Corneal surface irregularities and episodic pain in a patient with mucolipidosis IV

Title

Corneal surface irregularities and episodic pain in a patient with mucolipidosis IV

Creator

Newman NJ; Starck T; Kenyon KR; Lessell S; Fish I; Kolodny EH

Publisher

Archives Of Ophthalmology

Date

1990

Subject

Child; Humans; Male; Preschool; Cataract Extraction; Conjunctiva/pathology; Cornea/pathology; Epithelium/pathology; Mucolipidoses/pathology; Pain/etiology; Q3 Literature Search; Tears/secretion

Description

Mucolipidosis IV is a lysosomal storage disease characterized by prominent involvement of the corneal epithelium. A 5-year-old boy with mucolipidosis IV experienced recurrent episodes of severe ocular pain, tearing, and ipsilateral facial flushing. This was suggestive of reflex sympathetic dystrophy, a syndrome of pain and sympathetic hyperactivity. The examination revealed marked corneal surface irregularities, corresponding to massive accumulations of intracytoplasmic storage material in the epithelium. Episodic pain in patients with mucolipidosis IV is an important symptom, presumably reflecting the distinctive corneal ultrastructural abnormality in this disease.
1990

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Type

Journal Article

Citation List Month

Backlog

Citation

Newman NJ; Starck T; Kenyon KR; Lessell S; Fish I; Kolodny EH, “Corneal surface irregularities and episodic pain in a patient with mucolipidosis IV,” Pediatric Palliative Care Library, accessed August 8, 2022, https://pedpalascnetlibrary.omeka.net/items/show/12559.

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