Joint status in Spanish haemophilia B patients assessed using the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score.

AIM: The use of musculoskeletal ultrasound (MSK-US) following protocols for haemophilic arthropathy and the Haemophilia Early Arthropathy Detection with Ultrasound (HEAD-US) score can help standardize monitoring in haemophilia. This study evaluated the joint status (elbows, knees and ankles) of patients with haemophilia B (HB) in Spain using MSK-US and the HEAD-US score. METHODS: Haemophilia B patients ≥14 years old were included in this observational, multicentre, cross-sectional study, regardless of their clinical condition, HB severity and treatment received. Two blinded observers were involved in image acquisition and scoring in each centre. RESULTS: Eighty-two patients from 12 centres were enrolled: 27% mild HB, 23% moderate, 50% severe HB. Mean age was 38.9 ± 16.4 years, 60% were treated on demand (OD) and 40% were on prophylaxis. HEAD-US was zero in all joints in 28.6% OD patients and 36.4% on prophylaxis. Mean scores significantly worsened with HB severity, except for the left knee. Patients on primary and secondary prophylaxis had significantly better joint health vs OD patients in all joints, except the right ankle. Among OD patients, those with severe disease presented significantly worse scores in all HEAD-US items related to permanent damage. CONCLUSION: Joint status of HB patients in Spain is influenced by severity and treatment modality, related to the development of arthropathy, which appears prevalent in OD patients with severe HB. Routine assessment with an imaging tool such as ultrasound and HEAD-US system may help to improve joint health by personalizing and adjusting treatment in this population.

Direct-Contact Cytotoxicity Evaluation of CoCrFeNi-Based Multi-Principal Element Alloys.

Transition metal multi-principal element alloys (MPEAs) are novel alloys that may offer enhanced surface and mechanical properties compared with commercial metallic alloys. However, their biocompatibility has not been investigated. In this study, three CoCrFeNi-based MPEAs were fabricated, and the in vitro cytotoxicity was evaluated in direct contact with fibroblasts for 168 h. The cell viability and cell number were assessed at 24, 96, and 168 h using LIVE/DEAD assay and alamarBlue assay, respectively. All MPEA sample wells had a high percentage of viable cells at each time point. The two quaternary MPEAs demonstrated a similar cell response to stainless steel control with the alamarBlue assay, while the quinary MPEA with Mn had a lower cell number after 168 h. Fibroblasts cultured with the MPEA samples demonstrated a consistent elongated morphology, while those cultured with the Ni control samples demonstrated changes in cell morphology after 24 h. No significant surface corrosion was observed on the MPEAs or stainless steel samples following the cell culture, while the Ni control samples had extensive corrosion. The cell growth and viability results demonstrate the cytocompatibility of the MPEAs. The biocompatibility of MPEAs should be investigated further to determine if MPEAs may be utilized in orthopedic implants and other biomedical applications.

Factor VIII delivery devices in haemophilia A. Barriers and drivers for treatment adherence / Dispositivos de administración de Factor VIII en la hemofilia A. Barreras y estímulos para la-adherencia

Introduction and objective: To capture the experience of haemophilia A patients with their devices for coagulation factor reconstitution, the barriers for treatment adherence; and to determine their preferences, presenting a new double-chamber syringe (DCS). Method: A cross-sectional research through a supervised survey and a DCS testing session. Results: Seventy-four (74) patients participated, 50% of them on prophylaxis treatment, and 7 years (median) with their usual treatment (IQR 17.25). In the survey, the DCS received the highest score (75/100, p < 0.001) and it was the most likely to be used in prophylactic treatment (p < 0.001). In the practical testing session (n = 29), 62.1% preferred the DCS, and required 43 seconds as median time (24.5-82) for preparation, vs. 4 minutes (1-15) with their usual treatment (p < 0.001). The favourable opinion of their doctor regarding prophylaxis was very influential on the attitude of participants towards treatment adherence (OR = 1.324, CI 95% = 1.040-1.685, p = 0.023). Conclusions: The DCS was the preferred device, and was likely to encourage prophylaxis (AU)

