mortality/aging
|
• 50%-60% die sometime between 1 and 6 months of age
|
vision/eye
|
• vascularized and proliferative corneal lesion developed in the central region of the cornea
|
|
• neutrophils and macrophages
|
|
• occasional dying keratinocytes are present
|
|
• severe corneal epithelial thickening is observed, occurring abruptly at the junction of the central and peripheral
|
|
• at 25 days, the corneal epithelium is slightly thinner and keratinocytes are less orderly
|
liver/biliary system
|
• slowly progressive hepatic inflammation
• Inflammatory foci are characterized by an increasing number of interstitial and perivascular macrophages with
fewer neutrophils
|
|
• capsular fibrosis and inflammation is also seen
|
cardiovascular system
|
• vascularized and proliferative corneal lesion developed in the central region of the cornea
|
immune system
|
• neutrophils and macrophages
|
|
• slowly progressive hepatic inflammation
• Inflammatory foci are characterized by an increasing number of interstitial and perivascular macrophages with
fewer neutrophils
|


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