{"id":28,"date":"2024-10-30T14:26:21","date_gmt":"2024-10-30T14:26:21","guid":{"rendered":"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/?page_id=28"},"modified":"2024-10-30T14:26:22","modified_gmt":"2024-10-30T14:26:22","slug":"interactivo","status":"publish","type":"page","link":"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/interactivo\/","title":{"rendered":"Interactivo"},"content":{"rendered":"<div id=\"acf-block-6722420defda9\" class=\"block mps-interactivo\">\n    <div class=\"mps-interactivo-landing\">\n        <div class=\"split-content\">\n            <div class=\"wrapper\">\n                <div class=\"inner-wrapper\">\n                    <div class=\"content-block\">     \n                        <h1><span>Conhe\u00e7a mais sobre as<\/span> Mucopolissacaridoses<\/h1>\n                        <p>As mucopolissacaridoses (MPS) s\u00e3o doen\u00e7as metab\u00f3licas gen\u00e9ticas raras, com incid\u00eancia de 1 caso a cada 22.500 nascidos vivos. Constituem um grupo de doen\u00e7as caracterizadas pela falta de uma enzima respons\u00e1vel por decompor as longas cadeias de mol\u00e9culas de a\u00e7\u00facar, que acabam acumulando nas c\u00e9lulas. <sup>1_2<\/sup><\/p>\n                        <p>Essas mol\u00e9culas, chamadas glicosaminoglicanos (GAGs), desempenham papel importante nos diversos tecidos do corpo, como \u00f3sseo, cartilaginoso, vascular. Pacientes com MPS apresentam ac\u00famulo progressivo de GAGs em diferentes \u00f3rg\u00e3os, levando ao quadro cl\u00ednico multissist\u00eamico da doen\u00e7a<sup>1<\/sup>.<\/p>\n                        <p>Conhe\u00e7a um pouco mais sobre os sinais e sintomas da MPS IV A cl\u00e1ssica e MPS VI n\u00e3o cl\u00e1ssica, al\u00e9m da possibilidade de navegar no nosso esqueleto interativo para ver mais de perto os sintomas \u00f3sseos das MPS.<\/p>\n                        <p>Caso voc\u00ea tenha mais d\u00favidas sobre as mucopolissacaridoses, como diagnostic\u00e1-las e manej\u00e1-las, disponibilizamos o nosso Servi\u00e7o de Informa\u00e7\u00f5es M\u00e9dicas BioMarin por meio do e-mail: <a href=\"mailto:medinfola@bmrn.com\">medinfola@bmrn.com<\/a>.<\/p>\n                    <\/div>\n                    <div class=\"content-block\">\n                        <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/landing-image.png\" alt=\"\">\n                    <\/div>\n                <\/div>\n            <\/div>          \n        <\/div>\n        <div class=\"block\">\n            <div class=\"wrapper\">\n                <div class=\"inner-wrapper\">\n                    <p class=\"inline-buttons section-selection\">\n                        <a class=\"button button-arrow\" href=\"#mps-interactivo-section-skeletal\"><strong>MPS<\/strong> Sistema Esquel\u00e9tico<\/a>\n                        <a class=\"button button-arrow\" href=\"#mps-interactivo-section-not-classic\"><strong>MPS VI<\/strong>  N\u00e3o Cl\u00e1ssico<\/a>\n                        <a class=\"button button-arrow\" href=\"#mps-interactivo-section-classic\"><strong>MPS IVA<\/strong> Cl\u00e1ssico<\/a>\n                    <\/p>\n                <\/div>\n            <\/div>\n        <\/div>\n    <\/div>      \n    \n    <div id=\"mps-interactivo-section-skeletal\" class=\"mps-interactivo-section\">\n        <div class=\"wrapper\">\n            <div class=\"section-background\">\n                <model-viewer src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/model\/esqueleto_biomarin.glb\" ar ar-modes=\"webxr scene-viewer quick-look\" camera-controls poster=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/model\/poster.webp\" shadow-intensity=\"1\" shadow-softness=\"1\" environment-image=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/model\/pillars_1k.hdr\" tone-mapping=\"agx\">\n                  <div class=\"progress-bar hide\" slot=\"progress-bar\">\n                      <div class=\"update-bar\"><\/div>\n                  <\/div>\n                  <button slot=\"ar-button\" id=\"ar-button\">\n                      View in your space\n                  <\/button>\n                  <div id=\"ar-prompt\">\n                      <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/model\/ar_hand_prompt.png\">\n                  <\/div>\n                <\/model-viewer> \n            <\/div>  \n            \n            <div class=\"section-hotspots\">\n                <div class=\"area area-head\">\n                    <a class=\"hotspot\" href=\"#skeletal-section-head\">\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                    <\/a>\n                <\/div>\n                <div class=\"area area-torso\">\n                    <a class=\"hotspot\" href=\"#skeletal-section-torso\">\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                    <\/a>\n                <\/div>\n                <div class=\"area area-legs\">\n                    <a class=\"hotspot\" href=\"#skeletal-section-legs\">\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                    <\/a>\n                <\/div>\n            <\/div>\n            \n            <div class=\"main-content scrolling-container\">\n                <h2>Mucopolissacaridoses (MPS) <span>Sistema Esquel\u00e9tico<\/span><\/h2>                        \n                                \n                <div id=\"skeletal-section-head\" class=\"box section\">\n                    <div class=\"box-options\">\n                        <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/skeleton-head.jpg\" alt=\"Cabe\u00e7a\">\n                        <h3>Cabe\u00e7a e Pesco\u00e7o<\/h3>\n                        <ul class=\"section-links\">\n                            <li>\n                                <a href=\"#skeletal-head-01\">Cabe\u00e7a<\/a>\n                            <\/li>\n                            <li>\n                                <span>Macrocefalia<\/span>\n                            <\/li>\n                            <li>\n                                <span>Sela turca em forma de J<\/span>\n                            <\/li>\n                            <li>\n                                <span>Espessamento craniano<\/span>\n                            <\/li>\n                            <li>\n                                <span>Instabilidade atlanto-axial<\/span>\n                            <\/li>\n                            <li>\n                                <span>Estreitamento do canal vertebral<\/span>\n                            <\/li>\n                        <\/ul>                                       \n                    <\/div>\n                    \n                    <div id=\"skeletal-head-01\" class=\"box-information\">\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p>Na radiografia lateral de cr\u00e2nio em paciente com MPS IVA, evidencia-se:<\/p>\n                                        <p><strong class=\"letter-featured letter-white\">A<\/strong> Sela turca em forma de J<\/p>\n                                        <p><strong class=\"letter-featured letter-pink\">B<\/strong> Espessamento do cr\u00e2nio<\/p>\n                                        <p><strong class=\"letter-featured letter-orange\">C<\/strong> Platispondilia (achatamento dos corpos vertebrais)<\/p>\n                                        <p><strong>Refer\u00eancia:<\/strong> Palmucci S., Attin\u00e0 G., Lanza M L, et al. Insights Imaging, 2013, Aug;4(4):443-59. Imagem de cortesia do Dr. Hugo Flores-Navarro<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/skeleton01.jpg\" alt=\"\">\n                                            <\/div>                                          \n                                        <\/figure>\n                                    <\/div>  \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>                                      \n                                                                                \n                <\/div>\n                \n                <div id=\"skeletal-section-torso\" class=\"box section\">\n                    <div class=\"box-options\">\n                        <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/skeleton-torso.jpg\" alt=\"Torso\">\n                        <h3>Torso e membros<\/h3>\n                        <ul class=\"section-links\">                          \n                            <li>\n                                <a href=\"#skeletal-torso-01\">Coluna Vertebral<\/a>\n                            <\/li>\n                            <li>\n                                <a href=\"#skeletal-torso-02\">M\u00e3os<\/a>\n                            <\/li>\n                            <li>\n                                <span>Pectus Carinatum<\/span>\n                            <\/li>\n                            <li>\n                                <span>Costelas em forma de remo<\/span>\n                            <\/li>\n                            <li>\n                                <span>Clav\u00edculas curtas e largas<\/span>\n                            <\/li>\n                            <li>\n                                <span>Cifoescoliose<\/span>\n                            <\/li>\n                            <li>\n                                <span>Irregularidades do corpo vertebral e hiperlordose lombar<\/span>\n                            <\/li>\n                        <\/ul>                                       \n                    <\/div>\n                    \n                    <div id=\"skeletal-torso-01\" class=\"box-information\">\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p>Radiografia AP e lateral da coluna de paciente com MPS IVA.