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Journal articles on the topic 'Fort Duchesne'

Author: Grafiati
Published: 4 June 2021
Last updated: 17 February 2022

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1

Mattiello-Sverzut, A., M. Souza, C. Baptista, M. Figueiredo, and R. Alvades. "Ankle-foot orthoses in Duchenne muscular dystrophy." Neuromuscular Disorders 25 (October 2015): S304. http://dx.doi.org/10.1016/j.nmd.2015.06.421.

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2

Timonen, Anne, Michele Lloyd-Puryear, David M. Hougaard, Liisa Meriö, Pauliina Mäkinen, Ville Laitala, Tuukka Pölönen, et al. "Duchenne Muscular Dystrophy Newborn Screening: Evaluation of a New GSP® Neonatal Creatine Kinase-MM Kit in a US and Danish Population." International Journal of Neonatal Screening 5, no. 3 (August 27, 2019): 27. http://dx.doi.org/10.3390/ijns5030027.

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Duchenne muscular dystrophy (DMD/Duchenne) is a progressive X-linked disease and is the most common pediatric-onset form of muscular dystrophy, affecting approximately 1:5000 live male births. DNA testing for mutations in the dystrophin gene confirms the diagnosis of this disorder. This study involves assessment of screening newborns for DMD using an immunoassay for muscle-type (MM) creatine kinase (CK) isoform—the GSP Neonatal CK-MM kit. Comparisons were made with CK activity determination by fluorescence measurement. In addition, the study evaluated the effect of gestational age, age of infant at time of sampling and how stable the CK-MM was over time. This assay discriminates well between normal, unaffected and Duchenne affected populations and is suitable for Duchenne newborn screening.
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3

Jomantiene, Rasa, Robert E. Davis, Ellen L. Dally, John L. Maas, and Joseph D. Postman. "The Distinctive Morphology of `Fragaria multicipita' Is Due to Phytoplasma." HortScience 33, no. 6 (October 1998): 1069–72. http://dx.doi.org/10.21273/hortsci.33.6.1069.

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The rare plant, Fragaria multicipita Fern., was characterized by an unusual vegetative morphology that was long presumed to be suggestive of an ice front relict. While an additional species of Fragaria would be a potential source of genetic diversity for enhancing cultivated strawberry germplasm, evidence now indicates that such potential is not present in F. multicipita. Grafting of F. multicipita to F. chiloensis Duchesne resulted in transmission of a subgroup 16SrVI-B phytoplasma to, and the development of multicipital growth in, F. chiloensis. The results indicated that F. multicipita is a phytoplasma-diseased aberrant growth form of F. virginiana Duchesne and is an unfounded taxon. It is apparent that this plant population offers no unique potential for increasing genetic diversity in cultivated strawberry germplasm, but the phytoplasma may be capable of infecting commercial strawberry.
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4

Bromwich, W., M. James, C. Stewart, N. Emery, and R. Quinlivan. "Outcomes using ambulatory Ankle Foot Orthoses in Duchenne Muscular Dystrophy." Gait & Posture 36 (June 2012): S94—S95. http://dx.doi.org/10.1016/j.gaitpost.2011.10.342.

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5

Fernandes, Naelka dos Anjos, Denise Caldeira Troise, Francis Meire Fávero, Sissy Veloso Fontes, and Acary Souza Bulle Oliveira. "A Importância das Órteses de Membros Inferiores na Distrofia Muscular de Duchenne." Revista Neurociências 20, no. 4 (December 31, 2012): 584–87. http://dx.doi.org/10.4181/rnc.2012.20.701.4p.

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Introdução. A habilidade motora do paciente com Distrofia Mus­cular de Duchenne decresce progressivamente e a fisioterapia é fun­damental no tratamento destes pacientes para manutenção da força muscular e amplitude de movimento, por meio de alongamentos di­ários e ortetização. Objetivo. Identificar a importância das principais órteses de membros inferiores utilizadas no tratamento de pacientes com Distrofia Muscular de Duchenne. Método. Foram realizadas buscas eletrônicas nas bases de dados MEDLINE, LILACS, SCIE­LO a fim de identificar os artigos científicos indexados e publicados de 1979 a 2010. Resultados. Dez artigos foram selecionados. Dos artigos selecionados, um era estudo retrospectivo, oito eram estudos prospectivos, um era um estudo prospectivo randomizado. Os artigos tratavam dos tipos de órteses mais utilizadas no tratamento de pacien­tes com Distrofia Muscular de Duchenne, e dos benefícios que o uso desses dispositivos proporcionava aos pacientes. Conclusões. A órtese mais utilizada é a Knee-Ankle-Foot (KAFO), a qual é importante para prevenção/minimização de contraturas e deformidades, prolongamen­to da marcha e ortostatismo dos pacientes.
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6

DeSandies, Kisha D., and Gabriella Madden. "Philadelphia College of Osteopathic Medicine Examines Embryonic Cells to Find the Origin of Muscles With Image Analysis Software." Microscopy Today 8, no. 7 (September 2000): 34–36. http://dx.doi.org/10.1017/s1551929500054687.

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Tens of thousands of Americans suffer from muscular dystrophy (MD), a disease that gradually deteriorates a person's skeletal muscle. While there is no effective cure, scientists know MD is caused by a genetic defect and are searching for treatments that will stop or retard the deterioration of muscle. In June, American and British researchers announced the success of a long-term treatment that repairs the genetic defect in Duchenne muscular dystrophy, the most common childhood form of MD.Last fall, these researchers injected a short strand of nucleic acid into the shin muscle of a six-week old golden retriever - which had a genetic defect that leads to Duchenne MD in dogs - in order to maintain normal levels of dystrophin, the muscle protein missing in Duchenne MD. Their goal was for the nucleic acid to trigger the dog's system to correct the genetic defect. Eleven months later, the injected muscle continues to show normal levels of dystrophin.
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7

Winarno, Soelatin, Indrawarman Indrawarman, Sabdo Waloejo, and Lydia Kristianti. "Progressive Muscular Dystrophy (Duchenne Type) (Case Report)." Paediatrica Indonesiana 16, no. 9-10 (September 17, 2019): 361–4. http://dx.doi.org/10.14238/pi16.9-10.1976.361-4.

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Clinical findings of two brothers suffering from progressive muscular dystrophy pseudohypertrophic type according to Duchenne are reported. Literatures dealing with its clinical classification, biochemical disturbances, hypotheses of the pathogenesis, management of treatment, mode of action of A.T.P. and the pedigree have been briefly reported. Progressive Muscular Dystrophy is a progressive disease affecting voluntary muscles; It is characterized by a decreased strength in the affected muscles with rapid or slow gradual progression. About 45% of the patients gave a history that at least another member of the family is affected by the disease. Pseudohypertrophic form (Duchenne type) is usually inherited as a recessive factor, often sexlinked.
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8

Culligan, Kevin, and Kay Ohlendieck. "Diversity of the Brain Dystrophin-Glycoprotein Complex." Journal of Biomedicine and Biotechnology 2, no. 1 (2002): 31–36. http://dx.doi.org/10.1155/s1110724302000347.

