Morphological analysis of patellofemoral joint in haemophilic arthropathy: A case-control study.

INTRODUCTION: Knees affected by haemophilic arthropathy exhibit distinct differences in both bone morphology and soft tissue behaviour. This study aims to analyse the morphological characteristics of the distal femur and patellofemoral joint in patients with haemophilia in comparison to normal healthy population. MATERIAL AND METHODS: Study was conducted as pair-matched case-control study with 43 individuals in both the haemophilia group and the control group. Patellar luxation, patellar tilt (PT), length of the patella in both axis (pAP, pML), depth and angle of trochlear sulcus (SD, SA), lateral trochlear inclination (LTI), medial and lateral femoral facet length (mFL, LFL), intercondylar depth (ID), transepicondylar axis (TEA) and lateral condyle length (LCL) were assessed on knee MRI. Correlation between Pettersson score and measured variables were also analysed. RESULTS: PT was medial sided in 10 (23.2%) cases in haemophilic group. Mean values of pAP, pML, PT were significantly lower in haemophilia group (p < .001, p: .007, p = .001 respectively). There were no significant changes in SA (p = .628), SD (p = .340), LTI (p = .685), LFL (p = .241) and MFC-LFC (p = .770) whilst mFL was significantly longer in haemophilia group (p = .009). ID (p < .001), TEA (p = .007) and LCL (p = .001) were all shorter in haemophilia group. Pettersson score was inversely correlated with pAP, pML, ID, TEA, LCL, pML/SA and ID/LCL. CONCLUSION: Morphological changes in haemophilic arthropathy involve a smaller and medially-tilted patella, narrowed lateral condyle and transepicondylar axis, combined with reduced intercondylar depth. These alterations must keep in mind especially in pre- and intraoperative assessments for arthroplasty procedures.

Comparison of Testicular Sonography and Elastography Findings With Semen Parameters in Cases Investigated for Infertility.

OBJECTIVES: This study aimed to investigate the correlation between testicular shear wave elastography (SWE) values and semen analysis results in men with infertility. METHODS: This was a retrospective case-control study. Patients were categorized as normal, abnormal, or azoospermic based on sperm analysis results. Testicular volume was measured using B-mode ultrasonography using the Lambert formula. Subsequently, 40-80 regions of interest measuring 1.5 × 1.5 mm were manually positioned in both testicles based on their size, and two-dimensional SWE was applied through virtual touch imaging quantification software. RESULTS: The patients had a mean age of 33.79 ± 6.3 years, with semen analysis revealing normal results in 15 patients (22.4%), pathological findings in 35 patients (52.2%), and azoospermia in 17 patients (25.4%). Right, left, total, and mean testicular volumes were significantly lower in patients with azoospermia compared to those in both normal and impaired semen parameters (P < .05). Conversely, testicular elastography scores were higher in patients with azoospermia than in the other groups (P < .05). The significant negative correlation between volume and elastographic findings remained independent of age (r = 0.4, P < .001). The accuracy rates for detecting impaired semen parameters and azoospermia were 94.3% and 94.1%, respectively, after considering factors such as age, testicular volume (right/left/total), and elastography (right/left/total). Notably, the total mean elastography score ranked first, with 100% in the independent normalized importance distribution of these variables. CONCLUSION: SWE can be used effectively alone or in combination with other diagnostic tools to evaluate histopathological changes in the testicles of male patients with infertility.

Preoperative radiotherapy with concomitant chemotherapy in extremity soft tissue sarcomas: long-term results of a single center.

