Bilateral lipid keratopathy with rheumatic fever in siblings.

PURPOSE: To report two unusual cases of lipid keratopathy in siblings with rheumatic fever and positive anti-phospholipid antibodies. METHODS: Clinical and histopathological examination along with complete systemic work-up was done after taking appropriate consent from the patients. A serum lipid profile showed elevated cholesterol and low-density lipoproteins. Systemic history revealed both the siblings were on treatment for rheumatic fever. A complete blood profile showed positive anti-phospholipid antibodies in one of them. RESULTS: Penetrating keratoplasty was performed in both cases one year apart. Histopathological examination showed lipid filled macrophages distributed throughout the corneal stroma. No episode of recurrence was noted in the follow-up period of three years. CONCLUSION: These two cases highlight the importance of a thorough family history and a full systemic work-up in a patient with bilateral symmetrical lipid keratopathy. The co-existence of rheumatic fever and anti-phospholipid syndrome has been studied in literature but, their presence along with lipid keratopathy has not been previously described. It requires further research whether they have an association or it was just an incidental finding.

Predictive performance of six mortality risk scores and the development of a novel model in a prospective cohort of patients undergoing valve surgery secondary to rheumatic fever.

BACKGROUND: Mortality prediction after cardiac procedures is an essential tool in clinical decision making. Although rheumatic cardiac disease remains a major cause of heart surgery in the world no previous study validated risk scores in a sample exclusively with this condition. OBJECTIVES: Develop a novel predictive model focused on mortality prediction among patients undergoing cardiac surgery secondary to rheumatic valve conditions. METHODS: We conducted prospective consecutive all-comers patients with rheumatic heart disease (RHD) referred for surgical treatment of valve disease between May 2010 and July of 2015. Risk scores for hospital mortality were calculated using the 2000 Bernstein-Parsonnet, EuroSCORE II, InsCor, AmblerSCORE, GuaragnaSCORE, and the New York SCORE. In addition, we developed the rheumatic heart valve surgery score (RheSCORE). RESULTS: A total of 2,919 RHD patients underwent heart valve surgery. After evaluating 13 different models, the top performing areas under the curve were achieved using Random Forest (0.982) and Neural Network (0.952). Most influential predictors across all models included left atrium size, high creatinine values, a tricuspid procedure, reoperation and pulmonary hypertension. Areas under the curve for previously developed scores were all below the performance for the RheSCORE model: 2000 Bernstein-Parsonnet (0.876), EuroSCORE II (0.857), InsCor (0.835), Ambler (0.831), Guaragna (0.816) and the New York score (0.834). A web application is presented where researchers and providers can calculate predicted mortality based on the RheSCORE. CONCLUSIONS: The RheSCORE model outperformed pre-existing scores in a sample of patients with rheumatic cardiac disease.

Rheumatic fever with severe carditis: still prevalent in the South West Pacific.

Rheumatic heart disease (RHD) has a worldwide prevalence of 33 million cases and 270 000 deaths annually, making it the most common acquired heart disease in the world. There is a disparate global burden in developing countries. This case report aims to address the minimal RHD coverage by the international medical community. A Tahitian boy aged 10 years was diagnosed with advanced heart failure secondary to RHD at a local clinic. Previous, subtle symptoms of changes in handwriting and months of fever had gone unrecognised. Following a rapid referral to the nearest tertiary centre in New Zealand, urgent cardiac surgery took place. He returned home facing lifelong anticoagulation. This case highlights the RHD burden in Oceania, the limited access to paediatric cardiac services in countries where the RHD burden is greatest and the need for improved awareness of RHD by healthcare professionals, and the general public, in endemic areas.

European Heart for Children: a story of humanity and success.

Since its foundation in 2009 and the last publications, European Heart for Children has grown substantially.

Acute rheumatic fever and rheumatic heart disease in resource-limited settings.

Poststreptococcal complications, such as acute rheumatic fever (ARF) and rheumatic heart disease (RHD), are common in resource-limited settings, with RHD recognised as the most common cause of paediatric heart disease worldwide. Managing these conditions in resource-limited settings can be challenging. We review the investigation and treatment options for ARF and RHD and, most importantly, prevention methods in an African setting.

