Evaluation of microvascular flow imaging combined with high-frequency ultrasonography in children with haemophilic arthropathy A / 中华实用儿科临床杂志

Objective:To explore the application of microvascular flow imaging (MVFI) combined with high-frequency ultrasonography in children with haemophilic arthropathy A (HAA).Methods:Retrospective study.A total of 82 children diagnosed with HAA in the First Affiliated Hospital of Zhengzhou University from October 2018 to October 2020 were recruited.The elbow, knee and ankle joints of each child were examined by high-frequency ultrasonography.The numbers of thickened synovial joints were recorded.Blood flow signals of the thickening of synovial joints was checked by the MVFI and power Doppler ultrasound (PDUS), respectively.Color flow signals were graded by the semi-quantitative scoring systems.The chi- square test and independent multi-group ordinal multi-category rank-sum test were used to compare the differences of MVFI and PDUS in the display of thickened synovial blood flow. Results:A total of 254 joints were involved in 82 children with HAA, including synovial hypertrophy in 188 joints, hydrops articuli in 146 joints, fibrotic septa in 66 joints, cartilage damage in 63 joints, haemosider indeposition in 45 joints, bone erosion in 25 joints, osteophytes in 15 joints and bone remodeling in 8 joints.Grade Ⅱ synovial thickened joints were the most common.The proportion of blood flow signals detected by PDUS in thickened synovial membranes was significantly higher than that detected MVFI (52.66% vs.70.21%, χ2=12.225, P<0.05). Numbers of grade 0 and Ⅰ joints with thickened synovial membranes detected by MVFI were less than those of PDUS, while the opposite result was obtained in detecting grade Ⅱ and Ⅲ joints ( H=21.158, P<0.05). Compared with PDUS, MVFI more sensitively visualized the blood flow of the thickened synovial membrane. Conclusions:MVFI can more prominently detect the thickened synovial blood flow in children with HAA.A combined application of MVFI and high-frequency ultrasonography contributes to the evaluation of children with HAA.

Rehabilitación física en pacientes con artropatía hemofílica: revisión sistemática y metaanálisis sobre dolor / Physical rehabilitation in haemophilic arthropathy: systematic review and pain-related meta-analysis

RESUMEN Introducción: La artropatía hemofílica (AH) cursa con diferentes manifestaciones clínicas importantes, como son las hemorragias articulares, el dolor, la disminución de la amplitud del movimiento y las alteraciones funcionales que pueden causar secuelas en la funciona lidad y movilidad. El ejercicio físico adaptado a los pacientes con hemofilia puede ser una adecuada estrategia terapéutica, que repercuta positivamente sobre la calidad de vida de dichos sujetos. Objetivos: Evaluar la eficacia de la rehabilitación física en el tratamiento de la artropatía hemofílica. Materiales y métodos: Se ha realizado una revisión sistemática y metaánálisis de ensayos clí nicos (seleccionados según criterios de elegibilidad). Para ello, se han utilizado las siguientes bases de datos: PEDro, Pubmed, Scopus y Web of Science. Se empleó la escala «PEDro¼ para evaluar la calidad metodológica de los estudios. Resultados: Tras aplicar los criterios de inclusión y exclusión, en la revisión final fueron incluidos siete artículos, los cuales aportaron resultados favorables sobre la fuerza y el diá metro muscular, el rango de movilidad, el estado articular y la calidad de vida. De ellos, dos estudios aportaron datos para metaanálisis, con resultados favorables sobre la variable dolor [Diferencia de medias estandarizada (DME) = -2,64; IC 95%: (-4,26; 1,03)]. Conclusiones: Se encontró evidencia sobre la eficacia de la rehabilitación física en el trata miento de la artropatía hemofílica. El ejercicio terapéutico (ET) es el principal tratamiento realizado; con este se obtuvieron mejoras significativas en distintas variables físicas.