PURPOSE: To assess oncological outcomes of patients receiving neoadjuvant radiochemotherapy (RCT) for soft tissue sarcoma (STS) of the extremities. METHODS: Patients who were treated with preoperative radiotherapy and concomitant chemotherapy-3 cycles of mitomycin/doxorubicin/cisplatin (MAP) or 2-4 cycles of doxorubicin/cisplatin (AP)-followed by surgery were analyzed retrospectively. Survival rates were estimated, and prognostic factors were identified. RESULTS: Between 1994 and 2017, a total of 108 patients were included. Median ages were 43 years and 51 years for patients receiving MAP and AP, respectively. The 5­year local relapse-free survival (LRFS), disease-free survival (DFS), disease-specific survival (DSS), and overall survival (OS) were 94.1, 63.6, 75.3, and 71.9%, respectively. In the multivariate analysis, significant predictors were identified as follows: de novo or R1/R2 resected tumor on admission before RCT (p = 0.017; hazard ratio [HR] 0.112, 95% confidence interval [CI] 0.019-0.675) and R0 resection after RCT (p = 0.010; HR 0.121, 95% CI 0.024-0.598) for LRFS; female gender (p = 0.042; HR 0.569, 95% CI 0.330-0.979) and liposarcoma histology (p = 0.014; HR 0.436, 95% CI 0.224-0.845) for DFS; liposarcoma histology (p = 0.003; HR 0.114, 95% CI 0.027-0.478) and AP regimen (p = 0.017; HR 0.371, 95% CI 0.165-0.836) for DSS; age ≤ 45 years (p = 0.043; HR 0.537, 95% CI 0.294-0.980) and liposarcoma histology (p = 0.006; HR 0.318, 95% CI 0.141-0.716) for OS, respectively. CONCLUSION: An increased risk for local failure seems to exist for patients with relapsed tumor on admission and having positive surgical margins after neoadjuvant RCT. Intensity of chemotherapy influenced DSS but not OS, which could be due to younger patients receiving MAP.

The use of radiomics and machine learning for the differentiation of chondrosarcoma from enchondroma.

PURPOSE: To construct and compare machine learning models for differentiating chondrosarcoma from enchondroma using radiomic features from T1 and fat suppressed Proton density (PD) magnetic resonance imaging (MRI). METHODS: Eighty-eight patients (57 with enchondroma, 31 with chondrosarcoma) were retrospectively included. Histogram matching and N4ITK MRI bias correction filters were applied. An experienced musculoskeletal radiologist and a senior resident in radiology performed manual segmentation. Voxel sizes were resampled. Laplacian of Gaussian filter and wavelet-based features were used. One thousand eight hundred eighty-eight features were obtained for each patient, with 944 from T1 and 944 from PD images. Sixty-four unstable features were removed. Seven machine learning models were used for classification. RESULTS: Classification with all features showed neural network was the best model for both readers' datasets with area under the curve (AUC), classification accuracy (CA), and F1 score of 0.979, 0.984; 0.920, 0.932; and 0.889, 0.903, respectively. Four features, including one common to both readers, were selected using fast correlation based filter. The best performing models with selected features were gradient boosting for Fatih Erdem's dataset and neural network for Gülen Demirpolat's dataset with AUC, CA, and F1 score of 0.990, 0.979; 0.943, 0.955; 0.921, 0.933, respectively. Neural Network was the second-best model for FE's dataset based on AUC (0.984). CONCLUSION: Using pathology as a gold standard, this study defined and compared seven well-performing models to distinguish enchondromas from chondrosarcomas and provided radiomic feature stability and reproducibility among the readers.

The value of ultrasonography in detecting early arthropathic changes and contribution to the clinical approach in patients of hemophilia.

PURPOSE\AIM: Hemophilia affects the blood clotting process, is a genetic disease characterized by recurrent bleeding. The hemophilia early arthropathy detection with ultrasound (HEAD-US) procedure and scoring method were designed for the detection of early changes in affected joints of patients. In this article, it was aimed to detect early arthropathic changes in the joints of hemophilia patients with the HEAD US scoring system and to investigate its clinical contribution. It was aimed to investigate the effectiveness of HEAD-US scoring in showing early joint damage in subclinical hemophilia cases and its contribution to treatment. METHODS: The present study included 50 hemophilia patients who were admitted to Departments of Pediatric and Adult Hematology for routine follow-up. During routine follow-up controls, patients were scored by physical examination and HJHS 2.1 and by ultrasonography and HEAD US. Statistical tests were used to analyze joint health status and the results of US examination in the patient group. RESULTS: A total of 294 joints (elbow n = 100, knee n = 94, ankle n = 100) were evaluated by ultrasonography. The mean HJHS and HEAD-US scores of the patients were 14.94 ± 15.18 and 15.6 ± 12.6, respectively. CONCLUSIONS: HEAD-US is accepted to be more sensitive than HJHS in detecting early signs of arthropathy. Detection of early abnormalities by ultrasonography will enable the development of individualized treatment protocols and to the prevention of arthropathy development.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome secondary to antimicrobial therapy in pediatric bone and joint infections.