[Arthrodesis for patients with rheumatic arthritis of the ankle and hindfoot. A reasonable option?]. / Arthrodesen bei Patienten mit rheumatisch bedingten Sprunggelenks- und Rückfußarthrosen. Eine sinnvolle Option?

BACKGROUND: Ankle and hindfoot deformities as well as degenerative changes are often found in patients with rheumatological diseases. They often suffer from severe pain and complain of increasing immobility. Corrective procedures with ankle or hindfoot arthrodesis are promising options. OBJECTIVES: This article presents epidemiological data and describes the clinical aspects, diagnostics and treatment options for patients with ankle and hindfoot osteoarthritis. MATERIALS AND METHODS: The retrospective results of 56 patients after ankle or hindfoot arthrodesis are presented. RESULTS: After an average follow-up of 52 months the majority of results were good or excellent with relief of pain and reconstruction of the function of the foot. CONCLUSION: Ankle or hindfoot arthrodesis represents a promising option for patients with severe osteoarthritis and can safeguard patients from increasing immobility.

Modified Blair ankle fusion for ankle arthritis.

OBJECTIVE: To investigate the clinical outcome of modified Blair ankle fusion for ankle arthritis. METHODS: Between November 2009 and June 2012, 28 patients with ankle arthritis were treated, among whom 11 had obvious foot varus deformity, and 17 were almost normal in appearance. There were 13 males and 15 females with an average age of 49.4 years (range, 23-67 years). The main symptoms included swelling, pain, and a limited range of motion of the ankles. The ankle joints functions were assessed by American Orthopedic Foot and Ankle Society (AOFAS) ankle and hindfoot score and visual analog scale (VAS) preoperatively and at 1 year follow-up. RESULTS: Twenty-eight patients were followed up for 19.8 months on average (range, 1-2 years). Superficial wound infection occurred in 3 cases, and was cured after debridement; the other incisions healed by first intention without complications. All ankles were fused at 1 year follow-up after operation. The symptom was relieved completely in all patients at last follow-up without complication of implant failure, or nonunion. The postoperative AOFAS ankle and hindfoot score was 83.13±3.76, showing significant difference when compared with the preoperative score (45.38±3.21, P<0.01). VAS was significantly decreased from 8.01±0.63 to 2.31±1.05 at 1 year follow-up (P<0.05). CONCLUSION: Modified Blair ankle fusion has the advantages of high feasiblity, less cost and rigid fixation. It shows high reliability in pain relief and may obtain a good clinical effectiveness.

The influence of preoperative deformity on valgus correction angle: an analysis of 503 total knee arthroplasties.

We prospectively studied variations in valgus correction angle (VCA) and the influence of preoperative limb deformity on VCA in 503 consecutive total knee arthroplasties done in 393 patients. The percentage of limbs that had VCA values less than 5° was 10.9%, and that with VCA values greater than 7° was 44.9%. The percentage of limbs with VCA greater than 7° was significantly more in varus knees, and that with VCA less than 5° was significantly more in valgus knees; preoperative deformity showed a significant correlation with VCA. Choosing a fixed-routine VCA of 5° to 7° may cause an unacceptable planning error that may be minimized by individualizing VCA or using computer navigation.

Functional disabilities and issues of concern for Asian patients before total ankle arthroplasty.

Total ankle arthroplasty is a commonly performed invasive procedure that can be distressing to patients. Therefore, surgeons should consider patients' issues of greatest interest and concerns at the time of surgery and the function that patients hope to recover. Many studies have reported surgeon concerns before total knee arthroplasty and total hip arthroplasty, but few have focused on patients. The purpose of this study was to evaluate patients' functional disabilities and issues of concern regarding total ankle arthroplasty.Between May 2008 and June 2010, eighty-five patients (52 men and 33 women; mean age, 60 years) were recruited for the study. All patients were asked to complete a questionnaire divided into 3 parts: sociodemographic data, current functional disabilities and their perceived importance, and issues concerning patients before total ankle arthroplasty. Regarding functional disability based on severity, the top 5 issues were limping, difficulty squatting, daytime pain, difficulty kneeling, and difficulty climbing stairs. Regarding functional disability based on perceived importance, the top 5 issues were daytime pain, limping, difficulty walking, difficulty kneeling, and difficulty working. Regarding issues of concern, the top 5 issues were pain intraoperatively, ability to walk as much as desired, ability to climb stairs, pain after discharge from the hospital, and pain immediately postoperatively. The most important issue before total ankle arthroplasty was pain. Patients had a strong interest in high ankle extension and increased range of motion due to the lifestyle and religious activities of Eastern populations.

