This multicenter, prospective trial was conducted to develop an effective and safe reinduction regimen for marrow-relapsed pediatric acute lymphoblastic leukemia (ALL) by modifying the dose of idarubicin. Between 2006 and 2009, the trial accrued 44 patients, 1 to 21 years old with first marrow-relapsed ALL. The reinduction regimen comprised prednisolone, vincristine, L-asparaginase, and idarubicin (10 mg/m²/week). The idarubicin dose was adjusted according to the degree of myelosuppression. The second complete remission (CR2) rate was 72.7%, obtained by 54.2% of patients with early relapse < 24 months after initial diagnosis and 95.0% of those with late relapse (P = 0.002). Five patients entered remission with extended treatment, resulting in a final CR2 rate of 84.1%. The CR2 rate was not significantly different according to the idarubicin dose. The induction death rate was 2.3% (1/44). The 5-year event-free and overall survival rates were 22.2% ± 6.4% and 27.3% ± 6.7% for all patients, 4.2% ± 4.1% and 8.3% ± 5.6% for early relapsers, and 43.8% ± 11.4% and 50.0% ± 11.2% for late relapsers, respectively. Early relapse and slow response to reinduction chemotherapy were predictors of poor outcomes. In conclusion, a modified dose of idarubicin was effectively incorporated into the reinduction regimen for late marrow-relapsed ALL with a low toxic death rate. However, the CR2 rate for early relapsers was suboptimal, and the second remission was not durable in most patients.

The pathophysiology of glandular dysfunction in Sjögren's syndrome (SS) has not been fully elucidated. Previously, we reported the presence of autoantibodies to AQP-5 in patients with SS, which was associated with a low resting salivary flow. The purpose of this study was to investigate the presence of anti-AQP1 autoantibodies. To detect anti-AQP1 autoantibodies, cell-based indirect immunofluorescence assay was developed using MDCK cells that overexpressed human AQP1. By screening 112 SS and 52 control sera, anti-AQP1 autoantibodies were detected in 27.7% of the SS but in none of the control sera. Interestingly, the sera that were positive for anti-AQP1 autoantibodies also contained anti-AQP5 autoantibodies in the previous study. Different from anti-AQP5 autoantibodies, the presence of anti-AQP1 autoantibodies was not associated with the salivary flow rate. Although anti-AQP1 autoantibodies are not useful as a diagnostic marker, the presence of autoantibodies to AQP1 may be an obstacle to AQP1 gene therapy for SS.
Aquaporin 1 ; Autoantibodies* ; Fluorescent Antibody Technique ; Fluorescent Antibody Technique, Indirect ; Genetic Therapy ; Humans ; Madin Darby Canine Kidney Cells ; Mass Screening

BACKGROUND/AIMS: To determine the prevalence of autonomic dysfunction among Korean patients with primary Sjogren's syndrome (pSS) and its associations with the clinical features of pSS. METHODS: We analyzed 154 participants from the Korean Initiative of primary Sjogren's Syndrome (KISS) as a prospective pSS cohort and 154 age- and sex-matched healthy controls. A standardized 5-minute, supine, resting heart rate variability (HRV) test was performed, and autonomic dysfunction was defined as standard deviation of normal-to-normal RR intervals (SDNN) < 30 ms in patients < 50 years old and SDNN < 20 ms in patients ≥ 50 years old. The associations between autonomic dysfunction and various clinical features of pSS were analyzed. RESULTS: The overall autonomic activity in patients with pSS was significantly lower than that in controls. Autonomic dysfunction with the HRV test was observed in 35.7% of the KISS participants and was associated with a higher European League Against Rheumatism Sjogren's Syndrome Patient Reported Index fatigue score (p = 0.024). Raynaud's phenomenon was a more frequent clinical presentation in pSS patients with autonomic dysfunction than in those without autonomic dysfunction (29.4% and 14.4%, respectively; p = 0.048). Decreased parasympathetic activity was observed in 41.6% of pSS patients. No differences were found in the oral and ocular signs of pSS according to the decreased parasympathetic activity. CONCLUSIONS: In Korean patients with pSS, decreased and imbalanced autonomic activity is prevalent and is associated with fatigue. However, an association between autonomic dysfunction and glandular manifestations was not detected.
Autonomic Nervous System ; Cohort Studies* ; Fatigue ; Heart Rate ; Humans ; Prevalence ; Prospective Studies* ; Rheumatic Diseases ; Sjogren's Syndrome*

