Use of Enrofloxacin and Hydrotherapy in the Management of Fibrodysplasia Ossificans Progressiva (FOP) in a Savannah Cat.

FOP is a rare genetic condition, described mainly in man and cats, characterized by progressive, painful debilitation and shortened lifespan. A 10-month-old neutered male Savannah cat was referred for progressive gait abnormalities and multifocal firm masses within the soft-tissues that were unresponsive to previous treatment. Diagnosis of FOP was based on histopathological evaluation of intralesional biopsies, which revealed osteo-cartilaginous metaplasia and fibrocellular proliferation with intralesional chondrogenesis and endochondral ossification. The cat was managed with 5 mg/kg BID enrofloxacin and hydrotherapy for 3 years until acute death. During that three-year period, the cat displayed consistent improvement in endurance, quality of life, and range of motion. Postmortem histopathology further confirmed the diagnosis of FOP via identification of intramuscular and intra-fascial ossification with lymphoplasmacytic infiltration, degeneration, and regeneration of adjacent myocytes. To the authors' knowledge, this is the first report of long-term enrofloxacin treatment and hydrotherapy for the management of FOP in a cat, leading to improved mobility and survival time, and the first report of FOP in an exotic breed cat.

Fibrodisplasia ossificante progressiva em pediatria: revisão integrativa com foco na enfermagem / Progressive ossifying fibrodysplasia in pediatrics: an integrative review focusing on nursing / Fibrodisplasia osificante progresiva en pediatría: revisión integradora centrada en la enfermería

Objetivo: identificar as ações de cuidado/assistência de enfermagem ao paciente pediátrico com FOP na Atenção Primária à Saúde. Método: revisão integrativa, com as buscas realizadas em outubro de 2020, em cinco bases de dados e literatura cinzenta. Com descritores nacionais e internacionais. A seleção dos estudos foi realizada por revisores independentes. Resultados: a partir de uma busca ampla foram identificados 1213 estudos e incluídos 2 artigos na amostra final. Seguiu-se três direcionamentos temáticos assistenciais: Contribuições de enfermagem no reconhecimento precoce da doença, a importância da educação em saúde, e a prevenção de traumatismos. Conclusão: os resultados obtidos agregaram conhecimento acerca da assistência aos pacientes pediátricos com FOP. Estudos científicos acerca dos cuidados de enfermagem a essa doença ainda são escassos, assim recomenda-se o desenvolvimento de novos estudos visto a significância do papel da enfermagem às doenças raras (AU).

Objective: to identify the nursing care/assistance to pediatric patients with FOP in Primary Health Care. Method: integrative review was conducted in October 2020, in five databases and gray literature. With national and international descriptors. The eligibility of studies was performed by independent reviewers. Results: of 1213 potential studies, 2 studies were included in the final sample. Three thematic care directions followed: Nursing contributions to the early recognition of the disease, the importance of health education, and the prevention of injuries. Conclusion: The results obtained added knowledge about the assistance to pediatric patients with FOP. Scientific studies about nursing care for this disease are still scarce, so it is recommended that new studies be developed, given the significance of nursing's role in rare diseases (AU).

Objetivo: identificar las acciones de atención/asistencia de enfermería a pacientes pediátricos con FOP en la Atención Primaria de Salud. Método: revisión integradora, las búsquedas se realizaron en octubre de 2020, en cinco bases de datos y en la literatura gris, con descriptores nacionales e internacionales. La selección de los estudios fue realizada por revisores independientes. Resultados: mediante una búsqueda amplia se identificaron 1213 estudios y se incluyeron 2 artículos en la muestra final. Se siguieron tres direcciones temáticas de atención: aportes de la enfermería para el reconocimiento temprano de la enfermedad; importancia de la educación para la salud; y prevención de traumatismos. Conclusión: los resultados aportaron conocimiento adicional sobre la atención a pacientes pediátricos con FOP. Los estudios científicos sobre la atención de enfermería a esta enfermedad aún son escasos, por lo que se recomienda desarrollar nuevos estudios, dada la importancia que tiene el papel de la enfermería en las enfermedades raras (AU).

