National strategic advocacy to manage patients with inherited bleeding disorders in low and lower-middle income countries.

INTRODUCTION: Inherited bleeding disorders (IBDs) including hemophilia, von Willebrand disease, platelet disorders, mucocutaneous bleeding disorders and coagulation factor deficiencies are rarely found and under-recognized in low and lower-middle-income countries. Some patients succumbed to serious bleeding without diagnosis and treatment during childhood. AREA COVERED: Diagnosis, management, and prevention should be integrated into the existing health care system. Although some countries have not implemented appropriate health care infrastructure, an initiative plan should be set up by cooperation of experienced experts and health care providers. Identification of patients with IBDs should be started in the antenatal setting to search for females at risk of carrier state. The investigations include bleeding assessment, mixing venous clotting time, coagulogram, coagulation factor assay and mutation detection. Genotypic analysis is helpful for confirming the definite diagnosis, carrier detection as well as prenatal diagnosis for females at risk of bearing an offspring with severe bleeding manifestations. Management involves replacement therapy ranging from blood component to virus-inactivated factor concentrate. Appropriate research is an essential backbone for improving patients' care. EXPERT OPINION: Effective national strategic advocacy to manage patients with IBDs requires intensive collaboration among policy makers, health care providers, patients, and family members.

A clinical prediction model to differentiate tuberculous spondylodiscitis from pyogenic spontaneous spondylodiscitis.

BACKGROUND: Microbiological diagnosis of tuberculous spondylodiscitis (TS) and pyogenic spontaneous spondylodiscitis (PS) is sometime difficult. This study aimed to identify the predictive factors for differentiating TS from PS using clinical characteristics, radiologic findings, and biomarkers, and to develop scoring system by using predictive factors to stratify the probability of TS. METHODS: A retrospective single-center study. Demographics, clinical characteristics, laboratory findings and radiographic findings of patients, confirmed causative pathogens of PS or TS, were assessed for independent factors that associated with TS. The coefficients and odds ratio (OR) of the final model were estimated and used to construct the scoring scheme to identify patients with TS. RESULTS: There were 73 patients (51.8%) with TS and 68 patients (48.2%) with PS. TS was more frequently associated with younger age, history of tuberculous infection, longer duration of symptoms, no fever, thoracic spine involvement, ≥3 vertebrae involvement, presence of paraspinal abscess in magnetic-resonance-image (MRI), well-defined thin wall abscess, anterior subligamentous abscess, and lower biomarker levels included white blood cell (WBC) counts, erythrocyte-sedimentation-rate (ESR), neutrophil fraction, and C-reactive protein (all p < 0.05). Multivariate logistic regression analysis revealed significant predictors of TS included WBC ≤9,700/mm3 (odds ratio [OR] 13.11, 95% confidence interval [CI] 4.23-40.61), neutrophil fraction ≤78% (OR 4.93, 95% CI 1.59-15.30), ESR ≤92 mm/hr (OR 4.07, 95% CI 1.24-13.36) and presence of paraspinal abscess in MRI (OR 10.25, 95% CI 3.17-33.13), with an area under the curve of 0.921. The scoring system stratified the probability of TS into three categories: low, moderate, and high with a TS prevalence of 8.1%, 29.6%, and 82.2%, respectively. CONCLUSIONS: This prediction model incorporating WBC, neutrophil fraction counts, ESR and presence of paraspinal abscess accurately predicted the causative pathogens. The scoring scheme with combination of these biomarkers and radiologic features can be useful to differentiate TS from PS.

Abductor muscle function after anterolateral approach in patients with unilateral end-stage hip osteoarthritis undergoing total hip arthroplasty: A prospective study.

