Search

Aims and Objectives: To develop and evaluate the effectiveness of a structured therapeutic communication module on psychological distress and perceived needs among caregivers of critically ill patients., Background: Caregivers of critically ill patients experience intense psychological distress, and their needs often go unexpressed or unidentified. Structured therapeutic communication enables nurses to explore and fulfill these needs., Method: A mixed-method study was conducted among 30 caregivers of critically ill patients. During phase one, a qualitative interview was conducted, and a structured therapeutic communication module was developed based on Hildegard Peplau's Interpersonal Relations Theory. In the second phase, one group pre-test and post-test design was adopted. The Hospital Anxiety and Depression Scale (HADS) and a Semi-structured interview schedule were used to assess psychological distress and perceived needs, respectively., Results: Half of (50 %) the caregivers reported a high level of anxiety before intervention, with a mean of 11.30 (SD: 4.0), and 66.7 % of them had a high level of depression, with a mean of 12.03 (SD: 0.08). There was a statistically significant difference in anxiety (CI: 0.451-2.016) and depression (CI: 0.261-1.538) before and after the intervention. The qualitative analysis revealed unmet needs perceived by caregivers., Conclusion: Using a structured therapeutic communication module helps nurses to alleviate the psychological distress experienced by caregivers of CCU patients., Relevance to Clinical Practice: Nurses need to be sensitive to the unexpressed needs of caregivers of critically ill patients. The structured therapeutic communication modules can be integrated into routine nursing care practice to ensure family-centered care., Competing Interests: Declaration of competing interest Nil., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Purpose: Hand and wrist injuries are common among competitive athletes and can have a substantial impact on playing time and future participation. The purpose of this study was to provide epidemiological data from the National Collegiate Athletic Association Injury Surveillance Program to correlate injury diagnosis with the need for surgery and time loss., Methods: Using the National Collegiate Athletic Association Injury Surveillance Program, this retrospective study extracted data of hand and wrist injuries for all 25 National Collegiate Athletic Association sports from the academic years 2004-2005 to 2013-2014. The "severe" category was defined as injuries resulting in the following: (1) surgery, (2) season-ending status, or (3) more than 30 days of playing time loss. The epidemiologic data included injury rate per 100,000 athlete exposures (defined as 1 athlete participating in 1 practice or competition) based on diagnoses and demographic information such as sports and sex. We used a Poisson regression model to estimate the incidence rate and 95% confidence interval., Results: Overall, 4,851 hand injuries were identified, with an injury rate of 41.2 per 100,000 athlete exposures. The most common diagnoses were metacarpal or phalangeal fractures (19.9%), lacerations or contusions (15.4%), and wrist sprains (14.7%). The surgical rate was 9.6%, and the season-ending rate was 5.8%. Severe injuries occurred in 17.5% of the hand and wrist injuries; within this subset, the most common diagnoses included metacarpal or phalangeal fractures (43.8%), scaphoid fractures (12.8%), and thumb ulnar collateral ligament tears (8.7%). Scaphoid fractures and metacarpal or phalangeal fractures had the highest surgical rate and season-ending rate among all the injuries., Conclusions: The injury rate of hand and wrist injuries is comparable with those of other common sports injuries. Approximately one fifth of the injuries were considered severe, which led to a high surgical rate, and these had a considerable impact on the athletes' ability to finish the season., Type of Study/level of Evidence: Outcome research level II., (Copyright © 2023. Published by Elsevier Inc.)

