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KERNICTERUS (CLINICAL PRESENTATION (BIND (ACUTE (3 Stages: STAGE 1 (First…
KERNICTERUS
CLINICAL PRESENTATION
BIND
ACUTE
3 Stages: STAGE 1 (First Few days) --> ALOC, Hypotonia, Poor Feeding.
STAGE 2 (Variable) --> Hypertonia of extensor muscles, Retrocollis, Opisthtotonus. Progression to long-term neurological deficits.
STAGE 3 (>1wk) --> Hypotonia
CHRONIC
Evolve slowly over the first several years of life.
First 12 Months: Hypotonia, Hyperreflexia, Delayed Motor Development
12 Months: Extrapyramidal (Athetosis, Chorea), Visual (Ocular Movement deficits) and Auditory deficits (most consistent feature, damage to Cochlear Nuclei or Auditory nerve)
Other: Cognitive function relatively spared. Dental enamel hypoplasia, potential green-stained teeth
AETIOLOGY 1
INCREASED PRODUCTION
HAEMOLYSIS
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-
RBC Membrane Defects (Spherocytosis, Elliptocytosis)
Enzyme Defects (G6PD, Pyruvate Kinase Deficiency)
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EXTRAVASATED BLOOD
Cephalohaematoma, Subgaleal Haemorrhage, Various Ecchymoses. Internal Bleeding (Pulmonary, Intraventricular)
OTHER
ENZYME INDUCTION (HO-1, any physiologic stressor)
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-
AETIOLOGY 2
DECREASED ELIMINATION
ALBUMIN BINDING (Hypoalbuminaemia, Displaced By drugs i.e Ceftriaxone or Sulfisoxazole)
HEPATIC UPTAKE & CONJUGATION (UDPGT Defects --> 1. Physiological Jaundice 2. Gilbert Syndrome 3. Criggler-Najjar Type 1 & 2)
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OTHER/SYSTEMIC: Hypothyroidism, Galactosaemia
Ix: BLOODS: FBC, Total and Direct Bilirubin, Blood Type (Maternal & Foetal), Retic count, Direct Coombs Test.
LP if Neurological Sx or Sepsis suspected
IMAGING: Head Ultrasonography to rule out Haemorrhage or Parenchymal Abnormalities. CT of little value, MRI may show intensity signals in Globus Pallidus.
OTHER: Brainstem auditory evoked response (BAER) to assess hearing
Rx: See Local guidelines. Usually trial of phototherapy --> Exchange Transfusion. Various drugs available (L-Aspatric Acid inhibits B-Glucuronidase, Sn-Mesoporphyrin inhibits HO-1)