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Pseudodendritic keratitis and systemic tyrosinemia.

K H Charlton, P S Binder, L Wozniak

    Ophthalmology
    |April 1, 1981
    PubMed
    Summary
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    Systemic tyrosinemia can mimic herpes simplex keratitis in children. Early diagnosis and a low-tyrosine diet are crucial for treating this metabolic disorder and its ocular manifestations.

    Area of Science:

    • Ophthalmology
    • Metabolic Disorders
    • Genetics

    Background:

    • Systemic tyrosinemia is a rare inherited metabolic disorder.
    • Herpes simplex keratitis is a common cause of corneal inflammation in children.

    Observation:

    • Three children with systemic tyrosinemia presented with symptoms mimicking herpes simplex keratitis.
    • Two patients received extensive treatment for presumed herpes simplex keratitis before diagnosis.
    • All patients exhibited pseudodendritic keratitis and hyperkeratotic skin lesions.

    Findings:

    • All three children responded positively to a low-tyrosine diet.
    • The corneal findings were consistent with Richner-Hanhart syndrome, a subtype of tyrosinemia.
    • Unlike previous reports, none of the affected children displayed mental retardation.

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    Implications:

    • Ophthalmologists should consider tyrosinemia in children with bilateral dendritiform keratitis.
    • Measuring serum tyrosine levels is essential for diagnosing this condition.
    • Timely diagnosis and dietary management can prevent severe ocular complications.