Marfan syndrome in an Iranian family: a case series

Marfan syndrome [MFS] is a genetic disorder which is inherited by autosomal dominant traits. In MFS, lens displacement and cardiovascular involvement are important causes of morbidity and mortality in the clinical course of the disease. In this case study, the ocular involvement in a family with severe penetration of MFS is reported. Twelve members of a family [father, two daughters, three sons, and six grandchildren] had MFS. Lens ectopia was the most common ophthalmic involvement among the family [100%]. Other ocular involvements were as follows; Hypoplastic iris or ciliary's muscle hypoplasia [50%], on gated eyeball [42%], flat cornea [30%], glaucoma and cataract [25%], retinal detachment [16%]. Three members of the family underwent eye surgery including lens extraction, glaucoma surgery and retinal surgery

Correlation between serum lipids and age-related macular degeneration: a case-control study

Age-related macular degeneration [AMD] is one of the leading causes of visual loss among people aged 65 yr and older. The pathophysiology of AMD is poorly understood. The purpose of this study was to evaluate the relationship between serum lipid concentrations and age-related macular degeneration. In this case-control study, total cholesterol [TCH], low density lipoprotein [LDL], high density lipoprotein [HDL] and triglyceride [TG] of 32 patients with AMD were compared with 32 subjects without AMD that were matched for age. Data were analyzed using Independent t and Chi-Square Tests. TCH, LDL, and TG serum concentrations were significantly higher in AMD patients compared with control group [P<0.001, P<0.001 and P<0.017 respectively]. There was no difference in HDL concentration between two groups [P=0.781]. High level of total cholesterol, TG and LDL were associated with increased risk of AMD. Results of this study suggest further research to better understand the underlying biological mechanisms of AMD related to serum cholesterol

Cardiovascular manifestation of a family with Marfan's syndrome

Marfan's syndrome [MFS] is a genetic disorder associated with autosomal dominant inheritance. In MSF, the most prevalent cause of death is cardiovascular involvement. Here we introduce a family with a severe penetration of MFS. Eleven members of this family have MFS [father, two daughters, three sons, and five grandchildren]. The most common cardiac involvement in our patients was mitral valve prolapse as manifested by mirtal regurgitation. At ten years' follow-up, two patients had aortic root dilatation running the risk of aortic dissection; they were, therefore, scheduled for cardiac surgery, during which the Bental procedure was successfully performed. Fortunately, all of the members of this family are currently alive

Clinical indications of penetrating keratoplasty: an epidemiological study in teaching hospitals of birjand

The arm of tfif's study was to determine indications of keratoplasty during 7 years /1999-vrv Emam-Rveza and Vali-AvSc teaching hospitals of Birjand University of medical sciences. Medical records of all patients who underwent penetrating keratoplasty [PK] in teaching hospitals of Birjand University of Medical Sciences from 1999 to 2006 were investigated retrospectively. The recorded data covered sex, age, indication, job and place of life of the patient. This data were analyzed regarding statistical significance was determined using X[2] analysis. During a 7-year period, total number of 120 patients were operated. The most common indication for PK was corneal opacity [62%], followed by keratoconus 20% and other 18% [Bolus keratopathy plus corneal dystrophia]. The major job group for keratoplasty was farmer and the keratoplasty in patients that live in village [56%] was more than the city [44%]. Corneal opacity was the leading indication for PK. The major cause of corneal scarring was trauma with Thorn barberry and corneal infection in our study