Background: Bone and joint infections are common in children, particularly those under 10 years of age. While antimicrobial therapy can often successfully treat these infections, surgical drainage may also be necessary. It is important to note that prolonged courses of treatment have been associated with adverse events and drug reactions. Among these, drug reactions with eosinophilia and systemic symptoms (DRESS) syndrome is particularly severe and potentially life-threatening. We aimed to evaluate the cases of DRESS syndrome that develop during the treatment of bone and joint infections. Methods: A retrospective study was conducted at a tertiary-level university hospital between 2015 and 2022 to determine the incidence and outcomes of definite DRESS Syndrome in children under 18 years of age with bone and joint infections. Results: Of 73 patients with bone and joint infections, 16 (21.9 %) children developed antimicrobial therapy-induced DRESS syndrome. Eight (50 %) of these children were boys; the mean age of the patients was 9.76 ± 5.5 years. DRESS syndrome occurred in 16 children, including 13 children with osteomyelitis, 1 child with osteomyelitis and septic arthritis, and 2 children with septic arthritis and sacroiliitis. The mean duration of intravenous antibiotic therapy was 40.6 ± 16.6 days; the mean hospital stay was 48.7 ± 23.7 days; the mean time for the development of DRESS syndrome after starting antibiotics was 19.6 ± 7.68 days. New onset fever (68.8 %) and rash (43.8 %) were the most common symptoms of DRESS Syndrome. Cefotaxime and vancomycin were drugs responsible for DRESS syndrome in 8 (50 %) of 16. The causative antibiotics were switched to another class of antibiotic, most commonly preferred was ciprofloxacin (n:5; 31.3 %). For children with persistent symptoms, steroids were used in 5 (31.25) patients. Conclusions: Clinicians should be aware of DRESS syndrome in children who develop fever and rash under long-term antibiotics and should check hematological and biochemical parameters to predict the severity of DRESS syndrome. In patients with persistent symptoms, steroids may be used to control the symptoms.

What has changed in the last 25 years in osteosarcoma treatment? A single center experience.

BACKGROUND: Osteosarcoma is the most common type of primary malignant bone tumor in the extremities. The main purpose of this study was to determine clinical features, prognostic factors, and treatment results of patients with osteosarcoma at our center. METHODS: We retrospectively analyzed the medical records of children with osteosarcoma between the years 1994-2020. RESULTS: 79 patients were identified (54.4% male, 45.6% female). The most common primary site was the femur (62%). Twenty-six of them (32.9%) had lung metastasis at diagnosis. The patients were treated between 1995- 2013 according to the Mayo Pilot II Study protocol, while the others were treated with the EURAMOS protocol between the years 2013-2020. Sixty-nine patients underwent limb salvage surgery as a local treatment, whereas seven underwent amputation. The median follow-up time was 53 months (2.5-265 months). The event-free survival (EFS) and overall survival (OS) rates at 5 years were 52.1% and 61.5%. The 5-year EFS and OS rates were 69.4% and 80% in females; 37.1% and 45.5% in males (p=0.008/p=0.001). The 5-year EFS and OS rates of the patients without metastasis were 63.2% and 66.3%; with metastasis 28.8% and 51.8% (p=0.002/p=0.05). For good-responders, the 5-year EFS and OS rates were 80.2% and 89.1%; while for poor-responders, 35% and 46.7% (p=0.001). Mifamurtide was used in addition to chemotherapy as of the year 2016 (n=16). The 5-year EFS and OS rates were 78.8% and 91.7%, respectively for the mifamurtide group; 55.1% and 45.9%, respectively for the non-mifamurtide group (p=0.015, p=0.027). CONCLUSIONS: Metastasis at diagnosis and poor response to preoperative chemotherapy were the most important predictors of survival. Females had a better outcome than males. In our study group, the mifamurtide group`s survival rates were significantly higher. Further large studies are needed to validate the efficacy of mifamurtide.

