Idiopathic Neonatal Hepatitis
Grace E. Kim, MD
Key Facts
Terminology
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Convenient term for clinical condition manifested by prolonged jaundice in neonates with variable but definable histologic picture
Microscopic Pathology
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Predominately lobular histological changes
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Syncytial giant cell hepatocytes with extramedullary hematopoiesis
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Canalicular cholestasis and scattered to absent necrotic hepatocytes
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Top Differential Diagnoses
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Biliary atresia is very important to exclude because early surgical intervention is required
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If neonatal hepatitis-like pattern of injury, use clinical information and molecular tests to exclude known disorders
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INH is diagnosis of exclusion
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The characteristic features of INH include lobular giant cell transformation and extramedullary hematopoiesis. Ductular reaction is absent, and hepatocyte necrosis may not be apparent. |
INH is primarily a lobular process with hepatocytes showing giant cell change and necrosis  along with cholestasis  . |
TERMINOLOGY
Abbreviations
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Idiopathic neonatal hepatitis (INH)
Definitions
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General term for clinical condition manifested by prolonged jaundice in neonates with variable but definable histologic picture
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Clinicopathologic picture is termed neonatal hepatitis syndrome
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Uniform clinical presentation but broad spectrum of causative disease processes
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INH is a diagnosis of exclusion
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ETIOLOGY/PATHOGENESIS
Unknown
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Many different insults linked to this pattern of injury; if known etiologies are excluded, then idiopathic
CLINICAL ISSUES
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