The study aims at formulation and optimization of resveratrol and humic acid co-encapsulated colloidal polymeric nanocarriers to improve stability, oral bioavailability, and antiradical activity of water-insoluble, resveratrol. The eudragit E100 polymeric material was used to fabricate resveratrol and humic acid co-encapsulated oral colloidal polymeric nanocarriers ( Res -HA-co-CPNs) using emulsification-diffusion-evaporation method. Taguchi orthogonal array design was employed to check the effect of formulation factors on in vitro physicochemical characteristics. The optimized formulation was further evaluated for oral bioavailability as well as for antiradical potential. Optimized Res -HA-co-CPNs demonstrated spherical and smooth surface including mean particle size, 120.56 ± 18.8 nm; polydispersity index, 0.122; zeta potential, +38.25 mV; and entrapment efficiency, 82.37 ± 1.49%. Solid-state characterization confirmed the amorphous characteristic of optimized Res -HA-co-CPNs. In vitro release profile of Res -HA-co-CPNs showed sustained release behavior up to 48 h and CPNs were found to remain stable at the refrigerated condition for 6 months. In vivo pharmacokinetic studies revealed significant ( p  < 0.05) improvement of ∼62.76-fold in oral bioavailability. The radical-scavenging activity was found to be increased with time and after 72 h, it was analogous to pure Res . IC 50 values were reported to be decreased with time. Henceforth, developed Res -HA-co-CPNs was proven to be a proficient dosage form to increase stability, oral bioavailability, and antiradical activity of resveratrol.HighlightsResveratrol-humic acid co-encapsulated colloidal polymeric nanocarriers ( Res -HA-co-CPNs) were fabricated by emulsification-diffusion-evaporation method and optimized by Taguchi orthogonal array design.The Res -HA-co-CPNs revealed favorable mean particle size and percent encapsulation efficiency with a spherical and smooth surface.The Res -HA-co-CPNs showed diffusion-controlled release of Res and were found to be stable at the refrigerated condition for 6 months.The optimized Res -HA-co-CPNs demonstrated significantly ( p  < 0.05) higher oral bioavailability with respect to pure Res and PM.The optimized Res -HA-co-CPNs demonstrated higher radical-scavenging activity with respect to time.

Introduction: standardised protocols for the care of geriatric hip fractures demonstrate improved patient outcomes with decreased cost. The purpose of this study is to evaluate outcomes of a standardised hip fracture protocol at an urban safety-net hospital., Methods: All trauma patients presenting to our urban safety-net hospital are included in a trauma database and inpatient outcomes recorded. A hip fracture protocol was introduced at our institution in 2015, which depended on admission to a monitored setting due to the absence of a geriatric co-management service. The database was queried to identify patients surgically treated for a geriatric hip fracture in the 3 years prior to protocol implementation (2012-2014) and patients treated in the 3 years following protocol implementation (2016-2018). Demographics, time to surgery, inpatient complications, and length of stay were compared between groups., Results: A total of 633 patients treated operatively for isolated hip fractures were identified, 262 patients in the 2012-2014 pre-protocol cohort, and 371 patients in the 2016-2018 protocol cohort. Following implementation of a hip fracture protocol the number of patients admitted to a surgical service increased from 198 (76%) to 348 (94%, p < 0.005) with the number of patients being admitted to a monitored setting increasing from 40 (15%) to 83 (22%, p = 0.026). The time to surgery was reduced to 2.75 days ( p = 0.054). The complication rate fell from 23% to 4% ( p < 0.0005). Length of stay was significantly reduced from 13.2 days to 12 days ( p = 0.045)., Conclusions: A hip fracture protocol including admission to a monitored setting can be effectively implemented at an urban safety-net hospital where geriatric co-management is not available. This resulted in a decrease in complications and length of stay. Additional interventions are required to decrease average time to surgery below 36 hours.

Objective: To describe clinical patterns, identify associated drugs, and perform causality assessment of cutaneous adverse drug reactions., Materials and Methods: In this prospective, observational study, patients with signs of cutaneous drug reaction from the department of dermatology of a tertiary teaching hospital were included. Patients with viral exanthemas were excluded. The patterns of cutaneous drug reactions, and associated drugs were noted. Naranjo scale was used for causality assessment of adverse drug reactions., Results: Sixty-five patients (mean age 38.1 years) were included. Skin lesions were seen after 2 days of drug consumption in 25 (38.5%) patients. Pigmentation, fixed drug eruption, and maculo-papular rashes were seen in 13 (20%), 22 (33.8%), and 12 (18.5%) patients, respectively. Thirty-five (53.85%) patients had other associated symptoms. Fever was seen in 22 (33.8%) patients. Thirty-two (49.2%) cases had exposure to antibacterial agents. Most commonly associated drugs with reactions were nonsteroidal anti-inflammatory agents 14 (21.5%) followed by beta-lactam antibiotics 12 (18.5%), and fluroquinolones 7 (10.8%). Fifty-seven (87.7%) adverse drugs reactions had "probable" association with the drug.", Conclusion: Fixed drug eruption is the commonest presentation of cutaneous adverse drug reactions and antimicrobial agents are most commonly associated with it. According to the Naranjo scale, the majority of the cutaneous drug reactions have a "probable" association with the offending drugs., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Indian Journal of Dermatology.)

