BACKGROUND/AIMS: This study compared the efficacy, compliance, and safety of bowel preparation between sodium picosulfate with magnesium citrate (SPMC) and oral sulfate solution (OSS). METHODS: A prospective randomized multicenter study was performed. Split preparation methods were performed in both groups; the SPMC group, 2 sachets on the day before, and 1 sachet on the day of the procedure, the OSS group, half of the OSS with 1 L of water on both the day before and the day of the procedure. The adenoma detection rate (ADR), adequacy of bowel preparation using the Boston Bowel Preparation Scale (BBPS) score, patient satisfaction on a visual analog scale (VAS), and safety were compared between the 2 groups. RESULTS: This study analyzed 229 patients (121 in the SPMC group and 108 in the OSS group). ADR showed no differences between 2 groups (51.7% vs. 41.7%, P>0.05). The mean total BBPS score (7.95 vs. 8.11, P>0.05) and adequate bowel preparation rate (94.9% vs. 96.3%, P>0.05) were similar between the 2 groups. The mean VAS score for taste (7.62 vs. 6.87, P=0.006) was significantly higher in the SPMC group than in the OSS group. There were no significant differences in any other safety variables between the 2 groups except nausea symptom (36.1% vs. 20.3%, P=0.008). CONCLUSIONS: Bowel preparation for colonoscopy using low volume OSS and SPMC yielded similar ADRs and levels of efficacy. SPMC had higher levels of satisfaction for taste and feeling than did OSS.
Adenoma ; Citric Acid ; Colonoscopy ; Compliance ; Humans ; Magnesium ; Nausea ; Patient Satisfaction ; Prospective Studies ; Sodium ; Visual Analog Scale ; Water

PURPOSE: Clinical implications of single patient classifier (SPC) and microsatellite instability (MSI) in stage II/III gastric cancer have been reported. We investigated SPC and the status of MSI and Epstein-Barr virus (EBV) as combinatory biomarkers to predict the prognosis and responsiveness of adjuvant chemotherapy for stage II/III gastric cancer. MATERIALS AND METHODS: Tumor specimens and clinical information were collected from patients enrolled in CLASSIC trial, a randomized controlled study of capecitabine plus oxaliplatin-based adjuvant chemotherapy. The results of nine-gene based SPC assay were classified as prognostication (SPC-prognosis) and prediction of chemotherapy benefit (SPC-prediction). Five quasimonomorphic mononucleotide markers were used to assess tumor MSI status. EBV-encoded small RNA in situ hybridization was performed to define EBV status. RESULTS: There were positive associations among SPC, MSI, and EBV statuses among 586 patients. In multivariate analysis of disease-free survival, SPC-prognosis [hazard ratio (HR): 1.879 (1.101–3.205), 2.399 (1.415–4.067), p=0.003] and MSI status (HR: 0.363, 95% confidence interval: 0.161–0.820, p=0.015) were independent prognostic factors along with age, Lauren classification, TNM stage, and chemotherapy. Patient survival of SPC-prognosis was well stratified regardless of EBV status and in microsatellite stable (MSS) group, but not in MSI-high group. Significant survival benefit from adjuvant chemotherapy was observed by SPC-Prediction in MSS and EBV-negative gastric cancer. CONCLUSION: SPC, MSI, and EBV statuses could be used in combination to predict the prognosis and responsiveness of adjuvant chemotherapy for stage II/III gastric cancer.
Biomarkers ; Capecitabine ; Chemotherapy, Adjuvant ; Classification ; Disease-Free Survival ; Drug Therapy ; Herpesvirus 4, Human ; Humans ; In Situ Hybridization ; Microsatellite Instability ; Microsatellite Repeats ; Multivariate Analysis ; Prognosis ; RNA ; Stomach Neoplasms

