Chih-kuang Chuang

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Previous studies on the incidence of the various types of mucopolysaccharidoses (MPS) in different populations have shown considerable variation. However, information regarding the incidence of MPS in the Asian population is lacking. An epidemiological study of the MPS disorders in Taiwan using multiple ascertainment sources was undertaken, and incidences(More)
To evaluate the prevalence of obstructive sleep apnea (OSA) and to clarify sleep characteristics in patients with mucopolysaccharidoses (MPS), we performed overnight polysomnographic studies in 24 patients (22 males and 2 females; 3 with MPS I, 15 with MPS II, 1 with MPS III, 1 with MPS IV, and 4 with MPS VI; mean age, 10.8 ± 6.0 years; age range, 2.0-23.7(More)
BACKGROUND The identification of acid mucopolysaccharide by the liquid chromatography/tandem mass spectrometry method (LC-MS/MS) of the predominant disaccharide units of glycosaminoglycans (GAGs) (chondroitin sulfate, CS; dermatan sulfate, DS; heparan sulfate, HS) after methanolysis is validated and applicable for mucopolysaccharidosis (MPS) type(More)
Indoxyl sulfate and p-cresylsulfate was associated with poor clinical outcome of uremia. We explored the relationship between the two toxins and renal function in chronic kidney disease (CKD) patients. This study enrolled 103 stable CKD patients (stage 3-5 and hemodialysis (HD) patients). Serum levels of indoxyl sulfate and p-cresylsulfate were measured(More)
BACKGROUND Patients with mucopolysaccharidoses (MPS) are associated with poor bone growth and mineralization, however, information regarding the assessment of bone mineral density (BMD) in relation to age and treatment in this disorder is limited. METHODS Dual energy x-ray absorptiometry (DXA) was performed in 30 patients with MPS (21 males and 9 females;(More)
OBJECTIVE p-Cresyl sulfate (PCS) and indoxyl sulfate (IS) have been reported to predict poor clinical outcomes in hemodialysis (HD) patients. However, little is known about the effect of the two toxins on peripheral arterial disease (PAD) and vascular access dysfunction. Our objective was to explore the association between the two toxins and PAD and(More)
INTRODUCTION Previous studies have shown that serum p-cresyl sulfate (PCS) and indoxyl sulfate (IS) were significantly related to clinical outcomes in patients on hemodialysis (HD). However, evidence for the relationship in elderly HD patients remains scarce. We explore whether the two toxins can predict clinical outcomes in elderly HD patients. MATERIAL(More)
BACKGROUND Hunter syndrome (mucopolysaccharidosis type II) is an X-linked recessive lysosomal storage disease caused by a defect of the iduronate-2-sulfatase (IDS) gene. The result is impaired IDS enzyme function. METHODS To characterize the biochemical and molecular defects in IDS-deficient patients and their families, we measured IDS enzyme activity by(More)
AIM Several different genetic defects have been found to result in the characteristic phenotypic expression of Prader-Willi syndrome (PWS). METHODS We performed a retrospective analysis of 67 cases of molecularly confirmed PWS diagnosed from January 1980 through July 2006 in five medical centres in Taiwan. Clinical manifestations were compared between(More)
AIM To compare the effects of i.v. iron sucrose and Fe chloride on the iron indices of haemodialysis patients with anaemia. METHODS One hundred and eight haemodialysis patients receiving recombinant human erythropoiesis-stimulating agent (ESA) (mean age 59.37 years) were enrolled and randomly assigned to an iron sucrose or an Fe chloride group. Iron(More)