Background: Insertional Achilles tendinopathy (IAT) is a challenging common lower extremity disorder, despite several treatment options described in literature. Open dorsal closing wedge calcaneal osteotomy or Zadek Osteotomy (ZO), for the treatment of the IAT has good clinical results but a high rate of postoperative complications. The purpose of this study is to describe percutaneous ZO for the treatment of the IAT and to evaluate its impact on the clinical and functional postoperative outcomes., Methods: Twenty-six consecutive patients presenting with unilateral IAT refractory to nonoperative measures were treated with percutaneous ZO. Visual Analogue Scale (VAS) and Foot Function Index Score (FFI) were recorded preoperatively and at final follow-up visit (12±3) months. Postoperative complications, satisfaction, and relief of the pain were also recorded., Results: The percutaneous ZO showed a significant improvement (p<0.0001) in preoperative to postoperative FFI (from 65±9 to 8±12) and VAS (from 9±1 to 1±2). Two postoperative complications (8%) were observed: a case of symptomatic non-union and hardware pain, both in healthy patients. The overall rate of satisfaction after surgery was (92%). The relief from pain was achieved after an average period of 12 weeks., Conclusions: ZO is a safe and effective procedure for the treatment of IAT. The use of a minimally invasive surgical approach is associated with excellent pain reduction (VAS score) and improved clinical function (FFI score). When compared to the open surgical approach, the percutaneous ZO may decrease recovery time and postoperative complications., Level of Evidence: III, retrospective case series., (Copyright © 2019 European Foot and Ankle Society. Published by Elsevier Ltd. All rights reserved.)

Background: Achilles injuries are devastating injuries, especially for competitive athletes. No studies have examined the outcomes of Achilles injuries in NCAA athletes. Therefore, a better characterization and understanding of the epidemiology is crucial., Methods: Achilles injuries across 16 sports among NCAA men and women during the 2004-2005 to 2013-2014 academic years were analyzed using the NCAA Injury Surveillance Program (NCAA-ISP). Achilles tendon injury rate (IR) per 100,000 athlete-exposures (AEs), operative rate, annual injury rate trends, reinjury rates, mechanism of injury, in-season status (pre/regular/post season), and time loss distributions were compiled and calculated. A sub-analysis of comparing gender and injury mechanism was also performed for both all injuries and severe injuries., Results: Overall, N = 255 Achilles injuries were identified with an injury rate (IR) of 2.17 (per 100,000 AEs). These injuries occurred most often in women's gymnastics (IR = 16.73), men's basketball (IR = 4.26), and women's basketball (IR = 3.32), respectively. N = 52 injuries were classified as severe injuries which have higher median time loss (48 days) and higher operative rate (65.4%). For severe Achilles injuries, female athletes had higher operative (77.8% vs. 58.8%) and higher time loss compared to male athletes (96 days vs. 48 days). Contact mechanisms were associated with a higher season-ending injury rate., Conclusion: Overall, 20.4% of Achilles injuries were considered severe with 65.6% operative rate. About 73.1% were season-ending injuries, and the remaining athletes have a median time loss of 48 days. Severe Achilles injuries create significant impact on playing time and career for NCAA athletes.