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 and after enzyme replacement therapy (ERT), and long-term outcomes in patients with infantile-onset PD (IOPD) and late-onset PD (LOPD) at a tertiary medical center. METHODS: The medical records of 5 Korean patients (2 male, 3 female patients) diagnosed with PD between 2002 and 2013 at Samsung Medical Center in Seoul, Republic of Korea were retrospectively reviewed for data, including clinical and genetic characteristics at diagnosis and clinical course after ERT. RESULTS: Common initial symptoms included hypotonia, cyanosis, and tachycardia in patients with IOPD and limb girdle weakness in patients with LOPD. Electrocardiography at diagnosis revealed hypertrophic cardiomyopathy in all patients with IOPD who showed a stable disease course during a median follow-up period of 10 years. Patients with LOPD showed improved hepatomegaly and liver transaminase level after ERT. CONCLUSION: As ERT is effective for treatment of PD, early identification of this disease is very important. Thus, patients with IOPD should be considered candidates for clinical trials of new drugs in the future.
alpha-Glucosidases ; Cardiomyopathy, Hypertrophic ; Child ; Cyanosis ; Diagnosis ; Electrocardiography ; Enzyme Replacement Therapy ; Extremities ; Female ; Follow-Up Studies ; Genotype ; Glycogen Storage Disease Type II ; Hepatomegaly ; Humans ; Liver ; Male ; Medical Records ; Muscle Hypotonia ; Republic of Korea ; Retrospective Studies ; Seoul ; Tachycardia

PURPOSE: Fibroblast growth factor 21 (FGF21) is a crucial metabolic regulator, with multiple favorable effects on glucose homeostasis and lipid metabolism. Since serum FGF21 level has been implicated as a potential marker for the early identification of metabolic syndrome (MetS), we investigated the association between serum FGF21 level and the development of MetS in a population-based prospective study. MATERIALS AND METHODS: We conducted a prospective study of 221 randomly sampled adults without MetS from a general population-based cohort study who were examined from 2005–2008 (baseline) and from 2008–2011 (follow-up). Baseline serum FGF21 levels were analyzed using enzyme-linked immunosorbent assay. RESULTS: During the average 2.8-year follow-up period, 82 participants (36.6%) developed new-onset MetS. Serum FGF21 levels were significantly higher in patients with new-onset MetS than in those without MetS (209.56±226.80 vs. 110.09±81.10, p < 0.01). In multivariate adjusted models, the odds for MetS development were greater in patients with serum FGF21 levels in the highest quartile, compared to those in the lowest quartile (3.84, 95% confidence interval: 1.59–9.28). CONCLUSION: Serum FGF21 level was an independent predictor for new-onset MetS in a population-based prospective study.
Biomarkers/blood ; Female ; Fibroblast Growth Factors/*blood ; Humans ; Male ; Metabolic Syndrome/*blood ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Prospective Studies

BACKGROUND AND PURPOSE: The primary mechanism underlying paramedian pontine infarction (PPI) is atheroma obliterating the perforators. Here, we encountered a patient with PPI in the post-stenotic area of basilar artery (BA) without a plaque, shown by high-resolution magnetic resonance imaging (HR-MRI). We performed an experiment using a 3D-printed BA model and a particle image velocimetry (PIV) to explore the hemodynamic property of the post-stenotic area and the mechanism of PPI. METHODS: 3D-model of a BA stenosis was reconstructed with silicone compound using a 3D-printer based on the source image of HR-MRI. Working fluid seeded with fluorescence particles was used and the velocity of those particles was measured horizontally and vertically. Furthermore, microtubules were inserted into the posterior aspect of the model to measure the flow rates of perforators (pre-and post-stenotic areas). The flow rates were compared between the microtubules. RESULTS: A recirculating flow was observed from the post-stenotic area in both directions forming a spiral shape. The velocity of the flow in these regions of recirculation was about one-tenth that of the flow in other regions. The location of recirculating flow well corresponded with the area with low-signal intensity at the time-of-flight magnetic resonance angiography and the location of PPI. Finally, the flow rate through the microtubule inserted into the post-stenotic area was significantly decreased comparing to others (P<0.001). CONCLUSIONS: Perforator infarction may be caused by a hemodynamic mechanism altered by stenosis that induces a recirculation flow. 3D-printed modeling and PIV are helpful understanding the hemodynamics of intracranial stenosis.
Basilar Artery ; Constriction, Pathologic ; Fluorescence ; Hemodynamics* ; Humans ; Infarction* ; Magnetic Resonance Angiography ; Magnetic Resonance Imaging ; Microtubules ; Plaque, Atherosclerotic ; Rheology ; Silicon ; Silicones

