Purpose: Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor of the kidney (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea. Materials and Methods: From January 2001 to December 2015, data of pediatric patients (0–18 years) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed. Results: Among 439 patients (male, 240), the most common tumor was WT (n=342, 77.9%), followed by RCC (n=36, 8.2%), CCSK (n=24, 5.5%), MRTK (n=16, 3.6%), CMN (n=12, 2.7%), and others (n=9, 2.1%). Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration was 88.5 months (range 0-211.6). Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, progressive disease, second malignant neoplasm, and treatment-related mortality. Five-year overall survival and event free survival were 97.2% and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively (p < 0.001). Conclusion The pediatric renal tumor types in Korea are similar to those previously reported in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumors included a variety of tumors and showed inferior outcome, especially MRTK. Further efforts are necessary to optimize the treatment and analyze the genetic characteristics of pediatric renal tumors in Korea.

Background: Postoperative thyroid stimulating hormone (TSH) suppression therapy is recommended for patients with intermediate- and high-risk differentiated thyroid cancer to prevent the recurrence of thyroid cancer. With the recent increase in small thyroid cancer cases, the extent of resection during surgery has generally decreased. Therefore, questions have been raised about the efficacy and long-term side effects of TSH suppression therapy in patients who have undergone a lobectomy. Methods: This is a multicenter, prospective, randomized, controlled clinical trial in which 2,986 patients with papillary thyroid cancer are randomized into a high-TSH group (intervention) and a low-TSH group (control) after having undergone a lobectomy. The principle of treatment includes a TSH-lowering regimen aimed at TSH levels between 0.3 and 1.99 μIU/mL in the low-TSH group. The high-TSH group targets TSH levels between 2.0 and 7.99 μIU/mL. The dose of levothyroxine will be adjusted at each visit to maintain the target TSH level. The primary outcome is recurrence-free survival, as assessed by neck ultrasound every 6 to 12 months. Secondary endpoints include disease-free survival, overall survival, success rate in reaching the TSH target range, the proportion of patients with major cardiovascular diseases or bone metabolic disease, the quality of life, and medical costs. The follow-up period is 5 years. Conclusion The results of this trial will contribute to establishing the optimal indication for TSH suppression therapy in low-risk papillary thyroid cancer patients by evaluating the benefit and harm of lowering TSH levels in terms of recurrence, metabolic complications, costs, and quality of life.

Background: Postoperative thyroid stimulating hormone (TSH) suppression therapy is recommended for patients with intermediate- and high-risk differentiated thyroid cancer to prevent the recurrence of thyroid cancer. With the recent increase in small thyroid cancer cases, the extent of resection during surgery has generally decreased. Therefore, questions have been raised about the efficacy and long-term side effects of TSH suppression therapy in patients who have undergone a lobectomy. Methods: This is a multicenter, prospective, randomized, controlled clinical trial in which 2,986 patients with papillary thyroid cancer are randomized into a high-TSH group (intervention) and a low-TSH group (control) after having undergone a lobectomy. The principle of treatment includes a TSH-lowering regimen aimed at TSH levels between 0.3 and 1.99 μIU/mL in the low-TSH group. The high-TSH group targets TSH levels between 2.0 and 7.99 μIU/mL. The dose of levothyroxine will be adjusted at each visit to maintain the target TSH level. The primary outcome is recurrence-free survival, as assessed by neck ultrasound every 6 to 12 months. Secondary endpoints include disease-free survival, overall survival, success rate in reaching the TSH target range, the proportion of patients with major cardiovascular diseases or bone metabolic disease, the quality of life, and medical costs. The follow-up period is 5 years. Conclusion The results of this trial will contribute to establishing the optimal indication for TSH suppression therapy in low-risk papillary thyroid cancer patients by evaluating the benefit and harm of lowering TSH levels in terms of recurrence, metabolic complications, costs, and quality of life.

Background: Conventional diagnostic approaches for adrenal tumors require multi-step processes, including imaging studies and dynamic hormone tests. Therefore, this study aimed to discriminate adrenal tumors from a single blood sample based on the combination of liquid chromatography-mass spectrometry (LC-MS) and machine learning algorithms in serum profiling of adrenal steroids. Methods: The LC-MS-based steroid profiling was applied to serum samples obtained from patients with nonfunctioning adenoma (NFA, n=73), Cushing’s syndrome (CS, n=30), and primary aldosteronism (PA, n=40) in a prospective multicenter study of adrenal disease. The decision tree (DT), random forest (RF), and extreme gradient boost (XGBoost) were performed to categorize the subtypes of adrenal tumors. Results: The CS group showed higher serum levels of 11-deoxycortisol than the NFA group, and increased levels of tetrahydrocortisone (THE), 20α-dihydrocortisol, and 6β-hydroxycortisol were found in the PA group. However, the CS group showed lower levels of dehydroepiandrosterone (DHEA) and its sulfate derivative (DHEA-S) than both the NFA and PA groups. Patients with PA expressed higher serum 18-hydroxycortisol and DHEA but lower THE than NFA patients. The balanced accuracies of DT, RF, and XGBoost for classifying each type were 78%, 96%, and 97%, respectively. In receiver operating characteristics (ROC) analysis for CS, XGBoost, and RF showed a significantly greater diagnostic power than the DT. However, in ROC analysis for PA, only RF exhibited better diagnostic performance than DT. Conclusion The combination of LC-MS-based steroid profiling with machine learning algorithms could be a promising one-step diagnostic approach for the classification of adrenal tumor subtypes.