Introducción y objetivo: Recabar la experiencia de pacientes con hemofilia A con sus dispositivos de reconstitución de factor de coagulación, barreras para la adherencia y determinar sus preferencias, presentando una nueva jeringa de doble cá- mara (JDC). Método: Investigación transversal mediante encuesta dirigida y sesión de prueba de la JDC. Resultados: Participaron 74 pacientes, el 50% en tratamiento con profilaxis, y 7 años (mediana) con su tratamiento habitual (RIC 17,25). En la encuesta, la JDC recibió la mayor puntuación (75/100, p < 0,001) y la mayor probabilidad de uso en profilaxis (p < 0,001). En la sesión práctica (n = 29), el 62,1% prefirió la JDC y necesitaron de mediana 43 segundos (24,5-82) para la preparación, vs. 4 minutos (1-15) con el tratamiento habitual (p < 0,001). La opinión favorable del médico respecto a la profilaxis resultó muy influyente en la actitud de los participantes hacia la adherencia (OR = 1,324, IC 95% = 1,040-1,685, p = 0,023). Conclusiones: La JDC fue el dispositivo preferido y se mostró con probabilidad de favorecer la profilaxis (AU)

Factor VIII delivery devices in haemophilia A. Barriers and drivers for treatment adherence.

INTRODUCTION AND OBJECTIVE: To capture the experience of haemophilia A patients with their devices for coagulation factor reconstitution, the barriers for treatment adherence; and to determine their preferences, presenting a new double-chamber syringe (DCS). METHOD: A cross-sectional research through a supervised survey and a DCS testing session. RESULTS: Seventy-four (74) patients participated, 50% of them on prophylaxis treatment, and 7 years (median) with their usual treatment (IQR 17.25). In the survey, the DCS received the highest score (75/100, p < 0.001) and it was the most likely to be used in prophylactic treatment (p < 0.001). In the practical testing session (n = 29), 62.1% preferred the DCS, and required 43 seconds as median time (24.5-82) for preparation, vs. 4 minutes (1-15) with their usual treatment (p < 0.001). The favourable opinion of their doctor regarding prophylaxis was very influential on the attitude of participants towards treatment adherence (OR = 1.324, CI 95% = 1.040-1.685, p = 0.023). CONCLUSIONS: The DCS was the preferred device, and was likely to encourage prophylaxis.

Introducción y objetivo: Recabar la experiencia de pacientes con hemofilia A con sus dispositivos de reconstitucion de factor de coagulacion, barreras para la adherencia y determinar sus preferencias, presentando una nueva jeringa de doble camara (JDC). Método: Investigacion transversal mediante encuesta dirigida y sesion de prueba de la JDC. Resultados: Participaron 74 pacientes, el 50% en tratamiento con profilaxis, y 7 anos (mediana) con su tratamiento habitual (RIC 17,25). En la encuesta, la JDC recibio la mayor puntuacion (75/100, p < 0,001) y la mayor probabilidad de uso en profilaxis (p < 0,001). En la sesion practica (n = 29), el 62,1% prefirio la JDC y necesitaron de mediana 43 segundos (24,5-82) para la preparacion, vs. 4 minutos (1-15) con el tratamiento habitual (p < 0,001). La opinion favorable del medico respecto a la profilaxis resulto muy influyente en la actitud de los participantes hacia la adherencia (OR = 1,324, IC 95% = 1,040-1,685, p = 0,023). Conclusiones: La JDC fue el dispositivo preferido y se mostro con probabilidad de favorecer la profilaxis.

Micosis superficiales en la población Yanomami de la región de Mawaca, estado Amazonas / Superficial mycoses in the Yanomami population of the Mawaca Region, Amazon State

Este trabajo tuvo como objetivo determinar la presencia de micosis superficiales (MS) en la etnia Yanomami de Mawaca, estado Amazonas. De 176 personas examinadas, 64 mostraron lesiones sospechosas de MS, obteniendo 65 muestras: 10 de pitiriasis versicolor (PV) y 55 de dermatofitosis. El estudio micológico consistió en realizar exámenes directos, cultivos en agares micológicos, perforación del pelo in vitro, pruebas de ureasa y agar púrpura de bromocresol-glucosa-sólidos lácteos (Agar BCP-MS-G). De las 10 muestras sospechosas de PV, 3 fueron positivas, dos de niños menores de 10 años y una de un adulto, todos con lesiones en cara. En 36 raspados obtenidos se observaron hifas hialinas septadas. Se aislaron 52 colonias sospechosas de dermatofitos y de éstos, uno fue compatible con el Complejo Microsporum gypseum, de una lesión en la rodilla de un adulto. Las 51 restantes pertenecieron al Complejo Trichophyton rubrum, de los cuales, nueve perforaron el pelo in vitro, 24 hidrolizaron la urea y ninguno alcalinizó el medio Agar BCP-MS-G; la forma clínica fue tinea corporis y las lesiones se localizaron principalmente en espalda y oreja en adultos y niños, respectivamente. Estos resultados revelan la existencia de MS en la población Yanomami, sirviendo de base para futuros estudios epidemiológicos.

