A Novel Diabetic Arthritic Model in Rats Induced by Streptozotocin, High-Fat Diet, and Complete Freund's Adjuvant.

Introduction: Ankle arthrodesis is one of the treatments of choice, particularly in late-stage and unstable diabetic Charcot arthropathy. Unfortunately, poor healing capacity might play a role in the high nonunion rate (10-40%). The advancement in regenerative medicine opens a new horizon for enhancing fusion after ankle arthrodesis in patients with poor healing capacity. However, a suitable small animal model is warranted to study the effectivity of these regenerative medicine approaches. Streptozotocin (STZ)-induced diabetes models and adjuvant-induced arthritis models with complete Freund's adjuvant are two established models. However, no study has combined those two models to make a diabetic arthritic model that more closely resembles the condition in Charcot arthropathy. Methods: Twenty male Sprague-Dawley rats were assigned into five groups, consisting of one control group, and four diabetic groups which were induced by STZ injection and a high-fat diet. Among these diabetic rats, two groups received complete Freund's adjuvant (CFA) injections to the left ankle of the hind limb. The control group, one of the diabetic-only groups, and one of the arthritic-diabetic-induced groups were euthanized at 4 weeks after STZ induction, and the remainder were euthanized 6 weeks after STZ induction. Clinical, radiological, and histological examinations were then compared in all five groups. Results: Diabetic status was successfully achieved in the model, which was maintained until the completion of the study. The CFA-induced ankles were significantly larger than the contralateral ankles in all groups (p<0.05). Histopathological evaluation confirmed arthritic changes in the CFA-induced group with less variability after 4 weeks of arthritis induction. Conclusion: This rat model of arthritic diabetic mimics the progressive and chronic nature of Charcot arthropathy in humans. This model can be further use to study treatments that might enhance the fusion rate in ankle arthrodesis in healing-defective patients such as those with diabetes. Level of Clinical Evidence: 5.

The Development of New Scoring System to Define the Presence of Instability and the Need of Fusion in Degenerative Lumbar Spinal Stenosis - Jakarta Instability Score.

STUDY DESIGN: Systematic review, expert opinion and Delphi technique, and validity and reliability studies. OBJECTIVE: We developed Jakarta Instability Score (JIS) to identify spinal instability and the need of fusion in degenerative lumbar spinal stenosis (LSS). METHODS: This study consisted of systematic review to find predictors of spinal instability, expert opinion and modified Delphi technique to develop JIS, and validity and reliability studies of the newly developed JIS. RESULTS: A total of 54 studies were included in the systematic reviews to obtain predictors of spinal instability. Through expert opinion and modified Delphi technique, JIS was developed and consisted of the clinical component (back pain), dynamic radiograph component (dynamic translation and angulation), and MRI component (facet joint effusion), each of the component would be scored, and the total scoring would be from 0 to 14. The final scoring would classify patients into three groups: stable group (score of 0 to 4) in which the fusion is not needed, potentially unstable group (score of 5 to 8) in which the decision of fusion is based on surgeon's clinical judgment, and unstable group (score of 9 to 14) in which the fusion is needed. Final step of study concluded that this JIS had a high validity and reliability. CONCLUSION: The newly developed JIS was a valid and reliable scoring system that could help to identify the presence of instability in LSS and can be used as a guideline to decide whether spinal fusion will be needed.

Scoliocorrector Fatma-UI for correction of adolescent idiopathic scoliosis: Development, effectivity, safety and functional outcome.

BACKGROUND: Adolescent idiopathic scoliosis remains a major problem due to its high incidence, high risk, and high cost. One of the aims of the management in scoliosis is to correct the deformity. Many techniques are available to correct scoliosis deformity; however, they are all far from ideal to achieve three-dimensional correction in scoliosis. AIM: To develop a set of tools named Scoliocorrector Fatma-UI (SCFUI) to aid three-dimensional correction and to evaluate the efficacy, safety, and functional outcome. METHODS: This study consists of two stages. In the first stage, we developed the SCFUI and tested it in finite element and biomechanical tests. The second stage was a single-blinded randomized clinical trial to evaluate the SCFUI compared to direct vertebral rotation (DVR). Forty-four subjects with adolescent idiopathic scoliosis were randomly allocated into the DVR group (n = 23) and SCFUI group (n = 21). Radiological, neurological, and functional outcome was compared between the groups. RESULTS: Finite element revealed the maximum stress of the SCFUI components to be between 31.2 - 252 MPa. Biomechanical analysis revealed the modulus elasticity of SCFUI was 9561324 ± 633277 MPa. Both groups showed improvement in Cobb angle and sagittal profile, however the rotation angle was lower in the SCFUI group (11.59 ± 7.46 vs 18.23 ± 6.39, P = 0.001). Neurological and functional outcome were comparable in both groups. CONCLUSION: We concluded that SCFUI developed in this study resulted in similar coronal and sagittal but better rotational correction compared to DVR. The safety and functional outcomes were also similar to DVR.