Objective: To identify the relationship between dysphagia, dietary level, and pulmonary function in post-stroke patients. Methods: Thirty-six post-stroke patients with dysphagia, who were hospitalized from June 2017 to October 2017 in the Department of Rehabilitation Medicine at a tertiary hospital, were analyzed retrospectively. The video-fluoroscopic swallowing study (VFSS) and videofluoroscopic dysphagia scale (VDS) were used to assess dysphagia. The vital capacity (VC) and peak cough flow (PCF) were used to assess the pulmonary function. Upon admission, the patients were divided into three groups according to their dietary level (tube feeding, dysphagia diet, and general diet). The correlation between dysphagia and pulmonary function was analyzed using an independent t-test test with the optimal points, and the relationship between the diet level and pulmonary function was evaluated using a one-way analysis of the variance. Results: Significant correlations between the pulmonary function and sub-items of VDS were found in “oral transit time” with VC, “vallecullar residue” and “aspiration” with PCF, and “triggering of pharyngeal swallow”, “VDS total score” with VC and PCF. The dietary levels upon admission had a significant correlation with VC and PCF. The VC among groups divided according to three diet levels showed statistically significant differences. Conclusion This study revealed the relationship between the pulmonary function and dysphagia in post-stroke patients. Moreover, the pulmonary function correlated with dietary level, even though it was not confirmed that it affected dietary levels. The clinical importance of the pulmonary function in post-stroke patients with dysphagia should be emphasized. In addition, a large-scale study is needed to determine the correlation between the pulmonary function and swallowing difficulty

BACKGROUND: von Willebrand disease (VWD) is one of the most common inherited bleeding disorders. However, the number of patients who register to the Korea Hemophilia Foundation (KHF) is much lower than the expected prevalence rate and only few hospitals perform tests for diagnosis autonomously. Thus, we surveyed practical realities of VWD in Yeungnam region. METHODS: Patients with VWD (N=267) who were diagnosed at eleven university hospitals from March 1995 to March 2018 were enrolled in this study. We evaluated the medical records from each hospital retrospectively. RESULTS: Two hundred and twenty-eight children and 39 adults met the diagnostic criteria for VWD. Seventy-eight (57.4%) patients had the blood type O. Fifty-eight patients were definite type 1 (21.7%), 151 were possible type 1 (56.6%), and the others were type 2. Abnormal laboratory findings were the most common factor for the diagnosis in children. VWF mutations were detected in 17 patients. Patients with a family history showed age of diagnosis of 9 y, which is higher than in those with no family history (6 yr), and also showed a higher rate of significant bleeding (32.1% vs. 14.2%). VWF:RCo and VWF:Ag tests were performed in-hospital at only 1 of 11 hospitals. Twelve of 267 patients were enrolled at the KHF (4.5%). CONCLUSION: A high rate of out-sourcing studies may result in inaccurate diagnosis. The registration rate to the KHF is still lower than the prevalence rate. A comprehensive nationwide registration system is necessary in order to identify the actual prevalence rate and promote the diagnosis of VWD in Korea.
Adult ; Child ; Diagnosis ; Hemophilia A ; Hemorrhage ; Hospitals, University ; Humans ; Korea ; Medical Records ; Prevalence ; Retrospective Studies ; von Willebrand Diseases

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

BACKGROUND: Even after pneumococcal vaccination introduction, Streptococcus pneumoniae (pneumoccocus) is still an important cause of respiratory and invasive severe infection. Pneumococcus is resided in nasal mucosa and local or systemic infection begins with the nasal mucosa damage. We studied the indirect effect of pneumococcal conjugate vaccine (PCV) on pneumococcal nasopharyngeal carriage rates, serotypes and antimicrobial susceptibility between vaccinate and non-vaccinated children. MATERIALS AND METHODS: From January 2010 to October 2010, 379 healthy children under 5 years old from three university hospitals were recruited. Fully vaccinated children over 3 time doses of PCV and children with no vaccination history of PCV were enrolled, and nasopharyngeal aspirations were obtained from these children. Serotypes using multibead serotyping assay with multiplex PCR and antimicrobial susceptibility was analyzed. Antimicrobial susceptibilities were determined by the CLIS guideline. RESULTS: Two hundred seventy six children were received pneumococcal vaccination while 103 were not. 137 pneumococci were isolated from nasopharyngeal aspiration specimens. Nasal carriage rate was significantly low in vaccinated group (P-value; 0.001). Nasopharyngeal carriage rate was 28.6% (79/276) in vaccinate group and 56.3% (58/103) in non-vaccinated group. Among those vaccinated group, 13.0% (36/276) of the serotypes were vaccine or vaccine related type with the most common type 19F. In contrast, 31.1% (32/103) of the serotypes in non vaccinated group were vaccine or vaccine related type with the most common type 6A. The resistant rate of penicillin was 90.5%. For antimicrobial susceptibility, amoxicillin and amoxicillin/clavulanate showed high susceptibility (73.0%), but 19F and 19A serotypes were all resistant against amoxicillin. CONCLUSIONS: High nasopharyngeal carriage rate in non vaccinated group corresponded to the result of past study. However, 19F and 19A still came up as problematic serotypes with a high carriage rate and antimicrobial resistance in both vaccinated and non vaccinated groups. Also, this study showed that the resistance rate of primary oral antimicrobial agents was increased in compared to past. For solving these problems, the selective antimicrobial use with establishment of high dose amoxicillin/clavulanate regimen and active PCV immunization should be needed. Furthermore, pneumococcal carriage and serotype study concerning with antimicrobial susceptibility should be conducted in the future in 10 or 13-valent PCV received children.
Amoxicillin ; Anti-Infective Agents ; Aspirations (Psychology) ; Child ; Hospitals, University ; Humans ; Immunization ; Multiplex Polymerase Chain Reaction ; Nasal Mucosa ; Penicillins ; Porphyrins ; Serotyping ; Streptococcus ; Streptococcus pneumoniae ; Vaccination

