Accurate detection of BCR-ABL fusion transcripts at and below molecular response (MR) 4 (0.01% International Scale [IS]) is required for disease monitoring in patients with chronic myeloid leukemia (CML). We evaluated the analytical performance of the QXDx BCR-ABL %IS (Bio-Rad, Hercules, CA, USA) droplet digital PCR (ddPCR) assay, which is the first commercially available ddPCR-based in vitro diagnostics product. In precision analysis, the %CV was 9.3% and 3.0%, with mean values of 0.031% IS and 9.4% IS, respectively. The assay was linear in the first order, ranging from 0.032% IS to 20% IS. The manufacturer-claimed limit of blank, limit of detection, and limit of quantification were verified successfully. There was a very strong correlation between the results of the QXDx BCR-ABL %IS ddPCR assay and the ipsogen BCR-ABL1 Mbcr IS-MMR (Qiagen, Hilden, Germany) real-time quantitative PCR assay (r=0.996). In conclusion, the QXDx BCR-ABL %IS ddPCR assay can provide reliable results for CML patients.

PURPOSE: Many studies have reported that patients with type 1 diabetes have reduced bone mineral density (BMD). We assessed bone status in prepubertal children with type 1 diabetes mellitus (type 1 DM) at initial diagnosis and investigated factors associated with BMD. METHODS: Prepubertal children (n=29) with newly diagnosed type 1 diabetes from 2006 to 2014 were included. Dual-energy X-ray absorptiometry measured regional and whole-body composition at initial diagnosis. BMD was compared with healthy controls matched for age, sex, and body mass index (BMI). RESULTS: The mean age of all subjects (16 boys and 13 girls) was 7.58±1.36 years (range, 4.8–11.3 years). Initial mean glycosylated hemoglobin (HbA1c) level was 12.2%±1.9%. The mean BMD z-scores of lumbar spine, femur neck, and total body were not significantly different between patients and controls. Three patients (10.3%) had low bone density (total body BMD standard deviation score [SDS] < -2.0). To identify determinants of lumbar spine BMD z-score, multivariate regression analysis was performed with stepwise variable selection of age, pubertal status, BMI SDS, insulin like growth factor-1, and HbA1c. Only BMI SDS was significantly correlated with lumbar spine BMD z-score (β=0.395, P=0.023). CONCLUSIONS: Prepubertal children with newly diagnosed type 1 DM had similar bone mass compared to healthy peers. However, patients with low BMI should be carefully monitored for bone density in type 1 DM.
Absorptiometry, Photon ; Body Mass Index ; Bone Density ; Child* ; Diabetes Mellitus, Type 1 ; Diagnosis ; Femur Neck ; Hemoglobin A, Glycosylated ; Humans ; Insulin ; Miners* ; Spine

Intracranial arterial stenosis usually occurs due to atherosclerosis and is considered the most common cause of stroke worldwide. Although the effectiveness of bypass surgery for ischemic stroke is controversial, the superficial temporal artery to the middle cerebral artery bypass for ischemic stroke is a common procedure. In our report, a 50-year-old man presented with sudden-onset left side weakness and dysarthria. An angiogram showed significant stenosis in the junction of the right cavernous-supraclinoid internal carotid artery and right pericallosal artery. Symptoms altered between improvement and deterioration. Magnetic resonance imaging showed a repeated progression of anterior cerebral artery (ACA) infarction despite maximal medical therapy. We performed a STA-ACA bypass with contralateral STA interposition. Postoperative course was uneventful with no further progression of symptoms. Thus, bypass surgery may be considered in patients with symptomatic stenosis or occlusion of the ACA, especially when patients present progressive symptoms despite maximal medical therapy.
Anterior Cerebral Artery ; Arteries ; Atherosclerosis ; Carotid Artery, Internal ; Cerebral Revascularization ; Constriction, Pathologic* ; Dysarthria ; Humans ; Infarction ; Magnetic Resonance Imaging ; Middle Aged ; Middle Cerebral Artery ; Stroke ; Temporal Arteries ; Transplants*

BACKGROUND: Brain magnetic resonance imaging (MRI) is routinely performed to identify brain lesions in girls with central precocious puberty (CPP). We aimed to investigate the prevalence and type of brain lesions among Korean girls with CPP and evaluate the need for routine brain MRI examinations. METHODS: This retrospective cross-sectional study evaluated data on 3,528 girls diagnosed with CPP from April 2003 to December 2016, and identified 317 girls who underwent sellar MRI. Exclusion criteria were patients with a known brain tumor or who did not undergo brain MRI due to refusal or the decision of the pediatric endocrinologist. RESULTS: Normal sellar MRI findings were observed in 291 of the 317 girls (91.8%). Incidental findings were observed in 26 girls (8.2%). None of the patients had pathological brain lesions. CONCLUSION: The prevalence of intracranial lesions among girls who were generally healthy and without neurological symptoms but diagnosed with CPP was lower than that previously reported. Furthermore, none of the identified lesions required treatment. It may be prudent to reconsider the routine use of brain MRI to screen all patients with CPP, especially if they are healthy and neurologically asymptomatic, and are girls aged 6–8 years.
Brain Neoplasms ; Brain* ; Cross-Sectional Studies ; Female* ; Humans ; Incidental Findings ; Magnetic Resonance Imaging ; Prevalence* ; Puberty, Precocious* ; Retrospective Studies

