Prefoldin 6 mediates longevity response from heat shock factor 1 to FOXO in C. elegans.

Heat shock factor 1 (HSF-1) and forkhead box O (FOXO) are key transcription factors that protect cells from various stresses. In Caenorhabditis elegans, HSF-1 and FOXO together promote a long life span when insulin/IGF-1 signaling (IIS) is reduced. However, it remains poorly understood how HSF-1 and FOXO cooperate to confer IIS-mediated longevity. Here, we show that prefoldin 6 (PFD-6), a component of the molecular chaperone prefoldin-like complex, relays longevity response from HSF-1 to FOXO under reduced IIS. We found that PFD-6 was specifically required for reduced IIS-mediated longevity by acting in the intestine and hypodermis. We showed that HSF-1 increased the levels of PFD-6 proteins, which in turn directly bound FOXO and enhanced its transcriptional activity. Our work suggests that the prefoldin-like chaperone complex mediates longevity response from HSF-1 to FOXO to increase the life span in animals with reduced IIS.

Clinical evaluation of usefulness and effectiveness of sitting type continuous passive motion machines in patients with total knee arthroplasty: a study protocol for a single-blinded randomized controlled trial.

BACKGROUND: Total knee arthroplasty (TKA) is an important management strategy for patients with knee osteoarthritis (OA) refractory to conservative management. Postoperative range of motion (ROM) exercise is important to recover patients' activities of daily living. Continuous passive motion (CPM) is a machine that provides passive ROM exercises of the knee joint in a pre-defined arc of motion. The short- and long-term effects of CPM exercise are controversial. We hypothesized that the inconsistent results of the CPM exercise are due to poor fitting of CPM machines and measurement errors. This study aims to present a protocol for investigating a new type of CPM machine that could be applied in a sitting position in comparison with the conventional type of CPM machine for patients with unilateral TKAs. METHODS: This study presents the protocol of a prospective, multicenter, single-blinded, three-armed randomized controlled trial (RCT). One hundred and twenty-six patients receiving unilateral TKAs will be recruited at the physical medicine and rehabilitation clinics of two urban tertiary medical hospitals. The patients were randomly divided into three groups with a 1:1:1 allocation. The intervention group will receive two weeks of post-operative rehabilitation using a new type of CPM machine. The control group will receive 2 weeks of post-operative rehabilitation using conventional CPM machines. The third group will receive post-operative rehabilitation with both types of CPM machines. The primary outcome will be the change in the passive ROM of the affected knee joint from baseline to 2 weeks after baseline assessment. The secondary outcomes will be pain and functional measurements, and will include patient-reported outcomes and performance tests surveyed at multiple time points up to 3 months after TKA. DISCUSSION: This is the first RCT to investigate the effect of a new type of CPM machine. The results of this RCT will determine whether the position of the patients during CPM exercise is important in post-operative rehabilitation protocols after TKAs and will provide evidence for the development of proper rehabilitation guidelines after TKAs. TRIAL REGISTRATION: Clinical Research Information Service of Republic of Korea, KCT0005520, Registered on 21 October 2020, https://cris.nih.go.kr/cris/search/detailSearch.do/21750.

Paediatric heart transplantation recipients ≥7 years of age receiving donors with pre-existing coronary atherosclerosis showed progressive coronary artery disease.

BACKGROUND: This study aimed to determine the effect of donor-transmitted atherosclerosis on the late aggravation of cardiac allograft vasculopathy in paediatric heart recipients aged ≥7 years. METHODS: In total, 48 patients were included and 23 had donor-transmitted atherosclerosis (baseline maximal intimal thickness of >0.5 mm on intravascular ultrasonography). Logistic regression analyses were performed to identify risk factors for donor-transmitted atherosclerosis. Rates of survival free from the late aggravation of cardiac allograft vasculopathy (new or worsening cardiac allograft vasculopathy on following angiograms, starting 1 year after transplantation) in each patient group were estimated using the Kaplan-Meier method and compared using the log-rank test. The effect of the results of intravascular ultrasonography at 1 year after transplantation on the late aggravation of cardiac allograft vasculopathy, correcting for possible covariates including donor-transmitted atherosclerosis, was examined using the Cox proportional hazards model. RESULTS: The mean follow-up duration after transplantation was 5.97 ± 3.58 years. The log-rank test showed that patients with donor-transmitted atherosclerosis had worse survival outcomes than those without (p = 0.008). Per the multivariate model considering the difference of maximal intimal thickness between baseline and 1 year following transplantation (hazard ratio, 22.985; 95% confidence interval, 1.948-271.250; p = 0.013), donor-transmitted atherosclerosis was a significant covariate (hazard ratio, 4.013; 95% confidence interval, 1.047-15.376; p = 0.043). CONCLUSION: Paediatric heart transplantation recipients with donor-transmitted atherosclerosis aged ≥7 years had worse late cardiac allograft vasculopathy aggravation-free survival outcomes.

