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Kernicterus - Pediatric - Lecture Slides, Slides of Pediatrics

Complete lecture series on Pediatrics is available on docity. You can read online and download it for free. This lecture keywords are: Kernicterus, Pathophysiology, Bilirubin Conjugation, Newborn Hyperbilirubinemia, Exaggerated Hyperbilirubinemia, Erythroblastosis Fetalis, Bilirubin Encephalopathy, Diagnosis, Hyperbilirubinemia, Incidence and Prognosis

Typology: Slides

2012/2013

Uploaded on 10/01/2013

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Download Kernicterus - Pediatric - Lecture Slides and more Slides Pediatrics in PDF only on Docsity!

Kernicterus

History

  • Earliest work on jaundice from Baumes-

1785, and Hervieux-

  • Kernicterus was first described by Johannes

Orth, 1875

  • He postulated that jaundice might have hematologic origins
  • He noted that the brain in jaundiced adults wasn’t affected
  • Christian Schmorl coined the term in 1904
  • Translated, Kernicterus means jaundice of the ―kern‖ or nuclear region of the brain

Bilirubin Conjugation

Pathophysiology

Newborn Hyperbilirubinemia

  • Relatively high hematocrit; more cells to

break down

  • UDP-Glucuronyl Transferase is not fully

functional until 3-4 months of life

  • Relative starvation state and slow transit

time, especially in breastfeeders

  • Breastmilk contains beta-glucuronidase;

enterohepatic circulation is increased

Pathophysiology

  • UCB is lipophilic and crosses the Blood-Brain Barrier
    • In vitro, free UCB will not precipitate out of solution unless in the presence of a polar lipid membrane
    • In theory, only free UCB crosses, albumin-bound does not.
    • BBB of infants is more permeable than adults, and acidosis causes it to be even more permeable.
  • UCB has an affinity for the basal ganglia, hippocampus, cranial nerve nuclei - Mechanism is widely studied, but still unknown
  • UCB interrupts metabolism in glial cells and causes apoptosis of neurons - Exact mechanisms are unknown, but definitely separate pathways. - Age of the cell is inversely proportional to susceptibility docsity.com

MRI of an infant who suffered from

severe Erythroblastosis Fetalis

Clinical Manifestations:

Bilirubin Encephalopathy

  • Chronic Encephalopathy: (Perlstein’s Tetrad: Extrapyramidal Abnormalities, Hearing Loss, Gaze abnormality, and Dental Dysplasia)
  • Extrapyramidal abnormalities: Facial grimacing, drooling, dysarthria, and athetosis--may develop by 18mo or delayed to 8or9 years.
  • Hearing loss is usually due to injury of the cochlear nuclei in the brainstem. It may be the only manifestation
  • Gaze abnormalities: Limitation of upward gaze, palsies
  • Cerebral cortex is relatively spared, so intelligence is often close to normal.

Diagnosis

  • Kernicterus is a pathologic diagnosis, not

clinical. Post-mortem exam of the brain is

the definitive diagnosis

  • Clinically, kernicterus is suspected based on

the history of hyperbilirubinemia and the

clinical manifestations as mentioned above.

  • The degree of hyperbilirubinemia does not correlate well with the development or severity of kernicterus.

Prevention: Treatment of

Hyperbilirubinemia

  • Phototherapy
    • Initiate based on UCB level and baby’s age
    • Isomerizes UCB to Lumirubin, soluble in water and excreted via the kidney
  • Exchange transfusion
    • Initiate if phototherapy fails, repeat as needed
    • Incidence of kernicterus has dropped since the advent
  • Sn-Mesoporphyrin
    • Inhibits Heme-oxygenase, which is the rate-limiting enzyme in heme catabolism.
    • Only case reports thus far, where exchange transfusion was refused

Treatment

  • Treatment of clinically suspected

kernicterus is centered around early

intervention from multiple disciplines

  • Neurodevelopmental Pediatrics
  • Neurology
  • Physical and Occupational Therapy
  • Audiology
  • Ophthalmology
  • Speech Therapy
  • School, County, EFMP

Follow Up: Spasticity/Dystonia

  • Botox, Baclofen for severe posturing
  • Physical therapy for training of muscles and

teaching stretching techniques

  • Occupational Therapy for independence and

activities of daily living

  • Speech Therapy
  • Equipment—Appropriate chairs, braces, etc

Follow Up: Gaze Abnormality

  • Early involvement of ophthalmology
    • Strabismus surgery as needed
    • Correction/patching as needed

Follow Up: Cognitive Delays

  • Cerebral cortex is usually relatively spared,

but cognitive delays may be present

  • Neurodevelopmental Pediatrician to continually reassess for these delays
  • Early diagnosis of learning disabilities
  • Early intervention, special schools, IEPs as appropriate