Topographic anatomy and intraoperative USG-guided foreign bodies extraction of neglected Molotov cocktail victim: A rare case report.

INTRODUCTION AND SIGNIFICANCE: Foreign body implantation resulting from explosive devices is an extraordinary occurrence that often leads to substantial morbidity among the affected individuals. Explosions caused by such devices generate a rapidly propagating blast wave emanating from the point of detonation. This study aims to present a case involving a patient who experienced multiple foreign body implantations as a consequence of a bomb explosion. CASE PRESENTATION: A 41-year-old male presented with a history of multiple foreign bodies retained within his body for the past 22 years, originating from a homemade explosive device. At present, he reports weakness in his lower extremities, numbness extending from the umbilical region down to the lower extremities, and fecal incontinence. The patient underwent a surgical procedure for the removal of these foreign bodies, guided by ultrasonography (USG), which lasted for a duration of 12 h. CLINICAL DISCUSSION: The presence of foreign bodies within the human body incites an inflammatory response. In preparation for surgery, topographic anatomy is delineated through the use of pre-operative CT scans to ascertain the precise locations of these foreign bodies. Subsequently, the removal of these foreign bodies is executed under the guidance of ultrasound. CONCLUSION: The extraction of multiple foreign bodies from a patient's body is an infrequent surgical procedure. Meticulous surgical planning, aided by the utilization of X-rays and CT scans for topographic anatomical mapping, is imperative. Employing real-time ultrasound guidance during the procedure serves to minimize blood loss and mitigate potential damage to adjacent structures, thereby enhancing patient safety and reducing the likelihood of surgical complications.

Clinical evaluation of intralesional umbilical cord-derived mesenchymal stem cells, conditioned medium and triamcinolone acetonide injection for keloid treatment: A pilot study.

Topical keloid therapy is performed with triamcinolone acetonide (TA) intralesional injection. However, the recurrence rate is high with various side effects. Mesenchymal stem cells (MSCs) have high proliferative abilities and reduce the activity and proliferation of fibroblast cells in keloids. To overcome the costs and limitations, conditioned medium (CM) is used. This study aims to evaluate feasibility of intralesional injection of umbilical cord MSC (UC-MSC) and conditioned medium (UC-CM) compared to TA for keloid therapy. Twenty-four patients with keloids who met the inclusion criteria were included, randomized into three treatment groups and then got assessed for the sociodemographic data, keloid volume, histopathology (type 1:3 collagen ratio), interleukin-10 (IL-10) levels and Patient and Observer Scar Assessment Scale (POSAS) score during visits. Largest volume regression occurred in the UC-MSC group, followed by UC-CM and then the TA group (UC-MSC: 45.32% ± 2.61%; UC-CM: 43.61% ± 3.67%; TA: 28.34% ± 3.81%; p = 0.003). Similar pattern was also observed in increase in IL-10 levels, the decrease in POSAS scores and the reduction of type 1:3 collagen ratio. Hence, UC-MSC and UC-CM are promisingly more effective than TA for keloid therapy, showcasing their superiority in reducing keloid volume, symptoms and type 1:3 collagen ratio, as well as increasing the levels of IL-10.

Radiological Outcomes of Reduction Surgery for Degenerative Lumbar Spondylolisthesis Using Long Arm Pedicle Screws.

Objective: Until now, the spondylolisthesis reduction technique has relied on posterior instrumentation using long arm pedicle screws. In this way, the segments will be brought into alignment with the other vertebrae with the pedicle mats being tightened. The aim of this study is to acknowledge whether reduction surgery for degenerative lumbar spondylolisthesis (DLS) using long arm pedicle screws is able to correct the listhesis and spinopelvic parameters. Methods: We carried out a retrospective study of patients with degenerative lumbar spondylolisthesis who went through reduction surgery using long arm pedicle screws in our institutions from January 2019 to March 2022. Preoperative and postoperative radiological outcomes consisting of listhesis and spinopelvic parameters were assessed. Results: We found a statistical difference between the magnitude of listhesis immediately after surgery and preoperatively (p<0.001), with a successful correction of 85.85%. There was significant decrease in the value of pelvic tilt (p=0.044) and increase in the value of sacral slope (p=0.008) after surgery. Conclusion: Reduction surgery using long arm pedicle screws for DLS was able to reduce the listhesis effectively up to 85.5%, and also to restore the parts of spinopelvic parameters, the pelvic tilt and sacral slope, approaching normal values.