The purpose of this study was to determine the presence of superficial mycoses (SM) in the Yanomami ethnicity at Mawaca, Amazon State. Of 176 persons examined, 64 showed suspicious SM lesions, from which we obtained 65 samples: 10 pytiriasis versicolor (PV) and 55 dermatophytosis. The mycological study consisted in carrying out direct examination, culture in mycological agar, in vitro hair perforation, and urease and bromocresol purple-milk solids glucose agar (BCP-MS-G Agar) tests. Of the 10 PV suspicious lesions, 3 were positive, two in children under 10 years old and one in an adult, all with face lesions. In 36 scrapings we saw septate hyaline hyphae. Fifty-two dermatophyte suspicious colonies were isolated and of these, one was compatible with the Microsporum gypseum Complex, from a lesion on the knee of an adult. The other 51 belonged to the Trichophyton rubrum Complex, of which nine were in vitro hair perforations, 24 hydrolized urea, and none alkalinized the BCP-MS-G agar medium; the clinical form was tinea corporis and the lesions were mainly localized at the back and ears in children and adults respectively. These results reveal the existence of SM in the Yanomami population, serving as basis for future epidemiological studies.

Infectious agents associated with schizophrenia: a meta-analysis.

Schizophrenia is a highly disabling and limiting disorder for patients and the possibility that infections by some microorganisms may be associated to its development may allow prevention and recovery. In the current study we have done a meta-analysis of studies that have assessed the possible association between detection of different infectious agents and schizophrenia. We report results that support the idea that there is a statistically significant association between schizophrenia and infection by Human Herpesvirus 2 (OR=1.34; CI 95%: 1.09-1.70; p=0.05), Borna Disease Virus (OR=2.03; CI 95%: 1.35-3.06; p<0.01), Human Endogenous Retrovirus W (OR=19.31; CI 95%: 6.74-55.29; p<0.001), Chlamydophila pneumoniae (OR=6.34; CI 95%: 2.83-14.19; p<0.001), Chlamydophila psittaci (OR=29.05; CI 95%: 8.91-94.70; p<0.001) and Toxoplasma gondii (OR=2.70; CI 95%: 1.34-4.42; p=0.005). The implications of these findings are discussed and further research options are also explicated.

Evaluation of compliance with the EGS guidelines in Spain, using Achievable Benchmarks of Care (ABC®) methodology: the IMCA Study.

PURPOSE: To evaluate compliance with diagnostic and therapeutic recommendations of the European Glaucoma Society (EGS) for the management of glaucoma in Spain, as determined using the Achievable Benchmarks of Care approach. METHODS: A panel of 3 experts extracted quality and process-of-care indicators from the EGS guidelines. Investigators from all over the country, representative of Spanish ophthalmologists, were invited to participate in the study, completing a questionnaire on quality indicators (pertaining to the center) and process-of-care indicators (pertaining to all patients) for the first, diagnostic visit (retrospective) and the study visit. RESULTS: A total of 495 Spanish ophthalmologists included 5,060 patients with glaucoma or intraocular hypertension. The treatment was initiated with monotherapy in 88.5% of the patients. Overall, the percentage compliance with diagnostic examinations was 84.6% for intraocular pressure measurement, 96.3% for optic nerve head evaluation, 76.6% for visual field evaluation, 29.5% for central corneal thickness evaluation, and 49.3% at the study visit. The benchmark performance was close to 100% for most examinations. Only 32.8% of the centres had a written protocol for the management of the patients with glaucoma, and in these centers the recommendations of the EGS were followed in a higher proportion (p<0.0001) than in the rest. CONCLUSIONS: The use of local protocols for the management of glaucoma seems to enhance compliance. Benchmark performance was close to 100% in most indicators, suggesting that EGS recommendations are attainable in Spain. The overall performance suggests that certain diagnostic procedures are, in practice, not as widely used as recommended.

Small rounded B-cell lymphoma of bone presented by limp, with a positive multifocal 99mTc MDP bone scintigraphy pattern and a negative 99mTc HMPAO-labeled leukocytes study.

In this article, we present a rare case of B-cell bone lymphoma in a child with multifocal asymptomatic lesions detected by bone scintigraphy and a chronic clinical history characterized by limping and fever episodes for over a year. Initially, multifocal osteomyelitis was suspected and antibiotic therapy was administered with no clinical improvement. The biopsy of the main lesion in the left distal femur along with bone marrow cytology established the final diagnosis. This rare case illustrates the utility of routinely low cost-effective nuclear medicine studies like whole body bone scan and 99mTc hexamethylpropyleneamine oxime-labeled leukocytes to orientate similar cases to a correct diagnosis.