Compressed Sensing Image Reconstruction of Ultrasound Image for Treatment of Early Traumatic Myositis Ossificans of Elbow Joint by Electroacupuncture.

This article conducts a retrospective analysis of 500 patients with posttraumatic elbow dysfunction admitted to our department from March 2019 to September 2020. The average time from injury to operation is 11 months (2-20 months). We adopt a personalized treatment method to completely remove the hyperplastic adhesion tissue and heterotopic ossification around the joint, remove part of the joint capsule and ligament, and release it to achieve maximum function. After the operation, an external fixator was used to stabilize the loosened elbow joint, and the patient was guided to perform rehabilitation exercises with the aid of a hinged external fixator, and celecoxib was used to prevent heterotopic ossification. Mayo functional scoring system was used to evaluate the curative effect before and after surgery. The rapid realization of ultrasound imaging under the framework of compressed sensing is studied. Under the premise of ensuring the quality of ultrasound imaging reconstruction, the theory of ultrasound imaging is improved, and a plane wave acoustic scattering ultrasound echo model is established. On this basis, the theory of compressed sensing is introduced, the mathematical model of compressed sensing reconstruction is established, and the fast iterative shrinkage thresholding algorithm (FISTA) of compressed sensing reconstruction is improved to reduce the computational complexity and the number of iterations. This article uses FISTA directly to reconstruct medical ultrasound images, and the reconstruction results are not ideal. Therefore, a simulation model of FISTA training and testing was established using the standard image library. By adding different intensities of noise to all images in the image library, the influence of noise intensity on the quality of FISTA reconstructed images is analyzed, and it is found that the FISTA model has requirements for the quality of the images to be reconstructed and the training set images. In this paper, Rob's blind deconvolution restoration algorithm is used to preprocess the original ultrasound image. The clarity of the texture details of the restored ultrasound image is significantly improved, and the image quality is improved, which meets the above requirements. This paper finally formed a reconstruction model suitable for ultrasound images. The reconstruction strategy verified by the ultrasound images provided by the Institute of Ultrasound Imaging of a medical university has achieved a significant improvement in the quality of ultrasound images.

Saracatinib is an efficacious clinical candidate for fibrodysplasia ossificans progressiva.

Currently, no effective therapies exist for fibrodysplasia ossificans progressiva (FOP), a rare congenital syndrome in which heterotopic bone is formed in soft tissues owing to dysregulated activity of the bone morphogenetic protein (BMP) receptor kinase ALK2 (also known as ACVR1). From a screen of known biologically active compounds, we identified saracatinib as a potent ALK2 kinase inhibitor. In enzymatic and cell-based assays, saracatinib preferentially inhibited ALK2, compared with other receptors of the BMP/TGF-ß signaling pathway, and induced dorsalization in zebrafish embryos consistent with BMP antagonism. We further tested the efficacy of saracatinib using an inducible ACVR1Q207D-transgenic mouse line, which provides a model of heterotopic ossification (HO), as well as an inducible ACVR1R206H-knockin mouse, which serves as a genetically and physiologically faithful FOP model. In both models, saracatinib was well tolerated and potently inhibited the development of HO, even when administered transiently following soft tissue injury. Together, these data suggest that saracatinib is an efficacious clinical candidate for repositioning in FOP treatment, offering an accelerated path to clinical proof-of-efficacy studies and potentially significant benefits to individuals with this devastating condition.

Challenges in the treatment of fibrodysplasia ossificans progressiva.

Fibrodysplasia ossificans progressiva (FOP), is a rare autosomal dominant connective tissue disease with a prevalence of 1 in 2 million. It is characterized by congenital foot deformities and multiple heterotopic ossifications in fibrous tissue. It usually starts with painful soft tissue swellings occurring with attacks at the ages of three or four. The attacks develop spontaneously or after minor trauma, and gradually turn into heterotopic ossifications that cause joint limitations, growth defects, skeletal deformities and chronic pain. The average life expectancy is forthy, and most of the patients are lost due to pulmonary complications. FOP is often misdiagnosed as fibromatosis, desmoid tumour or cancer, bunion, myositis, arthritis and rheumatic diseases. After clinical suspicion, confirmatory genetic analysis should be used for the diagnosis. The treatment of FOP is currently supportive. An effective, proven method has not yet been established. Herein, we present an 18-year-old female patient with FOP who underwent different treatment modalities in a 5-year period. This case-based review reveals all available treatment approaches with at least 6-month follow-up for FOP in the literature.