First metatarsophalangeal (MTP) joint arthrodesis is a treatment option for patients with arthritic hallux valgus (HV). Nickel-titanium staples allow continuous compression throughout the fusion site and have been shown to achieve successful union in many procedures. However, their efficacy has not been tested in patients with underlying HV deformity. Three cases of severe HV deformity that underwent first MTP arthrodesis with 2 nickel-titanium staples placed 60° from each other and had failure are reported. The authors believe this construct does not provide adequate rotational control for first MTP arthrodesis in patients with severe HV deformity. [Orthopedics. 2019; 42(4):e402-e404.]., (Copyright 2019, SLACK Incorporated.)

Background: Anti-TNF (Tumor necrosis factor) therapy is effective in treating pediatric patients with refractory rheumatic disease. There is however a concern that anti-TNF usage may increase the risk of malignancy. Reports on specific types of malignancy in this patient population have been emerging over the past decade, but there is a need for additional malignancy reports, as these events are rare. Therefore, a retrospective chart review was performed on the biologic database of pediatric rheumatology patients at The Hospital for Sick Children (SickKids) from 1997 to 2013 for neoplasms, patient demographic information and rheumatologic treatment course., Findings: 6/357 (1.68%) rheumatology patients treated with anti-TNF therapy between 1997 and 2013 developed neoplasms. One patient had two malignancies. One patient had a benign neoplasm. Cases were exposed to etanercept, infliximab or both. Neoplasms developed late after anti-TNF exposure (median 5.0 years) and infliximab treatment was associated with a shorter time to malignancy. The neoplasms identified were as follows: 2 renal clear cell carcinoma, 1 pilomatricoma, 1 nasopharyngeal carcinoma, 1 Ewing's sarcoma, 1 hepatic T-cell lymphoma, 1 lymphoproliferative disease., Conclusions: The malignancy rate at our centre is low, however more than half of the neoplasms identified were rare and unusual in the pediatric population. The 5-year malignancy-free probability for patients with juvenile idiopathic arthritis (JIA) treated with biologic therapy was 97% from our database. Long-term screening for rare neoplasms is important as part of the safety monitoring for any pediatric rheumatology patient receiving anti-TNF therapy.

Nerve injury is a relatively rare, yet potentially devastating complication of total hip arthroplasty (THA). Incidence of this ranges from 0.6 to 3.7%, and is highest in patients with developmental hip dysplasia and previous hip surgery. Apart from patient and surgeon dissatisfaction, this complication can have medico-legal consequences. Therefore, the purpose of this study was to review the risk factors, etiology, diagnostic options, management strategies, prognosis, and prevention measures of nerve injuries associated with THA. We specifically evaluated the: 1) sciatic nerve; 2) femoral nerve; 3) obturator nerve; 4) superior gluteal nerve; and 5) the lateral femoral cutaneous nerve.

Objectives: To evaluate bone marrow aspirate concentrate (BMAC) use in the treatment of AFF., Design: Retrospective case control., Setting: Level 1 trauma center., Patients: Complete AFF, defined according to American Society of Bone and Mineral Research (ASBMR) criteria, from September 2009 to April 2015 with minimum 1-year follow-up., Intervention: Operative treatment with antegrade intramedullary nails. Beginning June 2014, BMAC from the ipsilateral iliac crest was added to all AFFs., Main Outcome Measurements: Time to union as determined by a blinded panel of 3 attending orthopaedic surgeons, union rates, complications., Results: Thirty-five patients with 36 AFFs were reviewed, of which 33 AFFs were included and 11 received BMAC. Alendronate was the most commonly prescribed bisphosphonate, with a similar mean duration of use in controls and BMAC cases (5.6 versus 6 years, P = 0.79). BMAC use significantly decreased time to union (3.5 versus 6.8 months, P = 0.004). Varus malreduction was associated with a significant delay in union (9.7 versus 4.7 months, P = 0.04). Overall, 1 year union rate was 86.2% and nonsignificantly higher in BMAC compared with controls (100.0% versus 77.3%, P = 0.11). Multivariate analysis revealed BMAC and varus malreduction as independent predictors of time to union. There were no complications related to BMAC use., Conclusion: Our findings support intramedullary nailing of AFFs as an effective treatment option with a low surgical complication rate and highlight the importance of avoiding varus malreduction. BMAC use significantly reduced time to fracture union without an increase in surgical complication rates., Level of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Objective: To determine cancer incidence in a large clinical juvenile-onset arthritis population., Methods: We combined data from 6 existing North American juvenile-onset arthritis cohorts. Patients with juvenile-onset arthritis were linked to regional cancer registries to detect incident cancers after cohort entry, defined as first date seen in the paediatric rheumatology clinic. The expected number of malignancies was obtained by multiplying the person-years observed (defined from cohort entry to end of follow-up) by the geographically matched age, sex and calendar year-specific cancer rates. The standardised incidence ratios (SIR; ratio of cancers observed to expected) were generated, with 95% CIs., Results: The 6 juvenile arthritis registries provided a total of 5294 patients. The mean age at cohort entry was 8.9 (SD 5.0) years and 68% of participants were female. The mean duration of follow-up was 6.8 years with a total of 36 063 person-years spanning 1978-2012. During follow-up, 9 invasive cancers occurred, compared with 10.9 expected (SIR 0.82, 95% CI 0.38 to 1.5). 3 of these were haematological (Hodgkin's, non-Hodgkin's lymphoma and leukaemia). 6 of the patients with cancer were exposed to disease-modifying drugs; 5 of these had also been exposed to biological agents., Conclusions: We did not clearly demonstrate an increase in overall malignancy risk in patients with juvenile-onset arthritis followed for an average of almost 7 years. 3 of the 9 observed cancers were haematological. 5 of the cancers arose in children exposed to biological agents. Longer follow-up of this population is warranted, with further study of drug effects.