Efficacy of radiosynovectomy in rheumatoid arthritis.

In this retrospective study, we evaluated the effect of radiosynovectomy of patients with rheumatoid arthritis. Radiosynovectomy was performed in 577 joints of 137 rheumatoid patients. We applied 185 MBq yttrium-90 in knees (n = 58), 74-111 MBq rhenium-186 colloids in ankle (n = 50), wrists (n = 43) and shoulders (n = 35), and 15 to 37 MBq in finger (n = 298) and toe joints (n = 46). The effect of radiosynovectomy was scored in 4 subjective categories: excellent response (no symptoms); good response (significant reduction of symptoms); moderate response (slight decrease); and bad response (no change or worsening), of pain and/or swelling in treated joint 3 months after the procedure. Excellent or good response was observed in 57% of treated knees, 63% of shoulders, 60% of wrists, 64% of ankles, 54% of thumb bases, 55% of MCP's, 54% of PIP's, 53% of DIP's, and 54% of MTP's. Side effects associated to the RSO, i.e., swelling or transient increase of pain, were recorded in 7% of the patients that resolved within 1 month. No patient had any non-reversible skin alteration after treatment, only slight erythema was observed in 5 patients. Radiosynovectomy is effective and safe in the treatment of rheumatoid arthritis.

Variability of medial and posterior offset in patients with fourth-generation stemmed shoulder arthroplasty.

PURPOSE: Most anthropometric data on the proximal humerus has been obtained from deceased healthy individuals with no deformities. Endoprostheses are implanted for primary and secondary osteoarthritis, rheumatoid arthritis,humeral-head necrosis, fracture sequelae and other humeral-head deformities. This indicates that pathologicoanatomical variability may be greater than previously assumed. We therefore investigated a group of patients with typical shoulder replacement diagnoses, including posttraumatic and rheumatic deformities. MATERIAL AND METHODS: One hundred and twenty-two patients with a double eccentrically adjustable shaft endoprosthesis served as a specific dimension gauge to determine in vivo the individual humeral-head rotation centres from the position of the adjustable prosthesis taper and the eccentric head. RESULTS: All prosthesis heads were positioned eccentrically.The entire adjustment range of the prosthesis of 12 mm medial/lateral and 6 mm dorsal/ventral was required. Mean values for effective offset were 5.84 mm mediolaterally[standard deviation (SD) 1.95, minimum +2, maximum +11]and 1.71 mm anteroposteriorly (SD 1.71, minimum −3,maximum 3 mm), averaging 5.16 mm (SD 1.76, minimum +2,maximum + 10). The posterior offset averaged 1.85 mm(SD 1.85, minimum −1, maximum + 6 mm). CONCLUSIONS: In summary, variability of the combined medial and dorsal offset of the humeral-head rotational centre determined in patients with typical underlying diagnoses in shoulder replacement was not greater than that recorded in the literature for healthy deceased patients.The range of deviation is substantial and shows the need for an adjustable prosthetic system.

Ceramic-on-ceramic total hip arthroplasty in patients younger than 20 years.

Total hip arthroplasty (THA) in the adolescent patient has historically shown relatively poor survivorship. This study reports the results of THA in young patients using contemporary ceramic bearings. Twenty-four THAs were performed using ceramic bearing surfaces in patients 20 years old or younger (mean, 16.4; range, 12-20). Average follow-up was 52 months (range, 25-123 months). The survival rate was 96%, with 1 revision for a loose acetabular component. Other complications included a peroneal nerve palsy that resolved and 2 dislocations in 1 patient. Postoperatively, the Modified Harris Hip Score mean was 93.4 (range, 66-100). This study shows promising results at short-term to midterm follow-up in very young patients who undergo THA using ceramic-on-ceramic components.

Febre reumática / Rheumatic fever

Ao final do curso acompanhamos vários casos de febre reumática no HUAP, e decidimos realizar uma breve revisão bibliográfica sobre o tema. Foram levantadas publicações nacionais e internacionais. Dividimos o tema, sob o ponto de vista didático, em 12 capítulos, desde a introdução até o tratamento cirúrgico. Concluímos que a doença, embora frequente vem diminuindo em nosso estado, graças à melhoria no nível primário dos servidores de saúde.

