[Fabry disease: a case report]

Fabry disease is a X-linked lysosomal storage disorder due to alpha galactoisidase A deficiency leading to abnormal accumulation of glycosphingolipids in different parts of body. This case report introduces a 35-year-old man with diffuse keratotic erythematous papules. Histopathological evaluation of the skin biopsy suggested the diagnosis of angiokeratoma. With attention to his nephropathy and the same skin lesions and renal involvement in his brother, which led him to receive renal transplantation, the diagnosis of Fabry disease was suggested

Prevalence of metabolic syndrome in a hemodialysis population

The role of metabolic syndrome [MS] in hemodialysis population has not been thoroughly studied. This study aimed to determine the prevalence of MS and to identify its correlates among hemodialysis patients. This cross-sectional study was conducted on patients in a hemodialysis center. The MS was defined according the Adult Treatment Panel III criteria. Clinical data of the patients were collected and blood samples were studied to measure fasting blood glucose and lipid profile. Eighty hemodialysis patients, including 47 men [58.8%] and 33 women [41.2%] with a mean age of 55.6 +/- 15.6 years, were enrolled in this study. Metabolic syndrome was diagnosed in 23 patients [28.7%]. Hypertension was present in 55 patients [68.8%]. Fifteen patients [18.8%] were diabetic, 24 [30.0%]] had a high serum triglyceride, 22 [27.5%] had a low high-density lipoprotein cholesterol, and 20 [25.0%] had evidence of abdominal obesity. Patients with MS had significantly higher body mass indexes [P < .001], fasting blood glucose levels [P < .001], and triglyceride levels [P = .004]. Metabolic syndrome was not associated with gender, age, and duration of hemodialysis. Men showed significant abnormality in glucose metabolism [P = .008]. Prevalence of low high-density lipoprotein cholesterol was significantly higher in the women than in the men [P = .02]. The prevalence of MS in our hemodialysis patients was relatively high, with the most common element being hypertension. We suggest that there needs to be a new set of criteria defined for MS in hemodialysis patients