One thousand one hundred and sixty-nine (1169) patients were examined in the Eye Clinic of University of Malaya Medical Centre over a period of three weeks to determine the prevalence of eye diseases and visual impairment. Age, gender, race, visual acuity and diagnosis of patients were noted from the case records. Cataract (385, 32.9%) was the most common eye disease seen in our study followed by glaucoma (274, 23.4%). Refractive errors were seen in 126 (10.8%) while diabetic retinopathy was noted in 113 (9.7%) patients. One hundred and fifteen (9.6%) patients had visual impairment and 11 (0.9%) had blindness in our study according to WHO classification of visual impairment. Refractive errors are the most common causes of visual impairment in children, while cataract, glaucoma and diabetic retinopathy account for visual impairment in elderly people. All these eye diseases are treatable and the severe eye conditions may be potentially preventable with early diagnosis.
Study site: Eye Clinic, University Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia
Introduction: Denys-Drash Syndrome (DDS) consists of a triad of pseudohermaphroditism, Wilms'tumor and nephropathy. This condition may manifest as a complete triad or in an incomplete form; with either one or a combination of the above features. The characteristic glomerular abnormality in DDS is diffuse mesangial sclerosis (DMS).Case report: We report two cases of DDS with focal membranoproliferative glomerulonephritis (MPGN). Both of our cases were males with ambiguous genitalia. They had a similar heterozygous germline mutation in exon 9 of WT1, c.1180C>T, p.R394W; a known mutation hotspot for DDS. Case 1 had nephropathy at the age of 4 years and Case 2 at 2.5 years with different rates of progression to end-stage renal failure. Conclusion: Our findings, in combination with other reports, illustrate the clinicopathological heterogeneity of DDS. There are no universal recommendations for optimal management of patients with DDS due to the inability to accurately predict affected individuals' progress.