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.

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.

PURPOSE: In infants and young children, acute bronchiolitis is a leading cause of hospitalization via emergency departments (EDs). We aimed to investigate factors associated with hospitalization via ED in children with acute bronchiolitis. METHODS: We reviewed medical records of children aged 36 months or younger with acute bronchiolitis who visited the ED from January to December 2017. The following clinical data were collected and analyzed: age, sex, premature birth history, symptoms, fever duration, presence of respiratory distress and radiographic lesion, and inflammatory markers. RESULTS: Of 780 children enrolled, 463 (59.4%) were hospitalized via the ED. The factor associated with the hospitalization were age ≤ 12 months (odd ratio [OR], 45.34; confidence interval [CI], 17.50-117.44), fever lasting ≥ 3 days (OR, 13.66; 95% CI, 6.46-28.87), respiratory rate ≥ 24 breaths per minute (OR, 6.88; 95% CI, 4.21-11.26), radiographic lesion (OR, 5.70; 95% CI, 2.62-12.40), and chest retraction (OR, 2.45; 95% CI, 1.11-5.41). CONCLUSION: In children with acute bronchiolitis who visit EDs, those having younger age, longer fever duration, respiratory distress or radiographic lesion may need hospitalization.
Bronchiolitis ; Child ; Emergencies ; Emergency Medicine ; Emergency Service, Hospital ; Fever ; Hospitalization ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Medical Records ; Pediatrics ; Premature Birth ; Respiratory Rate ; Thorax

PURPOSE: To investigate the relationship of improvement in erectile function (EF) with improvement in lower urinary tract symptoms (LUTS) and to assess the contribution of tamsulosin dose to the improvement of EF apart from the indirect influence of LUTS improvement in men with LUTS and erectile dysfunction (ED). MATERIALS AND METHODS: Fifty patients received tamsulosin 0.2 mg/d for the first 4 weeks and were subsequently divided into two groups by patient-reported outcomes. Nonescalators were maintained starting dose and escalators increased to 0.4 mg for the remaining 8 weeks. International Prostatic Symptom Score (IPSS) and International Index of Erectile Function (IIEF-5), and underwent uroflowmetry were evaluated at baseline, and weeks 4 and 12. RESULTS: LUTS parameters were significantly improved in both groups but insignificant between the 2 groups. The degree of the improvement in the total IPSS and in the voiding, storage, and quality of life (QoL) subscores were significantly correlated with the degree of the improvement in EF; this was especially prominent in patients successfully treated LUTS. The escalators experienced a significantly greater increase in IIEF-5 scores than did the nonescalators (3.3 vs. 1.5). CONCLUSIONS: Dose escalation provided similar LUTS improvement in patients with refractory to starting dose. The improvements of LUTS were correlated with the improvement of EF. The increase in the IIEF-5 score was significantly higher in escalators. These findings imply that tamsulosin may contribute to the improvement in EF through the improvement of LUTS and QoL and direct relaxation of the corpus cavernosum in a dose-dependent fashion.
Elevators and Escalators ; Erectile Dysfunction ; Humans ; Lower Urinary Tract Symptoms ; Male ; Prostatic Hyperplasia ; Quality of Life ; Relaxation ; Sulfonamides

BACKGROUND/AIMS: Transient elastography as performed using the Fibroscan(R) is a useful noninvasive method for evaluating hepatic fibrosis. However, recent studies have found that liver stiffness measurement (LSM) values are inappropriately elevated in acute hepatitis or in the acute flare state of chronic hepatitis, suggesting that the LSM value obtained by the Fibroscan(R) is not a reliable marker for fibrosis. We retrospectively evaluated the clinical factors influencing the LSM value obtained using transient elastography as performed using the Fibroscan(R) in patients with chronic liver disease. METHODS: A total of 298 patients who were followed in Kungpook National University Hospital from November 2007 to May 2008 due to previously established liver cirrhosis or chronic liver disease were investigated using the Fibroscan(R), laboratory test, ultrasound, and/or abdominal computed tomography. RESULTS: The 298 patients were aged 47.8+/-12.9 years (mean+/-SD). The cut-off value for a diagnosis of liver cirrhosis was 12.5 kPa (as used in previous studies). Thirty-six patients (15%) and 202 patients (85%) with chronic liver disease without clinical manifestation of cirrhosis had LSMs of >12.5 kPa and <12.5 kPa, respectively. Multivariate analysis revealed that LSM values were unusually increased in patients with chronic liver disease who were older (P=0.007) or who had increased gamma gultamyltranspetidase (GGT) (P=0.022), decreased albumin (P=0.015), or increased total bilirubin (P=0.009). CONCLUSIONS: This study reveals that age, GGT, and albumin are clinical factors influencing LSM values. This reinforces the need to interpret LSM values in the context of a defined diagnosis, biochemical data, radiologic examination, and other clinical findings.
Adult ; Age Factors ; Bilirubin/metabolism ; Biological Markers/blood ; Chronic Disease ; *Elasticity Imaging Techniques ; Female ; Hepatitis/diagnosis/ultrasonography ; Humans ; Liver Cirrhosis/diagnosis/ultrasonography ; Liver Diseases/diagnosis/radiography/*ultrasonography ; Male ; Middle Aged ; Retrospective Studies ; Serum Albumin/metabolism ; Tomography, X-Ray Computed ; gamma-Glutamyltransferase/metabolism