Background: Total hip arthroplasty (THA) is a successful procedure for treating end-stage hip osteoarthritis (OA). Regarding the surgical approach for THA, the anterolateral (AL) approach, which requires anterior hemimyotomy of the gluteus medius muscle, has shown a long-term favorable outcome. However, to date, complete information related to hip abductor muscle outcomes after the AL approach is unavailable. This study therefore aimed to evaluate the postoperative outcome of patients who undergo THA using the AL approach in terms of hip abductor muscle recovery, pain, function, and muscle healing status. Methods: Twenty patients diagnosed with unilateral end-stage hip OA underwent cementless THA with the AL approach. All patient procedures were performed by a single surgeon. Preoperative and postoperative data were collected at 2-week, 6-week, 3-month, and 6-month follow-up periods. Hip abductor muscle power was measured via handheld dynamometer. The healing of the musculotendinous repair was evaluated with magnetic resonance imaging at 9 months. Results: After THA, hip abductor muscle power in the operated hip significantly increased as early as 3 months post-procedure when compared with the preoperative value (p < 0.05). The other parameters-including pain score, Harris hip score, and WOMAC score-significantly improved as early as 2 weeks post-operation (p < 0.05). In all patients, MRI scans showed good healing of the muscle repair site without a gap in the gluteus medius muscle. However, three patients (15%) had some fibrosis and tendon swelling at the repair site. Conclusion: This study demonstrated that patients with end-stage hip OA could experience significantly improved hip abductor motor function as early as 3 months after undergoing THA with the AL approach. Moreover, despite patients experiencing anterior hemimyotomy of the gluteus medius muscle, no significant complications emerged at the muscle repair site in the AL approach.

Comprehensive and long-term outcomes of enzyme replacement therapy followed by stem cell transplantation in children with Gaucher disease type 1 and 3.

BACKGROUND: Gaucher disease (GD) is a lysosomal storage disorder, characterized by hepatosplenomegaly, pancytopenia, bone diseases, with or without neurological symptoms. Plasma glucosylsphingosine (lyso-Gb1), a highly sensitive and specific biomarker for GD, has been used for diagnosis and monitoring the response to treatment. Enzyme replacement therapy (ERT) is an effective treatment for the non-neurologic symptoms of GD. Neuronopathic GD (type 2 and 3) accounts for 60%-70% of the Asian affected population. METHODS: We explored combination therapy of ERT followed by hematopoietic stem cell transplantation (HSCT) and its long-term outcomes in patients with GD type 3 (GD3). RESULTS: Four patients with GD3 and one with GD type 1 (GD1) underwent HSCT. The types of donor were one matched-related, one matched-unrelated, and three haploidentical. The age at disease onset was 6-18 months and the age at HSCT was 3.8-15 years in the patients with GD3. The latest age at follow-up was 8-22 years, with a post-HSCT duration of 3-14 years. All patients had successful HSCT. Chronic graft-versus-host disease occurred in one patient. The enzyme activities were normalized at 2 weeks post HSCT. Lyso-Gb1 concentrations became lower than the pathological value. All of the patients are still alive and physically independent. Most of them (4/5) returned to school. None of the patients with GD3 had seizures or additional neurological symptoms after HSCT, but showed varying degrees of cognitive impairment. CONCLUSIONS: ERT followed by HSCT could be considered as an alternative treatment for patients with GD3 who have a high risk of fatal neurological progression.

Iliopsoas haemorrhage complicated by femoral neuropathy in patients with haemophilia: a case series report.

OBJECTIVES: The study aimed to determine the incidence of femoral neuropathy in patients with haemophilia exhibiting iliopsoas haemorrhage. METHODS: Patients with iliopsoas haemorrhage confirmed by ultrasonography or CT scan were studied retrospectively. RESULTS: A total of 44 episodes of iliopsoas haemorrhage occurred in 20 patients with haemophilia (A17, B3). Most episodes in patients without inhibitors (14/16 = 87.5%) were adequately treated followed by prophylaxis. However, 11 of 28 episodes (39.3%) in patients with inhibitors were adequately treated and no prophylaxis was provided. An appropriate rehabilitation programme was arranged during hospitalisation and follow-up. Femoral neuropathy was observed in 28 of 44 episodes, while 16 episodes of persistent femoral neuropathy from previous bleeding were excluded. As a result, 11 of 28 episodes (39.3%) of femoral neuropathy were similarly found amongst patients with and without inhibitors. The mean time of onset and resolution of femoral neuropathy were 3.7 (1.8) and 23.4 (20.5) days after the onset of iliopsoas haemorrhage, respectively. Patients receiving inadequate and delayed replacement had a significantly higher rate of femoral neuropathy than those who received adequate and prompt replacement. CONCLUSION: Femoral neuropathy following iliopsoas haemorrhage was common in haemophilia patients with and without inhibitors.