Recent studies have noted an increased risk of low energy subtrochanteric and femoral shaft fractures termed "atypical femur fractures" (AFFs) associated with long-term bisphosphonate use. As such, many clinicians have begun recommending a "drug holiday" to reduce the risks associated with long-term bisphosphonate use. We present two cases of AFFs occurring during a 4-year or greater drug holiday following long-term bisphosphonate use. These findings highlight the need to reevaluate optimal bisphosphonate therapy duration, dosage, as well as initiation and duration of a drug holiday with continued monitoring in the prevention of AFFs.

PURPOSE. To compare the rates of joint hypermobility in patients with and without anterior cruciate ligament (ACL) injury. METHODS. Records of 135 men and 75 women (mean age, 24.6 years) who underwent ACL reconstructions were reviewed and compared with 55 male and 35 female controls with no knee injury. Joint hypermobility was evaluated by 2 examiners using the Beighton score. The maximum score was 9, and a score of 4 or greater was defined as hypermobility. RESULTS. The mean time from injury to presentation was 18 days. A non-contact mechanism of injury was more common. The inter-observer reliability was 0.7. 127 (60.5%) of the patients with ACL injury and 23 (25.5%) of the controls had hypermobility (p<0.01). Among them, 58% and 24% were men and 65% and 29% were women, respectively. Female gender was associated with hypermobility. Patients with ACL injury were more likely to have joint hypermobility with an odds ratio of 4.46. CONCLUSION. Joint hypermobility was more common in patients with ACL injury.

Aim: To track the short-term neck narrowing changes in Birmingham metal-on-metal hip resurfacing (MOMHR) patients., Methods: Since 2001, the Center for Hip and Knee Replacement started a registry to prospectively collect data on hip and knee replacement patients. From June 2006 to October 2008, 139 MOMHR were performed at our center by two participate surgeons using Birmingham MOMHR prosthesis (Smith Nephew, United States). It is standard of care for patients to obtain low, anteriorposterior (LAP) pelvis radiographs immediately after MOMHR procedure and then at 3 mo, 1 year and 2 year follow up office visits. Inclusion criteria for the present study included patients who came back for follow up office visit at above mentioned time points and got LAP radiographs. Exclusion criteria include patients who missed more than two follow up time points and those with poor-quality X-rays. Two orthopaedic residency trained research fellows reviewed the X-rays independently at 4 time points, i.e., immediate after surgery, 3 mo, 1 year and 2 year. Neck-to-prosthesis ratio (NPR) was used as main outcome measure. Twenty cases were used as subjects to identify the reliability between two observers. An intraclass correlation coefficient at 0.8 was considered as satisfied. A paired t-test was used to evaluate the significant difference between different time points with P < 0.05 considered to be statistically significant., Results: The mean NPRs were 0.852 ± 0.056, 0.839 ± 0.052, 0.835 ± 0.051, 0.83 ± 0.04 immediately, 3 mo, 1 year and 2 years post-operatively respectively. At 3 mo, NPR was significantly different from immediate postoperative X-ray (P < 0.001). There was no difference between 3 mo and 1 year (P = 0.14) and 2 years (P = 0.53). Femoral neck narrowing (FNN) exceeding 10% of the diameter of the neck was observed in only 4 patients (5.6%) at two years follow up. None of these patients developed a femoral neck fracture (FNF)., Conclusion: Femoral neck narrowing after MOMHR occurred as early as 3 mo postoperatively, and stabilized thereafter. Excessive FNN was not common in patients within the first two years of surgery and was not correlated with risk of FNF.