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Duchenne muscular dystrophy (DMD), the most common inherited neuromuscular disorder, is characterized by progressive muscle wasting and weakness. One third of Duchenne patients suffer a moderate to severe, nonprogressive form of mental retardation. Mutations in the DMD gene are thought to be responsible, with the shorter isoforms of dystrophin implicated in its molecular brain pathogenesis. It is becoming clear that region-specific variations in dystrophin isoforms delegate the composition of the dystrophin-glycoprotein complex in brain, and hence, the function of the specific membrane assembly. Here we summarize the recent advances in the understanding of brain dystrophin, dystrophin-related proteins and dystrophin-associated proteins.
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9

Mackie, Gregory. "“THE MODERN IDEA UNDER AN ANTIQUE FORM”: AESTHETICISM AND THEATRICAL ARCHAEOLOGY IN OSCAR WILDE'S DUCHESS OF PADUA." Theatre Survey 53, no. 2 (August 28, 2012): 219–39. http://dx.doi.org/10.1017/s0040557412000063.

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During his final years in exile, Oscar Wilde derived as much income as he could from selling the rights to his as-yet-unpublished writings. Although at that time he was as pragmatic in his approach to the business of authorship as he had been during the height of his dramatic career in the early 1890s, Wilde nonetheless resisted publishing one of his earliest plays, the 1883 blank-verse tragedy The Duchess of Padua. In an 1898 letter to Robert Ross, Wilde noted of the play (which was finally produced in 1891) that “The Duchess is unfit for publication—the only one of my works that comes under that category. But there are some good lines in it.” Wilde had not always had such a dim view of his second completed play. Indeed, he once promoted it as “the masterpiece of all my literary work, the chef-d'oeuvre of my youth” and had worked hard to see it produced. Literary history, however, has tended to concur with Wilde's more mature assessment of the play's artistic merits. Katharine Worth, one of the few critics to assess the play in detail, suggests that it “is the one completed play of Wilde's which can scarcely be imagined in a modern performance.” Josephine M. Guy and Ian Small place the play among a group of early works by Wilde that “have been judged by modern critics to be failures.” According to their view, The Duchess “is seen as an embarrassment.” This essay instead regards The Duchess as an uneven experiment in both staging aestheticism and late Victorian theatrical “archaeology,” a practice that sought to mount historical dramas with as much accuracy and precision in costume and design as possible. In a letter to Mary Anderson, the American actress whom he hoped would star in the play, Wilde contextualized the spectacle to which he aspired in The Duchess: “the essence of art is to produce the modern idea under an antique form.”
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10

Leitch, Khristinn Kellie, Naweed Raza, Doug Biggar, Derek Stephen, James G. Wright, and Benjamin Alman. "Should Foot Surgery Be Performed for Children With Duchenne Muscular Dystrophy?" Journal of Pediatric Orthopaedics 25, no. 1 (January 2005): 95–97. http://dx.doi.org/10.1097/00004694-200501000-00021.

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11

Scher, David M., and Scott J. Mubarak. "Surgical Prevention of Foot Deformity in Patients With Duchenne Muscular Dystrophy." Journal of Pediatric Orthopaedics 22, no. 3 (May 2002): 384–91. http://dx.doi.org/10.1097/01241398-200205000-00024.

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12

Leitch, Khristinn Kellie, Naweed Raza, Doug Biggar, Derek Stephen, James G. Wright, and Benjamin Alman. "Should Foot Surgery Be Performed for Children With Duchenne Muscular Dystrophy?" Journal of Pediatric Orthopaedics 25, no. 1 (January 2005): 95–97. http://dx.doi.org/10.1097/01241398-200501000-00021.

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13

Qaniah, Fadhilah Ahmad. "Ekspresi Duchenne Smile dan Suasana Kota: Observasi di Beberapa Pusat Kota yang Sedang Tahap Pembangunan Ulang." Jurnal Planologi 18, no. 1 (April 30, 2021): 65. http://dx.doi.org/10.30659/jpsa.v18i1.11683.

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ABSTRAKSuasana kota umumnya tercipta dari karakteristik fisik berbagai tata ruang kota dan interaksi sosial di dalamnya. Komponen visual yang dirasakan memberikan beragam sensasi yang mengarahkan individu menangkap kualitas dari situasi secara emosional yang mampu mempengaruhi mood atau suasana hati mereka. Di saat interaksi sosial terjadi, ekspresi tersenyum duchenne dapat terjadi ketika individu merasakan emosi-emosi positif. Namun, suasana kota dapat berubah di saat ada pembangunan ulang fasilitas publik. Hal tersebut dapat mempengaruhi bentuk tata ruang kota dan potensi interaksi sosial yang dapat mempengaruhi suasana hati individu. Penelitian ini bertujuan untuk melihat seberapa banyak ekspresi senyum duchenne yang ada pada area kota yang ramai di pusat kota Kendari dan Jakarta Selatan. Penulis memilih pusat kota Kendari dan Jakarta Selatan karena memiliki fasilitas publik yang baik. Adapun kota Kendari saat ini membangun ulang beberapa pusat kotanya sejak tahun 2019. Hasil penelitian memperlihatkan, kedua area kota memiliki jumlah frekuensi senyuman duchenne yang sama banyaknya di area suasana jenis consumerism dan peaceful and quiet. Namun, ada perbedaan jumlah di area suasana jenis vibrant dan historic, karena beberapa fasilitas di kota tua dan pusat kota Kendari saat observasi berada di tahap pembangunan ulang. Suasana kota merupakan hal yang penting untuk image kota karena memberikan pengalaman emosi positif bagi orang yang singgah atau berinteraksi sosial di lingkungan perkotaan.Katakunci: duchenne, suasana kota, vibrant, peaceful and quiet, consumerism, historic ABSTRACTThe atmosphere of the city created from pshysical characteristics of various urban spatial plans and social interaction in it. The perceived visual component provided sensations that directs individual to capture quality of situation emotionally which can affect their mood. When social interaction occurs, duchenne smile expressions can appear when individual feel positive emotion. However, city atmosphere can change when there is unfinished redevelopment of public facilities. This can affect the form of urban spatial and potential social interactions that can affect individual moods. This study aims to see how much duchenne smile expressions appear in downtown area in Kenadi and South Jakarta. The author chose city center of Kendari and Jakarta because they have good public facilites. The city of Kendari is currently rebuilding several of its facilities in city centers since 2019. Result show that both city areas had same number of duchenne smiles in areas of consumerism and peaceful & quiet type. However, there are differences in the number of vibrant and historic areas, its perhaps because some of the facilities in the old town and downtown Kendari at the time of observatio were in the reconstruction stage. City atmosphere is important for the image of the city becauses it provides positive emotional experiences for people who stop by or interact socially in urban environment.Keyword: duchenne, city atmosphere, vibrant, peaceful and quiet, consumerism, historic
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14

Cohen-Sobel, E., V. Darmochwal, M. Caselli, S. Najjar, C. Lambert, and E. ]. Cohen-Sobol E [corrected to Cohen-Sobel. "Atypical case of Becker's muscular dystrophy. Early identification and management." Journal of the American Podiatric Medical Association 84, no. 4 (April 1, 1994): 181–88. http://dx.doi.org/10.7547/87507315-84-4-181.