Ultrasonographic measurement of abdominal and gluteal-femoral fat thickness as a predictor for android/gynoid ratio.

OBJECTIVE: To evaluate the use of ultrasonography (US) as an alternative to dual-energy x-ray absorptiometry (DXA) to predict the percentage ratio of android/gynoid (A/G) fat mass. METHODS: This was a cross-sectional study. Twenty-eight participants included in the study underwent whole-body DXA examinations and the A/G ratio was calculated. Soft-tissue US was performed in several standardised anthropometric areas of the body. Correlation analysis between abdominal and gluteal-femoral fat thickness based on US and A/G ratio was conducted using the Pearson or Spearman test depending on the data normality. Multiple regression analysis using the backward stepwise method was performed to establish an equation for estimating the A/G ratio. RESULTS: There was a strong and significant correlation between fat thickness in the six anthropometric areas and the A/G ratio in female participants. The analysis revealed three anthropometric areas: upper abdomen (S4), lower abdomen (S5), and mid-xiphoid-umbilical region (S7), that can accurately predict the A/G ratio by 82.3%. (P < 0.05). However, no such correlation was found in male participants. CONCLUSIONS: US measurement of fat thickness can predict A/G ratio in the female population. However, this method is not recommended for men.

Risk of developing cognitive impairment on patients with chronic obstructive pulmonary disease: A systematic review.

BACKGROUND: The increasing number of chronic obstructive pulmonary disease (COPD) incidence has led to a great negative impact on older people's lives. This chronic disease was a critical and independent risk factor for cognitive function impairment in the elderly with mild cognitive impairment as a frequent feature. This systematic review aimed to examine the risk of developing cognitive impairment in COPD. METHODS: A structured search of the literature was conducted using the Preferred Reporting Items for Systematic Reviews and Meta-analyses statement guideline, with a pre-determined search strategy starting from study identification, title and abstract screening, eligibility assessment, and inclusion of relevant study. The search was conducted in PubMed and MEDLINE via EBSCOhost, with restriction to human studies. The studies from inception until January 12, 2021. RESULTS: Five original articles were included. Most studies found that patients with COPD had a higher chance of developing cognitive impairment, especially when patients were followed up for more than 5 years. We discovered that the risk of cognitive impairment seemed to be correlated with the length of time spent following the participants, with the highest risk of cognitive impairment being identified in those who had the longest observation period. It is critical to conduct cognitive screening from the time a diagnosis of COPD is obtained and on a continuing basis in order to recognize and treat these individuals appropriately. CONCLUSION: There is a potential association between COPD and mild cognitive impairment. We encourage more studies to be done with higher sensitivity and specificity cognitive screening tools in the future to build better evidence and qualify to be analyzed quantitatively with meta-analysis.

Hemosiderin deposition evaluation in hemophilic ankle joints: association between US finding and gradient-recalled echo MR imaging sequence.

BACKGROUND: Repeated bleeding in hemophilic arthropathy (HA) may result in severe degenerative changes and joint destruction. The gradient-recalled echo (GRE) sequence MR is proved to be the best method to detect hemosiderin deposition. However, MR is not widely available in developing countries, including Indonesia. Some studies have proposed ultrasonography (US) as an alternative tool in evaluating hemophilic joint. However, there is still some disagreement on the ability of US to detect hemosiderin deposition. OBJECTIVE: To evaluate the association between US and GRE-sequence MR imaging in detecting hemosiderin deposition in hemophilic ankle joint. MATERIAL AND METHODS: A total of 102 sites from 17 ankle joints of 11 boys with severe hemophilia A underwent US examination using a high-frequency linear array transducer. GRE-sequence MR examination was performed in sagittal view consistent with the sites scanned by US. Both examinations were performed on the same day, but MR interpretation was performed blindly at different times. The association between US and GRE-sequences in detecting hemosiderin deposition was analyzed using McNemar's test. RESULTS: Statistical analysis showed a significant association (p value < 0.001) between US and GRE MR in detecting hemosiderin deposition, but the association is weak (R = 0.26). Sensitivity and specificity of US for detecting hemosiderin deposition were 46.84% (95%CI: 35.51-58.40) and 95.65% (95%CI: 78.05-99.89), respectively, with positive predictive value 97.37% (95%CI: 84.29-99.61), negative predictive value 34.38% (95%CI: 29.50-39.60) and accuracy 57.84% (95%CI: 47.66-67.56). CONCLUSION: There was a weak association between US and GRE-sequences in detecting hemosiderin deposition of hemophilic ankle joint. â€‹â€‹.

