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To retrospectively explore the correlation between anterior cruciate ligament (ACL)-ruptured knees, stability of ACL-rupture knee and posterior tibial slope (PTS).From January 2008 to October 2012, 150 knees with ACL rupture underwent arthroscopic surgery for ACL reconstruction. A control group was established for subjects undergoing arthroscopic surgery without ACL rupture during the same period. PTS was measured on a digitalized lateral radiograph. Lachman and mechanized pivot shift tests were performed for assessing the stability of knee.There was significant difference (P = 0.007) in PTS angle between the patients with ACL rupture (9.5 ± 2.2 degrees) and the control group (6.6 ± 1.8 degrees). Only among females, increased slope of tibial plateau had effect on the Lachman test. There was a higher positive rate of pivot shift test in patients of increased posterior slope in the ACL rupture group.Increased posterior tibial slope (6.6) appears to contribute to non-contact ACL injuries in females. And the changes of tibial slope have no effect upon the Lachman test. However, large changes in tibial slope affect pivot shift.

The treatment of community-acquired pneumonia (CAP) is complicated by the growing threat of antimicrobial resistance and the tendency to rely on empirical therapy. This study investigated the etiologic agents of adult CAP in Taiwan and the susceptibility of Streptococcus pneumoniae isolates from these patients.A collaborative group was established in the emergency department to conduct a prospective study of the etiology of adult CAP. The etiologic agent was determined by a combination of microscopic, culture, serologic and antigen detection methods. Pneumococcal susceptibility testing was performed to determine the extent of penicillin resistance.A total of 100 consecutive cases of mild to moderate adult CAP prior to the severe acute respiratory syndrome epidemic were enrolled. The etiologic agent was determined in 72% of cases. The 5 most common causative pathogens were S. pneumoniae (26%), Mycoplasma pneumoniae (20%), Chlamydia pneumoniae (13%), Haemophilus influenzae (9%), and Klebsiella pneumoniae (5%). Atypical pathogens accounted for 40% of CAP. Bacteremic pneumonia was diagnosed in 6.2% of cases. Co-infections with 2 or more pathogens were found in 16% of the cases. Among the 20 isolates of S. pneumoniae, 85% (17/20) were susceptible to penicillin, 3 (15%) were intermediate, and none were resistant to penicillin.S. pneumoniae, M. pneumoniae and C. pneumoniae were the 3 leading causes of mild to moderate CAP in Taiwan. This study indicates that penicillin-resistant S. pneumoniae play a very limited role in this condition in adults.

Background and objectives Intensified immunosuppression in steroid-resistant nephrotic syndrome is broadly applied, with disparate outcomes. This review of patients from the United Kingdom National Study of Nephrotic Syndrome cohort aimed to improve disease stratification by determining, in comprehensively genetically screened patients with steroid-resistant nephrotic syndrome, if there is an association between response to initial intensified immunosuppression and disease progression and/or post-transplant recurrence. Design, setting, participants, & measurements Pediatric patients with steroid-resistant nephrotic syndrome were recruited via the UK National Registry of Rare Kidney Diseases. All patients were whole-genome sequenced, whole-exome sequenced, or steroid-resistant nephrotic syndrome gene-panel sequenced. Complete response or partial response within 6 months of starting intensified immunosuppression was ascertained using laboratory data. Response to intensified immunosuppression and outcomes were analyzed according to genetic testing results, pattern of steroid resistance, and first biopsy findings. Results Of 271 patients, 178 (92 males, median onset age 4.7 years) received intensified immunosuppression with response available. A total of 4% of patients with monogenic disease showed complete response, compared with 25% of genetic-testing-negative patients (P=0.02). None of the former recurred post-transplantation. In genetic-testing-negative patients, 97% with complete response to first intensified immunosuppression did not progress, whereas 44% of nonresponders developed kidney failure with 73% recurrence post-transplant. Secondary steroid resistance had a higher complete response rate than primary/presumed resistance (43% versus 23%; P=0.001). The highest complete response rate in secondary steroid resistance was to rituximab (64%). Biopsy results showed no correlation with intensified immunosuppression response or outcome. Conclusions Patients with monogenic steroid-resistant nephrotic syndrome had a poor therapeutic response and no post-transplant recurrence. In genetic-testing-negative patients, there was an association between response to first intensified immunosuppression and long-term outcome. Patients with complete response rarely progressed to kidney failure, whereas nonresponders had poor kidney survival and a high post-transplant recurrence rate. Patients with secondary steroid resistance were more likely to respond, particularly to rituximab.