Effectiveness of prophylactic double-looped wiring in cementless Hip arthroplasty: A biomechanical study in osteoporotic bone model using impaction simulation system.

BACKGROUND: Periprosthetic femoral fracture (PFF) is well-known complication in cementless hip arthroplasty (HA), especially in the elderly with osteoporosis. To prevent intraoperative PFFs during HA, prophylactic cerclage wiring on the calcar area during femoral broaching and implant insertion is recommended. However, biomechanical data on the benefits of cerclage wiring in osteoporotic bone during impaction are limited and controversial. This study aimed to assess the efficacy of prophylactic double-looped cerclage wiring (PDLCW) during femoral broaching in the osteoporotic bone model with an impaction simulation system. METHODS: Fifteen osteoporotic femur models were prepared and allocated to three groups; control group (no cerclage), PW group (received PDLCW), and CC+PW group (prepared as having calcar crack and received PDLCW). All femurs were broached under impaction force that sequentially increased until the visible fracture or calcar crack propagation was visible. The primary outcomes were mallet impaction force (MIF-CF) and mallet velocity (MV-CF) at the time of calcar fracture. The secondary outcomes were calcar fracture pattern and subsidence during calcar fracture (S-CF). RESULTS: PW group showed a significant increase in average MIF-CF, by 40.4% and 120.2% (p<0.001) compared to the control and CC+PW groups, respectively. The average MV-CF in PW group was also significantly greater, 13.1% and 64.6% (p<0.001), compared to the control and CC+PW groups, respectively. Control group revealed significantly greater incidence of complete calcar fracture (60% vs. 0% vs. 0%, p = 0.024), and the highest S-CF compared to those in the PW and CC+PW groups (10.6 ±â€¯6.0 mm, 6.7 ±â€¯4.4 mm, and 1.3 ±â€¯2.0 mm, p = 0.020). CONCLUSIONS: This study showed that PDLCW significantly improved hoop stress resistance by increasing the calcar fracture threshold related to mallet impaction, decreasing the risk of complete calcar fracture.

Validation of the height-width measurement in Perthes disease among interpreters of different experience levels.

The epiphyseal height and metaphyseal width ratio estimated lateral pillar involvement with limited generalizability among various interpreters. The aim of the study was to evaluate the reliability and the validity of height-width ratio measurement in Perthes disease among interpreters of different experience levels. A cross-sectional study was conducted between 2019 and 2020. We included four groups of interpreters: orthopaedic residents, orthopaedic fellows, radiology residents and radiology fellows who were unaware of radiographic study materials. Each interpreter blindedly evaluated Perthes hip radiographs twice at 1-month intervals using the height-width ratio method. Patients' and interpreters' characteristics, height-width ratio and height-width ratio converted to lateral pillar classification (A, B and C) were collected. Intra- and interobserver reliability validated with the paediatric orthopaedist were estimated. Twenty-four interpreters assessed 18 Perthes radiographs. Intraobserver level of agreement (95% confidence interval) for height-width ratio was 0.022 (-0.017 to 0.062), -0.027 (-0.074 to 0.019), -0.010 (-0.095 to 0.074) and 0.019 (-0.109 to 0.146); and interobserver reliability compared with the paediatric orthopaedist was -0.007 (-0.091 to 0.077), 0.003 (-0.056 to 0.061), -0.021 (-0.077 to 0.035) and -0.002 (-0.090 to 0.086) for orthopaedic residents and fellows, radiology residents and fellows, respectively. Kappa statistics of height-width ratio converting to lateral pillar classification indicated intraobserver agreement of orthopaedic residents and fellows, radiology residents and fellows was 0.83, 0.75, 0.54 and 0.91; and interobserver agreement compared with the paediatric orthopaedist was 0.92, 0.83, 0.42 and 0.83, orderly. Height-width ratio and lateral pillar estimation by orthopaedic and radiology trainees have moderate to excellent reliability. Level of evidence: Level II - diagnostic study.

Correlation between full-thickness degenerative supraspinatus tear and radiographic parameters including the acromiohumeral centre edge angle and the greater tuberosity angle.