Pancreatic metastases from primary breast cancer are very rare. We report a case of pancreatic metastasis from invasive ductal carcinoma 13 years after the initial diagnosis of breast cancer. When the pancreatic mass was discovered, it was believed to be a primary pancreatic cancer due to the long interval from the initial diagnosis of breast cancer to metastasis. However, it was confirmed as metastatic breast cancer based on the pathology after surgical removal. Follow-up imaging has shown no recurrence.
Breast Neoplasms ; Breast* ; Carcinoma, Ductal* ; Diagnosis ; Follow-Up Studies ; Neoplasm Metastasis* ; Pancreatic Neoplasms ; Pathology ; Recurrence

“Clinical Practice Guideline for Stroke Rehabilitation in Korea 2016” is the 3rd edition of clinical practice guideline (CPG) for stroke rehabilitation in Korea, which updates the 2nd edition published in 2014. Forty-two specialists in stroke rehabilitation from 21 universities and 4 rehabilitation hospitals and 4 consultants participated in this update. The purpose of this CPG is to provide optimum practical guidelines for stroke rehabilitation teams to make a decision when they manage stroke patients and ultimately, to help stroke patients obtain maximal functional recovery and return to the society. The recent two CPGs from Canada (2015) and USA (2016) and articles that were published following the 2nd edition were used to develop this 3rd edition of CPG for stroke rehabilitation in Korea. The chosen articles' level of evidence and grade of recommendation were decided by the criteria of Scotland (2010) and the formal consensus was derived by the nominal group technique. The levels of evidence range from 1++ to 4 and the grades of recommendation range from A to D. Good Practice Point was recommended as best practice based on the clinical experience of the guideline developmental group. The draft of the developed CPG was reviewed by the experts group in the public hearings and then revised. “Clinical Practice Guideline for Stroke Rehabilitation in Korea 2016” consists of ‘Chapter 1; Introduction of Stroke Rehabilitation’, ‘Chapter 2; Rehabilitation for Stroke Syndrome, ‘Chapter 3; Rehabilitation for Returning to the Society’, and ‘Chapter 4; Advanced Technique for Stroke Rehabilitation’. “Clinical Practice Guideline for Stroke Rehabilitation in Korea 2016” will provide direction and standardization for acute, subacute and chronic stroke rehabilitation in Korea.
Canada ; Consensus ; Consultants ; Humans ; Korea* ; Practice Guidelines as Topic ; Rehabilitation* ; Scotland ; Specialization ; Stroke*

BACKGROUND/AIM: Adenocarcinoma arising from the ampulla of Vater is a rare disease and has limited data regarding outcome of palliative chemotherapy. We investigated the efficacy and safety of capecitabine plus oxaliplatin (XELOX) in patients with advanced ampullary adenocarcinoma. METHODS: From October 2006 to January 2014, we retrospectively analyzed 28 patients with advanced ampullary adenocarcinoma treated by XELOX regimen at single institution. All the patients had histologically confirmed stage IV or recurrent ampullary adenocarcinoma. XELOX was administered in outpatient clinic every 3 weeks according to the following protocol: oral administration of capecitabine 750 mg/m² twice a day on days 1-14 and intravenous injection of oxaliplatin 130 mg/m² on day 1. RESULTS: With follow-up of median 24.6 months (range 4.0–78.0 months), median progression-free survival (PFS) was 4.8 months (range 0.7–26.1 months), and median overall survival (OS) was 11.9 months (range 2.0–36.0 months). One patient (4%) achieved complete response and 5 patients (18%) showed partial response. There were no significant differences for PFS and OS according to response by chemotherapy. The most common grade 3 adverse events in patients were nausea and vomiting (10.7%). There was no treatment-related mortality. CONCLUSIONS: XELOX regimen is well tolerated and show moderate activity against advanced ampullary adenocarcinoma.
Adenocarcinoma* ; Administration, Oral ; Ambulatory Care Facilities ; Ampulla of Vater* ; Antineoplastic Agents ; Capecitabine* ; Disease-Free Survival ; Drug Therapy ; Follow-Up Studies ; Humans ; Injections, Intravenous ; Mortality ; Nausea ; Rare Diseases ; Retrospective Studies ; Vomiting