Background: To study the response to pamidronate using whole body magnetic resonance imaging (WB-MRI) in children with chronic non-bacterial osteitis (CNO) in a tertiary health centre. Methods: The medical records of children under the age of sixteen with a diagnosis of chronic non-bacterial osteitis between 2005 and 2018 were reviewed. All those who were treated with pamidronate were included and relevant data was collected. Response to therapy was determined based on the status of lesions on WB- MRI. Results: Forty six patients were included in the study. Pre- and post-treatment WB-MRI was available in forty patients. Cumulative lesions pre-treatment were 150 and reduced to 45 (30%) post-treatment. Seventeen patients (42.5%) had a good response with complete resolution of all lesions and nine patients (22.5%) worsened during or following treatment with pamidronate. Vertebral disease had a good response and 82.3% of the lesions resolved completely. Conclusion: Our study describes the experience with pamidronate in a tertiary health centre using WB-MRI as a marker of disease activity. Pamidronate was well tolerated in our cohort and treatment response was fairly good. Significance and innovation: 1. Bisphosphonates can be used in the treatment of CNO when response to NSAIDs is suboptimal. 2. In the presence of spinal or mandibular lesions bisphosphonates were used as first line. 3. Treatment was escalated to a TNF blocker when response to bisphosphonates was suboptimal.

The scope of the study was to evaluate the probability of the adequacy of water intake among residents of São Paulo, and to investigate if the quality of diet differs according to the adequacy of water intake. Data were extracted from the Health Survey of São Paulo, a cross-sectional, population-based study. Water intake (drinking water beverages and food) was evaluated using two 24-hours dietary recalls and a supplementary dietary intake questionnaire. Adequacy and classification of water intake was assessed using the corresponding Adequate Intake for sex and age of the individual. Dietary quality was evaluated using the revised Brazilian Healthy Eating Index (BHEI). The associations were tested using the Theil-Sen median test and logistic regression models. A low probability of adequate water intake was observed, and this decreased with advancing age. Except among the elderly, among whom no association was observed, a positive association between water intake and the BHEI was observed, and for the total and integral fruit components, which was maintained after confusion adjustments. Among adults, it was observed that those who ingested less water had even lower scores for saturated fats. The residents of São Paulo have low water intake, and the quality of diet is worse among those who drink less water.O objetivo foi avaliar se a qualidade da dieta varia de acordo com a adequação da ingestão de água em residentes de São Paulo. Trata-se do Inquérito de Saúde de São Paulo, estudo transversal de base populacional. A ingestão de água total (potável, de bebidas e de alimentos) foi avaliada por meio de dois recordatórios alimentares de 24h e questionário complementar sobre a dieta, e a qualidade da dieta foi avaliada por meio do índice de qualidade da dieta revisado (IQDR) para a população brasileira. A Adequate Intake foi usada para estratificar os indivíduos. As associações foram testadas pelo teste Theil-Sen e pelo modelo de regressão logística. Observou-se ingestão de água de 2,20 L/d, e que a qualidade da dieta “necessita de modificações”. Exceto nos idosos em que não foi observada nenhuma associação, nos demais grupos mostrou-se que há associação positiva da ingestão de água com o IQDR, bem como com os componentes frutas totais e integrais. Estas associações foram mantidas mesmo após ajustes para variáveis de confusão. Nos adultos, observou-se que aqueles que ingeriram menos água tiveram menores pontuações para gorduras saturadas. A qualidade da dieta dos residentes de São Paulo é pior entre os que ingerem menos água.

Juvenile idiopathic arthritis (JIA) is the commonest rheumatic disease in children and JIA-associated uveitis its most frequent extra-articular manifestation. The uveitis is potentially sight-threatening and thus carries a considerable risk of morbidity with associated reduction in quality of life. The commonest form of uveitis seen in association with JIA is chronic anterior uveitis, which is almost always asymptomatic in the initial stages. Therefore, screening for JIA-associated uveitis in at-risk patients is essential. The aim of early detection and treatment is to minimise intraocular inflammation and to avoid complications that lead to visual loss, which can result from both disease activity and medications. The sight-threatening complications of JIA-associated uveitis include cataracts, glaucoma, band keratopathy, and macular oedema. There is increasing evidence for the early introduction of systemic immunosuppressive therapies to reduce topical and systemic use of glucocorticoids. A recently published randomised controlled trial of adalimumab in JIA-associated uveitis now provides convincing evidence for the use of this biologic in patients who fail to respond adequately to methotrexate. Tocilizumab and abatacept are being investigated as alternatives in children inadequately treated with anti–tumour necrosis factor drugs.