Objective: To compare the American College of Rheumatology paediatric (ACRp) response criteria and conventional radiography with MRI findings in a cohort of patients with juvenile idiopathic arthritis., Methods: Forty consecutive patients (30 girls, 10 boys; median age 10.8 years) with arthritis of the wrist starting treatment with disease-modifying antirheumatic drugs or biological agents were recruited. At 1-year follow-up the treatment response was assessed by ACRp criteria and radiographic progression using the adapted Sharp/van der Heijde method. Wrist MRIs were evaluated using both the paediatric-MRI and the OMERACT rheumatoid arthritis MRI scores. Sensitivity to change of clinical and imaging variables was assessed by standardised response mean (SRM) and relative efficiency (RE) was used to compare SRMs., Results: ACRp90 responders showed a significantly higher decrease in MRI synovitis score (median change -4) than non-responders (median change 0), ACRp30-50 responders (median change 0) and ACRp70 responders (median change -1) (p=0.0006, Kruskal-Wallis test). Non-responders showed significantly higher radiographic progression than ACRp90 responders (pB=0.016). The MRI synovitis score showed a greater responsiveness to change (SRM 1.69) compared with the majority of ACR core set of variables. MRI erosion scores were less responsive than conventional radiography in detecting destructive changes (RE <1). MRI follow-up revealed no signs of inflammation in four out of 24 wrists with clinically inactive disease., Conclusion: Only ACRp90 responders showed a significant decrease in synovitis and the halting of structural damage, suggesting that levels of response higher than ACRp30 are more appropriate for assessing drug efficacy. The excellent responsiveness of MRI and its ability to detect subclinical synovitis make it a promising outcome measure.

Objective: To put forward a new concept--Blau arteritis, a form of large-vessel vasculitis phenotypically related to Takayasu disease but genetically and clinically part of an expanded phenotype of Blau syndrome., Methods: We provide a clinical description of a new case and summarize previously published cases of arteritis associated with Blau syndrome. Genetic testing was performed by direct sequencing of exon 4 of the NOD2 gene., Results: The case described and those reviewed from the literature demonstrate the emerging phenotype of Takayasu-like arteritis in patients with Blau syndrome. Although most patients described to date depict an otherwise classic Blau syndrome phenotype, the current case was atypical in that the predominant features were arteritic. A novel substitution, G464W, in a highly conserved position near the nucleotide oligomerization domain of the NOD2 protein is also described., Conclusion: Blau arteritis can be observed in the context of both typical and atypical (incomplete) Blau syndrome. The associated mutation in the NOD2 gene raises the question of the potential importance of this gene among patients with "primary" forms of Takayasu arteritis.