Role of osteoclasts in heterotopic ossification enhanced by fibrodysplasia ossificans progressiva-related activin-like kinase 2 mutation in mice.

Fibrodysplasia ossificans progressiva (FOP) is a disorder of skeletal malformations and progressive heterotopic ossification. The constitutively activating mutation (R206H) of the bone morphogenetic protein type 1 receptor, activin-like kinase 2 (ALK2), is responsible for the pathogenesis of FOP. Although transfection of the causal mutation of FOP into myoblasts enhances osteoclast formation by transforming growth factor-ß (TGF-ß), the role of osteoclasts in heterotopic ossification is unknown. We therefore examined the effects of alendronate, SB431542 and SB203580 on heterotopic ossification induced by the causal mutation of FOP. Total bone mineral content as well as numbers of tartrate-resistant acid phosphatase (TRAP)-positive multinucleated and alkaline phosphatase (ALP)-positive cells in heterotopic bone were significantly higher in muscle tissues implanted with ALK2 (R206H)-transfected mouse myoblastic C2C12 cells than in the tissues implanted with empty vector-transfected cells in nude mice. Alendronate, an aminobisphosphonate, did not affect total mineral content or numbers of TRAP-positive multinucleated and ALP-positive cells in heterotopic bone, which were enhanced by the implantation of ALK2 (R206H)-transfected C2C12 cells, although it significantly decreased serum levels of cross-linked C-telopeptide of type I collagen, a bone resorption index. Moreover, neither SB431542, an inhibitor of TGF-ß receptor type I kinase, nor SB203580, an inhibitor of p38 mitogen-activated protein kinase, affected the increase in heterotopic ossification due to the implantation of ALK2 (R206H)-transfected C2C12 cells. In conclusion, the present study indicates that osteoclast inhibition does not affect heterotopic ossification enhanced by FOP-related mutation.

Myositis ossificans of the serratus anterior as a rare complication of massage: a case report.

INTRODUCTION: Myositis ossificans usually occurs in the vicinity of the elbow, knee joints, or hip joints, following obvious trauma or surgery. This is the first report on myositis ossificans of the serratus anterior. CASE PRESENTATION: In this report we present a case of myositis ossificans within the serratus anterior which developed as a complication due to long-term nape massage. The patient was a 29-year-old Han woman. Because heterotopic ossificans constricted her brachial plexus the surface of her right upper arm was slightly numb; the symptom disappeared after surgery. CONCLUSION: This case highlights that myositis ossificans can occur in the serratus anterior following long-term nape massage.

Myositis ossificans in the paraspinal muscles of the neck after acupuncture: a case report.

BACKGROUND CONTEXT: Although traumatic myositis ossificans (MO) has been reported occasionally, MO of paraspinal muscles has been rarely seen in the cervical spine after minor injury. This is difficult to distinguish from benign and malignant soft lesions in cases of a lack of definite trauma history. PURPOSE: We report a case of MO in the cervical paraspinal muscle after acupuncture and describe methods for diagnosis and proper treatment, including classification, etiology, and radiologic and histologic features. STUDY DESIGN: Case report. METHODS: A 26-year-old woman complained of posterior neck pain that had began 2 months earlier and neck swelling after acupuncture. No abnormal finding existed on the X-ray except soft tissue swelling. Magnetic resonance imaging was evaluated because of constant neck pain. To obtain more accurate assessment, computed tomography-guided biopsy was performed and a diagnosis of MO was made. RESULTS: The patient was conservatively treated through rest and analgesics. Posterior neck pain and swelling improved for a several months. The hyperdensity was comparable with the bony density, and the size of the calcified lesion on X-ray diminished until the last follow-up. CONCLUSIONS: Myositis ossificans that can occur after acupuncture should be recognized as a possible cause of persistent neck pain and swelling despite no definite trauma after thorough evaluation of the neoplasm and infection.