Pulmonary benign metastasizing leiomyoma (PBML) is defined as metastasis of a leiomyoma to lung tissue. It was first reported in 1937. P BML is known as a benign disease, but can undergo malignant transformation. Only 1 case of the malignant transformation of PBML to leiomyosarcoma has been reported previously. In this report, we present a case of malignant transformation of PBML.
Leiomyoma* ; Leiomyosarcoma ; Lung ; Lung Neoplasms ; Neoplasm Metastasis

OBJECTIVE: To investigate the interobserver and test-retest reproducibility of T1ρ and T2 measurements of lumbar intervertebral discs using 3T magnetic resonance imaging (MRI). MATERIALS AND METHODS: This study included a total of 51 volunteers (female, 26; male, 25; mean age, 54 ± 16.3 years) who underwent lumbar spine MRI with a 3.0 T scanner. Amongst these subjects, 40 underwent repeat T1ρ and T2 measurement acquisitions with identical image protocol. Two observers independently performed the region of interest measurements in the nuclei pulposi of the discs from L1–2 through L5–S1 levels. Statistical analysis was performed using intraclass correlation coefficient (ICC) with a two-way random model of absolute agreement. Comparison of the ICC values was done after acquisition of ICC values using Z test. Statistical significance was defined as p value < 0.05. RESULTS: The ICCs of interobserver reproducibility were 0.951 and 0.672 for T1ρ and T2 mapping, respectively. The ICCs of test-retest reproducibility (40 subjects) for T1ρ and T2 measurements were 0.922 and 0.617 for observer A and 0.914 and 0.628 for observer B, respectively. In the comparison of the aforementioned ICCs, ICCs of interobserver and test-retest reproducibility for T1ρ mapping were significantly higher than T2 mapping (p < 0.001). CONCLUSION: The interobserver and test-retest reproducibility of T1ρ mapping were significantly higher than those of T2 mapping for the quantitative assessment of nuclei pulposi of lumbar intervertebral discs.
Humans ; Intervertebral Disc Degeneration ; Intervertebral Disc* ; Magnetic Resonance Imaging* ; Male ; Spine ; Volunteers

PURPOSE: Proper femoral tunnel position is important for anatomical reconstruction of the anterior cruciate ligament (ACL). The purpose of this study was to evaluate the positions of femoral and tibial tunnels created using an accessory anteromedial portal technique in single bundle ACL reconstruction. MATERIALS AND METHODS: The femoral tunnel was targeted at the mid-portion of the ACL bundles. We evaluated postoperative computed tomography scans of 32 patients treated by ACL reconstruction using a free-hand low accessory anteromedial portal technique. On the tibial side, the tunnel position was evaluated using Tsukada's method. On the femoral side, the position was evaluated using 1) the quadrant method, 2) Mochizuki's method, 3) Mochizuki's method, and 4) Takahashi's method. Tunnel obliquity was also evaluated. RESULTS: The mean tibial tunnel position was located at 44.6%+/-2.5% anterior from the anterior margin and 48.0%+/-3.0% in medial from the medial margin. The mean femoral tunnel position was located at the center between the anteromedial and posterolateral bundles: Quadrant method, 26.7%+/-2.7%/30.0%+/-2.9%; Watanabe's method, 37.7%+/-2.5%/26.6%+/-2.2%; Mochizuki's method, 38.7%+/-2.7%; Takahashi's method, 21.8%+/-2.2%. The mean femoral tunnel obliquity was 57.7degrees+/-6.2degrees in the sagittal plane and 49.9degrees+/-5.6degrees in the coronal plane. CONCLUSIONS: In anatomic single bundle ACL reconstruction, the low anteromedial portal technique can restore accurate position of the native footprint. Accurate femoral tunnel position facilitates recovery of stability and decreases graft failure rate.
Anterior Cruciate Ligament ; Anterior Cruciate Ligament Reconstruction ; Arthroscopy ; Humans ; Knee ; Transplants