Tuberculous septic arthritis of the hip with large abscess formation mimicking soft tissue tumors: A case report.

BACKGROUND: Tuberculous septic arthritis is an infection that occurs inside the joint or synovial fluid and joint tissues caused by Mycobacterium tuberculosis. It may show wide variability of clinical symptoms and imaging appearance, ranging from asymptomatic with a normal radiographic examination to severe joint pain along with joint destruction, osteomyelitis, and abscess formation. This article presents radiographic and MR imaging appearance from a case of tuberculous septic arthritis with large abscess formation mimicking soft tissue tumor. CASE PRESENTATION: We reported a 32-year-old female with a slowly enlarging lump on her right proximal thigh within the last 4 months along with slowly progressing joint pain. Both radiographic and MR images showed destruction of the femoral head and acetabular roof, with a formation of large rim-enhanced abscess that extending superficially and distally until mid-thigh. The patient underwent open drainage surgery and excisional biopsy. Histopathological examination showed chronic granulomatous inflammation caused by tuberculous infection. CONCLUSION: MR imaging combined with radiographic and clinical information played a very important role in the diagnosis of tuberculous septic arthritis with abscess, and to differentiate it from soft tissue neoplasms.

Correlation between Hemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score and Hemophilia Joint Health Score (HJHS) in patients with hemophilic arthropathy.

BACKGROUND: Hemophilic arthropathy, a condition manifested as joint destruction due to spontaneous joint bleeding, is one complication of hemophiliac patients. Early detection and intervention may improve the outcome, in which ultrasonography can be an ideal modality with the introduction of HEAD-US (Hemophilia Early Arthropathy Detection with Ultrasound) protocol. Studies have shown US benefit in hemophiliac patients, including its potential as an alternative for the Hemophiliac Joint Health Score (HJHS) system. However, many of the studies were conducted in countries with better management of hemophilia using prophylaxis treatment. It is unclear whether HEAD-US has a correlation with HJHS in countries using episodic treatment only, like in Indonesia. PURPOSE: This study aimed to explore the correlation between HEAD-US and HJHS in hemophiliac patients with joint problems in Indonesia. MATERIALS AND METHODS: A cross-sectional correlation study between HEAD-US and HJHS was performed with primary data collected from 120 hemophilic patients. US examination was performed on elbow, knee and ankle joints using the HEAD-US scoring method by a musculoskeletal radiologist. HJHS examination was conducted by a trained physiotherapist and a medical rehabilitation specialist. All examiner is member of multidisciplinary Hemophiliac Management Team in Cipto Mangunkusumo General Hospital in Jakarta, Indonesia. RESULTS: The mean age of the participant was 9.3 (5-14) years old. The median score of HEAD-US was 8 (1-28) with most of the joint abnormalities found on the ankles. The median score of HJHS was 3 (0-35), with most joint abnormalities found on the knees. There was a moderate correlation between HEAD-US and HJHS score (p < 0.05, r = 0.65). CONCLUSION: HEAD-US shows a moderate correlation to HJHS in hemophiliac patients who received episodic treatment. HEAD-US can provide additional value in the anatomical evaluation of the joint and could be complementary to HJHS in assessing the joint status in hemophilic patient.

Ultrasonography evaluation of the normal ulnar nerve in adult: Comparison of the cross-sectional area at elbow extension and flexion.