ABSTRACT Introduction: Haemophilic arthropathy presents with different important clinical disorders, such as joint disease, pain, decreased range of motion, and functional alterations that can produce limitations in functionality and mobility. The physical exercise adapted to patients with haemophilia can be an adequate therapeutic strategy, having a positive impact on the quality of life of these subjects. Objectives: To identify the published clinical trials that evaluate the efficacy of physical rehabilitation in the treatment of haemophilic arthropathy. Materials and methods: A systematic review and meta-analysis of clinical trials was con ducted (using pre-defined eligibility criteria). The literature search was performed in the databases: PEDro, Pubmed, Scopus, and Web of Science. The quality of the methods used in the studies was evaluated using the PEDro scale. Results: After applying the inclusion and exclusion criteria, 7 studies were included in this review, providing favourable results on muscle strength and circumference, range of motion, joint disease, and quality of life. Moreover, 2 articles contributed information to the meta-analysis, showing favourable results on pain [Standardised mean difference (SMD) = -2.64; 95% CI: (-4.26; 1.03)]. Conclusions: This systematic review found evidence on the efficacy of physical rehabilitation in the treatment for haemophilic arthropathy. Therapeutic exercise is the main treatment carried out, obtaining significant improvements in the different physical outcomes.

Hemofilia y osteoporosis / Haemophilia and osteoporosis

Una relación entre la hemofilia y la osteoporosis ha sido sugerida, lo cual ha conducido a la iniciativa de realizar tanto revisiones como estudios acerca de este tema. Las hemofilias son un trastorno de la coagulación hereditario, causado por deficiencia o defecto en la actividad coagulante de los factores VIII (hemofilia A) y IX (hemofilia B). La hemartrosis o hemorragia dentro de las articulaciones representa entre el 65-80% de todas las hemorragias en hemofílicos y determinan en gran parte el deterioro en la calidad de vida por su curso crónico e incapacitante. La osteoporosis es una enfermedad esquelética sistémica caracterizada por compromiso de la resistencia ósea que aumenta el riesgo de fractura. La hemofilia y la osteoporosis tienen varios aspectos en común: ambas enfermedades están acompañadas de dolor crónico, invalidez, pérdida de la independencia, aumento de la mortalidad y tienen un impacto negativo sobre la calidad de vida de los pacientes. La osteoporosis es la enfermedad metabólica ósea más frecuente en la población mayor, con alcances socioeconómicos importantes. No obstante, las hemofilias son menos comunes, pero los costos de su tratamiento global son más elevados. Aparte de estas obvias similitudes entre la hemofilia y la osteoporosis, se considera que hay una fuerte relación entre estas dos entidades más que la que se suponía antes, y los pacientes con hemofilia severa y artropatía hemofílica tienen mayor riesgo de tener un pico de masa ósea bajo. La hemofilia está asociada a varios factores que predicen un pico de masa ósea bajo y, ciertos de estos factores, también pueden predecir un aumento en la pérdida de la masa ósea; sin embargo, aún no se ha demostrado que la pérdida ósea sea mayor en hemofílicos más que en los controles sanos, ni el grado en el cual el pico de masa ósea baja confiere un aumento en el riesgo de fractura. Se necesitan estudios prospectivos antes de establecer guías acerca de la prevención o tratamiento de la osteoporosis en hemofílicos, las cuales no pueden solamente basarse en el resultado de la densitometría ósea (DMO) aislada. [Sossa CL, Solano MH, Wandurraga EA, Jiménez SI, Galvis HD, Pérez CA. Hemofilia y osteoporosis. MedUNAB 2008; 11:218-224]

A relationship between haemophilia and osteoporosis has been suggested. This association has driven an initiative to do literature reviews and clinical trials. Haemophilia is a disease characterized by inherited coagulation disorders caused by factor VIII (haemophilia A) and factor IX (haemophilia B) defect or deficiency. Haemarthrosis or bleeding inside the articulation represents between 65 and 80% of all bleeding episodes in haempohilics and cause a decrease in the quality of life because of the chronic and incapacitating properties. Osteoporosis is a bone disease characterized by a progressive decrease in bone quality and increase in the fracture risk. Haemophilia and osteoporosis have various common features; both diseases are associated with chronic pain, disability, lost of independency, increase in mortality and a negative impact in the quality of life. Osteoporosis is a bone metabolic disease more often presented in the elder population with a high socioeconomic burden. Even that haemophilia is a less frequent disease than osteoporosis the cost of treatment is more expensive than osteoporosis. It has been described that patients with severe haemophilia and haemophilic arthropathy are at risk of a low bone mass peak; however currently there has not been confirmed by clinical trials that haemophilic patients have a higher risk of fractures and decreased bone mass than healthy controls, and more prospective trials are needed before conclusions to develop clinical guidelines. [Sossa CL, Solano MH, Wandurraga EA, Jiménez SI, Galvis HD, Pérez CA. Haemophilia and osteoporosis. MedUNAB 2008; 11:218-224].