BACKGROUND AND PURPOSE: The primary mechanism underlying paramedian pontine infarction (PPI) is atheroma obliterating the perforators. Here, we encountered a patient with PPI in the post-stenotic area of basilar artery (BA) without a plaque, shown by high-resolution magnetic resonance imaging (HR-MRI). We performed an experiment using a 3D-printed BA model and a particle image velocimetry (PIV) to explore the hemodynamic property of the post-stenotic area and the mechanism of PPI. METHODS: 3D-model of a BA stenosis was reconstructed with silicone compound using a 3D-printer based on the source image of HR-MRI. Working fluid seeded with fluorescence particles was used and the velocity of those particles was measured horizontally and vertically. Furthermore, microtubules were inserted into the posterior aspect of the model to measure the flow rates of perforators (pre-and post-stenotic areas). The flow rates were compared between the microtubules. RESULTS: A recirculating flow was observed from the post-stenotic area in both directions forming a spiral shape. The velocity of the flow in these regions of recirculation was about one-tenth that of the flow in other regions. The location of recirculating flow well corresponded with the area with low-signal intensity at the time-of-flight magnetic resonance angiography and the location of PPI. Finally, the flow rate through the microtubule inserted into the post-stenotic area was significantly decreased comparing to others (P<0.001). CONCLUSIONS: Perforator infarction may be caused by a hemodynamic mechanism altered by stenosis that induces a recirculation flow. 3D-printed modeling and PIV are helpful understanding the hemodynamics of intracranial stenosis.
Basilar Artery ; Constriction, Pathologic ; Fluorescence ; Hemodynamics* ; Humans ; Infarction* ; Magnetic Resonance Angiography ; Magnetic Resonance Imaging ; Microtubules ; Plaque, Atherosclerotic ; Rheology ; Silicon ; Silicones

PURPOSE: Although the possibility of distal radius fractures is strong in hemodialysis patients, there are many difficult problems such as the bleeding tendency, hypervascularity and injury to arteriovenous fistula. We studied the outcomes of open reduction and volar locking plate fixation of the distal radius fractures in hemodyalisis patients with ipsilateral arteriovenous fistula. METHODS: From 2007 to 2009, a retrospective chart review was performed of eleven hemodialysis patients who underwent volar locking plate fixation for treatment of distal radius fractures. Eight of them were female and three were male and mean age was 68 years (range, 57-81 years). Mean follow-up period was 19 months (range, 12-28 months). All patients had osteoporosis with mean T-score of -2.7. All operations were performed on the next day of hemodialysis. Scheduled hemodialysis was possible on the next day of surgery without splint. We analyzed radiographic results, the wrist range of motion, Mayo wrist score and disabilities of the arm, shoulder and hand (DASH) score at the last follow-up. RESULTS: All fractures achieved anatomical reduction and united at final follow-up. Complications such as hematoma or occlusion of arteriovenous fistula were not observed. Mean Mayo wrist score was 78 and mean DASH score was 22. CONCLUSION: Volar locking plate fixation for distal radius fractures provides immediate support for continuing hemodialysis and exercise. Open reduction of the fractures and the use of tourniquet don't seem to increase the vascular complications like hematoma and fistula occlusion.
Arm ; Arteriovenous Fistula ; Female ; Fistula ; Follow-Up Studies ; Hand ; Hematoma ; Hemorrhage ; Humans ; Male ; Osteoporosis ; Radius Fractures* ; Radius* ; Range of Motion, Articular ; Renal Dialysis* ; Retrospective Studies ; Shoulder ; Splints ; Tourniquets ; Wrist