Clinical implication of intraoperative ventricular-arterial coupling in pediatric patients undergoing ventricular septal defects repair: A retrospective cohort study.

BACKGROUND: Ventricular-arterial coupling is the ratio of arterial elastance to ventricular end-systolic elastance. AIMS: The objective of this study was to determine the clinical implication of intraoperative ventricular-arterial coupling derived from the pressure-area relationship using transesophageal echocardiography. METHODS: This retrospective study reviewed the medical records of 72 pediatric patients with ventricular septal defects who underwent corrective surgery with cardiopulmonary bypass. The single-beat modified method was used to assess ventricular-arterial coupling. Logistic regression analyses were performed to determine the correlation between ventricular-arterial coupling and early postoperative outcomes, including the maximum vasoactive-inotropic score, length of mechanical ventilation, and length of hospital stay. RESULTS: Ventricular-arterial coupling after cardiopulmonary bypass significantly increased (from 1.0 ± 0.4 to 1.4 ± 0.8, p < .001), indicating a disproportionate increase in the arterial elastance index (from 11.5 ± 5.1 to 19.8 ± 7.5 mmHg/cm2 /m2 , p < .001) compared with the ventricular end-systolic elastance index (from 13.0 ± 6.9 to 16.9 ± 9.0 mmHg/cm2 /m2 , p < .001). Logistic regression analyses revealed that high postoperative ventricular-arterial coupling was independently associated with higher postoperative maximum vasoactive-inotropic score (>10; odds ratio [OR], 8.04; 95% confidence interval [CI], 1.38-46.85, p = .020), longer postoperative mechanical ventilation (>15 h; OR: 11.00; 95% CI: 1.26-96.45, p = .030), and longer postoperative hospital stay (>7 days; OR: 2.98; 95% CI: 1.04-8.58, p = .043). CONCLUSIONS: Ventricular-arterial coupling can be easily obtained from the intraoperative transesophageal echocardiography in pediatric patients undergoing ventricular septal defects repair. High postoperative ventricular-arterial coupling is strongly associated with worse early postoperative outcomes. Ventricular-arterial coupling shows promise as an intraoperative analysis tool that can provide insight into the impact of interventions on cardiovascular performance and identify potential targets for treatment in this population.

Predictive Parameters of Decreased Left Ventricular Global Longitudinal Strain at 1 Month After Pediatric Heart Transplantation.

Previous reports indicate that the decreased left ventricular global longitudinal strain (LVGLS) seen in the early postoperative period of pediatric heart transplant patients generally recovers over the course of 1-2 years. In this study, we investigate the predictive capacity of preoperative parameters on the LVGLS decline seen at 1 month post transplant. Forty-six transplant subjects with 2D echocardiographic images sufficient for speckle tracking echocardiography were enrolled. We excluded patients diagnosed with cardiac allograft vasculopathy or with an episode of rejection 1 month before or after their echocardiographic examinations. The mean LVGLS was significantly reduced at 1 month when compared to 1 year following transplant (- 15.5% vs. - 19.4%, respectively, p < 0.001). The predictors of LVGLS that decline at 1 month were the LV mass z-score [odds ratio (OR) 1.452; 95% confidence interval (CI) 1.007-2.095, p = 0.046], recipient age (OR 1.124; 95% CI 1.015-1.245, p = 0.025), and donor age (OR 1.081; 95% CI 1.028-1.136, p = 0.002) in the univariate logistic regression analyses. Although multivariate analysis yielded no significant predictors, higher LV mass z-scores showed a trend associated with the decline of LVGLS (p = 0.087). The donor/recipient weight ratio was associated with the LV mass z-score (R2 = 0.412, p < 0.001).

Machine-learning algorithm that can improve the diagnostic accuracy of septic arthritis of the knee.

