Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030552 (paresis)
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Vogt-Koyanagi-Harada (VKH) disease is an autoimmune disease involving pigmented tissue in eyes, auditory system, skin, and central nervous system. The pathogenesis is a result of T cell lymphocyte reaction against melanocyte component, tyrosinase and tyrosinase-related protein. This disease uniquely affected pigmented race in Asia and native America, mostly women aged 20-50. We reported an 8-years-old boy complained for visual disturbance since 6 weeks prior admission. Two years earlier, the parents noted the patient eyes were looked red when photographed (suggesting a dilated pupil) preceded by whitened on the right forehead and nose and whitened hair, eyebrow and eyelashes. The examination showed a vitiligo and skin atrophy on right frontal and right nasal, poliosis on the eyebrows, eyelids and hair. The diameter of right pupil was 8 mm, with a paresis on 3rd, 4th, 6th, and 9th nerves. Fundus examination revealed sunset glow appearance. The visual acuity on the right eye: 2/60, left eye: 1-0. There's positive serology for antitoxoplasma IgG, anti Rubella IgG, anti CMV IgG. The patient was diagnosed with a VKH disease and then prescribed with methylprednisolone 1 mg/kg/day. The patient also had further follow up with dermatovenerologist and ophthalmologist. The eye examination revealed an improvement on right eye panuveitis, with a remaining keratic precipitate in the endothelium, and minimal flare and cell on the anterior chamber. ENT consultation revealed no ear inflammation or hearing disturbance.
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PMID:Vogt-Koyanagi-Harada disease in an 8-year-old boy. 2205 4