BACKGROUND: There are many single-gene causes of steroid-resistant nephrotic syndrome (SRNS) and the list continues to grow rapidly. Prompt comprehensive diagnostic testing is key to realising the clinical benefits of a genetic diagnosis. This report describes a bespoke-designed, targeted next-generation sequencing (NGS) diagnostic gene panel assay to detect variants in 37 genes including the ability to identify copy number variants (CNVs).METHODS: This study reports results of 302 patients referred for SRNS diagnostic gene panel analysis. Phenotype and clinical impact data were collected using a standard proforma. Candidate variants detected by NGS were confirmed by Sanger sequencing/Multiplex Ligation-dependent Probe Amplification with subsequent family segregation analysis where possible.RESULTS: Clinical presentation was nephrotic syndrome in 267 patients and suspected Alport syndrome (AS) in 35. NGS panel testing determined a likely genetic cause of disease in 44/220 (20.0%) paediatric and 10/47 (21.3%) adult nephrotic cases, and 17/35 (48.6%) of haematuria/AS patients. Of 71 patients with genetic disease, 32 had novel pathogenic variants without a previous disease association including two with deletions of one or more exons of NPHS1 or NPHS2.CONCLUSION: Gene panel testing provides a genetic diagnosis in a significant number of patients presenting with SRNS or suspected AS. It should be undertaken at an early stage of the care pathway and include the ability to detect CNVs as an emerging mechanism for genes associated with this condition. Use of clinical genetic testing after diagnosis of SRNS has the potential to stratify patients and assist decision-making regarding management.

Haemophagocytic syndrome, or haemophagocytic lymphohistiocytosis (HLH), is a hyperinflammatory disorder characterised by uncontrolled activation of the immune system. It can result from mutations in multiple genes involved in cytotoxicity or occur secondary to a range of infections, malignancies or autoimmune rheumatic diseases. In the latter case, it is also known as macrophage activation syndrome (MAS). Characteristic features are persistent fever, hepatosplenomegaly, petechial/purpuric rash, progressive cytopenias, coagulopathy, transaminitis, raised C reactive protein, falling erythrocyte sedimentation rate, hypertriglyceridaemia, hypofibrinogenaemia and extreme hyperferritinaemia often associated with multi-organ impairment. Distinguishing HLH from systemic sepsis can present a major challenge. Criteria for diagnosis and classification of HLH and MAS are available and a serum ferritin >10 000 µg/L is strongly supportive of HLH. Without early recognition and appropriate treatment, HLH is almost universally fatal. However, with prompt referral and advancements in treatment over the past two decades, outcomes have greatly improved.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, with JIA-associated uveitis its most common extra-articular manifestation. JIA-associated uveitis is a potentially sight-threatening condition and thus carries a considerable risk of morbidity. The aetiology of the condition is autoimmune in nature with the predominant involvement of CD4(+) T cells. However, the underlying pathogenic mechanisms remain unclear, particularly regarding interplay between genetic and environmental factors. JIA-associated uveitis comes in several forms, but the most common presentation is of the chronic anterior uveitis type. This condition is usually asymptomatic and thus screening for JIA-associated uveitis in at-risk patients is paramount. Early detection and treatment aims to stop inflammation and prevent the development of complications leading to visual loss, which can occur due to both active disease and burden of disease treatment. Visually disabling complications of JIA-associated uveitis include cataracts, glaucoma, band keratopathy and macular oedema. There is a growing body of evidence for the early introduction of systemic immunosuppressive therapies in order to reduce topical and systemic glucocorticoid use. This includes more traditional treatments, such as methotrexate, as well as newer biological therapies. This review highlights the epidemiology of JIA-associated uveitis, the underlying pathogenesis and how affected patients may present. The current guidelines and criteria for screening, diagnosis and monitoring are discussed along with approaches to management.

Macrophage activation syndrome (MAS) is a potentially life-threatening complication of rheumatic diseases such as systemic juvenile idiopathic arthritis (sJIA) and systemic lupus erythematosus. It is often considered a type of secondary hemophagocytic lymphohistiocytosis (HLH) and results from over-activation of T lymphocytes and macrophages leading to a "cytokine storm". Characteristic features are persistent fever, lymphadenopathy, hepatosplenomegaly, cytopenias (anemia, leucopenia, thrombocytopenia), raised C-reactive protein, falling erythrocyte sedimentation rate, hypofibrinogenemia, transaminitis, hypertriglyceridemia and extreme hyperferritinemia often associated with multi-organ impairment. Key to its management is early recognition of MAS which may be difficult due to similarity to systemic sepsis or flares of the underlying rheumatic disease. To aid with this process, criteria for the diagnosis of MAS in patients with sJIA derived by international consensus have been published. Although bone marrow biopsy showing hemophagocytosis is strongly supportive it is not essential for diagnosis. Together with appropriate supportive care, first-line treatment is high-dose intravenous corticosteroids with cyclosporin or intravenous immunoglobulin (IVIg) added if there is not initial response. Although etoposide is used by hematologists in treatment of HLH, there are concerns regarding organ toxicity and bone marrow suppression which weigh against its use in initial management of MAS. With increasing understanding of the pathogenesis of MAS, use of drugs targeting specific cytokines has been reported in case series. The relatively rapid effectiveness of anakinra, a recombinant IL-1 receptor antagonist, has been documented. Further studies of this and other biologic agents are required to identify the most effective and safest treatment option for refractory MAS.