PURPOSE: Prompt diagnosis and treatment of septic arthritis of the knee is crucial. Nevertheless, the quality of evidence for the diagnosis of septic arthritis is low. In this study, the authors developed a machine learning-based diagnostic algorithm for septic arthritis of the native knee using clinical data in an emergency department and validated its diagnostic accuracy. METHODS: Patients (n = 326) who underwent synovial fluid analysis at the emergency department for suspected septic arthritis of the knee were enrolled. Septic arthritis was diagnosed in 164 of the patients (50.3%) using modified Newman criteria. Clinical characteristics of septic and inflammatory arthritis were compared. Area under the receiver-operating characteristic (ROC) curve (AUC) statistics was applied to evaluate the efficacy of each variable for the diagnosis of septic arthritis. The dataset was divided into independent training and test sets (comprising 80% and 20%, respectively, of the data). Supervised machine-learning techniques (random forest and eXtreme Gradient Boosting: XGBoost) were applied to develop a diagnostic model using the training dataset. The test dataset was subsequently used to validate the developed model. The ROC curves of the machine-learning model and each variable were compared. RESULTS: Synovial white blood cell (WBC) count was significantly higher in septic arthritis than in inflammatory arthritis in the multivariate analysis (P = 0.001). In the ROC comparison analysis, synovial WBC count yielded a significantly higher AUC than all other single variables (P = 0.002). The diagnostic model using the XGBoost algorithm yielded a higher AUC (0.831, 95% confidence interval 0.751-0.923) than synovial WBC count (0.740, 95% confidence interval 0.684-0.791; P = 0.033). The developed algorithm was deployed as a free access web-based application ( www.septicknee.com ). CONCLUSION: The diagnosis of septic arthritis of the knee might be improved using a machine learning-based prediction model. LEVEL OF EVIDENCE: Diagnostic study Level III (Case-control study).

Autogenous Bone Graft With Kirschner Wire Fixation Could Be a Reliable Treatment Option for Scaphoid Nonunion in Adolescents.

BACKGROUND: Scaphoid nonunion in adolescents is rare, and most treatments include prolonged immobilization and screw fixation. Many studies have shown that Kirschner wires (K-wires) show comparable outcomes in screw fixation in adult scaphoid nonunion. However, few studies have reported K-wire fixation results in the treatment of adolescent scaphoid nonunion. The purpose of this study was to evaluate the clinical and radiologic results after bone graft and K-wire fixation for scaphoid nonunion in adolescents. METHODS: We retrospectively reviewed 12 adolescent patients, mean age 15.4 (12 to 17) years, who underwent surgical treatment of scaphoid nonunion. Autogenous bone grafts with K-wire fixation were performed for all patients. Radiologic results, including bone union and degenerative changes, were evaluated with serial radiographs. Clinical results, including range of motion, grip strength, and a visual analogue scale for pain, were assessed. RESULTS: All patients received <3 years of postoperative follow-up assessments. Stable bony union was achieved in all patients. Radiologic bony unions were identified at an average of 11.4 weeks postoperatively (range, 9 to 15 wk). The mean active range of motion of the injured wrist at 3 years postoperatively was 215 degrees (range, 185 to 230 degrees). None of the patients were treated for wrist pain or needed medication during follow-up. Three patients experienced intermittent pain (visual analogue scale 1) after heavy work. The grip strength was reduced compared with the uninjured hand (31.8 and 32.8 kg, respectively), but there was no statistically significant difference (P=0.19). All patients had the K-wire removed at 12 weeks postoperatively. There was no degenerative change in the scaphoid at the final follow-up radiograph. CONCLUSION: Autogenous bone graft with K-wire fixation could be a reliable treatment option of scaphoid nonunion in adolescents. LEVEL OF EVIDENCE: Level IV-retrospective case series.

Comparable Outcome with a Faster Engraftment of Optimized Haploidentical Hematopoietic Stem Cell Transplantation Compared with Transplantations from Other Donor Types in Pediatric Acquired Aplastic Anemia.