Impact of the unplanned excision on the oncological outcomes of patients with soft tissue sarcomas: a single-center retrospective review of 490 patients.

OBJECTIVE: This study aimed to (1) compare the oncological results of patients who underwent re-excision after unplanned excision with those who underwent planned excision and (2) analyze the impact of local recurrences on oncological outcomes. METHODS: Patients with soft tissue sarcoma who had been treated in our center between 2000 and 2018 were retrospectively reviewed. Patients were divided into two groups: Group PE (Planned excision; n=345) and group UE (Unplanned excision; n=145). Two groups were compared in terms of local recurrence-free survival (LRFS), metastasis-free survival (MFS), and overall survival (OS). Local recurrences effects over MFS and OS were also analyzed. RESULTS: There were 26 (17.9%) local recurrences in the UE group and 30 (8.7%) local recurrences in the PE group (P=0.005). There was no difference in MFS and OS between study groups (P=0.278 and P=0.848, respectively). Five years MFS rates of UE and PE groups were 76.4% and 73.6%, and five-year OS rates of UE and PE groups were 70.3% and 73.9%, respectively (P=0.417, P=0.656). Patients with local recurrence had a 1.96 times higher risk of metastasis than patients without local recurrence (P=0.008). Patients with local recurrence had 1.65 times higher risk of mortality than patients without local recurrence (P=0.047). CONCLUSION: Although local recurrence is much more common in the UE group, this outcome does not seem to affect MFS or OS. These results indicate that similar outcomes can be achieved if UE patients are referred and appropriately treated with wide re-resections. LEVEL OF EVIDENCE: Level III, Therapeutic Study.

Magnetic Resonance Imaging Signal Changes Mimicking Bone Metastasis in Patients Receiving Bisphosphonate Therapy

Bisphosphonates are inorganic pyrophosphate agents that reduce bone turnover. These agents reduce bone pain and delay skeletal complications, such as fractures in patients with metastatic lytic lesions, malignant-related hypercalcemia, multiple myeloma, Paget's disease of bone, and osteoporosis. Osteonecrosis, developing in the jaw bones specifically, has been described as a complication associated with the use of bisphosphonates. In this report, we presented osteonecrosis-like magnetic resonance imaging findings that can be confused with bone metastasis in two patients who underwent long-term bisphosphonate treatment and the value of bone scan and 18flor-fluorodeoxyglucose positron emission tomography/computerized tomography in the differential diagnosis.

Diffuse midline glioma in Ollier disease: A case report and a brief review of the literature.

Ollier disease is a rare condition presenting with enchondromas in an irregular distribution within the medullary cavity of bones. The disease is well known for sarcomatous transformation to chondrosarcomas. It also increases the risk of other malignancies like leukemia, ovarian tumors, and glial tumors. Central nervous system malignancies associated with Ollier disease are thought to arise by somatic IDH mosaicism with their atypical features of distribution, multifocality, and age of onset. We present a case with imaging consistent with diffuse midline glioma in a patient with Ollier disease. We conclude with a brief review of the literature on Ollier Disease with a focus on central nervous system malignancies, tumorigenesis and pathophysiology.