BACKGROUND: Many radiographic parameters associated with the extrinsic cause of supraspinatus tears have been proposed. The aim of this study was to assess the relationship between a full-thickness degenerative supraspinatus tear (FTDST) and the patient's radiographic parameters, including the acromiohumeral centre edge angle (ACEA) and the greater tuberosity angle (GTA). METHODS: A retrospective study was conducted. We included 116 patients who underwent shoulder arthroscopic surgery at our institute. The case group included FTDST patients, whereas the control group also included patients without evidence of supraspinatus tears. In each patient, the ACEA and GTA values were measured and analyzed by two independent observers. Intra- and interobserver reliabilities were assessed. Multivariate regression analysis was performed. RESULTS: The ACEA values were significantly increased in the FTDST group with a mean of 26.44° ± 9.83° compared with 16.81° ± 7.72° in the control group (P < 0.001). Multivariate regression analysis also showed that higher ACEA values were associated with an FTDST (odds ratio 1.16 per degree, P = 0.01). For GTA values, a statistically significant difference was found with a mean of 70.92° ± 6.64 compared with 67.84° ± 5.56 in the control group (P = 0.02). However, stepwise regression analysis did not indicate that GTA was a predictor of FTDST. CONCLUSIONS: Our study demonstrated that the presence of increased ACEA values is an independent significant risk factor for the presence of FTDSTs. Consequently, GTA values may be less helpful in assessing the risk of FTDST, especially in this specific population.

Development of prediction model for osteoporotic vertebral compression fracture screening without using clinical risk factors, compared with FRAX and other previous models.

This study developed a prediction model to assess the need for asymptomatic osteoporotic vertebral compression fracture (OVCF) screening in women without using clinical risk factors. Our results demonstrated that the combination of age, height loss, and femoral neck T-score can predict OVCF comparable to previous models, including FRAX. PURPOSE: Osteoporotic vertebral compression fracture (OVCF) is a major fracture in osteoporosis patients. Early detection of OVCF can reduce the risk of subsequent fractures and death. Many existing diagnostic tools can screen for the risk of osteoporotic fracture but none aim to identify OVCF. The objective of this research is to study a predictive model for capturing OVCF and compare it with previous models. METHODS: A retrospective review was conducted that included women aged ≥ 50 years who underwent dual-energy X-ray absorptiometry and vertebral fracture screening between 2012 and 2019. The data included age, height, weight, history of height loss (HHL), and bone mass density (BMD). Receiver operating characteristic analysis and univariate and multivariate logistic regression were performed. The predictive OVCF model was formulated, and the result was compared to other models. RESULTS: A total of 617 women, a 179 of which had OVCFs, were eligible for analysis. Multivariate regression analysis showed age > 65, height loss > 1.5 cm, and femoral neck T-score < -1.7 as independent risk factors for OVCF. This model revealed comparable performance with FRAX. The model without BMD revealed superior performance to FRAX and other standard osteoporosis assessment models. CONCLUSIONS: BMD and vertebral fracture screening should be eligible for individual women age > 65 years with an HHL more than 1.5 cm, regardless of BMD. Vertebral fracture assessment should be additionally conducted on these women with a femoral neck T-score less than -1.7.

Is continuous locking suture with braided suture sufficient for arthrotomy repair in the conventional TKR? A randomized controlled trial study.

INTRODUCTION: Medial-parapatellar-arthrotomy is the standard approach for total knee replacement(TKR). No studies have clarified the outcomes as quadriceps-strength-recovery (QS) and safety of Continuous-locking-suture-technique(CLS) for the arthrotomy-repair. METHODS: Patients were randomly assigned into a CLS(n = 40) and an interrupted-horizontal-mattress(IHM, n = 40). QS, visual-analog-scale(VAS), modified-timed-up-and-go(TUGT) test, Western-Ontario-and McMasters-Universities-Osteoarthritis-Index[WOMAC] and Knee-Society-Score[KSS] were followed for 6 months'. RESULTS: A significantly-shorter capsular-closure-time in CLS(233 ± 40 VS 388 ± 47 sec)(p < 0.0001). There were insignificant difference in QS, VAS, TUGT, WOMAC and KSS during the 6-month follow-up period(p > 0.05 all). No wound complications were found. CONCLUSION: CLS with braided-suture is safe and effective as demonstrated a recovery of the QS and knee function outcome comparable to IHM. TRIAL REGISTRATION: This study was registered in Thai Clinical Trials Registry on December 2015 (https://www.clinicaltrials.in.th). The registration number was TCTR20151208003.

