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71.
Sung Yoon Cho Rimm Huh Mi Sun Chang Jieun Lee Younghee Kwun Se Hyun Maeng Su Jin Kim Young Bae Sohn Sung Won Park Eun-Kyung Kwon Sun Ju Han Jooyoun Jung Dong-Kyu Jin 《Journal of Korean medical science》2014,29(2):254-260
Hunter syndrome (or mucopolysaccharidosis type II [MPS II]) arises because of a deficiency in the lysosomal enzyme iduronate-2-sulfatase. Short stature is a prominent and consistent feature in MPS II. Enzyme replacement therapy (ERT) with idursulfase (Elaprase®) or idursulfase beta (Hunterase®) have been developed for these patients. The effect of ERT on the growth of Korean patients with Hunter syndrome was evaluated at a single center. This study comprised 32 patients, who had received ERT for at least 2 yr; they were divided into three groups according to their ages at the start of ERT: group 1 (<6 yr, n=14), group 2 (6-10 yr, n=11), and group 3 (10-20 yr, n=7). The patients showed marked growth retardation as they got older. ERT may have less effect on the growth of patients with the severe form of Hunter syndrome. The height z-scores in groups 2 and 3 revealed a significant change (the estimated slopes before and after the treatment were -0.047 and -0.007, respectively: difference in the slope, 0.04; P<0.001). Growth in response to ERT could be an important treatment outcome or an endpoint for future studies.
Graphical Abstract
相似文献72.
73.
Jung Inn Sohn Woong-Ki Hong Su Seok Choi Harry J. Coles Mark E. Welland Seung Nam Cha Jong Min Kim 《Materials》2014,7(3):2044-2061
Diverse functionalities of liquid crystals (LCs) offer enormous opportunities for their potential use in advanced mobile and smart displays, as well as novel non-display applications. Here, we present snapshots of the research carried out on emerging applications of LCs ranging from electronics to holography and self-powered systems. In addition, we will show our recent results focused on the development of new LC applications, such as programmable transistors, a transparent and active-type two-dimensional optical array and self-powered display systems based on LCs, and will briefly discuss their novel concepts and basic operating principles. Our research will give insights not only into comprehensively understanding technical and scientific applications of LCs, but also developing new discoveries of other LC-based devices. 相似文献
74.
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76.
Song CS Park DI Yoon MY Seok HS Park JH Kim HJ Cho YK Sohn CI Jeon WK Kim BI 《Digestive diseases and sciences》2012,57(4):1033-1038
Background
Recent studies have suggested that a higher red blood cell distribution width (RDW) is associated with disease activity in patients with inflammatory bowel disease (IBD). However, the RDW in IBD patients without anemia has not been investigated. 相似文献77.
Klebsiella is an opportunistic pathogen that is known to cause septicemia, pneumonia, urinary tract infections, hepatobiliary tract infections, and soft tissue infections in patients who have severe underlying diseases or are under immunosuppression. Most Klebsiella species found are Klebsiella pneumoniae, and Klebsiella oxytoca is rarely cultured in humans. We report a case of a 48-year-old man presenting with a soft fluctuating mass on his forehead. The lesion was an abscess, and percutaneous drainage yielded pus from which K. oxytoca was isolated. Parenteral levofloxacin was administrated leading to resolution of infection signs. Because of the rarity of the pathogen, evaluation for underlying illnesses was done, and the patient was diagnosed with type 2 diabetes mellitus. This is the first report of a patient with previously undiagnosed diabetes who was found with an extraorbital abscess caused by K. oxytoca, which we present with a review of diagnosis, pathogenesis, and treatment. 相似文献
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79.
Lee JI Oh SJ Ha WC Kwon HS Sohn TS Son HS Cha BY 《Diabetes research and clinical practice》2012,95(1):42-47
Aim
To evaluate the association between serum 25-hydroxyvitamin D [25(OH)D] and arterial stiffness in patients with type 2 diabetes.Methods
Serum 25(OH)D was measured in a cross-sectional sample of 131 men and 174 women aged 30 years and over in Korea. Arterial stiffness was assessed by pulse wave velocity (PWV) obtained with a VP-2000 pulse wave unit. Fasting plasma glucose, insulin, lipid profile, HbA1c, calcium, phosphorous, and HS-CRP were measured.Results
The prevalence of vitamin D deficiency was high (85.9%). Those with lower vitamin D levels had increased PWV. Using multivariate regression analysis, low 25(OH)D concentrations independently predicted PWV (p < 0.001) in people with type 2 diabetes after adjustment for other risk factors such as age, smoking, hypertension, HS-CRP, diabetes duration, hypertension duration, HbA1c, and BMI.Conclusions
Vitamin D deficiency is common in type 2 diabetes, and a low 25(OH)D level is significantly associated with increased arterial stiffness in these patients. Vitamin D may influence the development of cardiovascular disease. Clinical intervention studies are needed to clarify whether treatment with vitamin D decreases the risk of cardiovascular disease in patients with type 2 diabetes. 相似文献80.