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A case of a child with Becker's muscular dystrophy is presented. Because of the genetic and clinical similarity with the more common Duchenne muscular dystrophy, these two diseases are compared. Since muscular dystrophy often initially presents with toe walking, flat-foot, and waddling gait, podiatrists may be the first physicians to see the child and provide early diagnosis.
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15

Gupta, Anupam, Shanti Prakash Arya, and Atchayaram Nalini. "Ankle-Foot Orthosis in Duchenne Muscular Dystrophy: Experience in a Multidisciplinary Neuromuscular Clinic." Archives of Physical Medicine and Rehabilitation 98, no. 10 (October 2017): e134. http://dx.doi.org/10.1016/j.apmr.2017.08.438.

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16

Oddoux, Sarah, Kristien J. Zaal, Victoria Tate, Aster Kenea, Shuktika A. Nandkeolyar, Ericka Reid, Wenhua Liu, and Evelyn Ralston. "Microtubules that form the stationary lattice of muscle fibers are dynamic and nucleated at Golgi elements." Journal of Cell Biology 203, no. 2 (October 21, 2013): 205–13. http://dx.doi.org/10.1083/jcb.201304063.

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Skeletal muscle microtubules (MTs) form a nonclassic grid-like network, which has so far been documented in static images only. We have now observed and analyzed dynamics of GFP constructs of MT and Golgi markers in single live fibers and in the whole mouse muscle in vivo. Using confocal, intravital, and superresolution microscopy, we find that muscle MTs are dynamic, growing at the typical speed of ∼9 µm/min, and forming small bundles that build a durable network. We also show that static Golgi elements, associated with the MT-organizing center proteins γ-tubulin and pericentrin, are major sites of muscle MT nucleation, in addition to the previously identified sites (i.e., nuclear membranes). These data give us a framework for understanding how muscle MTs organize and how they contribute to the pathology of muscle diseases such as Duchenne muscular dystrophy.
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17

Taktak, Diane M., and Peter Bowker. "Lightweight, modular knee-ankle-foot orthosis for duchenne muscular dystrophy: Design, development, and evaluation." Archives of Physical Medicine and Rehabilitation 76, no. 12 (December 1995): 1156–62. http://dx.doi.org/10.1016/s0003-9993(95)80126-x.

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18

Bencze, M., J. Meng, V. Pini, F. Conti, F. Muntoni, and J. Morgan. "Necroptosis, a programmed form of necrosis, participates in muscle degeneration in Duchenne muscular dystrophy." Neuromuscular Disorders 27 (October 2017): S98. http://dx.doi.org/10.1016/j.nmd.2017.06.029.

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19

Bencze, Maximilien, Veronica Pini, Virginie Mariot, Julie Dumonceaux, Francesco Conti, Francesco Muntoni, Frederic Relaix, François Jerôme Authier, and Jenny Morgan. "Necroptosis, a programmed form of necrosis, participates in muscle degeneration in Duchenne muscular dystrophy." Morphologie 102, no. 338 (September 2018): 145–46. http://dx.doi.org/10.1016/j.morpho.2018.07.110.

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20

Bencze, M., J. Meng, V. Pini, F. Conti, F. Muntoni, and J. E. Morgan. "Necroptosis, a programmed form of necrosis participates in muscle degeneration in Duchenne muscular dystrophy." Neuromuscular Disorders 27 (March 2017): S7. http://dx.doi.org/10.1016/s0960-8966(17)30236-5.

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21

Garralda, M. Elena, Francesco Muntoni, Anna Cunniff, and Angeles Diaz Caneja. "Knee–ankle–foot orthosis in children with duchenne muscular dystrophy: User views and adjustment." European Journal of Paediatric Neurology 10, no. 4 (July 2006): 186–91. http://dx.doi.org/10.1016/j.ejpn.2006.07.002.

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22

Bromwich, W., N. Emery, C. Stewart, M. James, and R. Quinlivan. "P09 A Novel Ankle foot orthoses/footwear combination to aid walking in Duchenne muscular dystrophy." Neuromuscular Disorders 20 (March 2010): S7. http://dx.doi.org/10.1016/s0960-8966(10)70024-9.

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23

Dubow, Jordan S., and James M. Meyer. "Corticosteroids in Duchenne Muscular Dystrophy—A Deflazacort Review." US Neurology 12, no. 01 (2016): 12. http://dx.doi.org/10.17925/usn.2016.12.01.12.

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Duchenne Muscular dystrophy (DMD) is a devastating disorder that is the most common and severe form of childhood onset muscular dystrophy. While guidelines exist that promote a multidisciplinary approach to treatment of DMD, surveys have identified inconsistent adherence to the recommendations. This is particularly highlighted by the variability in the prescriptive habits of corticosteroids, which have a long history of literature supporting their use in DMD. Relatively recent publications have uncovered differences between countries in the prescription patterns of steroid regimens. Currently, treatment recommendations lack comparative data on the efficacy and safety of alternative dosing regimens of corticosteroids. New research is being conducted to help understand the benefits and risks of the most commonly used glucocorticoid regimens in DMD. Deflazacort is a glucocorticoid corticosteroid that is currently seeking approval in the U.S. This article summarizes the relevant published literature on deflazacort to help inform DMD-treating physicians on its impact on the disease.
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24

Autelli, R., L. Persson, and F. M. Baccino. "Cloning and expression of two ornithine decarboxylase forms from HMOA cells." Biochemical Journal 312, no. 1 (November 15, 1995): 13–16. http://dx.doi.org/10.1042/bj3120013.

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In HMOA cells [Mamont, Duchesne, Grove and Tardif (1978) Exp. Cell Res. 115, 387-393] the half-life of ornithine decarboxylase (ODC) is 8-14 h instead of 15 min as in the Hepatoma Tissue Culture parental cells, due to a single amino acid substitution [Miyazaki, Matsufuji, Murakami and Hayashi (1993) Eur. J. Biochem. 214, 837-844]. We demonstrate for the first time that HMOA cells possess two forms of ODC mRNA that are translated into two proteins differing greatly in turnover rates. We have cloned and transfected the cDNAs for the two ODC forms into COS-1 cells for a direct measurement of their turnover rate. The variant ODC form was much more stable than the wild-type protein, with a half-life of 14 h as compared with 2.5 h.
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25

Marquis-Nicholson, Renate, Daniel Lai, Chuan-Ching Lan, Jennifer M. Love, and Donald R. Love. "A Streamlined Protocol for Molecular Testing of the DMD Gene within a Diagnostic Laboratory: A Combination of Array Comparative Genomic Hybridization and Bidirectional Sequence Analysis." ISRN Neurology 2013 (February 7, 2013): 1–7. http://dx.doi.org/10.1155/2013/908317.

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Purpose. The aim of this study was to develop a streamlined mutation screening protocol for the DMD gene in order to confirm a clinical diagnosis of Duchenne or Becker muscular dystrophy in affected males and to clarify the carrier status of female family members. Methods. Sequence analysis and array comparative genomic hybridization (aCGH) were used to identify mutations in the dystrophin DMD gene. We analysed genomic DNA from six individuals with a range of previously characterised mutations and from eight individuals who had not previously undergone any form of molecular analysis. Results. We successfully identified the known mutations in all six patients. A molecular diagnosis was also made in three of the four patients with a clinical diagnosis who had not undergone prior genetic screening, and testing for familial mutations was successfully completed for the remaining four patients. Conclusion. The mutation screening protocol described here meets best practice guidelines for molecular testing of the DMD gene in a diagnostic laboratory. The aCGH method is a superior alternative to more conventional assays such as multiplex ligation-dependent probe amplification (MLPA). The combination of aCGH and sequence analysis will detect mutations in 98% of patients with the Duchenne or Becker muscular dystrophy.
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McMillan, Hugh J., Craig Campbell, and Jean K. Mah. "Duchenne Muscular Dystrophy: Canadian Paediatric Neuromuscular Physicians Survey." Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 37, no. 2 (March 2010): 195–205. http://dx.doi.org/10.1017/s0317167100009926.