Using iron sucrose-labeled adipose-derived mesenchymal stem cells in 1.5 and 3 T MRI tracking: An in vitro study.

OBJECTIVES: The objective of this study was to investigate iron sucrose labeling in mesenchymal stem cell (MSCs) tracking. BACKGROUND: Adipose-derived mesenchymal stem cell-based therapy is a promising strategy for promoting musculoskeletal repair. METHODS: Iron sucrose-labeled adipose-derived mesenchymal stem cells (IS-labeled ASCs) were tracked using T2-and T2∗-weighted sequences by 1.5 and 3 T MRI in an in vitro model. ASCs were isolated from cosmetic liposuction specimens. ASCs from passages 4-6 were labeled with iron sucrose (Venofer®) which was added to the cell culture medium. Pre- and post-iron sucrose labeled ASCs were evaluated for cell surface immunophenotypes. Cell viability as well as chondrogenic, adipogenic and osteogenic differentiation of IS-labeled-ASCs were evaluated. The IS-labeled ASCs were titrated into microtubes at 1 × 103, 1 × 104, 1 × 105 and 1 × 106 cells/ml/microtube and their intensities were determined by 1.5 and 3T MRI using T2-and T2∗-weighted sequences. RESULTS: The expression markers of IS-labeled ASCs from flow cytometry were equivalent to control. The mean cell viability was 97.73 ± 2.06%. Cell differentiations of IS-labeled ASCs were confirmed in each lineage using specific staining solutions. T2∗-weighted sequences (T2∗) were able to detect iron sucrose labeled-ASCs at a minimum of 1 × 105 cells/ml/microtube using 1.5 and 3T MRI, but the detection sensitivity was lower with T2-weighted sequences (T2). CONCLUSIONS: Iron sucrose incubation is a safe alternative method for ASCs labeling and tracking using MRI following treatment. Clinicians and researchers should be able to visualize the location of ASCs engraftment without secondary surgical investigation involving tissue sampling.

Innovative Force-PRO device to measure force and implant position in total hip arthroplasty.

Total hip arthroplasty (THA) is the appropriate treatment for hip pain, dislocation, and dysfunction. THA refers to surgery to replace a hip implant, which is an effective way to recover normal hip function. The design of an implant imitates hip functions and allows bone growth in the implant area. However, it should be noted that the implant can dislocate after surgery. The main factor that should be considered during surgery is the correct position of the implant component. The acetabular cup of the hip implant should be positioned at [Formula: see text] anteversion and [Formula: see text] inclination. The evaluation of the implant inclination and anteversion during the operation decrease the risk of the implant dislocation after surgery. Developing a new innovative Force-PRO device can aid the doctor in evaluating the force on the surface of the acetabular liner and the angle of the acetabular liner during the hip implant operation. This device consists of two main sensors-force sensors and inertial measurement unit sensors. Furthermore, the 3D printings of an implant's parts should be specifically designed to integrate with these sensors. To develop the graphical user interface application, C[Formula: see text] should be the programming language of use. The graphical user interface application communicates between the device and user via a wireless communication system. CT-based imaging and force gauge measurement are the methods to evaluate the efficiency of this device. For this purpose, the sterile method is considered.

Height-Width Ratio of Proximal Femoral Epiphysis: Estimation of Lateral Pillar Involvement in Bilateral Perthes Disease.

