIC
    COAGULOPATHY METABOLISM

    “HALLMARK”
    MANAGEMENT

    1. DIAGNOSTIC

    2. SUPPORTIVE

    3. UNDERLYNG : - Antiviral for HSV and HBV


    -Steroid for AIH
    - NTBC for Tyrosinemia
    - NAC for acetaminophen overdose
    - etc
    4. LIVER TRANSPLANTATION

    # NTBC: 2(nitro-4 -trifluoromethylbenzoil )1-3- cyclohexanedione


    # NAC : N-Acetylcysteine
    SUPPORTIVE
    1. VENTILATION
    2. WATER & ELECTROLYTES
    3. COAGULOPATHY:
    - Vitamin K
    - Transfusion : Fresh Frozen Plasma
    4. BLOOD AMMONIA (NH3)  :
    - lactulose
    - neomycine  Nephrotoxic
    5. REDUCING INTRACRANIAL PRESSURE :
    - cerebral edem : steroid, mannitol, etc.
    - hypokarbia  Cerebral Blood Flow 
    6. MONITORED CLOSELY FOR INFECTION
    7. HEPATOTOXIC DRUGS WITHDRAWN :
    - eg : Sedative
    The King’s College Criteria for Liver Transplantation

    Acetaminophen Non-acetaminophen

    @ pH < 7,3(irrespective of grade @ PT> 100 seconds (INR>6,5)


    encephalopathy) or all 3 of the (irrespective of grade encephalopathy)
    following or any 3 of the following
    = Grade III- IV encephalopathy = Age <10 or > 40 years
    = PT> 100 seconds ( INR> 6,5) = Etiology: non-A,non-B hepatitis,
    = Serum creatinine > 300µmol/L halothane,
    ( 3,4 mg/dL) idiosyncratic drug reaction,
    Wilson’s disease
    = Periode of jaundice to encephalopathy
    > 7 days
    = PT>50 seconds (INR>3,5 )
    = Serum bilirubin> 300 µmol/L(17,5 mg/dL)
    ENCEPHALOPATHY

    FATTY DEGENERATION
    REYE SYNDROME

    BLOOD NH3

    Mitochondrial dysfunction

    HYPOGLYCEMIA

    ACUTE

    SALICYLIC ACID
    Th 1. CONTROL OF INTRACRANIAL PRESSURE 

    2. WATER & ELECTROLYTES

    3. ADEQUATE OXYGENATION

    4. CONTROL OF HYPOGLICEMIA

    5. COAGULOPATHY IS MANAGED

    6. EXCHANGE TRANSFUSION

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