Haploidentical family donors have been used as an alternative source in hematopoietic cell transplantation for patients with severe aplastic anemia. We evaluated and compared the outcomes of transplantation in pediatric acquired severe aplastic anemia based on donor type. Sixty-seven patients who underwent transplantation between 1998 and 2017 were included. Fourteen patients received grafts from matched sibling donors, 21 from suitable unrelated donors, and 32 from haploidentical family donors. Ex vivo CD3+ or αß+ T cell-depleted grafts were used for haploidentical transplantation. Sixty-five patients (97.0%) achieved neutrophil engraftment at a median of 11 days. Haploidentical transplantation resulted in significantly faster neutrophil engraftment at a median of 10 days, compared with 14 days in cases of matched sibling donors and 12 days in cases of unrelated donor recipients. Nine patients experienced graft failure, and 5 of 7 who underwent a second transplantation are alive. There was no difference in the incidence of acute or chronic graft-versus-host disease based on donor type. The 5-year overall survival and failure-free survival rates were 93.8% ± 3.0% and 83.3% ± 4.6%, respectively, and there was no significant survival difference based on donor type. The survival outcomes of haploidentical transplantation in patients were comparable with those of matched sibling or unrelated donor transplantation. Optimized haploidentical transplantation using selective T cell depletion and conditioning regimens including low-dose total body irradiation for enhancing engraftment may be a realistic therapeutic option for pediatric patients with severe aplastic anemia.

Reconstitution of T and NK cells after haploidentical hematopoietic cell transplantation using αß T cell-depleted grafts and the clinical implication of γδ T cells.

To investigate reconstitution of T and NK cells after αß T lymphocyte-depleted haploidentical hematopoietic cell transplantation (HHCT) and the clinical implications of γδ T cells, we analyzed 50 pediatric patients who received 55 HHCTs using αß T cell-depleted grafts. The number of CD3+ T cells and CD8+ T cells recovered rapidly and reached donor levels at days 180 and 60, respectively. Recovery of NK cells was rapid, and the median of NK cells at day 14 was comparable to the donor level. At day 14, median percentage of γδ T lymphocytes was 70.5%. After day 14, the percentage of γδ T cells gradually decreased, while the percentage of αß T cells gradually increased. Patients with a low percentage (≤21%) of γδ T cells at day 30 had significantly higher incidence of cytomegalovirus (CMV) reactivation compared to patients with a high percentage (>70%) of γδ T cells (P < .01). In patients with acute leukemia, patients with high percentage of γδ T cells at day 30 showed significantly higher relapse-free survival compared to those with low percentage of γδ T cells (P = .02). Data suggest that early recovery of γδ T cells decreases the risk of CMV reactivation and leukemia relapse.

Depletion of αß+ T cells for a haploidentical hematopoietic stem cell transplantation in children.

A consistent and reproducible depletion technique is crucial for the successful transplantation of an ex vivo depleted graft. Our aim was to evaluate the efficacy of an ex vivo technique for depletion of αß+ T cells using a biotinylated anti-TCRαß monoclonal antibody, which was performed by one clinical nurse specialist. Between 2012 and 2017, 119 depletion procedures from 216 apheresis using the anti-TCRαß monoclonal antibody were performed on 105 pediatric patients. The median log depletion of αß+ T cells was 4.0 (range, 2.5-5.0). The median recovery rates of CD34+ , NK, and γδ+ T cells were 90.4%, 74.9%, and 75.9%, respectively. The efficacy of depletion of αß+ T cells significantly improved over time and the duration of the depletion procedure significantly decreased over time. Our study demonstrated that this procedure for depletion of αß+ T cells by skilled staff is highly effective at depleting target cells and obtaining CD34+ progenitor cells.

Hepatic Stiffness Using Shear Wave Elastography and the Related Factors for a Fontan Circulation.

Hepatic problems related to a Fontan circulation have been highlighted and elastography using ultrasound is a non-invasive tool that can measure the severity of hepatic stiffness. We investigated the hepatic stiffness using shear wave elastography (SWE) and related factors in patients with a Fontan circulation. This study enrolled 64 patients with a Fontan circulation who underwent cardiac catheterization and abdominal ultrasound from 2011 to 2015. The correlation between the laboratory tests, hemodynamic factors by cardiac catheterization, and SWE was evaluated. The patients were classified into non-cirrhotic level (≥ 2.0 m/s) and cirrhotic level (< 2.0 m/s) groups by the SWE value. The mean age was 17.6 years and the mean duration after the Fontan operation was 12.1 years. The mean value of SWE in patients (1.95 m/s) was higher than the normal (< 1.3 m/s). The SWE was higher in patients without than those with a fenestration (2.03 vs. 1.75 m/s, P = 0.003). In a multiple regression analysis between SWE and other factors, the CVP, fenestration, and lipoprotein Apo B had a significant correlation. In a multivariate analysis of cirrhotic level group, the CVP was the only significant factor. The hepatic stiffness had significantly progressed in most patients with a Fontan circulation. A low CVP and Fontan circulation with a fenestration might reduce the progression of the hepatic stiffness.

