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Clinical and molecular characterization of Korean children with infantile and late-onset Pompe disease: 10 years of experience with enzyme replacement therapy at a single center

Kim MS, Song A, Im M, Huh J, Kang IS, Song J, Yang A, Kim J, Kwon EK, Choi EJ, Han SJ, Park HD, Cho SY, Jin DK

PURPOSE: Pompe disease (PD) is an autosomal recessive disorder caused by a deficiency of acid alpha-glucosidase resulting from pathogenic GAA variants. This study describes the clinical features, genotypes, changes before...
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Relationship of Nurses' Perception of the Joint Commission International Hospital Accreditation, Nursing Performance, Self-concept and Retention Intention

Kwon EK, Lee EJ

PURPOSE: This study was done to identify the relationships of perception of JCI (Joint Commission International) hospital accreditation, nursing performance, self-concept and retention intention in nurses. METHODS: Self-administered questionnaires were used...
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Factors Influencing Use of Medical Alert Identification in Diabetic Patients Using Insulin

Kwon EK, Lee CK, Sim KH

PURPOSE: The aim of this study was to identify perception, use status and factors influencing use of medical alert identification (ID) in diabetic patients who are on insulin. METHODS: A survey...
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Impact of Enzyme Replacement Therapy on Linear Growth in Korean Patients with Mucopolysaccharidosis Type II (Hunter Syndrome)

Cho SY, Huh R, Chang MS, Lee J, Kwun Y, Maeng SH, Kim SJ, Sohn YB, Park SW, Kwon EK, Han SJ, Jung J, Jin DK

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...
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Extrinsic compression of left iliac vein does not predict the development of post thrombotic syndrome in left side deep venous thrombosis

Park SJ, Park HJ, Kwon EK, Kim SJ, Cho HR

  • KMID: 2324837
  • J Korean Surg Soc.
  • 2012 Jun;82(6):370-373.
PURPOSE: Left side deep venous thrombosis (DVT) is associated with May-Thurner's anatomical variation and is often instigated by invasive treatment. The aim of this study is to analyze the influence...
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A study of the relationship between clinical phenotypes and plasma iduronate-2-sulfatase enzyme activities in Hunter syndrome patients

Lee OJ, Kim SJ, Sohn YB, Park HD, Lee SY, Kim CH, Ko AR, Yook YJ, Lee SJ, Park SW, Kim SH, Cho SY, Kwon EK, Han SJ, Jin DK

PURPOSE: Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a rare lysosomal storage disorder caused by iduronate-2-sulfatase (IDS) deficiency. MPS II causes a wide phenotypic spectrum of symptoms ranging...
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Impact of Distance from the Right Iliac Artery to the Lumbar Vertebra in Left Side Deep Venous Thrombosis

Kim SJ, Park SJ, Kwon EK, Park HJ, Rho SY, Hwang JC, Cho HR

PURPOSE: The aim of this study is to evaluate how much influence iliac vein compression exerts on the clinical feature of deep venous thrombosis (DVT). METHODS: One hundred five cases of...
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Apoptosis and Expression of AQP5 and TGF-beta in the Irradiated Rat Submandibular Gland

Choi JH, Wu HG, Jung KC, Lee SH, Kwon EK

  • KMID: 2128576
  • Cancer Res Treat.
  • 2009 Sep;41(3):145-154.
PURPOSE: To evaluate the effect of X-ray irradiation on apoptosis and change of expression of aquaporin 5 (AQP5) and transforming growth factor-beta(TGF-beta) in the rat submandibular gland (SMG). MATERIALS AND...
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The Determinants of Job Satisfaction of Nurses: Focused on Work Rewards

Yom YH, Kwon SB, Lee YY, Kwon EK, Ko JW

PURPOSE: The purpose of this study was to investigate the determinants of job satisfaction of hospital nurses. The focus was on work rewards. A causal model of job satisfaction of...
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The Usefulness of the Computed Tomography for Diagnosing DeepVenous Thrombosis of the Lower Extremities

Yi JH, Park SJ, Kwon EK, Oh YJ, Kang TW, Hwang JC, Kang BS, Choi SH, Cho HR

  • KMID: 2096863
  • J Korean Soc Vasc Surg.
  • 2009 May;25(1):12-16.
PURPOSE: This study aims to access the usefulness of indirect computed tomographic venography (CTV) after performing computed tomographic pulmonary angiography (CTPA) to detect deep venous thrombosis. METHODS: Eighty six patients who...
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Effects and adverse-effects of growth hormone therapy in children with Prader-Willi syndrome: A two year study