PURPOSE: It is known that the loss of diurnal rhythm of testosterone by age is related to late-onset hypogonadism (LOH). Currently testosterone replacement therapy (TRT) has been recommended only in men with hypogonadism. We evaluated the effectiveness and safety of TRT for men with LOH symptoms and the loss of diurnal rhythm of total testosterone but normal values of total testosterone. MATERIALS AND METHODS: We enrolled 62 patients in whom the difference in testosterone between morning and evening was lower than 108 ng/dl, whose morning values were higher than 300 ng/dl, and who were diagnosed with LOH using the Androgen Deficiency in Aging Male (ADAM) questionnaire. Among the 62 patients enrolled, 44 completed the daily application of 1% testosterone gel or the intramuscular injection of long-acting testosterone undecanoate for the full 20-week period. We compared the data at baseline, and the 8th and 20th week using the Aging Males' Symptoms (AMS) scale, the International Index of Erectile Function (IIEF)-15, the International Prostate Symptoms Score (IPSS), and the serum levels of total testosterone, prostate specific antigen (PSA), complete blood cell count (CBC), and lipid profile. RESULTS: The mean age was 54.9+/-7.2 years. Subjects main symptoms were sexual dysfunction and decrease of ejaculate volume. AMS scales before and after TRT were 41.3+/-18.5 and 35.8+/-19.7 (p<0.05). IIEF total scores before and after TRT were 29.7+/-13.7 and 38.9+/-17.4 (p<0.001). However, 18 patients (40.9%) were not satisfied with TRT and only 11% were fully satisfied. Total testosterone and estradiol were higher after TRT but the other values had not changed. The most common adverse event (27.3%) was erythrocystosis (18.2%). CONCLUSIONS: TRT could induce total testosterone to reach the mid-normal level and was relatively effective for aging male symptoms and sexual function. It is essential for physician to inform patients about potential adverse events and the low satisfaction rate associated with TRT even though TRT has generally been effective.
Aging ; Blood Cell Count ; Circadian Rhythm ; Estradiol ; Hormone Replacement Therapy ; Humans ; Hypogonadism ; Injections, Intramuscular ; Male ; Prostate ; Prostate-Specific Antigen ; Surveys and Questionnaires ; Reference Values ; Testosterone ; Weights and Measures

Bordetella (B) bronchiseptica is a common veterinary pathogen, but has rarely been implicated in human infections. Most patients with B. bronchiseptica infections are compromised clinically such as in patients with a malignancy, AIDS, malnutrition, or chronic renal failure. We experienced a case of relapsing peritonitis caused by B. bronchiseptica associated with continuous ambulatory peritoneal dialysis (CAPD). A 56-yr-old male, treated with CAPD due to end stage renal disease (ESRD), was admitted with complaints of abdominal pain and a turbid peritoneal dialysate. The culture of peritoneal dialysate identified B. bronchiseptica. The patient was treated with a combination of intraperitoneal antibiotics. There were two further episodes of relapsing peritonitis, although the organism was sensitive to the used antibiotics. Finally, the indwelling CAPD catheter was removed and the patient was started on hemodialysis. This is the first report of a B. bronchiseptica human infection in the Korean literature.
Anti-Bacterial Agents/pharmacology/therapeutic use ; Bordetella Infections/*diagnosis/microbiology ; Bordetella bronchiseptica/*metabolism ; Fibrosis ; Humans ; Kidney Failure/microbiology ; Male ; Middle Aged ; Peritoneal Dialysis, Continuous Ambulatory/*methods ; Peritoneum/pathology ; Peritonitis/*microbiology ; Recurrence