Risk of reoperation for aortic bioprosthesis dysfunction.

BACKGROUND AND AIM OF THE STUDY: The risk of reoperative valve replacement for failed aortic bioprosthesis may be overestimated, this being a dominant factor when selecting an initial prosthesis to be implanted in patients aged <70 years. The study aims were, first, to analyze the mortality and morbidity of redo aortic bioprosthesis replacement in the current era, and second, to identify preoperative risk factors and evaluate the EuroSCORE. METHODS: A total of 156 consecutive patients (111 men, 45 women; mean age 60.9 years; range: 23-87 years) who underwent reoperation for failed aortic bioprosthesis between 1990 and 2006 was reviewed in this retrospective, single-center study. Surgery was undertaken due to bioprosthesis degeneration (82.7%), bacterial prosthetic endocarditis (14.1%), paravalvular leak (1.3%) and other causes (1.9%). Emergency procedures were performed in 9% of patients. Associated procedures were coronary artery bypass grafting in 7.7% of patients, ascending aortic graft in 7%, and complete aortic root replacement in 6.4%. The predictive mortality was 8% according to the Additive EuroSCORE, and 15% according to the Logistic EuroSCORE. RESULTS: Overall, the operative mortality was 3.8% (n = 6), and postoperative morbidity was low. The only multivariable predictor was emergency surgery (OR = 15.22, 95% CI = 1.68-86.43; p = 0.02). A mortality trend was associated with atrial fibrillation and NYHA class III/IV, but this was not statistically significant (p = 0.09 and p = 0.06, respectively). Associated procedures were not significant risk factors for mortality. CONCLUSION: Reoperation for aortic bioprosthesis dysfunction can be performed with a low risk of mortality. It appears that this risk is overestimated by the EuroSCORE. Those patients who wish to avoid postoperative anticoagulant therapy may choose to receive this type of valve, even if reoperation is foreseeable.

Comparison of mechanical and biological prostheses when used to replace heart valves in children and adolescents with rheumatic fever.

OBJECTIVE: To assess the outcomes in children and adolescents with rheumatic fever of the implantation of mechanical as opposed to biological heart valves. METHODS: We assessed 73 patients with rheumatic heart disease under the age of 18 years, who underwent replacement of heart valves between January, 1996, and December, 2005, at the National Institute of Cardiology in Rio de Janeiro, Brazil. Of the group, 71 patients survived, and were divided into a group of 52 receiving mechanical prostheses, and 19 with biological prostheses. We compared endpoints between the groups in terms of mortality, reoperation, haemorrhage, and stroke. Survival curves were estimated using the Kaplan-Meier method and were compared by the Mantel (log-rank) test. RESULTS: Overall mortality was 8.2%. In those receiving mechanical prostheses, 2 (3.8%) patients died, 5 (9.6%) underwent reoperation, 2 (3.8%) suffered severe haemorrhage, and 3 (5.8%) had strokes. In those receiving biological valves, 2 (10.5%) patients died, and 4 (21%) underwent reoperation. After 2, 4, and 8 years, overall survival was 96%, 93% and 86%, respectively, with a borderline difference between the groups (p = 0.06). The probabilities of remaining free from reoperation (p = 0.13), and from combined endpoints, showed no statistically significant difference between the groups (p = 0.28). CONCLUSIONS: Patients with mechanical prostheses had lower mortality and required fewer reoperations, but when all combined endpoints were considered, the groups did not differ. The biological prosthesis proved to be a good option for cardiac surgery in children and adolescents with difficulties or risks of anticoagulation.

Epidemiology and prevention of valvular heart diseases and infective endocarditis in Africa.

Valvular heart diseases in Africa affect mainly children and young adults and are a result of rheumatic fever. Rheumatic fever is a preventable disease, but in Africa the combination of a lack of resources, lack of infrastructure, political, social and economic instability, poverty, overcrowding, malnutrition and lack of political will contributes to the persistence of a high burden of rheumatic fever, rheumatic valvular heart diseases and infective endocarditis. Combating and eradicating rheumatic fever and rheumatic heart diseases requires economic development and implementation of best practices of primary and secondary prevention measures. The barriers to achieving this goal in Africa are numerous, but not insurmountable.