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Background:Duchenne muscular dystrophy (DMD) is the most common form of muscular dystrophy in childhood.Method:To assess the current care of paediatric DMD patients in Canada, a questionnaire was mailed to 17 physicians who were members of the Canadian paediatric neuromuscular group. Areas of enquiry included; 1) multidisciplinary team composition; 2) means of DMD diagnosis; 3) corticosteroid use; surveillance and management for: 4) orthopaedic, 5) respiratory and 6) cardiac complications and 7) health maintenance (nutrition & immunizations).Results:Completed surveys were returned by 14/17 (82%) of physicians. Twelve respondents followed DMD patients. All centres had multidisciplinary teams, including respirology (11/12), child neurology or physiatry (11), physiotherapy (9), occupational therapy (9) and orthopaedic surgery (7). Deflazacort 0.9mg/kg/d was used at all centres, which was continued after loss of independent ambulation (11), along with routine calcium and vitamin D supplementation (10). Night splints were prescribed at all centres. Routine surveillance studies included pulmonary function testing (11), sleep studies (10), EKG/echocardiogram (10), bone density (DEXA) scans (10), spine radiography (9), and dietician referral (4).Conclusion:Paediatric DMD patients are receiving relatively consistent care in multidisciplinary clinics across Canada, in accordance with recommended guidelines for DMD.
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27

ZAV’YALOVA, Oksana O. "SALONS AND CIRCLES IN RUSSIA IN THE MIDDLE OF THE XIX CENTURY AS A FORM OF INTERACTION BETWEEN POWER AND PUBLIC." Historical and social-educational ideas 10, no. 6/1 (January 18, 2019): 19–28. http://dx.doi.org/10.17748/2075-9908-2018-10-6/1-19-28.

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The article presents an analysis of the functioning of salons and circles in Russia in the second half of the 1850s as a form of interaction between government and society in this period. On the basis of published sources of personal origin and archival materials, the main stages of activity the St. Petersburg circle of brothers Milyutins and salon of Grand Duchess Elena Pavlovna were reviewed. They brought together progressive government and public figures who advocated the implementation of reforms. It is concluded that with the advent of Alexander II to power and the launch of the modernization process in Russia, participants in the St. Petersburg circle are reoriented from scientific and educational issues to politically significant issues. Also during this period, close cooperation was established between the circle of brothers Milyutins and the salon of Grand Duchess Elena Pavlovna in the framework of the development of a project on the abolition of serfdom in the Karlovka estate. In the conditions of the sociopolitical rise of the second half of the 1850s salons and circles in Russia became centers of informal influence on the official course of the Russian government and turned into discussion «platforms», in which discussion and development of reform projects.
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Nicholson, C., M. Main, M. Kinali, F. Muntoni, and E. Mercuri. "G.P.12.12 Predictive factors for progressive foot deformity in non-ambulant boys with Duchenne muscular dystrophy." Neuromuscular Disorders 18, no. 9-10 (October 2008): 807. http://dx.doi.org/10.1016/j.nmd.2008.06.284.

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29

de Souza, Mariana Angélica, Marisa Maia Leonardi Figueiredo, Cyntia Rogean de Jesus Alves de Baptista, Robson Devanir Aldaves, and Ana Claudia Mattiello-Sverzut. "Beneficial effects of ankle–foot orthosis daytime use on the gait of Duchenne muscular dystrophy patients." Clinical Biomechanics 35 (June 2016): 102–10. http://dx.doi.org/10.1016/j.clinbiomech.2016.04.005.

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30

Waris, Risda, and A. Mumtihanah Mursyid. "Aktivitas Antiinflamasi Ekstrak Etanolik Daun Arbenan [Duchesnea indica (Jacks.) Focke]." Jurnal Fitofarmaka Indonesia 8, no. 1 (January 29, 2021): 18–22. http://dx.doi.org/10.33096/jffi.v8i1.722.

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Arbenan leaf is a medicinal plant that used in the healing of inflammation. The content of phenolic compounds, flavonoids, steroids and tannins contained in arbenan plants which play an important role in alternative medicine. Currently, scientific work of phytotherapy is an important alternative route in finding effective natural medicines with minimal side effects. The aim of this study was to determine the phytochemical properties and to determine the anti-inflammatory effect of arbenan leaf [Duchesnea indica (Jacks.) Focke] extract. In this study, nine male Wistar rats were tested in three treatment groups. Group I Na CMC, group II Na diclofenac and group III arbenan leaf extract. Inflammation in rats by inducing 1% carrageenan as much as 0.10 mL. The volume of edema per hour is known from the difference in the volume of the foot at certain hours with the volume of the normal foot. The AUC value of edema volume was calculated by the trapezoid method every one hour and the percen of anti-inflammatory power was calculated. The statistical analysis of the Least Significance Different (LSD) AUC value from the edema volume data showed that the two treatment groups, namely the Na diclofenac and the arbenan leaf extract group, were significantly different from the Na CMC group (P <0.05). So that both treatment groups have anti-inflammatory effects. The results showed that the ethanolik extract of arbenan leaves contained phenolic compounds, flavonoids and tannins. Then the ethanolic extract of arbenan leaves has anti-inflammatory activity of 0.31%.
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Hellebrekers, Danique M. J., Judith M. Lionarons, Catharina G. Faber, Sylvia Klinkenberg, Johan S. H. Vles, and Jos G. M. Hendriksen. "Instruments for the Assessment of Behavioral and Psychosocial Functioning in Duchenne and Becker Muscular Dystrophy; a Systematic Review of the Literature." Journal of Pediatric Psychology 44, no. 10 (August 20, 2019): 1205–23. http://dx.doi.org/10.1093/jpepsy/jsz062.

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Abstract Objective This systematic review aims to provide an overview of instruments used to assess behavioral and psychosocial functioning of patients with Duchenne and Becker muscular dystrophy, as well as to review the psychometric properties and applicability of these instruments. Methods Five databases (Embase, Psyc.info, ERIC, Pubmed/Medline, and Cochrane) were searched from inception to June, 2018. Potential articles were rated by two independent reviewers. A predefined PROSPERO form (CRD42017074518) was used to extract data from included articles. Results Sixty-one instruments were used in 54 studies. The Child Behavior Checklist is commonly used, but it lacks disease specific psychometric information. Sixteen instruments that contained disease specific psychometric information were included for final evaluation. The results displayed three instruments that are potentially valid for screening of psychosocial problems: The Psychosocial Adjustment and Role Skills Scale 3rd edition, the Pediatric Quality of Life Inventory Generic module, and the Life Satisfaction Index for Adolescents with Duchenne muscular dystrophy. Appropriate instruments for screening of behavioral problems may be: the Strengths and Difficulties Questionnaire, the Generalized Anxiety Disorder-7 item questionnaire, and the Patient Health Questionnaire-9 item questionnaire. Conclusions Further research on psychometric properties of screening instruments is crucial to ascertain a gold standard for clinical and research purposes. Meanwhile, for definite diagnostics purposes we recommend a multimethod, multisource, multisetting assessment in this high-risk population.
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Verma, Ritu, Lily Pal, Rakesh Pandey, and Vimal Kumar Paliwal. "Enzyme Histochemical Assessment of Mitochondrial Functions in Patients with Myopathic form of Limb-Girdle Syndrome." ISRN Pathology 2011 (November 24, 2011): 1–8. http://dx.doi.org/10.5402/2011/204940.

