ABSTRACT
PURPOSE: To describe the clinical characteristics and treatment outcomes of Vogt-Koyanagi-Harada (VKH) disease over a 12-year period. METHODS: A retrospective chart review was used to identify VKH patients, from January 1999 to December 2011. RESULTS: In total, 154 patients (308 eyes) were diagnosed with VKH. The mean age at diagnosis was 33.8 ± 13 years. Mean baseline best-corrected visual acuity (BCVA) was 20/125; (0.8 ± 0.6 logMAR). Recurrent episodes occurred in 107 (54%) patients. Oral prednisone was administered in all patients. At the last visit, the mean BCVA was 20/50 (0.4 ± 0.7 logMAR). The most common complications post-treatment were: glaucoma [104 (33.8%) eyes] and cataract [84 (27.2%) eyes]. CONCLUSIONS: In Saudi Arabia, VKH-related uveitis is predominant in young females. Bilateral panuveitis is the most common ocular manifestation and near 50% of eyes present with exudative retinal detachment. Oral prednisone was the primary treatment and the majority of eyes maintained 20/50 or better vision.
Subject(s)
Uveomeningoencephalitic Syndrome/diagnosis , Acute Disease , Administration, Oral , Adolescent , Adult , Aged , Antibiotics, Antitubercular , Child , Chronic Disease , Drug Therapy, Combination , Female , Fluorescein Angiography , Glucocorticoids/therapeutic use , Humans , Male , Middle Aged , Mycophenolic Acid/therapeutic use , Prednisolone/therapeutic use , Recurrence , Retrospective Studies , Tertiary Care Centers , Tomography, Optical Coherence , Treatment Outcome , Uveomeningoencephalitic Syndrome/drug therapy , Visual Acuity/physiologyABSTRACT
Carbonic anhydrase II (CAII) deficiency is an autosomal recessive disorder manifest by osteopetrosis, renal tubular acidosis, and cerebral calcification. Other features include growth failure and mental retardation. Complications of the osteopetrosis include frequent bone fractures, cranial nerve compression, and dental mal-occlusion. A hyper-chloremic metabolic acidosis, sometimes with hypokalemia, occurs due to renal tubular acidosis that may be proximal, distal, or more commonly, the combined type. Such patients may present with global hypotonia, muscle weakness or paralysis. We report a case of CA II deficiency with recurrent attacks of acute paralysis which was misdiagnosed initially as Guillian-Barre syndrome.
