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: Dexrazoxane has been used as an effective cardioprotector against anthracycline cardiotoxicity. This study intended to analyze cardioprotective efficacy and secondary malignancy development, and elucidate risk factors for secondary malignancies in dexrazoxane-treated pediatric patients. MATERIALS AND METHODS: Data was collected from 15 hospitals in Korea. Patients who received any anthracyclines, and completed treatment without stem cell transplantation were included. For efficacy evaluation, the incidence of cardiac events and cardiac event-free survival rates were compared. Data about risk factors of secondary malignancies were collected. RESULTS: Data of total 1,453 cases were analyzed; dexrazoxane with every anthracyclines group (D group, 1,035 patients) and no dexrazoxane group (non-D group, 418 patients). Incidence of the reported cardiac events was not statistically different between two groups; however, the cardiac event-free survival rate of patients with more than 400 mg/m2 of anthracyclines was significantly higher in D group (91.2% vs. 80.1%, p=0.04). The 6-year cumulative incidence of secondary malignancy was not different between both groups after considering follow-up duration difference (non-D, 0.52%±0.37%; D, 0.60%±0.28%; p=0.55). The most influential risk factor for secondary malignancy was the duration of anthracycline administration according to multivariate analysis. CONCLUSION: Dexrazoxane had an efficacy in lowering cardiac event-free survival rates in patients with higher cumulative anthracyclines. As a result of multivariate analysis for assessing risk factors of secondary malignancy, the occurrence of secondary malignancy was not related to dexrazoxane administration.
Anthracyclines ; Cardiotoxicity ; Dexrazoxane ; Disease-Free Survival ; Follow-Up Studies ; Humans ; Incidence ; Korea ; Multivariate Analysis ; Neoplasms, Second Primary ; Risk Factors* ; Stem Cell Transplantation

BACKGROUND: Hip pain is a common musculoskeletal complaint in general practice. Although comprehensive diagnostic approach on hip pain is mandatory for adequate treatment, un- or mis-diagnosis is not rare in primary care. The aim of this study was to analyze descriptively un- or mis-diagnosed hip pain cases referred from primary care to a tertiary hospital, especially in young adults ≤ 50 years old. METHODS: We retrospectively analyzed a consecutive cohort of 150 patients (≤ 50 years old) with chronic hip pain (≥ 6 weeks), which was not diagnosed or misdiagnosed based on the information provided on the referral form. RESULTS: Overall an average 32 cases/month were referred due to hip pain without a diagnosis or with an incorrect diagnosis. Among them, 150 patients were enrolled in this study and 146 (97.3%) could be allocated to a specific disease by using data from routine clinical practice. Four common final diagnoses were femoroacetabular impingement (FAI) syndrome (55.3%), hip dysplasia (HD, 13.3%), referred pain from the lumbar spine (9.3%), and spondyloarthritis (SpA, 7.3%). In patients with FAI syndrome, 37 (44.0%) had pincer-type FAI and 33 (39.8%) had combined-type. Although the pain site or gender was not tightly clustered, the distribution of final diagnosis was significantly different according to hip pain location or gender. Especially, SpA or HD was not observed in younger women subgroup or elder men subgroup, respectively, when stratified by the mean age of participants. CONCLUSION: Most (> 80%) young patients with hip pain, a difficult issue to diagnosis for many primary physicians, had FAI syndrome, HD, spine lesions, and SpA. This study could give a chance to feedback information about cases with un- or mis-diagnosed hip pain, and it suggests that primary physicians need to be familiar with the diagnostic approach for these 4 diseases.
Cohort Studies ; Diagnosis ; Female ; Femoracetabular Impingement ; General Practice ; Hip Dislocation ; Hip* ; Humans ; Male ; Pain, Referred ; Primary Health Care ; Referral and Consultation ; Retrospective Studies* ; Spine ; Tertiary Care Centers ; Young Adult*