BACKGROUND: The standard evaluation of epiphyseal involvement in Perthes disease is lateral pillar classification. However, it needs to be compared with contralateral normal hip leading to limited use in bilateral disease. We, therefore, develop a ratio between epiphyseal height and metaphyseal width of affected hips to estimate lateral pillar involvement. This study aimed to assess the height-width ratio of the proximal femoral epiphysis in non-Perthes children, and to find the relationship between the height-width ratio and lateral pillar classification in Perthes disease. METHODS: A cross-sectional study was conducted between 2009 and 2015. Phase I included children aged 2 to 15 years who did not have Perthes disease. Phase II included children aged 2 to 15 years who had Perthes disease. Other abnormal proximal femoral epiphysis was excluded. Lateral pillar height and metaphyseal width were independently measured twice by 2 assessors in each phase. Intraobserver and interobserver levels of agreement, height-width ratio and cut-off points to differentiate lateral pillar types were determined. RESULTS: There were 69 children (87 hip radiographs) who had non-Perthes hips, and 18 boys with Perthes disease (20 hip radiographs). Height-width ratio in the non-Perthes group increased from 0.38 to 0.48 at 2 to 10 years of age and remained constant until maturity. Average height-width ratio in lateral pillar A/non-Perthes hip was 0.47±0.05, lateral pillar B or B/C 0.32±0.05, and lateral pillar C 0.18±0.05. Intraobserver and interobserver level of agreements of height-width ratio in Perthes disease were 0.007 (95% confidence interval, -0.030 to 0.043) and 0.006 (95% confidence interval, -0.119 to 0.107), respectively. Cut-off values to differentiate lateral pillar A and B or B/C was 0.40, and to differentiate lateral pillar B or B/C, and C was 0.25 with 90% accuracy and area under receiver operating characteristic curve of 0.9. CONCLUSIONS: Height-width ratio is useful for grading severity in unilateral and bilateral Perthes disease. It has excellent reliability and validity with exact cutoff values to estimate lateral pillar classification. LEVEL OF EVIDENCE: Level II-diagnostic study.

The comparisons between thermography and ultrasonography with physical examination for wrist joint assessment in juvenile idiopathic arthritis.

OBJECTIVE: This study aimed to assess infrared thermography (IRT) and ultrasonography (US) for detecting wrist arthritis in juvenile idiopathic arthritis (JIA) patients. Although IRT could help us in detecting joint inflammation, IRT studies in JIA patients with wrist arthritis are still limited. Currently, no validated US criteria exist for detecting arthritis, and the most useful parameters between gray-scale ultrasound (GSUS) or power Doppler ultrasound (PDUS) remain unclear. APPROACH: Forty-six JIA patients were included in this study. Detecting wrist arthritis at varying degrees using IRT and US were compared with physical examination. MAIN RESULTS: Sixteen patients had previous wrist arthritis that is currently inactive and 30 still had wrist arthritis. The median ages (IQR) were 7.7 (4.3) and 10.2 (4.8) years, respectively. Fifteen healthy participants were included, with a median age (IQR) of 9.2 (2.0) years. Using IRT, mean temperature (T mean) and maximum temperature (T max) at skin surface in the region of interest (ROI) in the arthritis group were higher than in the inactive group and the healthy controls with p < 0.05. When patients with arthritis were subgroup analyzed by disease severity based on physical examination, the moderate to severe arthritis had T mean and T max higher than the mild arthritis group with statistical significance. The heat distribution index (HDI), two standard deviations of all pixel temperature values in the ROI, in the moderate to severe arthritis group was higher than in the healthy controls (p = 0.027). The receiver operating characteristic analysis in arthritis detection revealed diagnostic sensitivity of 85.7% and 71.4% and specificity of 80.0% and 93.3% at cut-off points of T mean ⩾ 31.0 °C and T max ⩾ 32.3 °C, respectively. For US, GSUS and PDUS are useful in detecting arthritis, providing high sensitivity (83.3%) and specificity (81.3%). SIGNIFICANCE: Our study demonstrated that both IRT and US were applicable tools for detecting wrist arthritis.

A retrospective study on 158 Thai patients with juvenile idiopathic arthritis followed in a single center over a 15-year period.

