Identification of a novel HLA-B allele, HLA-B*54:47, in a Korean individual.

B*54:47 allele differs from B*54:01:01:01 in codon 74 in exon 2.

Nutritional Status and Dietary Management According to Hemodialysis Duration.

As the incidence of chronic diseases such as diabetes and hypertension increases, complications such as decreased renal function are also increasing in many patients. Nutritional management in hemodialysis patients is a very important factor for prognosis and quality of life. The purpose of this study was to investigate the differences in nutritional status and dietary management according to hemodialysis duration. A total of 145 patients were divided into 4 groups according to hemodialysis duration: less 1 year (D1), 1-5 years (D2), and above 5 years (D3). The rates of protein-energy wasting were 31.1% in D1 group, 49.5% in D2 group, and 47.6% in D3 group. However, there was no significant difference between the 3 groups. Nutrient intake analysis showed that protein, iron, and vitamin C were significantly lower in the D3 group than in the D1 group. Protein intake in all 3 groups was insufficient compared to the recommended dietary amount for dialysis patients. The most difficult aspect in dietary management was cooking with low sodium. In the D3 group, which had the longest duration of dialysis, the practice of diet therapy and self-perceived need for nutrition education was lowest. Observations of nutritional status are necessary to maintain the health status of dialysis patients. In addition, education plans should be prepared to mediate the nutrient intakes and identify the patient's difficulties and provide practical help.

Fabry disease with aortic regurgitation.

Aortic regurgitation is not so rare in patients with Fabry disease. Enzyme replacement therapy has become the standard medical care for Fabry disease in recent years. A 31-year-old man with Fabry disease, treated with recombinant alpha-galactosidase enzyme replacement for 19 months was admitted for evaluation of exertional dyspnea. Cardiac workup revealed left ventricular hypertrophy, increased left ventricular size, and moderate to severe aortic regurgitation. He underwent mechanical valvular replacement and heart biopsy. Histology of his aortic valve showed myxoid degeneration of valve leaflets. His heart muscle revealed extensive hypertrophy with vacuolization and the absence of lamellar bodies. We report a case of Fabry disease with aortic regurgitation in a man who underwent valvular replacement operation during enzyme replacement therapy.

The role of mesenchymal stem cells in the functional improvement of chronic renal failure.

The most commonly used therapeutic targets in nephrology are the reduction of injury, the delay of progression, or renal replacement therapy. Many animal and human studies demonstrated the role of stem cells in repair and regenerations of kidney. Mesenchymal stem cells (MSCs) have shown to improve outcome of acute renal injury models. It is controversial whether MSCs can reduce injury following a toxic/ischemic event and delay renal failure in chronic kidney disease. We evaluated the hypothesis that the treatment with MSCs could improve renal function and attenuate injury in chronic renal failure (CRF). Sprague-Dawley female rats (8 weeks old, 182.2 +/- 7.2 g) underwent modified 5/6 nephrectomy. Rats in the MSC group received an injection of MSCs (1 x 10(6) cells) via tail vein 1 day after nephrectomy. Blood and urine samples were collected after 7 days and every month thereafter. The kidneys of rats were removed for histologic evaluation after 24-h urine collection and blood sampling. The Y-chromosome stain using fluorescent in situ hybridization was performed to verify the presence of male MSCs in the kidney of female recipients. No significant differences in blood urea nitrogen and creatinine concentration were observed between the MSC group and the untreated CRF group. However, the weight gain in the MSC group was greater than those in the CRF group after 4 months. Proteinuria in the MSC group was less than that in the CRF group over time. Y chromosome was detected in the kidney of MSC group. Although no significances were observed between these two groups, the histologic analysis suggests that MSCs have positive effect against glomerulosclerosis. These results suggest that MSCs help preserve renal function and attenuate renal injury in CRF.