Objectives: To evaluate the performance of the Birmingham Vasculitis Activity Score (BVAS) v3 and the Disease Extent Index (DEI) for the assessment of disease activity in 4 primary childhood (c-) systemic vasculitides., Methods: Patients fulfilling the EULAR/PRINTO/PRES (Ankara) c-vasculitis classification criteria for Henoch-Schönlein purpura (HSP), childhood (c) polyarteritis nodosa (c-PAN), c-Wegener's granulomatosis (c-WG) and c-Takayasu arteritis (c-TA) with disease duration at the time of diagnosis ≤3 months were extracted from the PRINTO database. The performance of the BVAS and DEI were examined by assessing convergent validity, the pattern of disease involvement, and responsiveness. We also evaluated alternative unweighted scoring methods for both tools., Results: The analysis set included 796 patients with 669 HSP, 80 c-PAN, 25 c-WG and 22 c-TA. The median age at diagnosis was 6.9 years (6.6-12) and median delay in making the diagnosis from the onset of signs/symptoms was 0.01 (0.003-0.027) years. A strong correlation was found between the BVAS and DEI (rs=0.78) while correlation with the physician global assessment was moderate (rs=0.48) with BVAS and poor with DEI (rs=0.25). Both the BVAS and DEI sub-scores and total scores were able to descrive the disease involvement in the 4 childhood vasculitides. Responsiveness was large (>1.5) for both tools. The performance characteristics of the BVAS and DEI with the unweighted methods were comparable., Conclusions: This study demonstrates that both the BVAS and DEI are valid tools for the assessment of the level of disease activity in a large cohort of childhood acute and chronic vasculitides.

Objective: To evaluate response to therapy over a 24-month period in a large prospective international cohort of patients with juvenile dermatomyositis (DM)., Methods: The study included 145 patients with recent-onset juvenile DM and 130 juvenile DM patients experiencing disease flare, all of whom were <18 years old. Disease activity parameters and therapeutic approaches in 4 geographic areas were analyzed at baseline and at 6, 12, and 24 months. Response was assessed according to the Pediatric Rheumatology International Trials Organization (PRINTO) juvenile DM response criteria, and data were reported "as observed" and in the intent-to-treat (ITT) population., Results: Patients with recent-onset juvenile DM at baseline had higher baseline disease activity and greater improvement over 24 months when compared to juvenile DM patients experiencing disease flare at baseline. Methotrexate (MTX) or high-dose corticosteroids were administered more frequently to patients with recent-onset juvenile DM, compared to juvenile DM patients experiencing disease flare, who were more likely to receive cyclosporine. Compared to patients from Western and Eastern Europe, a higher proportion of patients from South and Central America and North America received pulse steroids, and the average steroid dosage was higher in the North American and South and Central American patients. The use of MTX was similar in all 4 regions, while cyclosporin A was more frequently used in Western Europe. In the "as observed" analysis, 57.9% of the patients with recent-onset juvenile DM and 36.4% of the patients experiencing disease flare (P<0.001) reached at least a 70% response by PRINTO criteria at 6 months; these proportions had increased at month 24 to 78.4% and 51.2%, respectively (P<0.001). Corresponding results of the ITT analysis were much lower, with only one-third of the patients able to maintain the initial assigned therapy over 24 months., Conclusion: Patients with recent-onset juvenile DM are more likely to achieve significant clinical improvement over 24 months, when compared to patients experiencing flares of juvenile DM. Internationally, various therapeutic approaches are used to treat this disease., (Copyright © 2011 by the American College of Rheumatology.)

Purpose: Resistant nonunion of distal femoral fractures in the elderly is a great challenge to treat because it is often associated with multiple problems like poor bone quality, arthritis, disuse osteopenia, joint contractures, and implant failure. We treated ten patients with megaprosthesis and report the outcome., Methods: Ten elderly patients of resistant nonunion of distal femoral fractures with arthritis knee were treated with megaprosthesis. The median patient age was 74 years (68-85 years). All these patients had undergone at least two prior operative procedures and were operated by us, 24-33 months following the first index surgery. Modular Resection System was used in all the patients., Results: Eight patients were available for follow-up. Median follow-up period was 4 years. Two patients required extended local wound care for wound skin necrosis. One patient sustained traumatic periprosthetic subtrochanteric fracture following a subsequent trauma. No thromboembolic complications were seen. All knees showed satisfactory alignment and range of motion. Postoperative, median knee society pain score, and knee society function score were 84 (80-88) and 88 (84-92) at last follow-up., Conclusion: Megaprosthesis can be offered as a one-stage salvage procedure for difficult nonunion in distal femoral fractures associated with bone loss, osteoporosis, and secondary knee arthritis.