We evaluated the incidence, clinical characteristics, and prognostic impact of calreticulin (CALR) mutations in essential thrombocythemia (ET) and primary myelofibrosis (PMF) patients. In all, 48 ET and 14 PMF patients were enrolled, and the presence of CALR mutations was analyzed by direct sequencing. Patients were classified into three subgroups according to Janus kinase 2 (JAK2) V617F and CALR mutation status, and their clinical features and prognosis were compared. CALR mutations were detected in 15 (24.2%) patients, and the incidence increased to 50.0% in 30 JAK2 V617F mutation-negative cases. These included 11 patients with three known mutations (c.1092_1143del [seven cases], c.1154_1155insTTGTC [three cases], and c.1102_1135del [one case]) and 4 patients with novel mutations. ET patients carrying CALR mutation were younger, had lower white blood cell counts, and experienced less thrombosis during follow-up than those carrying JAK2 V617F mutation, while both patient groups showed similar clinical features and prognosis. In ET patients without JAK2 V617F mutation, CALR mutation did not significantly affect clinical manifestation and prognosis. In conclusion, CALR mutation analysis could be a useful diagnostic tool for ET and PMF in 50% of the cases without JAK2 V617F mutations. The prognostic impact of CALR mutations needs further investigation.
Adult ; Aged ; Calreticulin/*genetics ; DNA Mutational Analysis ; Exons ; Female ; Genotype ; Humans ; INDEL Mutation ; Janus Kinase 2/genetics ; Male ; Middle Aged ; Primary Myelofibrosis/diagnosis/epidemiology/*genetics ; Prognosis ; Republic of Korea ; Tertiary Care Centers ; Thrombocythemia, Essential/diagnosis/epidemiology/*genetics ; Young Adult

BACKGROUND: It is important to differentiate Graves' disease from that of painless thyroiditis in patients with thyrotoxicosis. In this study, we evaluated the usefulness of total T3 to free T4 ratio in making a differential diagnosis between Graves' disease and painless thyroiditis. METHODS: We reviewed medical records of thyrotoxic patients, who had been diagnosed with Graves' disease or painless thyroiditis, from October 2009 to July 2011. We assessed clinical characteristics, serum levels of total T3, free T4, thyroid stimulating hormone, thyrotropin-binding inhibitory immunoglobulin, and findings of 99mTechnetium thyroid scan. We analyzed the total T3/free T4 ratios between Graves' disease and painless thyroiditis patients. RESULTS: A total of 76 untreated thyrotoxic patients "49 Graves' disease and 27 painless thyroiditis" were examined. The total T3, free T4 levels and the total T3/free T4 ratios were significantly higher in patients with Graves' disease than in those with painless thyroiditis (P < 0.001). In the total T3/free T4 ratio > 73, the possibility of Graves' disease was significantly higher than in painless thyroiditis (sensitivity, 75.5%; specificity, 70.3%). The sensitivity and specificity of the total T3/free T4 ratio in patients with free T4 < 3.6 ng/dL have been increased (sensitivity, 100%; specificity, 71.4%). CONCLUSION: The total T3/free T4 ratios was useful for making a differential diagnosis between Graves' disease and painless thyroiditis.
Diagnosis, Differential ; Graves Disease ; Humans ; Immunoglobulins ; Immunoglobulins, Thyroid-Stimulating ; Medical Records ; Sensitivity and Specificity ; Thyroid Gland ; Thyroiditis ; Thyrotoxicosis ; Thyrotropin

Traumatic anterior dislocation of the hip is rare. Bilateral traumatic anterior dislocation is an even rarer injury; indeed, only 5 cases have been reported in the English literature. We describe a case of a bilateral traumatic anterior dislocation of the hip and a concomitant unstable lumbar burst fracture following a mechanism of injury distinctly different from other reports.
*Accidents, Occupational ; Acetabulum/injuries ; Buttocks/*injuries ; Fractures, Bone/complications/etiology/radiography ; Hip Dislocation/etiology/*radiography ; Humans ; Lumbar Vertebrae/*injuries ; Lumbosacral Region/*injuries ; Male ; Middle Aged ; Spinal Fractures/etiology/*radiography