Hematopoietic stem cell transplantation in pediatric patients with acute myeloid leukemia without favorable cytogenetics.

Intensified chemotherapy, HSCT, and supportive care improve the survival of pediatric patients with AML. However, no consensus has been reached regarding the role of HSCT in patients without favorable cytogenetics. We evaluated OS and EFS according to prognostic factors that affect clinical outcomes, including cytogenetics risk group, conditioning regimen, donor type, disease status at the time of HSCT, and number of chemotherapy cycles prior to HSCT in 65 pediatric patients with AML without favorable cytogenetics who underwent HSCT. Fifteen of the 65 patients died: three of TRM and 12 of disease-related mortality. The 5-year OS and EFS were 78.0% and 72.0%, respectively, and the 5-year cumulative relapse and TRM rates were 26.9% and 5.1%, respectively. Survival rates were not influenced by cytogenetic group (intermediated vs. poor), donor type (related vs. unrelated), transplant type (myeloablative vs. reduced-intensity conditioning), or number of pretransplant chemotherapy cycles (≤3 vs. >3 cycles). The low TRM rate and encouraging outcomes suggest that HSCT may be a feasible treatment for pediatric patients with AML without favorable cytogenetics.

Temperature Dependence According to Grain Boundary Potential Barrier Variation in Vertical NAND Flash Cell with Polycrystalline-Silicon Channel.

We investigate the electrical characteristics according to changing temperature on trap distribution in the energy gap of grain boundary (GB) and interface trap density (D(it)) between polycrystalline-silicon (poly-Si) channel and tunnel oxide in Vertical NAND (VNAND) flash cell with poly-Si channel. We confirmed that there are two factors changing GB potential barrier height such as trap distribution in GB and D(it) using technology computer-aided design (TCAD) simulation. Also, we found that the electrical characteristics according to changing temperature are significantly dependent on height and position of GB potential barrier in VNAND flash cell with poly-Si channel. We expect that it is required to develop more accurate extraction method for trap distribution in each GB and D(it) for better understanding temperature dependence of electrical characteristics in VNAND Flash cell.

Ultrasonography-guided reduction of pediatric radial neck fractures.

BACKGROUND: Treatment of displaced and angulated radial neck fractures in children is controversial and challenging. Numerous studies have been conducted regarding treatment algorithms and surgical techniques that use fluoroscopy. However, ultrasonography (US)-guided reduction of pediatric radial neck fractures has not been reported yet. We aimed to determine the safety and efficacy of US-guided reduction and fixation of radial neck fractures in children. METHODS: Among 28 cases of radial neck fracture from 2014 to 2016, 12 were classified as type III or IV according to the Judet classification. All 12 patients underwent US-guided reduction and percutaneous fixation with Kirschner wire and follow-up for more than 6 months. US was used primarily to monitor the angulation and reduction of the radial neck. Fluoroscopy was applied to confirm the fixation with Kirschner wire. Dose area product (DAP; mGy/cm2) was measured to assess per-procedure radiation dose. Radiological and clinical results were evaluated at 6 months after the surgery by using the Metaizeau criteria. RESULTS: Of the patients, 4 were boys and 8 were girls, with a mean age of 7.7 years (range, 5-11 years). Judet type III fractures accounted for 83% of all injuries. The mean preoperative radial angulation was 62.5° (range: 46°-76°). The mean postoperative radial angulation was 5.6° (range: 2°-9°). The mean fluoroscopy time was 31 s (range: 10-73 s), and the mean DAP was 10.7 mGy/cm2 (range: 7.2-18.7 mGy/cm2). The mean follow-up period was 18.3 months (range, 8-24 months). According to the Metaizeau criteria, 10 cases were excellent and 2 cases were good at the last follow-up. CONCLUSIONS: US-guided reduction and percutaneous fixation is safe and reliable option to treat displaced radial neck fractures in children.

Percutaneous Cementoplasty For Acetabulum In Patients With Bone Metastasis.