Mucopolysaccharidosis (MPS) III has 4 enzymatically distinct forms (A, B, C, and D), and MPS IIIC, also known as Sanfilippo C syndrome, is an autosomal recessive lysosomal storage disease caused by a deficiency of heparan acetyl-CoA:alpha-glucosaminide N-acetyltransferase (HGSNAT). Here, we report a case of MPS IIIC that was confirmed by molecular genetic analysis. The patient was a 2-yr-old girl presenting with skeletal deformity, hepatomegaly, and delayed motor development. Urinary excretion of glycosaminoglycan (GAG) was markedly elevated (984.4 mg GAG/g creatinine) compared with the age-specific reference range (<175 mg GAG/g creatinine), and a strong band of heparan sulfate was recognized on performing thin layer chromatography. HGSNAT enzyme activity in leukocytes was 0.7 nmol/17 hr/mg protein, which was significantly lower than the reference range (8.6-32 nmol/17 hr/mg protein). PCR and direct sequencing of the HGSNAT gene showed 2 mutations: c.234+1G>A (IVS2+1G>A) and c.1150C>T (p.Arg384*). To the best of our knowledge, this is the first case of MPS IIIC to be confirmed by clinical, biochemical, and molecular genetic findings in Korea.
Acetyltransferases/*genetics ; Asian Continental Ancestry Group/*genetics ; Base Sequence ; Child, Preschool ; Chromatography, Thin Layer ; Female ; Glycosaminoglycans/urine ; Heparitin Sulfate/chemistry/metabolism ; Humans ; Leukocytes/immunology/metabolism ; Mucopolysaccharidosis III/*diagnosis/genetics/radiography ; Mutation ; Republic of Korea ; Sequence Analysis, DNA

Posttransplant lymphoproliferative disorder (PTLD) is documented as one of the serious complications leading to mortality particularly in organ transplant recipients receiving immunosuppressive therapy. Extant literature confirms beyond doubt that the most common site of involvement of PTLD is lymph nodes, and rarely involved is the gastrointestinal tract. It is a well-known fact that Epstein-Barr virus (EBV) is a risk factor for PTLD development. In this study, we report a case of PTLD presented as small bowel perforation without EBV infection after long-term immunosuppressive therapy in a renal transplant recipient.
Epstein-Barr Virus Infections ; Gastrointestinal Tract ; Herpesvirus 4, Human ; Kidney Transplantation ; Lymph Nodes ; Lymphoproliferative Disorders ; Risk Factors ; Transplants

BACKGROUND: The occurrence of malignancy following kidney transplantation has been estimated three to five times the incidence compared to that of the general population. It is estimated that particularly in renal cell carcinoma (RCC), the relative risk increases. The aim of this study was to analyze the characteristics, risk factors, and prognosis of RCC following kidney transplantation. METHODS: Total number of 3,272 kidney recipients who underwent transplantation from April 1979 to December 2012 and patients who had RCC following kidney transplantation were retrospectively reviewed and analyzed. RESULTS: We found that among 232 cases of posttransplant malignancies, 25 recipients were diagnosed with RCC. We have observed in our study that it took an average of 175.2+/-71.0 months to develop RCC after their first kidney transplantation. However, with longer follow up period, interval incidence of RCC increased. Fourteen patients (56%) were diagnosed with RCC 15 years after transplantation. We also found that with reference to the risk factor analysis for posttransplant RCC, the long-term follow-up period was the only independent risk factor. In our study, 21 patients with RCC were treated with radical nephrectomy. Of them, 16 patients survived, and four RCC-related deaths occurred. Furthermore, the patient survival rate of RCC recipients was lower than that of the nonmalignancy group despite the graft survival rate were not different. CONCLUSIONS: We conclude that the incidence of RCC increased in a time-dependent manner following kidney transplantation. Therefore, we strongly recommend the procedure of regular-interval screening for the patients who are on compulsive long-term immunosuppression.
Carcinoma, Renal Cell ; Follow-Up Studies ; Graft Survival ; Humans ; Immunosuppression ; Incidence ; Kidney ; Kidney Transplantation ; Mass Screening ; Nephrectomy ; Prognosis ; Retrospective Studies ; Risk Factors ; Survival Rate ; Transplants