Clinically applicable antianginal agents suppress osteoblastic transformation of myogenic cells and heterotopic ossifications in mice.

Fibrodysplasia ossificans progressiva (FOP) is a rare autosomal dominant disorder characterized by progressive heterotopic ossification. FOP is caused by a gain-of-function mutation in ACVR1 encoding the bone morphogenetic protein type II receptor, ACVR1/ALK2. The mutant receptor causes upregulation of a transcriptional factor, Id1. No therapy is available to prevent the progressive heterotopic ossification in FOP. In an effort to search for clinically applicable drugs for FOP, we screened 1,040 FDA-approved drugs for suppression of the Id1 promoter activated by the mutant ACVR1/ALK2 in C2C12 cells. We found that that two antianginal agents, fendiline hydrochloride and perhexiline maleate, suppressed the Id1 promoter in a dose-dependent manner. The drugs also suppressed the expression of native Id1 mRNA and alkaline phosphatase in a dose-dependent manner. Perhexiline but not fendiline downregulated phosphorylation of Smad 1/5/8 driven by bone morphogenetic protein (BMP)-2. We implanted crude BMPs in muscles of ddY mice and fed them fendiline or perhexiline for 30 days. Mice taking perhexiline showed a 38.0 % reduction in the volume of heterotopic ossification compared to controls, whereas mice taking fendiline showed a slight reduction of heterotopic ossification. Fendiline, perhexiline, and their possible derivatives are potentially applicable to clinical practice to prevent devastating heterotopic ossification in FOP.

Pathogenic mutation of ALK2 inhibits induced pluripotent stem cell reprogramming and maintenance: mechanisms of reprogramming and strategy for drug identification.

Fibrodysplasia ossificans progressiva (FOP) is a rare congenital disorder characterized by progressive ossification of soft tissues. FOP is caused by mutations in activin receptor-like kinase 2 (ALK2) that cause its constitutive activation and result in dysregulation of BMP signaling. Here, we show that generation of induced pluripotent stem cells (iPSCs) from FOP-derived skin fibroblasts is repressed because of incomplete reprogramming and inhibition of iPSC maintenance. This repression was mostly overcome by specific suppression of ALK2 expression and treatment with an ALK2 inhibitor, indicating that the inhibition of iPSC generation and maintenance observed in FOP-derived skin fibroblasts results from constitutive activation of ALK2. Using this system, we identified an ALK2 inhibitor as a potential candidate for future drug development. This study highlights the potential of the inhibited production and maintenance of iPSCs seen in diseases as a useful phenotype not only for studying the molecular mechanisms underlying iPS reprogramming but also for identifying drug candidates for future therapies.

The extremes in orthopaedics! Whom to blame?

Traditional medicines especially the herbal paste is routinely prescribed by the herb sellers. The unsupervised combinations and preparations are easily available in our part of world. The demand and supply of such irrational combination is only based on the principle that anything natural is safe. Drugs and preparations made by the people who lack the scientific knowledge of traditional herbal medicines adversely affect the consumer. We put forward few complications that resulted after herbal paste application in those who already had visited the specialist but opted the easily available unsupervised herbal preparations.

Improvement of mouth opening for a patient with fibrodysplasia ossificans progressiva: a case report.

The aim of this article is to describe the care of a patient with fibrodysplasia ossificans progressiva (FOP) and to provide dentists with a guide for how to safely care for patients with FOP. Treatment improved the patient's limited mouth opening. FOP is a rare autosomal dominant disorder characterized by congenital malformation of the fingers and toes by heterotopic ossification progressiva of the connective tissue. This ossification causes a limitation in osteoradicular mobility, mainly affecting the spine, shoulders, hips, and peripheral joints. The disease can manifest from pregnancy until adulthood, with no greater prevalence associated with race or gender. Although rare, the disease can be easily identified by its clinical features, and diagnosis can be confirmed by a radiographic examination. There is no known effective treatment for this disease. All therapeutic treatment must be conservative to avoid any condition that may cause heterotopic ossification. Guidelines to prevent new ossifications are important for patients with FOP. Dental professionals should be cautious in planning treatment, avoiding anesthesia, especially in the mandible, to prevent ankylosis of the temporo-mandibular joints. The prevention of dental caries is essential to avoid the need for more invasive treatment.