BACKGROUND: This study examines the clinical and radiologic results of ceramic-on-ceramic total hip arthroplasties with regard to wear, osteolysis, and fracture of the ceramic after a minimum follow-up of six years. METHODS: We evaluated the results of a consecutive series of 148 primary ceramic-on-ceramic total hip arthroplasties that had been performed between May 2001 and October 2005 in 142 patients. The mean age was 57.2 years (range, 23 to 81 years). The mean follow-up period was 7.8 years (range, 6.1 to 10.1 years). Preoperative diagnosis was avascular necrosis in 77 hips (52%), degenerative arthritis in 36 hips (24.3%), femur neck fracture in 18 hips (12.2%), rheumatoid arthritis in 15 hips (10.1%), and septic hip sequelae in 2 hips (1.4%). Clinical results were evaluated with the Harris hip score, and the presence of postoperative groin or thigh pain. Radiologic analysis was done with special attention in terms of wear, periprosthetic osteolysis, and ceramic failures. RESULTS: The mean Harris hip score improved from 58.3 (range, 10 to 73) to 92.5 (range, 79 to 100) on the latest follow-up evaluation. At final follow-up, groin pain was found in 4 hips (2.7%), and thigh pain was found in 6 hips (4.1%). Radiologically, all femoral stems demonstrated stable fixations without loosening. Radiolucent lines were observed around the stem in 25 hips (16.9%), and around the cup in 4 hips (2.7%). Endosteal new bone formation was observed around the stem in 95 hips (64.2%) and around the cup in 88 hips (59.5%). No osteolysis was observed around the stem and cup. There were 2 hips (1.4%) of inclination changes of acetabular cup, 2 hips (1.4%) of hip dislocation, 1 hip (0.7%) of ceramic head fracture, and 1 hip (0.7%) of squeaking. The Kaplan-Meier survival rate of the prostheses was 98.1% at postoperative 7.8 years. CONCLUSIONS: The ceramic-on-ceramic total hip arthroplasty produced excellent clinical results and implant survival rates with no detectable osteolysis on a minimum six-year follow-up study. The ceramic-on-ceramic couplings could be a reasonable option of primary total hip arthroplasty for variable indications.
Adult ; Aged ; Aged, 80 and over ; Arthroplasty, Replacement, Hip/adverse effects/instrumentation/*methods ; *Ceramics ; Female ; Follow-Up Studies ; Hip Joint/radiography/surgery ; *Hip Prosthesis ; Humans ; Kaplan-Meier Estimate ; Male ; Middle Aged ; Pain, Postoperative/etiology ; Prosthesis Failure ; Range of Motion, Articular

PURPOSE: There was a global increase in the prevalence of oseltamivir-resistant influenza viruses during the 2007-2008 influenza season. This study was conducted to investigate the occurrence and characteristics of oseltamivir-resistant influenza viruses during the 2007-2008 and 2008-2009 influenza seasons among patients who were treated with oseltamivir (group A) and those that did not receive oseltamivir (group B). METHODS: A prospective study was conducted on 321 pediatric patients who were hospitalized because of influenza during the 2007-2008 and 2008-2009 influenza seasons. Drug resistance tests were conducted on influenza viruses isolated from 91 patients. RESULTS: There was no significant difference between the clinical characteristics of groups A and B during both seasons. Influenza A/H1N1, isolated from both groups A and B during the 2007-2008 and 2008-2009 periods, was not resistant to zanamivir. However, phenotypic analysis of the virus revealed a high oseltamivir IC50 range and that H275Y substitution of the neuraminidase (NA) gene and partial variation of the hemagglutinin (HA) gene did not affect its antigenicity to the HA vaccine even though group A had a shorter hospitalization duration and fewer lower respiratory tract complications than group B. In addition, there was no significant difference in the clinical manifestations between oseltamivir-susceptible and oseltamivir-resistant strains of influenza A/H1N1. CONCLUSION: Establishment of guidelines to efficiently treat influenza with oseltamivir, a commonly used drug for treating influenza in Korean pediatric patients, and a treatment strategy with a new therapeutic agent is required.
Child ; Drug Resistance ; Hemagglutinins ; Hospitalization ; Humans ; Influenza, Human ; Inhibitory Concentration 50 ; Neuraminidase ; Orthomyxoviridae ; Oseltamivir ; Prevalence ; Prospective Studies ; Respiratory System ; Seasons ; Viruses ; Zanamivir

The ulcerative colitis is a chronic inflammatory bowel disease with an unknown etiology. The major symptoms of ulcerative colitis are diarrhea, abdominal pain and hematochezia. However, arthritis, skin disorders, hepatobiliary inflammation and uveitis are occasionally recognized as systemic complications. Although there are few reports of coexistent pulmonary and inflammatory bowel disease, the lung is not generally considered to be a target organ in ulcerative colitis. We report a patient with ulcerative colitis-related bronchilolitis obliterans organizing pneumonia confirmed by video-assisted thoracoscopic surgery, who responded to corticosteroid therapy.
Abdominal Pain ; Arthritis ; Colitis, Ulcerative ; Cryptogenic Organizing Pneumonia ; Diarrhea ; Gastrointestinal Hemorrhage ; Humans ; Inflammation ; Inflammatory Bowel Diseases ; Lung ; Pneumonia ; Skin ; Thoracic Surgery, Video-Assisted ; Ulcer ; Uveitis