PURPOSE: Eribulin mesilate was approved for the treatment of patients with locally advanced or metastatic breast cancer (MBC), who had received at least two chemotherapeutic regimens, including anthracycline and taxane. On the other hand, the efficacy and safety information of eribulin in Korean patients is limited by the lack of clinical trials. MATERIALS AND METHODS: In this multicenter, open-label, single-arm, phase IV study, locally advanced or MBC patients were enrolled between June 2013 and April 2014 from 14 centers in Korea. One point four mg/m2 dose of eribulin was administered on days 1 and 8 of every 21 days. The primary endpoint was the frequency and intensity of the treatment emergent adverse event. The secondary endpoint was the disease control rate, which included the rate of complete responses, partial responses, and stable disease. RESULTS: A total of 101 patients received at least one dose of eribulin and were included in the safety set. The patients received a total of 543 treatment cycles, with a median of three cycles (range, 1 to 31 cycles). The most common adverse event was neutropenia (91.1% of patients, 48.3% of cycles). The frequent non-hematological adverse events included alopecia, decrease in appetite, fatigue/asthenia, and myalgia/arthralgia. The peripheral neuropathy of any grade occurred in 27 patients (26.7%), including grade 3 in two patients. Disease control rate was 52.7% and 51.3% of patients in the full analysis set and per-protocol set, respectively. CONCLUSION: This study demonstrated the feasible safety profile and activity of eribulin in Korean patients with MBC.
Alopecia ; Appetite ; Breast Neoplasms* ; Breast* ; Clinical Study* ; Hand ; Humans ; Korea ; Mesylates* ; Neoplasm Metastasis ; Neutropenia ; Peripheral Nervous System Diseases

BACKGROUND: This study was conducted to analyze the distribution of the psychosocial well-being index among adults living in two rural communities in Korea and to examine its correlation with lifestyle variables such as sleep duration, regular exercise, and sedentary time. METHODS: Using the cohort data of the Atherosclerosis Risk of a Rural Area Korean General Population, this study examined 3631 participants living in Wonju and Pyeongchang in Gangwon Province; their preliminary data were established from 2005 to 2007 while their follow-up data were collected 3 years later. This study investigated demographic characteristics, lifestyle habits, disease history, Psychosocial Well-being Index-Short Form (PWI-SF) scores, sleep duration, regular exercise, and sedentary time during work. Using repeated measures ANOVA, this study examined how the variables and PWI-SF scores changed over the course of 3 years and identified the correlation between them based on mixed model analysis. Afterwards, using the generalized estimation equation, this study identified each variable's risk towards the PWI-SF high-risk group and performed a stratified analysis by occupation after dividing the participants into farmers and non-farmers. RESULTS: The PWI-SF high-risk group was found to be 18.9% of the participants from preliminary data and 15.5% from follow-up data. The odds ratio towards the PWI-SF high-risk group was 1.503 (95% CI 1.241–1.821) in the short sleep duration group and 1.327 (95% CI 1.136–1.550) in the non-regular exercise group. A stratified analysis by occupation showed that middle and long sedentary time in the white-collar group increased the risk toward the PWI-SF high-risk group. CONCLUSIONS: Short sleep duration, no regular exercise, and long sedentary time in the white-collar group were identified as risk factors toward the PWI-SF high-risk group in the rural communities, and policy interventions are needed to address this issue.
Adult* ; Atherosclerosis ; Cohort Studies ; Farmers ; Follow-Up Studies ; Gangwon-do ; Humans ; Korea ; Life Style ; Occupations ; Odds Ratio ; Risk Factors ; Rural Population