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Isolated mitochondrial myopathy is characterized by slowly progressive limb-girdle muscle weakness and resembles other muscle disorders like muscular dystrophy or inflammatory myopathy on clinical grounds. Identification of abnormal mitochondria in the muscle tissue is required for the diagnosis of isolated mitochondrial myopathy. Therefore, this study was done with aim to identify patients with isolated mitochondrial myopathy among those with limb-girdle muscle syndromes of undefined cause. Forty-eight consecutive patients with limb-girdle muscle disease from 2008 to 2010 were screened for Duchenne/Becker muscular dystrophy gene deletion, metabolic myopathy, and drug-induced and endocrine causes. Twenty patients without an identifiable cause were subjected to muscle biopsy for hematoxylin and eosin staining and enzyme histochemistry. Clinical, biochemical, and electrophysiological features in all these patients with limb-girdle muscle disease were nonspecific, and no conclusion regarding the underlying cause could be drawn from these investigations. On hematoxylin and eosin staining, 12 patients were diagnosed as muscular dystrophy, inflammatory myopathy with characteristic appearance of polymyositis was diagnosed in 4 patients, and 3 patients had normal muscle histology. After enzyme histochemistry, one patient was identified having mitochondrial myopathy. A brief case summary of the only patient diagnosed as isolated mitochondrial myopathy in our study is presented.
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Dudkina, N. A. "Multiple sclerosis: environmental risk factors." Neurology Bulletin XXX, no. 1-2 (March 15, 1998): 42–43. http://dx.doi.org/10.17816/nb80849.

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The author represented materials on incidence of progressive muscular distrophy in the region of Tver. 168 patients were investigated with different forms of disease. The most frequent form of disease was Erbs athophy (in 60%); incidence of Duchennes and Landuzys forms was the same (in 20%). Compared with the Russias indices of incidence, significant prevailing of this pathology in the region of Tver was revealed.
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Ogundele, Michael, Jesslyn S. Zhang, Mansi V. Goswami, Marissa L. Barbieri, Utkarsh J. Dang, James S. Novak, Eric P. Hoffman, Kanneboyina Nagaraju, and Yetrib Hathout. "Validation of Chemokine Biomarkers in Duchenne Muscular Dystrophy." Life 11, no. 8 (August 13, 2021): 827. http://dx.doi.org/10.3390/life11080827.

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Duchenne muscular dystrophy (DMD) is a progressive muscle disease involving complex skeletal muscle pathogenesis. The pathogenesis is triggered by sarcolemma instability due to the lack of dystrophin protein expression, leading to Ca2+ influx, muscle fiber apoptosis, inflammation, muscle necrosis, and fibrosis. Our lab recently used two high-throughput multiplexing techniques (e.g., SomaScan® aptamer assay and tandem mass tag-(TMT) approach) and identified a series of serum protein biomarkers tied to different pathobiochemical pathways. In this study, we focused on validating the circulating levels of three proinflammatory chemokines (CCL2, CXCL10, and CCL18) that are believed to be involved in an early stage of muscle pathogenesis. We used highly specific and reproducible MSD ELISA assays and examined the association of these chemokines with DMD pathogenesis, age, disease severity, and response to glucocorticoid treatment. As expected, we confirmed that these three chemokines were significantly elevated in serum and muscle samples of DMD patients relative to age-matched healthy controls (p-value < 0.05, CCL18 was not significantly altered in muscle samples). These three chemokines were not significantly elevated in Becker muscular dystrophy (BMD) patients, a milder form of dystrophinopathy, when compared in a one-way ANOVA to a control group but remained significantly elevated in the age-matched DMD group (p < 0.05). CCL2 and CCL18 but not CXCL10 declined with age in DMD patients, whereas all three chemokines remained unchanged with age in BMD and controls. Only CCL2 showed significant association with time to climb four steps in the DMD group (r = 0.48, p = 0.038) and neared significant association with patients’ reported outcome in the BMD group (r = 0.39, p = 0.058). Furthermore, CCL2 was found to be elevated in a serum of the mdx mouse model of DMD, relative to wild-type mouse model. This study suggests that CCL2 might be a suitable candidate biomarker for follow-up studies to demonstrate its physiological significance and clinical utility in DMD.
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Gupta, Anupam, Atchayaram Nalini, Shanti Prakash Arya, Seena Vengalil, Meeka Khanna, Rashmi Krishnan, and Arun B. Taly. "Ankle-Foot Orthosis in Duchenne Muscular Dystrophy: A 4 year Experience in a Multidisciplinary Neuromuscular Disorders Clinic." Indian Journal of Pediatrics 84, no. 3 (November 5, 2016): 211–15. http://dx.doi.org/10.1007/s12098-016-2251-7.

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Ushakova, Rima A., Svetlana P. Bochkareva, and Anna A. Vereshhinskaja. "Children who have cytolysis syndrome in debut of genetic diseases: analysis of primary morbidity." Russian Pediatric Journal 1, no. 4 (January 15, 2021): 18–24. http://dx.doi.org/10.15690/rpj.v1i4.2192.

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Background. Cytolysis syndrome often helps to suspect liver pathology. However, a rare genetic disease may manifest under the guise of increased transaminases. No true etiology of the disease is then identified with the standard examination algorithm. It is not recognized for a long time. Patients diagnosed with unspecified hepatitis receive irrational treatment, which in turn leads to deterioration in the quality of medical care.Purpose. To analyze the medical history, laboratory test results and list of clinical symptoms in pediatric patients with genetic diseases in the debut, and to correlate an identified symptom complex with increased transaminases.Methods. The article presents the results of a retrospective study of 17 randomly selected cases of children with a first established diagnosis of Duchenne — Becker muscular dystrophy, glycogen storage disease, mucopolysaccharidosis followed up in 2010–2019. We used data from patient complaints, anamnesis vitae and anamnesis morbi, laboratory test results and list of clinical symptoms in the debut of genetic diseases. The statistical processing was performed by using parametric and nonparametric methods. Confirmation of the diagnosis was obtained by molecular genetic techniques and the test for expression of urine glycosaminoglycans. Confirming diagnostic technologies were used in the laboratory of molecular diagnostics and the laboratory of inherited metabolic diseases at the Bochkov Research Centre for Medical Genetics (Moscow).Results. A comparative analysis of medical history and clinical and laboratory data was performed in 9 patients with Duchenne — Becker muscular dystrophy, 5 patients with glycogen storage disease, and 3 children with mucopolysaccharidosis. Prolonged neonatal jaundice was observed in 22.2% of newborns with Duchenne muscular dystrophy. In myopathies, elevated transaminases originate from destroyed muscle fibers and are not associated with the breakdown of the liver cells. This fact is also confirmed by our discovery of a direct correlation between AST (r = 0.76) and ALT (r = 0.72) values with high activity of creatine phosphokinase (CPK), p < 0.05. Prolonged neonatal jaundice was observed in 40% of children with glycogen storage disease. Hepatomegaly was detected in all cases, due to which the volume of the abdomen increases giving a specific form to patients against the background of overweight, lag in physical development due to low growth in 80% of cases and a “puppet face” in 100% of children. There is strong correlation between the increased alkaline phosphatase and AST (r = 0.78), ALT (r = 0.81), p < 0.05. In the third group, there are three children with mucopolysaccharidosis. We did not find any significant increase in transaminases in this group of children.Conclusion. Against the background of progressive Duchenne — Becker muscular dystrophy, hyperenzymemia is detected in each male patient. There is correlation between elevated transaminases and high creatine phosphokinase. Cytolysis syndrome was found only in some cases of glycogen storage disease, and hyperenzymemia occurs against the background of typical clinical symptoms of the disease. In cases of mucopolysaccharidosis we have found no elevated transaminases, but there is hepatomegaly. In case of prolonged unexplained cytolysis syndrome, the pediatrician should conduct a diagnostic search to identify or exclude genetic diseases.
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Law, D. J., D. L. Allen, and J. G. Tidball. "Talin, vinculin and DRP (utrophin) concentrations are increased at mdx myotendinous junctions following onset of necrosis." Journal of Cell Science 107, no. 6 (June 1, 1994): 1477–83. http://dx.doi.org/10.1242/jcs.107.6.1477.