Kim SJ, Cho JB, Kwak MJ, Kwon EK, Paik KH, Jin DK

PURPOSE: The objective of this study was to evaluate the effects and adverse side-effects of growth hormone (GH) therapy in children with Prader-Willi syndrome (PWS). METHODS: Forty-one patients who had been...
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Peptide YY, Cholecystokinin, Insulin and Ghrelin Response to Meal did not Change, but Mean Serum Levels of Insulin is Reduced in Children with Prader-Willi Syndrome

Paik KH, Jin DK, Lee KH, Armstrong , Lee JE, Oh YJ, Kim S, Kwon EK, Choe YH

Prader-Willi syndrome (PWS) is a contiguous gene syndrome characterized by uncontrollable eating or hyperphagia. Several studies have confirmed that plasma ghrelin levels are markedly elevated in PWS adults and children....
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Marked Suppression of Ghrelin Concentration by Insulin in Prader-Willi Syndrome

Paik KH, Lee MK, Jin DK, Kang HW, Lee KH, Kim AH, Kim C, Lee JE, Oh YJ, Kim S, Han SJ, Kwon EK, Choe YH

The plasma ghrelin has been reported to be elevated in Prader-Willi syndrome (PWS) and modulated by insulin. It was hypothesized that insulin might have a more pronounced effect on reducing...
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Effect of Flavopiridol on Radiation-induced Apoptosis of Human Laryngeal and Lung Cancer Cells

Kim S, Kwon EK, Lee SH, Park HJ, Wu HG

  • KMID: 2198499
  • J Korean Soc Ther Radiol Oncol.
  • 2007 Dec;25(4):227-232.
PURPOSE: To investigate the flavopiridol effect on radiation-induced apoptosis and expression of apoptosis- related genes of human laryngeal and lung cancer cells. MATERIALS AND METHODS: A human laryngeal cancer cell line,...
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A Study on Subjective Perception of Their Disease by Adolescents with IDDM

Choi MH, Kwon EK, Kim SK

  • KMID: 2291990
  • J Korean Acad Child Health Nurs.
  • 2006 Jul;12(3):322-332.
Purpose: This study was done to identify types of subjective perception of their disease by adolescents who have insulin dependent diabetes mellitus (IDDM) in order to better understand these adolescents...
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Clinical and Laboratory Features of Korean Mucopolysaccharidoses (MPSs)

Sohn WY, Lee JH, Paik KH, Kwon EK, Kim AH, Jin DK

  • KMID: 2279075
  • Korean J Pediatr.
  • 2005 Oct;48(10):1132-1138.
PURPOSE: The mucopolysaccharidoses (MPSs) are a heterogeneous group of lysosomal storage disorders. They are caused by a deficiency of the enzymes involved in the degradation of glycosaminoglycans. Early recognition is...
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Clinical Characteristics and Genetic Analysis of Prader-Willi Syndrome

Lee JE, Moon KB, Hwang JH, Kwon EK, Kim SH, Kim JW, Jin DK

  • KMID: 2207178
  • J Korean Pediatr Soc.
  • 2002 Sep;45(9):1126-1133.
PURPOSE: Prader-Willi syndrome(PWS) is a complex disorder affecting multisystems with characteristic clinical features. Its genetic basis is an expression defect in the paternally derived chromosome 15q11-q13. We analyzed the clinical...
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Detection of black-pigmented bacteria in infected root canals

Kwon EK, Kim ES, Kwak JS, Lee H, Lee SJ, Im MK

Black-pigmented bacteria have been implicated in the endodontic infections. This group of microorganisms includes Porphyromonas endodontalis, Porphyromonas gingivalis, Prevotella intermedia, and Prevotella nigrescens. The organisms display a wide variety of...
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Clinical Evaluation of Hypoglycemic Symptoms Reported by Children with Insulin-dependent Diabetes Mellitus and by Their Parents

Lee BE, Park HY, Cheon CM, Kwon EK, Song SM, Kim JS, Jin DK

  • KMID: 2335532
  • J Korean Pediatr Soc.
  • 2001 Apr;44(4):450-455.
PURPOSE: The following study has been carried out to find the symptoms of hypoglycemia and the symptoms of transient focal neurologic deficit, which were complained by insulin dependent diabetes mellitus...
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