The purpose of this study is to demonstrate the surgical technique and to show the results of percutaneous cementoplasty (PC) for acetabular metastases using lateral approach under regional anesthesia. Forty-two cases underwent PC for acetabular metastases. The PC was performed using spinal anesthesia, lateral approach and fluoroscopic guidance. We assessed visual analogue scale (VAS) and revised musculoskeletal tumor society (MSTS) rating system and maximum standardized uptake value (SUVmax) of the acetabular lesion using F-18-FDG PET/CT before and after the PC. The mean injected volume of polymethylmethacrylamide to the pelvis was 21±11.8 ml. The mean of regional VAS (6.2±1.1 vs. 3.1±2.7, p<0.001), MSTS (10.3±3.9 vs. 18.3±3.2, p<0.001) and local SUVmax (8.6±5.2 vs. 5.7±3.6 , p = 0.012) on PET/CT showed significant reductions after surgery. Twenty-three patients (55%) died of disease at mean 11.8±4.8 months after surgery. PC using lateral approach and regional anesthesia could be a simple and safe surgical method for relieving pain and maintaining skeletal stability against acetabular metastasis.

Risk Factors for Femoral Head Deformity in the Early Stage of Legg-Calvé-Perthes Disease: MR Contrast Enhancement and Diffusion Indexes.

PURPOSE: To determine whether changes in diffusion and/or contrast enhancement are of prognostic value in the early stage of Legg-Calvé-Perthes disease (LCPD). MATERIALS AND METHODS: This study was approved by the institutional review board, and written informed consent was obtained from patient parents. Diffusion and contrast agent-enhanced magnetic resonance (MR) imaging studies were performed in 46 children (37 boys and nine girls; mean age, 7.5 years [age range, 3.3-11.9 years]) with unilateral LCPD at the early stage before development of extensive femoral head deformity. The degree of contrast enhancement was measured on the contrast-enhanced MR images, and the apparent diffusion coefficient (ADC) value was measured on the ADC map at various regions of interest in the proximal femur. The association of the MR imaging parameters that compared the affected side with the contralateral normal side with the femoral head deformity index value above 0.3 at 2 years was investigated. RESULTS: Increased diffusion in the metaphysis (P = .003) and decreased contrast enhancement in the central epiphysis (P = .034) were the significant prognostic indicators of subsequent femoral head deformation. ADC in the metaphysis 45% higher and a contrast enhancement in the central epiphysis 37% lower than those of the contralateral normal side are associated with a nonfavorable prognosis. For diffusion MR imaging, sensitivity was 83% (15 of 18), specificity was 86% (24 of 28), positive predictive value was 79% (15 of 19), negative predictive value was 89% (24 of 27), and accuracy was 85% (39 of 46). For contrast-enhanced MR imaging, sensitivity was 78% (14 of 18), specificity was 64% (18 of 28), positive predictive value was 58% (14 of 24), negative predictive value was 82% (18 of 22), and accuracy was 70% (32 of 46). CONCLUSION: Diffusion and contrast-enhanced MR imaging are potentially useful to assess risk of later development of femoral head deformity.

Successful use of brentuximab vedotin for refractory anaplastic large cell lymphoma as a bridging therapy to haploidentical stem cell transplantation and maintenance therapy post-transplantation.

Brentuximab vedotin (BV) is a monoclonal antibody-drug conjugate that targets CD30, and has been reported to be effective for relapsed/refractory anaplastic large cell lymphoma. We here report a patient who experienced multiple relapses after conventional chemotherapy and autologous hematopoietic stem cell transplantation (HSCT). He achieved a complete metabolic response with BV and proceeded to undergo haploidentical HSCT. After HSCT, he received three doses of BV to prevent an early relapse, and remains in remission 16 months post-transplantation. Our case suggests the potential use of BV both as a bridging therapy to allogeneic HSCT and as a maintenance therapy post-transplantation.

Surgical Results of Anomalous Origin of One Pulmonary Artery Branch from the Ascending Aorta.