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Duchenne muscular dystrophy (DMD) and the myopathy seen in the mdx mouse both result from absence of the protein dystrophin. Structural similarities between dystrophin and other cytoskeletal proteins, its enrichment at myotendinous junctions, and its indirect association with laminin mediated by a transmembrane glycoprotein complex suggest that one of dystrophin's functions in normal muscle is to form one of the links between the actin cytoskeleton and the extracellular matrix. Unlike Duchenne muscular dystrophy patients, mdx mice suffer only transient muscle necrosis, and are able to regenerate damaged muscle tissue. The present study tests the hypothesis that mdx mice partially compensate for dystrophin's absence by upregulating one or more dystrophin-independent mechanisms of cytoskeleton-membrane association. Quantitative analysis of immunoblots of adult mdx muscle samples showed an increase of approximately 200% for vinculin and talin, cytoskeletal proteins that mediate thin filament-membrane interactions at myotendinous junctions. Blots also showed an increase (143%) in the dystrophin-related protein called utrophin, another myotendinous junction constituent, which may be able to substitute for dystrophin directly. Muscle samples from 2-week-old animals, a period immediately preceding the onset of muscle necrosis, showed no significant differences in protein concentration between mdx and controls. Quantitative analyses of confocal images of myotendinous junctions from mdx and control muscles show significantly higher concentrations of talin and vinculin at the myotendinous junctions of mdx muscle. These findings indicate that mdx mice may compensate in part for the absence of dystrophin by increased expression of other molecules that subsume dystrophin's mechanical function.
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38

Bakker, J. P. J., I. J. M. de Groot, H. Beckerman, B. A. de Jong, and G. J. Lankhorst. "The effects of knee-ankle-foot orthoses in the treatment of Duchenne muscular dystrophy: review of the literature." Clinical Rehabilitation 14, no. 4 (August 2000): 343–59. http://dx.doi.org/10.1191/0269215500cr319oa.

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39

Debes, Mario A., Ingrid G. Orce, Ana C. Luque, Ana C. Luque, Juan C. Díaz-Ricci, Atilio P. Castagnaro, and Marta E. Arias. "First report of Duchesnea indica f. albocaput (Rosaceae) in Northwestern Argentina." Boletín de la Sociedad Argentina de Botánica 53, no. 1 (April 20, 2018): 83–91. http://dx.doi.org/10.31055/1851.2372.v53.n1.19890.

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: The genus Duchesnea includes two species originally from India: D. indica and D. chrysantha. In Northwestern Argentina the monitoring of wild strawberry-like species was carried out; during 2002- 2016, many populations of D. indica and none of D. chrysantha were discovered. Red- and white-fruited plants of D. indica were collected from disturbed areas and ex situ conserved in green-house and in nursery conditions. Were also consulted materials from different national and international herbaria, and we only found reports of D. indica with red fruit for Argentina. In the present work, we report for the first time the presence of D. indica f. albocaput in Argentina and South America, cohabiting with populations of D. indica f. indica in the underwoods of Tucumán. This finding broadens the distribution range of D. indica f. albocaput, cited as endemic to Japan by Naruhashi 1992. The morphological and anatomical characters of the two botanical forms of D. indica (red fruit genotypes and white fruit genotypes) are also presented: fruit color, number of leaflets, and crystal form
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Anggraeny, Ebta Narasukma, Endang Sri Sunarsih, Patricia Sanggita Listyoputri Wibowo, and Novi Elisa. "Aktivitas Antioksidan Jus Stroberi (Fragaria ananassa Duchessne) Terhadap Kadar SGPT, SGOT dan MDA pada Tikus Jantan Galur Wistar yang Diinduksi Isoniazid." JURNAL ILMIAH SAINS 21, no. 1 (March 1, 2021): 17. http://dx.doi.org/10.35799/jis.21.1.2021.30337.

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ABSTRAKIsoniazid adalah obat anti tuberkulosis yang digunakan baik sebagai monoterapi atau kombinasi. Penggunaan isoniazid dalam waktu lama dapat menyebabkan hepatotoksik. Hepatotoksik disebabkan oleh hasil metabolisme isoniazid di hepar berupa hidrazin dan asetilhidrazin. Radikal bebas tersebut yang menyebabkan tingginya reactive oxygen species (ROS) didalam tubuh. Tingginya radikal bebas menyebabkan peningkatan kadar SGPT dan SGOT yang merupakan indikator adanya kerusakan hepar. Tingginya radikal bebas dalam tubuh dapat dilihat dari paramter MDA. Hal tersebut dapat diatasi dengan pemberian antioksidan eksogen seperti jus stroberi (Fragaria ananassa Duchessne). Tujuan penelitian ini untuk mengetahui skrinning fitokimia jus stroberi dan pengaruh pemberian jus stroberi terhadap kadar SGPT, SGOT dan MDA pada tikus yang diinduksi Isoniazid. Perlakuan diberikan selama 14 hari dengan pembagian kelompok yaitu kontrol normal, kontrol negatif, kontrol positif, dosis 3g/kgBB, 6g/kgBB, dan 9g/kgBB. Pengambilan data dilakukan pada hari 1, hari 15, dan hari 29. Hasil penelitian dapat disimpulkan bahwa jus stroberi dapat menurunkan kadar SGPT, SGOT dan MDA pada tikus yang diinduksi isoniazid dengan dosis efektif sebesar 3g/kgBB tikus.Kata kunci: Isoniazid; jus stroberi; MDA; SGPT; SGOT;Effect of Strawberry Juice (Fragraria ananassa Duchessne) against SGPT, SGOT and MDA levels in Isoniazide-Induced Wistar Male Rats ABSTRACTIsoniazid is an anti-tuberculosis drug that is used either as monotherapy or in combination. Prolonged use of isoniazid can cause hepatotoxicity. Hepatotoxicity is caused by the hepatic isoniazid metabolism in the form of hydrazine and acetylhydrazine. These free radicals cause high reactive oxygen species (ROS) in the body. The high level of free radicals causes an increase in SGPT and SGOT levels, which are indicators of liver damage. The high level of free radicals in the body can be seen from the MDA parameter. This can be overcome by giving exogenous antioxidants such as strawberry juice (Fragaria ananassa Duchessne). The purpose of this study was to determine the phytochemical screening of strawberry juice and the effect of giving strawberry juice on the levels of SGPT, SGOT and MDA in rats induced by Isoniazid. The treatment was given for 14 days divided into groups, namely normal control, negative control, positive control, dose of 3g / kg, 6g / kg, and 9g / kg of body weight. Data were collected on day 1, day 15, and day 29. The results of this study concluded that strawberry juice can reduce levels of SGPT, SGOT and MDA in isoniazid-induced rats with an effective dose of 3 g/kgBW rats.Keywords: Isoniazid; Strawberry juice; MDA; SGPT; SGOT
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41