Purpose: In this review we describe the general methodology and the results of the international projects, conducted by the Paediatric Rheumatology International Trials Organisation (PRINTO), in collaboration with the Paediatric Rheumatology Collaborative Study Group (PRCSG). The aim of these projects were to identify and validate criteria for the evaluation of response to therapy in clinical trials and in daily clinical practice in patients with the three major paediatric rheumatic diseases (PRD): juvenile idiopathic arthritis (JIA), juvenile dermatomyositis (JDM) and juvenile systemic lupus erythematosus (JSLE)., Methods: The methodological approach to identify and validate outcome measures can be divided into three main phases: (1) the development of a preliminary core set of measures to evaluate the outcome (e.g. response to therapy, remission criteria, disease activity or damage etc.) through literature review and consensus techniques; (2) a large-scale data collection for a prospectively evidence-based validation of the preliminary findings; (3) the final development of a validated criteria for the evaluation of the outcome., Results: The core sets for three diseases included domains that are common to all diseases (physician's global assessment of disease activity; parent's global assessment of the overall patient's well-being; disability and/or health-related quality of life) plus additional domains that are specific for each disease. In order to be classified as a responder to a given treatment, a patient should demonstrate a different minimum level of improvement (≥30% in JIA, ≥20% in JDM, and ≥50% in JSLE) with no more than one of the remaining variables worsening by more than 30%., Conclusions: The proposed core sets and definitions of improvement incorporate clinically meaningful change in a composite endpoint for the evaluation of global response to therapy in the major PRD. The definitions are proposed for use in PRD clinical trials and may help physicians to decide if a child has responded adequately to therapy.

We report a four year old boy who presented with liver failure secondary to anti-thrombin III deficiency related Budd Chiari syndrome. He was treated with TIPSS (transjugular intrahepatic porto systemic shunt) which reversed the encephalopathy, normalised the liver function and improved growth, pre-empting the need for a liver transplantation. This is the first reported case of TIPSS in a child with a fulminant presentation of Budd-Chiari Syndrome.

Objectives: To study the prevalence of joint hypermobility in children from Mumbai, India and to study its association with malnutrition., Methods: In a cross-sectional field study from September '02 to February '03 in Mumbai, 829 children of the lower urban socio-economic strata, between 3 and 19 years of age were evaluated independently by two observers for hypermobility using the Beighton 9-point scoring system. A score of >or= 4/9 was considered positive. Their nutritional status was stratified using standard Indian growth charts and hypermobility was quantified in various nutritional groups. Musculoskeletal symptoms were assessed by a questionnaire given to parents. Standard tests of significance (Chi square test, p<0.05-significant) were applied., Results: 58.7% of the population studied, had a Beighton score >or= 4/9. There was a declining prevalence of joint hypermobility noted with increasing age. Near equal sex incidence was noted. A higher incidence of finger signs was noted in comparison to elbow hyperextension, knee hyperextension and hands-to-floor. 26% of the hypermobile population had musculoskeletal symptoms as compared with 17.2% of the non-hypermobile population (p<0.05). A positive Beighton score was found in 452/734 (61.5%) children with Grade 3 and 4 malnutrition in comparison to 35/95 (36.8%) children with normal nutrition or mild grades (Grade 1 and 2) of malnutrition (p<0.05). In the group with Grade 3 and 4 malnutrition, 26.1% of those hypermobile had musculoskeletal symptoms in comparison to 17.7% of their non-hypermobile counterparts (p<0.05)., Conclusions: In our study population: 1. A high prevalence of hypermobility using Beighton's score was noted; 2. Finger signs of the Beighton score were more common than the other signs; 3. Moderate and severe malnutrition were associated with hypermobility; 4. Musculoskeletal symptoms were linked to joint hypermobility; 5. Moderate and severely malnourished hypermobile children were more likely to have musculoskeletal symptoms as compared to their non-hypermobile counterparts.