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/Aims: The prospective Crohn’s Disease Clinical Network and Cohort Study is a nationwide multicenter cohort study of patients with Crohn’s disease (CD) in Korea, aiming to prospectively investigate the clinical features and long-term prognosis associated with CD. Methods: Patients diagnosed with CD between January 2009 and September 2019 were prospectively enrolled. They were divided into two cohorts according to the year of diagnosis: cohort 1 (diagnosed between 2009 and 2011) versus cohort 2 (between 2012 and 2019). Results: A total of 1,175 patients were included, and the median follow-up duration was 68 months (interquartile range, 39.0 to 91.0 months). The treatment-free durations for thiopurines (p<0.001) and anti-tumor necrosis factor agents (p=0.018) of cohort 2 were shorter than those of cohort 1. Among 887 patients with B1 behavior at diagnosis, 149 patients (16.8%) progressed to either B2 or B3 behavior during follow-up. Early use of thiopurine was associated with a reduced risk of behavioral progression (adjusted hazard ratio [aHR], 0.69; 95% confidence interval [CI], 0.50 to 0.90), and family history of inflammatory bowel disease was associated with an increased risk of behavioral progression (aHR, 2.29; 95% CI, 1.16 to 4.50). One hundred forty-one patients (12.0%) underwent intestinal resection, and the intestinal resection-free survival time was significantly longer in cohort 2 than in cohort 1 (p=0.003). The early use of thiopurines (aHR, 0.35;95% CI, 0.23 to 0.51) was independently associated with a reduced risk of intestinal resection. Conclusions The prognosis of CD in Korea appears to have improved over time, as evidenced by the decreasing intestinal resection rate. Early use of thiopurines was associated with an improved prognosis represented by a reduced risk of intestinal resection.

Purpose: Acute promyelocytic leukemia (APL) is a rare disease in children and there are some different characteristics between children and adult. We aimed to evaluate incidence, clinical characteristics and treatment outcomes of pediatric APL in Korea. Materials and Methods: Seventy-nine pediatric APL patients diagnosed from January 2009 to December 2016 in 16 tertiary medical centers in Korea were reviewed retrospectively. Results: Of 801 acute myeloid leukemia children, 79 (9.9%) were diagnosed with APL. The median age at diagnosis was 10.6 years (range, 1.3 to 18.0). Male and female ratio was 1:0.93. Thirty patients (38.0%) had white blood cell (WBC) count greater than 10×109/L at diagnosis. All patients received induction therapy consisting of all-trans retinoic acid and chemotherapy. Five patients (6.6%) died during induction chemotherapy and 66 patients (86.8%) achieved complete remission (CR) after induction chemotherapy. The causes of death were three intracranial hemorrhage, one cerebral infarction, and one sepsis. Five patients (7.1%) suffered a relapse during or after maintenance chemotherapy. The estimated 4-year event-free survival and overall survival (OS) rates were 82.1%±4.4%, 89.7%±5.1%, respectively. The 4-year OS was significantly higher in patients with initial WBC < 10×109/L than in those with initial WBC ≥ 10×109/L (p=0.020). Conclusion This study showed that the CR rates and survival outcomes in Korean pediatric APL patients were relatively good. The initial WBC count was the most important prognostic factor and most causes of death were related to serious bleeding in the early stage of treatment.

Purpose: Effectiveness and safety of clofarabine (one of the treatment mainstays in pediatric patients with relapsed/refractory acute lymphoblastic leukemia [ALL]) was assessed in Korean pediatric patients with ALL to facilitate conditional coverage with evidence development. Materials and Methods: In this multicenter, prospective, observational study, patients receiving clofarabine as mono/combination therapy were followed up every 4-6 weeks for 6 months or until hematopoietic stem cell transplantation (HSCT). Response rates, survival outcomes, and adverse events were assessed. Results: Sixty patients (2-26 years old; 65% B-cell ALL, received prior ≥ 2 regimen, 68.3% refractory to previous regimen) were enrolled and treated with at least one dose of clofarabine; of whom 26 (43.3%) completed 6 months of follow-up after the last dose of clofarabine. Fifty-eight patients (96.7%) received clofarabine combination therapy. Overall remission rate (complete remission [CR] or CR without platelet recovery [CRp]) was 45.0% (27/60; 95% confidence interval [CI], 32.4 to 57.6) and the overall response rate (CR, CRp, or partial remission [PR]) was 46.7% (28/60; 95% CI, 34.0 to 59.3), with 11 (18.3%), 16 (26.7%), and one (1.7%) patients achieving CR, CRp, and PR, respectively. The median time to remission was 5.1 weeks (95% CI, 4.7 to 6.1). Median duration of remission was 16.6 weeks (range, 2.0 to 167.6 weeks). Sixteen patients (26.7%) proceeded to HSCT. There were 24 deaths; 14 due to treatment-emergent adverse events. Conclusion Remission with clofarabine was observed in approximately half of the study patients who had overall expected safety profile; however, there was no favorable long-term survival outcome in this study.