AIM: To determine the outcomes of juvenile idiopathic arthritis (JIA) in Thai children. METHODS: A retrospective cohort study. All JIA patients in a rheumatology clinic, Ramathibodi Hospital, between July 1997 and December 2012 were enrolled. The patient data were reviewed from medical records. At the most recent follow-up visit, patient outcomes were assessed in three aspects: disease status, functional outcomes and structural damage. RESULTS: Of 168 patients, 158 (94.0%) were assessed in disease status and functional outcomes, with 114 patients (67.9%) assessed in three aspects over 4 years of disease. The most common JIA category was systemic JIA (SJIA) (33.8%), followed by enthesitis-related arthritis (ERA) (24.8%), oligoarthritis (18.5%), rheumatoid factor (RF)-negative polyarthritis (15.3%), RF-positive polyarthritis (7.6%) and one undifferentiated arthritis. SJIA had the highest remission rate due to early diagnosis and prompt treatment compared to other categories, whereas RF-positive polyarthritis carried the worst prognosis in three aspects, followed by ERA. Moreover, ERA patients had the highest failure rate in conventional therapy, half of whom had combined treatment with biologic agents and presence of human leukocyte antigen (HLA)-B27 was a predictor for biologic treatment in ERA patients. In addition, disease duration > 2 years or failure of conventional therapy was a predictor of structural bone damage. CONCLUSIONS: SJIA had the highest remission rate, whereas RF-positive polyarthritis had the worst outcome in three aspects. Prolonged disease duration or failure of conventional therapy was a predictor of structural bone damage, while HLA-B27 was a predictor for biologic treatment in ERA patients.

Traumatic Injury in a Child with Scurvy: A Case Report.

This case report aimed to describe the clinical presentation, treatments and prognosis of a child who had scurvy and traumatic injury of the left thigh. A 30-month-old boy had presented with left hip pain two weeks after falling down on the floor while walking. He developed pain, warmness of the left hip and thigh, and finally was unable to bear weight. He also had a high fever gingival hemorrhage, dental caries, petechiae, positive rolling test and limited range of motion of the left hip. The radiographs revealed Wimberger's ring and Frenkel line as scurvy. Vitamin C supplement had been prescribed for one week. However, there was no clinical response and magnetic resonance imaging (MR) suggested subperiosteal abscess as well as osteomyelitis of bilateral femurs and tibias. Debridement and biopsy of the left femur were performed and found only subperiosteal blood. A clinical improvement was noted on the second day after surgery. Vitamin C level was reported at 0.03 mg/dl which was very low. Bacterial culture was negative and the pathological findings were callus formation with hemorrhage. The patient continued the treatment for two months and all conditions were healed eventually. In severe scurvy with trauma, prolonged subperiosteal hematoma was susceptible to infection, and may need debridement simultaneously with vitamin C supplement to shorten the clinical course.

PRIMARY STERNAL TUBERCULOSIS: A CASE REPORT AND LITERATURE REVIEW.

Mycobacterium tuberculosis can infect multiple organs and may rarely cause osteomyelitis of the sternum. We report a patient with primary sternal tuberculosis who presented with a chest wall mass. A core needle biopsy confirmed the diagnosis-of tuberculosis. We review the literature regarding tuberculous osteomyelitis of the sternum.

Paget's disease of bone in ethnic Thai presented with urologic symptoms and misleading as metastatic prostate cancer: report of a case with a review of the literature.

Paget's disease of bone is common in some parts of Europe and in countries inhabited by European emigrants. In Western Countries, Paget's bone disease is one of the priorities in differential diagnosis for elderly patients who present osteoblastic lesions, so it is unlikely to be overlooked, even though patients may present symptoms unrelated to bone lesions. However in Asian countries where Paget's disease is rare, metastatic prostate cancer appears to be the most common cause for osteoblastic lesions, thus, the Paget's disease is unlikely to be of much concern. This may lead to undue emphasis on investigations to support the diagnosis of prostate cancer. In this report, a 69 year-old man presented pain in the right leg and difficulty urinating. The plain film showed osteoblastic lesions of the right pelvic bone and lumbar spine. The pertinent routine laboratory findings revealed increased levels of serum alkaline phosphatase 125 U/L (normal 27-86). Metastatic prostate cancer was highly suspected and investigations focused upon this, including a serum prostate specific antigen assay, a transurethral cystoscopic examination, an intravenous pyelogram, and an ultrasonogram of the prostate gland. However all of these investigations failed to support prostate cancer. Bone biopsy was performed twice, resulting in a report supporting Paget's disease of bone. The patient was treated with alendronate for three months. Radiologic findings, six months later, showed signs of improvement. He died one year later of heart failure that could plausibly had been a cardiovascular complication of Paget's disease. Better awareness of Paget's disease in Thailand and other Asian countries should reduce the incidence of unnecessary investigations and avoid a misleading diagnosis, which could lead to inappropriate treatment for metastatic prostatic cancer and undesirable psychological impact associated with being misinformed regarding malignancy.