OBJECTIVE: Autonomic symptoms are commonly observed in patients with Parkinson's disease (PD) and often limit the activities of daily living. The Scale for Outcomes in Parkinson's disease-Autonomic (SCOPA-AUT) was developed to evaluate and quantify autonomic symptoms in PD. The goal of this study was to translate the original SCOPA-AUT, which was written in English, into Korean and to evaluate its reliability and validity for Korean PD patients. METHODS: For the translation, the following processes were performed: forward translation, backward translation, expert review, pretest of the pre-final version and development of the final Korean version of SCOPA-AUT (K-SCOPA-AUT). In total, 127 patients with PD from 31 movement disorder clinics of university-affiliated hospitals in Korea were enrolled in this study. All patients were assessed using the K-SCOPA-AUT and other motor, non-motor, and quality of life scores. Test-retest reliability for the K-SCOPA-AUT was assessed over a time interval of 10−14 days. RESULTS: The internal consistency and reliability of the K-SCOPA-AUT was 0.727 as measured by the mean Cronbach's α-coefficient. The test-retest correlation reliability was 0.859 by the Guttman split-half coefficient. The total K-SCOPA-AUT score showed a positive correlation with other non-motor symptoms [the Korean version of non-motor symptom scale (K-NMSS)], activities of daily living (Unified Parkinson's Disease Rating Scale part II) and quality of life [the Korean version of Parkinson's Disease Quality of Life 39 (K-PDQ39)]. CONCLUSION: The K-SCOPA-AUT had good reliability and validity for the assessment of autonomic dysfunction in Korean PD patients. Autonomic symptom severities were associated with many other motor and non-motor impairments and influenced quality of life.
Activities of Daily Living ; Humans ; Korea ; Movement Disorders ; Parkinson Disease ; Quality of Life ; Reproducibility of Results

With recent advances in molecular diagnostic methods and targeted cancer therapies, several molecular tests have been recommended for gastric cancer (GC) and colorectal cancer (CRC). Microsatellite instability analysis of gastrointestinal cancers is performed to screen for Lynch syndrome, predict favorable prognosis, and screen patients for immunotherapy. The epidermal growth factor receptor (EGFR) tyrosine kinase inhibitor has been approved in metastatic CRCs with wildtype RAS (KRAS and NRAS exon 2–4). A BRAF mutation is required for predicting poor prognosis. Additionally, amplification of human epidermal growth factor receptor 2 (HER2) and MET is also associated with resistance to EGFR inhibitor in metastatic CRC patients. The BRAF V600E mutation is found in sporadic microsatellite unstable CRCs, and thus is helpful for ruling out Lynch syndrome. In addition, the KRAS mutation is a prognostic biomarker and the PIK3CA mutation is a molecular biomarker predicting response to phosphoinositide 3-kinase/AKT/mammalian target of rapamycin inhibitors and response to aspirin therapy in CRC patients. Additionally, HER2 testing should be performed in all recurrent or metastatic GCs. If the results of HER2 immunohistochemistry are equivocal, HER2 silver or fluorescence in situ hybridization testing are essential for confirmative determination of HER2 status. Epstein-Barr virus–positive GCs have distinct characteristics, including heavy lymphoid stroma, hypermethylation phenotype, and high expression of immune modulators. Recent advances in next-generation sequencing technologies enable us to examine various genetic alterations using a single test. Pathologists play a crucial role in ensuring reliable molecular testing and they should also take an integral role between molecular laboratories and clinicians.