BACKGROUND: Cross-sectional area (CSA) measurement of the ulnar nerve in the adult population by using ultrasonography (US) at elbow extension and flexion has previously been reported, but not much evidence showed a significant difference between elbow extension and flexion position. PURPOSE: To compare the ulnar nerve CSA between elbow extension and flexion position. METHODS: A comparative cross-sectional study was conducted by involving 36 healthy adults with normally functioning ulnar nerve proven by Nerve Conduction Study (NCS) or Electroneurography. The ulnar nerve CSA was measured on each elbow by using US at the level of the medial epicondyle, 2 cm distal and 2 cm proximal from the medial epicondyle. RESULTS: The average ulnar nerve CSA at the medial epicondyle, 2 cm distal and proximal to the medial epicondyle at elbow extension respectively were 5.95 ±â€¯0.74 mm2, 6.27 ±â€¯0.92 mm2, and 5.92 ±â€¯0.73 mm2. At elbow flexion, the average ulnar nerve CSA at the positions was 5.70 ±â€¯0.83 mm2, 5.23 ±â€¯0.87 mm2, dan 5.73 ±â€¯0.71 mm2 respectively. The CSA of the ulnar nerve at elbow extension was significantly larger compared to the flexion position in the three areas observed in this study (p < 0.001). CONCLUSION: The CSA of the ulnar nerve at elbow extension position was larger compared to the flexion position. Elbow position should be considered in measuring CSA of the ulnar nerve.

Thromboembolism in Malignant Musculoskeletal Tumour: A Literature Review.

Malignant musculoskeletal tumour may cause considerable burden to general health. The fast growth combined with the tumour characteristics and its invasion capability resulted in the poor prognosis of malignant musculoskeletal tumour. Malignant musculoskeletal tumour may cause significant disability by destroying normal tissue that plays important role in body kinematics. Thromboembolism, including deep vein thrombosis, pulmonary embolism, and other kinds of venous thromboembolism, is one of the most underestimated complications of musculoskeletal tumour. Normally, thrombosis ensues when pathologic factors overcame the body hemostatic regulatory capabilities, which will predispose the body to the formation of thrombus. Venous thromboembolism in musculoskeletal tumour may develop as a result of interaction between the tumour pathologic capabilities and its interaction with normal bodily functions. In this study, we reviewed the burden of musculoskeletal tumour and its complication on global health. Then, the review will focus on the pathologic and clinical aspect of thromboembolism in malignant musculoskeletal tumour, including pathophysiology, diagnosis, and treatment based on recent findings and literature.

Computed tomography evaluation of fat infiltration ratio of the multifidus muscle in chronic low back pain patients.

BACKGROUND AND OBJECTIVES: Fat infiltration of multifidus muscle is an important parameter to assess the efficacy of spinal stabilization training in chronic low back pain (LBP) patients. As a CT scan shows a specific attenuation value for fat, it can be used as a ratio to evaluate fat infiltration of the muscle relative to its cross-sectional area. This study aims to compare the fat infiltration ratio of multifidus muscle between subjects with and without chronic LBP in Indonesia. METHODS: Comparative cross-sectional study of 20 subjects with chronic LBP and 20 subjects without LBP. Fat infiltration ratio calculation of the multifidus muscle was obtained from the database of abdominal CT at the level of the superior and inferior endplate of L4 and L5 vertebral body. RESULTS: The fat infiltration ratio of multifidus muscle in the chronic LBP subjects group was significantly higher than the subjects group without NPB (p < 0.05). The cut-off value of the fat infiltration ratio at the level of the inferior endplate of L4 was 0.125 (75 % sensitivity and 80 % specificity). CONCLUSION: The calculation of the multifidus muscle fat infiltration ratio at the inferior endplate L4 using CT is a potential method to evaluate multifidus muscle quality in chronic LBP patients.

Non-contrast versus contrast-enhanced MR in the diagnosis of spondylitis: A quantitative concordance-analysis.

INTRODUCTION: : Magnetic Resonance (MR) imaging using gadolinium contrast media is an essential imaging modality in diagnosing spondylitis. However, gadolinium contrast is not widely available in Indonesia and relatively expensive. Many MR studies in Indonesia are performed without contrast administration. It is unclear how confident non-contrast MR can diagnose tuberculous spondylitis in comparison to standard contrast MR. PURPOSES: : This study aims to evaluate the concordance between the contrast MR and non-contrast spine MR in diagnosing tuberculous spondylitis. We also evaluate the interobserver agreement between the general radiologist and musculoskeletal radiologist in interpreting non-contrast MR of spondylitis. MATERIALS AND METHODS: : A cross-sectional study using secondary data was performed to evaluate the concordance between the MR results regarding the usage of contrast media in diagnosing spondylitis. The inclusion criteria were patients over 17 years old who underwent complete sequences of contrast-enhanced MR examination of the spine, referred to radiology with the clinical diagnosis of suspected tuberculous spondylitis, spondylodiscitis, or both. All of the non-contrast and contrast-enhanced MR results were read and interpreted by two independent observers, a musculoskeletal radiologist and a general radiologist, blindly. The interobserver agreement analysis of the MR examination was conducted using Kappa and McNemar test. RESULTS: : There was no significant difference between the contrast and non-contrast MR in diagnosing spondylitis (P= 0.368) and no significant difference in the interpretation of MR between the first and the second observer (P =  0.343). The concordance between the contrast and non-contrast spine MR in diagnosing spondylitis (R: 0.88, P < 0.001) and the interpretation of MR between both observers (R: 0.65, P < 0.001) were showed in this study. CONCLUSION: : There is a high concordance between the contrast and non-contrast MR in diagnosing tuberculous spondylitis. Although contrast MR is preferred as the standard imaging method of spondylitis, in case gadolinium contrast is unavailable, non-contrast MR can still provide valuable information in diagnosing spondylitis.