Pizzey, J. A., J. A. Witkowski, and G. E. Jones. "Spreading behaviour of cultured fibroblasts from carriers of Duchenne muscular dystrophy." Journal of Cell Science 87, no. 1 (February 1, 1987): 163–69. http://dx.doi.org/10.1242/jcs.87.1.163.

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Cultured skin fibroblasts from patients with Duchenne muscular dystrophy (DMD) are more sensitive than normal cells to prolonged exposure to the ionophore monensin. In a cell spreading assay in which cells were preincubated with monensin and subsequently allowed to adhere to and spread on a glass substratum in serum-free medium for 100 min, the mean transformed cell area of normal and DMD cells was 5.97 +/− 0.11 and 5.29 +/− 0.03, respectively. Cultured fibroblasts from carriers of DMD yielded a value of 5.59 +/− 0.03, which is intermediate between, and significantly different from, the values for both normal and DMD cultures. This result would be predicted on the basis of random X-chromosome inactivation in female carriers of this disorder. However, comparison of DMD carrier cell spreading data with data obtained from pooled and summated measurements taken from separate experiments using either normal or DMD fibroblasts suggest a more complex situation. Examination of the variance of the means of cell area for the true carrier population and the summated normal and DMD population provides evidence suggesting that some form of cellular interaction may occur between the two cell genotypes in culture.
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42

Peter, Angela K., Jamie L. Marshall, and Rachelle H. Crosbie. "Sarcospan reduces dystrophic pathology: stabilization of the utrophin–glycoprotein complex." Journal of Cell Biology 183, no. 3 (November 3, 2008): 419–27. http://dx.doi.org/10.1083/jcb.200808027.

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Mutations in the dystrophin gene cause Duchenne muscular dystrophy and result in the loss of dystrophin and the entire dystrophin–glycoprotein complex (DGC) from the sarcolemma. We show that sarcospan (SSPN), a unique tetraspanin-like component of the DGC, ameliorates muscular dystrophy in dystrophin-deficient mdx mice. SSPN stabilizes the sarcolemma by increasing levels of the utrophin–glycoprotein complex (UGC) at the extrasynaptic membrane to compensate for the loss of dystrophin. Utrophin is normally restricted to the neuromuscular junction, where it replaces dystrophin to form a functionally analogous complex. SSPN directly interacts with the UGC and functions to stabilize utrophin protein without increasing utrophin transcription. These findings reveal the importance of protein stability in the prevention of muscular dystrophy and may impact the future design of therapeutics for muscular dystrophies.
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43

Wefki Abdelgawwad Shousha, Ahmed Abdelgawwad, Maria Sanfilippo, Antonio Sabba, and Paolo Pinchera. "Sugammadex and Reversal of Neuromuscular Block in Adult Patient with Duchenne Muscular Dystrophy." Case Reports in Anesthesiology 2014 (2014): 1–3. http://dx.doi.org/10.1155/2014/680568.

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Duchenne’s muscular dystrophy (DMD) is the most common and severe form of myopathy. Patients with DMD are more sensitive to sedative, anesthetic, and neuromuscular blocking agents which may result in intraoperative and early postoperative cardiovascular and respiratory complications, as well as prolonged recovery from anesthesia. In this case report, we describe a 25-year-old male patient admitted for cholecystectomy under general anesthesia. We induced our anesthesia by oxygen, propofol, fentanyl, and rocuronium bromide. Maintenance was done by fentanyl, rocuronium bromide, sevoflurane, and O2. We report in this case the safety use of sugammadex to antagonize the neuromuscular block and rapid recovery in such category of patients.
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Martini, Joyce, Mariana Callil Voos, Michele Emy Hukuda, Maria Bernadete Dutra de Resende, and Fátima Aparecida Caromano. "Compensatory movements during functional activities in ambulatory children with Duchenne muscular dystrophy." Arquivos de Neuro-Psiquiatria 72, no. 1 (January 2014): 5–11. http://dx.doi.org/10.1590/0004-282x20130196.

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Objective: During the transitional phase (ambulatory to non-ambulatory), synergies characterize the evolution of Duchenne muscular dystrophy (DMD). This study was performed to describe and quantify compensatory movements while sitting down on/rising from the floor and climbing up/down steps. Method: Eighty videos (5 children × 4 assessments × 4 tasks) were recorded quarterly in the year prior to gait loss. Compensatory movements from the videos were registered based on the Functional Evaluation Scale for DMD. Results: The most frequently observed compensatory movements were upper limb support on lower limbs/floor/handrail during all the tasks and lumbar hyperlordosis, trunk support on handrail, equinus foot, increased base of support, non-alternated descent, and pauses while climbing up/down steps. Conclusion: Climbing up/down steps showed a higher number of compensatory movements than sitting down on/rising from the floor, which seemed to be lost before climbing up/down steps in ambulatory children with DMD.
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Senesac, Claudia R., Donovan J. Lott, Sean C. Forbes, Sunita Mathur, Ishu Arpan, Emily S. Senesac, Glenn A. Walter, and Krista Vandenborne. "Longitudinal Evaluation of Muscle Composition Using Magnetic Resonance in 4 Boys With Duchenne Muscular Dystrophy: Case Series." Physical Therapy 95, no. 7 (July 1, 2015): 978–88. http://dx.doi.org/10.2522/ptj.20140234.

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Background Duchenne muscular dystrophy (DMD), an inherited recessive X chromosome-linked disease, is the most severe childhood form of muscular dystrophy. Boys with DMD experience muscle loss, with infiltration of intramuscular fat into muscles. Objectives This case series describes the progression of DMD in boys using magnetic resonance imaging (MRI) and magnetic resonance spectroscopy (MRS). Magnetic resonance results are then compared with an established functional timed test. Methods Four boys with DMD and 4 healthy age-matched controls were chosen from a larger cohort. Boys with DMD were assessed at 4 time points over 2 years, with controls assessed at baseline only. Progression of the disease was documented by assessing the plantar flexors using MRI and MRS techniques and by assessing ambulation using the 30-Foot Fast Walk Test. Results Transverse relaxation time (T2) values were elevated in all boys with DMD at baseline. The lipid ratio increased rapidly as the disease progressed in 2 boys. Discrete changes in T2 in the other 2 boys with DMD indicated a slower disease progression. Magnetic resonance imaging and MRS allowed monitoring of the disease over all time periods regardless of ambulation status. Limitations The magnetic resonance data were collected with 2 different magnets at 2 different field strengths (1.5 and 3.0 T). Although we corrected for this difference, care must be taken in interpreting data when different image collection systems are used. This was a case series of 4 boys with DMD taken from a larger cohort study. Conclusions Magnetic resonance imaging and MRS are objective, noninvasive techniques for measuring muscle pathology and can be used to detect discrete changes in both people who are ambulatory and those who are nonambulatory. These techniques should be considered when monitoring DMD progression and assessing efficacy of therapeutic interventions.
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Vose, Heather M. "A Sixteenth-century Assessment of the French Church in the Years 1521–4 by Bishop Guillaume Briçonnet of Meaux." Journal of Ecclesiastical History 39, no. 4 (October 1988): 509–19. http://dx.doi.org/10.1017/s0022046900040574.