We reviewed our surgical experience with anomalous origin of one pulmonary artery from the ascending aorta (AOPA). From 1989 to 2012, 12 children (five neonates) aged 3-734 days (mean 152 ± 222) with AOPA underwent operations. Eight patients had right AOPA, and four patients had left AOPA. The majority of the patients had elevated right ventricular pressure, with 58 % (7 of 12) demonstrating suprasystemic right ventricular pressure. Surgery was performed by direct anastomosis (group 1) in seven patients and by employing an autologous patch (group 2) in five patients. There were two postoperative mortalities caused by heart failure and pulmonary hypertensive crisis. The mean follow-up duration was 12.6 ± 8 years. Catheterization showed that the right ventricle-to-systemic pressure ratio decreased following operation (preoperative vs. postoperative; 1.13 ± 0.19 vs. 0.48 ± 0.03, p = 0.043). There was no difference in the perfusion of the affected lung as measured by the final lung perfusion scan, between the two groups (group 1 vs. group 2; 50.0 ± 10.3 vs. 42.7 ± 28.7 %, p = 0.158). Two patients required reoperations for pulmonary regurgitation and pulmonary artery stenosis. There were two catheter-based interventions. At 20 years, survival by the Kaplan-Meier was 91.7 ± 8.0 %, freedom from reoperation was 80.0 ± 17.9 %, and freedom from catheter intervention was 80.8 ± 12.2 %. Early repair of AOPA improves right ventricular pressure and overall hemodynamics with excellent survival and low risk of reintervention. The type of surgical repair did not significantly affect the long-term outcomes (measured via lung perfusion scan).

Percutaneous epiphysiodesis using transphyseal screws in the management of leg length discrepancy: optimal operation timing and techniques to avoid complications.

BACKGROUND: Percutaneous epiphysiodesis using transphyseal screws (PETS) has been used to manage leg length discrepancy (LLD) in growing children. The purposes of this study were to analyze effects of PETS on LLD, its associated complications, to determine optimal operation timing, and find ways of preventing complications. PATIENTS AND METHODS: The data of 59 patients obtained up to screw removal or at skeletal maturity were retrospectively analyzed. Retrospective growth calculations were done using multiplier method. The efficacy of LLD correction was calculated, and the predicted segment length with the index operation was compared with the final measured length and final LLD was measured. The screw insertion angle in 3-dimension was calculated, and it was correlated with the efficacy. Complications associated with screw design and the techniques used were analyzed. RESULTS: The LLD correction efficacy averaged 75.5% (5.0 to 114.0) at the distal femur and 78.9% (11.0 to 111.0) at the proximal tibia. However, mean final LLD was 3.0 mm (range, -10.0 to 16.7 mm), presumably because operations were performed on average 1.3 years earlier than estimated by growth calculation. Three-dimensional screw insertion angle was positively correlated with LLD correction efficacy. Complications were closely related to the screw design and the implantation techniques. CONCLUSIONS: PETS provides a minimally invasive and effective means of LLD correction. In view of its delayed effect, we recommend that PETS be performed at least 1 year earlier than estimated optimal epiphysiodesis timing. The careful selection of screw design and length and accurate screw placement are the keys to successful results. LEVEL OF EVIDENCE: Level IV prognostic studies.

Elevated preoperative systemic inflammatory markers predict poor outcome in localized soft tissue sarcoma.

BACKGROUND: Systemic inflammation has been implicated in cancer development and progression. This study sought to determine whether systemic inflammatory markers can predict postoperative outcome in soft tissue sarcoma (STS). METHODS: A total of 162 cases of primary, localized STS were reviewed. Patients with evidence of infectious or inflammatory diseases were excluded. The mean follow-up period was 46.7 months. The level of serum C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), and neutrophil-to-lymphocyte ratio (NLR) measured before surgery were evaluated for association with disease-specific survival and local recurrence. RESULTS: The mean values of CRP, ESR, and NLR were 0.79 mg/dL, 18.8 mm/h, and 2.12, respectively. Cutoff values derived from receiver-operating characteristic curve analysis were 0.20 mg/dL for CRP, 10.0 mm/h for ESR, and 2.50 for NLR. On univariate analysis, all inflammatory markers were associated with disease-specific survival (CRP: P = 0.007; ESR: P = 0.022; NLR: P = 0.030). On multivariate analysis, the CRP level (P = 0.019) and ESR (P = 0.013) remained significant. Elevation of multiple markers was a more significant prognostic factor than elevation of a single marker (P = 0.001). However, none of the inflammatory markers was associated with local recurrence (CRP: P = 0.345; ESR: P = 0.271; NLR: P = 0.570). Histologic grade was strongly correlated with inflammatory marker values (NLR: P < 0.001; ESR: P = 0.002; CRP: P = 0.007). CONCLUSIONS: Preoperative systemic inflammatory status, assessed by using multiple serum markers, predicted disease-specific survival in STS.