Background: Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. Methods: We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. Results: A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. Conclusion This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

BACKGROUND/AIMS: Three-dimensional (3D) imaging is gaining popularity and has been partly adopted in laparoscopic surgery or robotic surgery but has not been applied to gastrointestinal endoscopy. As a first step, we conducted an experiment to evaluate whether images obtained by conventional gastrointestinal endoscopy could be used to acquire quantitative 3D information. METHODS: Two endoscopes (GIF-H260) were used in a Borrmann type I tumor model made of clay. The endoscopes were calibrated by correcting the barrel distortion and perspective distortion. Obtained images were converted to gray-level image, and the characteristics of the images were obtained by edge detection. Finally, data on 3D parameters were measured by using epipolar geometry, two view geometry, and pinhole camera model. RESULTS: The focal length (f) of endoscope at 30 mm was 258.49 pixels. Two endoscopes were fixed at predetermined distance, 12 mm (d12). After matching and calculating disparity (v2-v1), which was 106 pixels, the calculated length between the camera and object (L) was 29.26 mm. The height of the object projected onto the image (h) was then applied to the pinhole camera model, and the result of H (height and width) was 38.21 mm and 41.72 mm, respectively. Measurements were conducted from 2 different locations. The measurement errors ranged from 2.98% to 7.00% with the current Borrmann type I tumor model. CONCLUSIONS: It was feasible to obtain parameters necessary for 3D analysis and to apply the data to epipolar geometry with conventional gastrointestinal endoscope to calculate the size of an object.
Aluminum Silicates ; Endoscopes ; Endoscopes, Gastrointestinal ; Endoscopy, Gastrointestinal ; Laparoscopy ; Pilot Projects ; Resin Cements

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare, autosomal-recessive disorder characterized by the clinical triad of indifference of pain, anhidrosis and heat intolerance.Because of their lack of autonomic response to noxious stimuli, the determination of adequate depth of anesthesia in the CIPA patient undergoing surgery is a major challenge.We experienced a patient with CIPA who had minor procedures three times under the general anesthesia, in which bispectral index (BIS) was maintained at 40-50 by adjusting sevoflurane concentrations with 50% nitrous oxide.The low end-tidal sevoflurane concentrations (<1.2 vol%) were required to keep the target BIS while vital signs remained stable throughout the surgery in each operation.BIS monitor may be a valuable tool to guide the depth of anesthesia in patients with CIPA.
Anesthesia ; Anesthesia, General ; Hereditary Sensory and Autonomic Neuropathies ; Hot Temperature ; Humans ; Hypohidrosis ; Indoles ; Methyl Ethers ; Organothiophosphorus Compounds ; Propionates ; Vital Signs

BACKGROUND: Desflurane is known to causes hypertension and tachycardia when its inspired concentration is rapidly increased. We determined whether nitrous oxide (N2O) or remifentanil alters cardiovascular responses to intubation and/or inhalation of high concentrations of desflurane during induction of anesthesia. METHODS: Sixty patients were assigned randomly into three groups (n = 20 each). Anesthesia was induced with thiopental 5 mg/kg followed by saline (control and N2O groups) or remifentanil 1microg/kg (remifentanil group). Tracheal intubation was facilitated with intravenous vecuronium 0.12 mg/kg and 12% desflurane was given soon after the intubation. In addition, 75% N2O was given beginning 3 min before the intubation in the N2O group. Systolic arterial pressure (SAP), heart rate (HR), and plasma catecholamine concentrations were determined. RESULTS: The intubation resulted in an immediate increase and an additional second increase of SAP and HR at 3 to 5 min after intubation in all groups. SAP but not HR in the N2O group and both SAP and HR in the remifentanil group at first and second peak responses were lower than in the control group. Norepinephrine increased at 1 min after intubation and increased further at 5 min in the control and N2O groups but only increased at 5 min in the remifentanil group. CONCLUSIONS: A biphasic pressor and tachycardiac response in response to intubation and desflurane were noted. Although N2O did not affect tachycardiac response, it suppressed the pressor responses and augmented norepinephrine release. However, remifentanil significantly attenuated hemodynamic and catecholamine responses to endotracheal intubation and desflurane.
Anesthesia ; Arterial Pressure ; Heart Rate ; Hemodynamics ; Humans ; Hypertension ; Inhalation ; Intubation ; Intubation, Intratracheal ; Isoflurane ; Nitrous Oxide ; Norepinephrine ; Piperidines ; Plasma ; Tachycardia ; Thiopental ; Vecuronium Bromide

BACKGROUND/AIMS: Efficacy of triple therapy with omeprazole, amoxicillin, and clarithromycin (OAC) for Helicobacter pylori (H. pylori) varies among different geographic regions and patient populations. And, there is no full consensus on how long this treatment should be maintained. We assessed the efficacy of the OAC regimen according to the treatment duration (7, 10 or 14 days) using 13C-urea breath test (13C-UBT). METHODS: Two hundred fifty five H. pylori positive patients with peptic ulcer were randomly assigned to a OAC (omeprazole 20 mg b.d., amoxicillin 1.0 g b.d. and clarithromycin 500 mg b.d.) 7, 10 or 14 days regimen. Eradication of H. pylori was assessed by 13C-UBT 4 weeks after the completion of therapy. RESULTS: 140 male and 115 female (mean age, 51.3) patients were enrolled. The overall eradication rate of H. pylori in each group was significantly higher in OAC 14 days regimen than OAC 7 and 10 days regimen (91.9% in OAC 14 days, 74.4% in OAC 7 days, and 80.2% in OAC 10 days, respectively, p<0.05). CONCLUSIONS: The overall eradication rate of H. pylori was highest in OAC 14 days regimen in our study. We have found significant differences in eradication rates with previous reports, using CLO and histology, in Korea. Thus, further studies focusing on the treatment period may be warranted.
Amoxicillin ; Breath Tests* ; Clarithromycin ; Consensus ; Female ; Helicobacter pylori* ; Helicobacter* ; Humans ; Korea ; Male ; Omeprazole ; Peptic Ulcer