Duodenal abscess is a rarely reported disease throughout the entire world. Duodenal abscesses are developed mostly from the complication of duodenal ulcer perforation, and only small percentage of duodenal abscesses are the result of cholecysto-duodenal fistula which was made by gall bladder perforation. We report a 84-year-old male patient who presented to the emergency department with severe anorexia and generalized weakness for 2 weeks. The upper gastrointestinal endoscopy done and revealed a protruding mass at the lesser curvature of the duodenal bulb. As soon as the mass was punched with a biopsy forceps, a large amount of abscess began to pour out into the intestinal lumen. Abdominal CT scan demonstrated the presence of an air-fluid level the in gall bladder and also abscess in the porta hepatitis which was located between the gall bladder and the duodenum. Because the patient refused any surgical intervention, we treated him conservatively with intravenous antibiotics. Patient's symptom of anorexia was slowly resolved, and patient was discharged 10 days later.
Abscess* ; Aged, 80 and over ; Anorexia ; Anti-Bacterial Agents ; Biopsy ; Duodenal Ulcer ; Duodenum ; Emergency Service, Hospital ; Endoscopy ; Endoscopy, Gastrointestinal ; Fistula ; Hepatitis ; Humans ; Male ; Surgical Instruments ; Tomography, X-Ray Computed ; Urinary Bladder

PURPOSE: The recommended treatments for iliac arterial occlusive disease are an endovascular procedure in TASC type A patients, but surgery in TASC type D patients. However, in TASC type B and C patients, more evidence is required to make any firm recommendations about the best treatments. This study aimed to evaluate the best treatments for patients classified using the TASC morphological stratification. METHOD: A retrospective review of the patients who had undergone stent insertion into iliac arterial occlusive lesions, between 1995 and 2004, at the Chonnam National University Hospital, was performed. All patients were also classified using the TASC morphologic stratification. The TASC type was determined from the initial peripheral angiography findings. Also, the stenosis-free patency was used as an objective end point to evaluate the hemodynamic outcome of stent insertion. RESULT: A total of 86 patients were included in the study. The number of patients classified as TASC types A, B, C and D were 1 25, 17, and 43, respectively. Stent insertion was successfully performed in all patients. The mean follow up period was 26 months. The 1-year stenosis-free patency rates were 94.4, 74.0, and 19.1% in TASC type B, C, and D, respectively. The 5-year patency rates were 50.4, 18.5, and 6.0% in TASC type B, C, and D, respectively. CONCLUSION: The TASC morphological stratification was a useful prognostic factor in relation to the short and long-term iliac stent patency. For TASC types B and C, more evidence and experience of endovascular therapy are needed to confirm the best treatment. For TASC type D, surgical treatment is strongly recommended.
Angiography ; Arterial Occlusive Diseases ; Consensus* ; Endovascular Procedures ; Follow-Up Studies ; Hemodynamics ; Humans ; Jeollanam-do ; Retrospective Studies ; Stents*

Meconium peritonitis is a rare disease in neonates, characterized by intraperitoneal calcification, numerous fibrosis with or without pseudocyst formation due to antenatal extravasation of meconium. Meconium peritonitis may result in a number of genital manifestations, including inguinal and scrotal or labial hydrocele containing meconium or calcifications. Recently, increased numbers of fetuses with meconium peritonitis have been prenatally diagnosed by ultrasonography. We report a case of meconium peritonitis in a neonate with ascites and hydrocele which was diagnosed by antenatal ultrasonography.
Ascites ; Fetus ; Fibrosis ; Humans ; Infant, Newborn ; Meconium* ; Peritonitis* ; Rare Diseases ; Ultrasonography

PURPOSE: To evaluate the risk period of osteonecrosis of the femoral head following steroid therapy. MATERIALS AND METHODS: Medical records data were reviewed and studied the duration and dosage of steroid therapy for 22 patients who were diagnosed as in the early stage osteonecrosis by MRI but were without positive findings on plain radiographs. RESULTS: The period from the onset of steroid therapy to the time of diagnosis by MRI ranged from one to 16 months (mean, 5.3 months). Twenty-one of the 22 patients were diagnosed within 12 months. The total dosage of steroid ranged from 1,800 to 15,505 mg of prednisolone or its equivalent (mean, 5,928 mg). CONCLUSION: The current study revealed that the risk period for developing femoral head osteonecrosis in patients receiving long-term steroid therapy is 12 months. Periodical follow-up and intensive screening are recommended within the first year of long-term steroid therapy to detect osteonecrosis of the femoral head at an early stage.
Diagnosis ; Follow-Up Studies ; Head* ; Humans ; Magnetic Resonance Imaging ; Mass Screening ; Medical Records ; Osteonecrosis* ; Prednisolone