Parosteal Osteosarcoma: A Benign-Looking Tumour, Amenable to a Variety of Surgical Reconstruction.

Osteosarcoma arising from cortical surface is classified into parosteal, periosteal and high-grade surface osteosarcoma. Along the spectrum, parosteal osteosarcoma occupies the well-differentiated end. It is a relatively rare disease entity, comprised only 4% of all osteosarcomas and barely reported in the literature. The objective of this study is to describe cases of parosteal osteosarcoma as well as a variety of treatment options amenable to such entity. Six cases of parosteal osteosarcoma were identified based on histopathological reports in a tertiary referral hospital in Jakarta, Indonesia between January 2001 and December 2019. The mean age was 29.8 years old; four of them (66.7%) were male. Distal end of femur was the most commonly involved bone (five cases, 83.3%). The patients were treated with wide excision followed by several different reconstruction methods: replacement with endoprosthesis, extracorporeal irradiation, knee arthrodesis, or prophylactic fixation. One of our patients presented with dedifferentiated component, and therefore was treated by limb ablation. While two cases died of pulmonary metastasis, other patients reported fair to excellent functional outcome.

Comparison of the efficacy and safety of 12-month low-dose factor VIII tertiary prophylaxis vs on-demand treatment in severe haemophilia A children.

INTRODUCTION: Prophylaxis has commonly become standard treatment for severe haemophilia patients. The World Federation of Hemophilia (WFH) recommends low-dose prophylaxis in countries with resource constraints. OBJECTIVE: To determine efficacy and safety of low-dose factor VIII (FVIII) tertiary prophylaxis compared to on-demand treatment in severe haemophilia A children in Indonesia. METHODS: Eligible patients were randomly assigned to prophylaxis and on-demand groups. Patients in the prophylaxis group received infusion of FVIII 10 IU/kg body weight, two times per week. Primary outcomes were the numbers of joint bleeding and total bleeding episodes; secondary outcomes were evidence of FVIII inhibitor, Hemophilia Joint Health Score (HJHS) and Hemophilia Early Arthropathy Detection Ultrasound (HEAD-US) score. Patients were monitored for 12 months. RESULTS: Fifty patients, all with tertiary prophylaxis, 4-18 years of age, were randomized into prophylaxis (n = 25) and on-demand (n = 25) groups. The mean follow-up time was 12.8 ± 0.86 vs 12.3 ± 0.54 months, respectively. Numbers of total and joint bleeding episodes were significantly lower in the prophylaxis group (P < 0.001, 95% CI -24.6;-10.7 and P < 0.001, 95% CI -14;-3, respectively). The prophylaxis group showed improvement of joint function (P = 0.004; CI 95% -3;-0.5); on the contrary, we found deterioration in the on-demand group (P = 0.001; CI 95% 1;3). HEAD-US scores showed improvement at month 6 in the prophylaxis group, but there was no significant difference between groups at month 12. CONCLUSION: Low-dose FVIII tertiary prophylaxis was effective in reducing joint bleeding episodes and improvement of HJHS compared to on-demand FVIII treatment in severe haemophilia A children.

Lipoblastoma and lipoblastomatosis of the lower leg.

Lipoblastoma is a benign lesion of immature fat cells that is found almost exclusively in pediatric population. This tumor is a rare tumor that occurs in infancy and early childhood, accounting for less than 1% of all childhood neoplasm. It is more common in male than in female and often presents as an asymptomatic, rapidly enlarging, soft lobular mass on the extremity. Although benign, it gives great difficulty in its management, due to its extensions into different facial planes, especially in lipoblastomatosis. Thus, complete surgical excision is the treatment of choice.