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‘C'est à vous, Madame, à qui je parle.’ So wrote the distinguished church statesman and reforming bishop of the Meaux diocese to Marguerite d'Angoulême, duchess of Alençon (later queen of Navarre), powerful sister of the Renaissance monarch François I, on 22 December 1521. The personal and emphatic form of address employed here by Guillaume Briçonnet arose from his concern that Marguerite should grasp firmly a neglected aspect of Christian doctrine: the role of the Spirit in the life of each believer as well as within the Ecclesia. By grace, came the episcopal injunction, Marguerite must recognise ‘le vray feu qui s'est logé, long temps a [i.e. il y a longtemps déja] dans vostre cceur’;and by this same grace French Christendom must acknowledge its state of desolation. An analysis of the 1521–4 exchange of letters between the duchess of Alençon and the bishop of Meaux reveals both the extent of Briçonnet's distress at what he saw as basic weaknesses in the French Church and his spiritual counsel aimed at rectifying a situation he held to be desperate.
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Lim, Kenji, Chantal Yoon, and Toshifumi Yokota. "Applications of CRISPR/Cas9 for the Treatment of Duchenne Muscular Dystrophy." Journal of Personalized Medicine 8, no. 4 (November 24, 2018): 38. http://dx.doi.org/10.3390/jpm8040038.

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Duchenne muscular dystrophy (DMD) is a fatal X-linked recessive neuromuscular disease prevalent in 1 in 3500 to 5000 males worldwide. As a result of mutations that interrupt the reading frame of the dystrophin gene (DMD), DMD is characterized by a loss of dystrophin protein that leads to decreased muscle membrane integrity, which increases susceptibility to degeneration. CRISPR/Cas9 technology has garnered interest as an avenue for DMD therapy due to its potential for permanent exon skipping, which can restore the disrupted DMD reading frame in DMD and lead to dystrophin restoration. An RNA-guided DNA endonuclease system, CRISPR/Cas9 allows for the targeted editing of specific sequences in the genome. The efficacy and safety of CRISPR/Cas9 as a therapy for DMD has been evaluated by numerous studies in vitro and in vivo, with varying rates of success. Despite the potential of CRISPR/Cas9-mediated gene editing for the long-term treatment of DMD, its translation into the clinic is currently challenged by issues such as off-targeting, immune response activation, and sub-optimal in vivo delivery. Its nature as being mostly a personalized form of therapy also limits applicability to DMD patients, who exhibit a wide spectrum of mutations. This review summarizes the various CRISPR/Cas9 strategies that have been tested in vitro and in vivo for the treatment of DMD. Perspectives on the approach will be provided, and the challenges faced by CRISPR/Cas9 in its road to the clinic will be briefly discussed.
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Cutrignelli, Annalisa, Francesca Sanarica, Antonio Lopalco, Angela Lopedota, Valentino Laquintana, Massimo Franco, Brigida Boccanegra, Paola Mantuano, Annamaria De Luca, and Nunzio Denora. "Dasatinib/HP-β-CD Inclusion Complex Based Aqueous Formulation as a Promising Tool for the Treatment of Paediatric Neuromuscular Disorders." International Journal of Molecular Sciences 20, no. 3 (January 30, 2019): 591. http://dx.doi.org/10.3390/ijms20030591.

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New scientific findings have recently shown that dasatinib (DAS), the first-choice oral drug in the treatment of chronic myeloid leukemia (CML) for adult patients who are resistant or intolerant to imatinib, is also potentially useful in the paediatric age. Moreover, recent preclinical evidences suggest that this drug could be useful for the treatment of Duchenne muscular dystrophy, since it targets cSrc tyrosin kinase. Based on these considerations, the purpose of this work was to use the strategy of complexation with hydroxypropyl-β-cyclodextrin (HP-β-CD) in order to obtain an aqueous preparation of DAS, which is characterized by a low water solubility (6.49 × 10−4 mg/mL). Complexation studies demonstrated that HP-β-CD is able to form a stable host-guest inclusion complex with DAS with a 1:1 apparent formation constant of 922.13 M−1, as also demonstrated by the Job’s plot, with an increase in DAS aqueous solubility of about 21 times in the presence of 6% w/v of HP-β-CD (0.014 mg/mL). The inclusion complex has been prepared in the solid state by lyophilization and characterized by Fourier Transform Infrared (FT-IR), Nuclear Magnetic Resonance (NMR), Differential Scanning Calorimetry (DSC) techniques, and its dissolution profile was studied at different pH values. Moreover, in view of potential use of DAS for Duchenne muscular dystrophy, the cytotoxic effect of the inclusion complex has been assessed on C2C12 cells, a murine muscle satellite cell line. In parallel, a one-week oral treatment was performed in wild type C57Bl/6J mice to test both palatability and the exposure levels of the new oral formulation of the compound. In conclusion, this new inclusion complex could allow the development of a liquid and solvent free formulation to be administered both orally and parenterally, especially in the case of an administration in paediatric age.
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49

Vitiello, Tibaudo, Pegoraro, Bello, and Canton. "Teaching an Old Molecule New Tricks: Drug Repositioning for Duchenne Muscular Dystrophy." International Journal of Molecular Sciences 20, no. 23 (November 30, 2019): 6053. http://dx.doi.org/10.3390/ijms20236053.

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: Duchenne muscular dystrophy (DMD) is one of the most severe forms of inherited muscular dystrophies. The disease is caused by the lack of dystrophin, a structurally essential protein; hence, a definitive cure would necessarily have to pass through some form of gene and/or cell therapy. Cell- and genetic-based therapeutics for DMD have been explored since the 1990s and recently, two of the latter have been approved for clinical use, but their efficacy is still very low. In parallel, there have been great ongoing efforts aimed at targeting the downstream pathogenic effects of dystrophin deficiency using classical pharmacological approaches, with synthetic or biological molecules. However, as it is always the case with rare diseases, R&D costs for new drugs can represent a major hurdle for researchers and patients alike. This problem can be greatly alleviated by experimenting the use of molecules that had originally been developed for different conditions, a process known as drug repurposing or drug repositioning. In this review, we will describe the state of the art of such an approach for DMD, both in the context of clinical trials and pre-clinical studies.
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Iwata, Y., T. Sakamoto, A. Maruyama, T. Tateishi, K. Yorimoto, H. Yajima, M. Wakita, et al. "S.P.15 Utility of subjective pain scales for assessments of standing with knee–ankle–foot-orthoses in Duchenne muscular dystrophy." Neuromuscular Disorders 22, no. 9-10 (October 2012): 880. http://dx.doi.org/10.1016/j.nmd.2012.06.254.

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