Myositis ossificans of the elbow after a trigger point injection.

Trigger point injection is a simple procedure that is widely performed for relieving pain. Even though there are several complications of trigger point injection, myositis ossificans has not been documented as one of its complications. We treated a patient who suffered from painful limitation of elbow motion and this was caused by myositis ossificans between the insertions of brachialis and supinator muscles after a trigger point injection containing lidocaine mixed with saline, and we also review the relevant medical literature.

Myositis Ossificans of the Elbow after a Trigger Point Injection

Trigger point injection is a simple procedure that is widely performed for relieving pain. Even though there are several complications of trigger point injection, myositis ossificans has not been documented as one of its complications. We treated a patient who suffered from painful limitation of elbow motion and this was caused by myositis ossificans between the insertions of brachialis and supinator muscles after a trigger point injection containing lidocaine mixed with saline, and we also review the relevant medical literature.

A 9-month-old phosphaturic mesenchymal tumor mimicking the intractable rickets.

Phosphaturic mesenchymal tumor is an extremely rare disease and is frequently associated with oncogenic osteomalacia showing paraneoplastic syndrome, which is characterized by phosphaturia, hypophosphatemia, normocalcemia, and decreased levels of 1,25-dihydroxyvitamin D3 associated with a tumor. A 2-year-old boy, who had a soft tissue tumor on his right thigh and previously diagnosed as myositis ossificans at 9-months-old, was presented with rachitic rosary and mildly enlarged tumor. Biochemical investigations showed hypophosphatemia, hyperphosphaturia, and an increased alkaline phosphatase level of 440 U/l (25-100 U/l), suggesting rickets, which was resistant to vitamin D dietary supplementation. We were certain of intractable rickets because of oncogenic hypophosphatemia and thus decided to excise the soft tissue mass. We observed laboratory improvement of rickets after 2 weeks. On the basis of surgical and histopathological examinations, the tumor was finally diagnosed as the phosphaturic mesenchymal tumor.

Fractured heterotopic bone in myositis ossificans traumatica.

OBJECTIVE: To discuss the case of a patient with an acute fracture occurring through preexisting, quiescent, posttraumatic heterotopic bone formation of the gastrocnemius muscle. The lesion demonstrated a previously unreported pattern of healing. This case serves to demonstrate an infrequent event with a rare and heretofore unreported sequella. CLINICAL FEATURES: A 54-year-old man with preexisting, mature heterotopic bone within the left gastrocnemius muscle experienced reinjury to the site. The preexisting heterotopic bone was a result of a sporting injury that had occurred 32 years previously. At the time of the new injury, the patient experienced immediate, severe pain. Radiographs demonstrated a nondisplaced oblique fracture through the preexisting heterotopic bone. INTERVENTION AND OUTCOME: The patient was treated with protective wrapping and physiotherapy modalities. Because only the heterotopic bone was involved, the leg was not placed in a cast. Follow-up radiographs obtained 14 weeks later showed callus formation about the fractured site and evidence of union. CONCLUSION: Fractures occurring through mature heterotopic bone formations, such as those seen in myositis ossificans traumatica, are infrequent; callus formation with union of the fractured fragments is an even rarer occurrence.

Chondrosarcoma and myositis ossificans.

OBJECTIVE: To discuss the clinical radiographic findings in a 70-yr-old woman suffering from chondrosarcoma. CLINICAL FEATURES: The patient experienced right SI pain present initially only at night. She later developed morning numbness. An X-ray examination revealed a flocculent calcification in the right buttock region. Computed tomographic scans confirmed the diagnosis. INTERVENTION AND OUTCOME: Initial palliative care continued until surgery was performed to resect the area. CONCLUSION: Chondrosarcoma is a severe disease that must be differentiated from myositis ossificans.