BACKGROUND/AIMS: Endogenous endophthalmitis (EE) is rare. However, the visual outcome of patients with EE is very poor. Many cases of EE caused by Gram-negative bacterial infections have recently been reported. This study was conducted to explore the most frequent pathogens, diagnosis, and treatment outcomes of EE. METHODS: A retrospective analysis was carried out in 23 patients diagnosed with EE through clinical manifestations and ophthalmic examinations in three hospitals between January 2000 and April 2011. Samples from 23 patients with EE were analyzed microbiologically. RESULTS: Pathogens were identified in 18 (78%) of the 23 blood, liver aspirate, and/or vitreous humor samples. Klebsiella pneumoniae was the most frequent organism (13/23, 57%). Abdomino-pelvic imaging (21/23, 91%) was performed to evaluate the primary site of infection. The most common primary infection was liver abscess (14/23, 61%). Despite administration of intravenous antibiotics and intravitreal injection, only six of 23 patients showed improvements in visual acuity. Thirteen (57%) experienced worse visual acuity. Four (17%) were eventually enucleated. CONCLUSIONS: In patients diagnosed with EE, abdomino-pelvic CT is required to exclude the presence of liver abscess. If a liver abscess is identified, percutaneous drainage should be considered. Considering the rapid progression and poor prognosis of EE, early diagnosis and immediate management are vital. We currently suggest that empiric antibiotics for treatment of EE should have activity against Gram-negative bacilli such as K. pneumoniae.
Anti-Bacterial Agents ; Drainage ; Early Diagnosis ; Endophthalmitis ; Gram-Negative Bacterial Infections ; Humans ; Intravitreal Injections ; Klebsiella ; Klebsiella pneumoniae ; Liver ; Liver Abscess ; Pneumonia ; Prognosis ; Retrospective Studies ; Visual Acuity ; Vitreous Body

BACKGROUND/AIMS: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are predominantly known as medication-induced diseases. However, at our institution, we have experienced more cases of non-drug-related SJS and TEN than expected. Therefore, we studied the difference between non-drug-related and drug-related SJS and TEN in terms of clinical characteristics and prognoses. METHODS: The etiologies, clinical characteristics, and treatment outcomes for 82 adult patients with SJS and TEN were retrospectively reviewed. RESULTS: A total of 71 patients (86.6%) were classified as having SJS, and the other 11 patients (13.4%) were classified as having TEN. Drug-related cases were more common (43, 52.4%) than non-drug-related cases (39, 47.6%). Anticonvulsants (12/82, 14.6%) and antibiotics (9/82, 11%) were the most common causative medications. Anemia (p = 0.017) and C-reactive protein of > or = 5 mg/dL (p = 0.026) were more common in the drug-related cases than in the non-drug-related cases. Intravenous steroid therapy was used as the main treatment regimen (70/82, 85.4%). Of the 82 patients, 8 (9.8%) died during the clinical course. A univariate analysis for mortality showed statistical significance for the following: kidney function abnormality, pneumonia, hemoglobin of < 10 g/dL, and combined underlying diseases. In a multivariate analysis, only pneumonia was statistically significant (odds ratio, 25.79; p = 0.009). CONCLUSIONS: Drugs were the most frequent cause of these diseases. However, non-drug-related causes also contributed to a significant proportion of cases. Physicians should keep this in mind when documenting patient history. In addition, early recognition and treatment may be important for better outcomes.
Adolescent ; Adult ; Aged ; Aged, 80 and over ; Chi-Square Distribution ; Epidermal Necrolysis, Toxic/diagnosis/*etiology/mortality/*therapy ; Female ; Humans ; Logistic Models ; Male ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Republic of Korea ; Risk Assessment ; Risk Factors ; Stevens-Johnson Syndrome/chemically induced/diagnosis/*etiology/mortality/*therapy ; Survival Analysis ; Treatment Outcome ; Young Adult