Impact of the HEAD-US Scoring System for Observing the Protective Effect of Prophylaxis in Hemophilia Patients: A Prospective, Multicenter, Observational Study

Objective: This study aimed to observe the preventive effect of prophylactic treatment on joint health in people with hemophilia (PwH) and to investigate the importance of integration of ultrasonographic examination into clinical and radiological evaluation of the joints. Materials and Methods: This national, multicenter, prospective, observational study included male patients aged ≥6 years with the diagnosis of moderate or severe hemophilia A or B from 8 centers across Turkey between January 2017 and March 2019. Patients were followed for 1 year with 5 visits (baseline and 3th, 6th, 9th, and 12th month visits). The Hemophilia Joint Health Score (HJHS) was used for physical examination of joints, the Pettersson scoring system was used for radiological assessment, point-of-care (POC) ultrasonography was used for bilateral examinations of joints, and the Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score was used for evaluation of ultrasonography results. Results: Seventy-three PwH, of whom 62 had hemophilia A and 11 had hemophilia B, were included and 24.7% had target joints at baseline. The HJHS and HEAD-US scores were significantly increased at the 12th month in all patients. These scores were also higher in the hemophilia A subgroup than the hemophilia B subgroup. However, in the childhood group, the increment of scores was not significant. The HEAD-US total score was significantly correlated with both the HJHS total score and Pettersson total score at baseline and at the 12th month. Conclusion: The HEAD-US and HJHS scoring systems are valuable tools during follow-up examinations of PwH and they complement each other. We suggest that POC ultrasonographic evaluation and the HEAD-US scoring system may be integrated into differential diagnosis of bleeding and long-term monitoring for joint health as a routine procedure.

False-positive I-131 Uptakes at Pulmonary Wedge-resection Site and Soft Tissue Lateral to the Femoral Heads in a Patient with Papillary Thyroid Carcinoma

A hyper-metabolic pulmonary nodule was detected on 18F-FDG PET/CT in a 65-year-old woman who had been followed up for 12 years without any complaints following treatment for papillary thyroid cancer (PTC). Wedge resection was performed to the pulmonary nodule and the pathologic examination revealed PTC metastasis. On the post-therapeutic I-131 scan after radioiodine treatment, focal I-131 uptake was detected at the site of pulmonary wedge resection. At first, this finding was thought to be related to the residual lesion but diagnostic CT demonstrated only focal traction bronchiectasis at that region. In addition, a false-positive I-131 uptake was also detected at the soft tissue just lateral to the femoral heads probably due to inflammation.

Swollen digits due to pachydermodactyly resembling inflammatory arthritis.

Pachydermodactyly is a rare digital fibromatosis, characterized by painless, fusiform swelling in proximal interphalangeal joints. Since it may be misdiagnosed as inflammatory arthritis, it is important to call physicians' attention to this rarely seen condition to avoid unnecessary immunosuppressive treatment. Hereby, we present a case of a pachydermodactyly that had been previously followed-up and treated as an inflammatory arthritis for 4 years. We describe the clinical, radiological, and dermoscopic features of the case.

Differences between pediatric extra-pulmonary and pulmonary tuberculosis: a warning sign for the future.

BACKGROUND: Tuberculosis (TB) remains a major global health problem. The childhood tuberculosis has some unique features different which makes the diagnosis more complicated. Here we described the epidemiologic, clinical and microbiologic features of children with extra pulmonary and pulmonary TB. METHODS: The data of the patients <14 years with active TB were collected and compared in pulmonary (PTB) and extrapulmonary TB (EXPTB) patients. RESULTS: A total of 128 cases was included. Forty-two cases occurred in children were < 5 years of age; 41 cases between 6-10 years and 45 cases > 10 years. PTB was present in 75,0% of the cases, and EXPTB was present in 25% of cases. There was no significant difference between the EXPTB and PTB by means of distribution of age groups (p=0,201). The rate of patients free of constitutional symptoms were significantly higher in EXPTB compared to PTB(p=0,000). There was no significant difference between EXPTB and PTB by means of sources detection(p=0,069). CONCLUSION: TB is still a major public health problem. EXPTB has an insidious and silent onset without any constitutional symptoms, and both microbiological confirmation and the source by an adult are not frequently found. Moreover, detection of the adult source is mandatory for controlling the TB disease in children.