<\/p>\n                                        <p><strong class=\"letter-featured letter-white\">A<\/strong> Bico anterior<\/p>\n                                        <p><strong class=\"letter-featured letter-pink\">B<\/strong> Platispondilia (achatamento dos corpos vertebrais)<\/p>\n                                        <p><strong class=\"letter-featured letter-orange\">C<\/strong> Costelas em forma de remo<\/p>\n                                        <p><strong>Refer\u00eancia:<\/strong> Palmucci S, Attina G, Lanza Mi, et al. Insights imaging 2013 Aug:4(4)443-59. Imagem de cortesia do Dr. Hugo Flores-Navarro<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/skeleton02.jpg\" alt=\"\">\n                                            <\/div>                                          \n                                        <\/figure>\n                                    <\/div>      \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                    <div id=\"skeletal-torso-02\" class=\"box-information\">\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p>Radiografia dorso-palmar de paciente com MPS IVA.<\/p>\n                                        <p><strong class=\"letter-featured letter-white\">A<\/strong> Falanges distais curtas em forma de bala<\/p>\n                                        <p><strong class=\"letter-featured letter-pink\">B<\/strong> Metacarpos curtos com estreitamento proximal<\/p>\n                                        <p><strong class=\"letter-featured letter-orange\">C<\/strong> Hipoplasia do carpo<\/p>\n                                        <p><strong>Refer\u00eancia:<\/strong> Palmucci S, Attina G, Lanza Mi, et al. Insights imaging 2013 Aug:4(4)443-59. Imagem de cortesia do Dr. Hugo Flores-Navarro<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/skeleton03.jpg\" alt=\"\">\n                                            <\/div>                                          \n                                        <\/figure>\n                                    <\/div>\n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                                                                \n                <\/div>\n                \n                <div id=\"skeletal-section-legs\" class=\"box section\">\n                    <div class=\"box-options\">\n                        <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/skeleton-legs.jpg\" alt=\"Membros\">\n                        <h3>Membros inferiores<\/h3>\n                        <ul class=\"section-links\">                          \n                            <li>\n                                <a href=\"#skeletal-legs-01\">Quadril<\/a>\n                            <\/li>\n                            <li>\n                                <span>Dor e displasia no quadril<\/span>\n                            <\/li>\n                            <li>\n                                <span>Dor no p\u00e9<\/span>\n                            <\/li>\n                        <\/ul>                                       \n                    <\/div>                                      \n                    \n                    <div id=\"skeletal-legs-01\" class=\"box-information\">\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p>Radiografia simples anteroposterior da pelve em paciente com MPS IVA.<\/p>\n                                        <p><strong class=\"letter-featured letter-white\">A<\/strong> Il\u00edacos arredondados<\/p>\n                                        <p><strong class=\"letter-featured letter-pink\">B<\/strong> Ep\u00edfise femoral proximal aplanada e irregular bilateral<\/p>\n                                        <p><strong class=\"letter-featured letter-orange\">C<\/strong> Necrose avascular<\/p>\n                                        <p><strong class=\"letter-featured letter-red\">D<\/strong> Coxa valga<\/p>\n                                        <p><strong>Refer\u00eancia:<\/strong> Adaptado de Flores-Navarro H, Colmenares-Bonilla D. J Orthop Case Rep. 2021 Dec; 11(12):47-51. Palmucci S, Attina G, Lanza Mi, et al. Insights imaging 2013 Aug:4(4)443-59. Imagem de cortesia do Dr. Hugo Flores-Navarro<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/skeleton04.jpg\" alt=\"\">\n                                            <\/div>                                          \n                                        <\/figure>\n                                    <\/div>  \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>                                      \n                    \n                <\/div>\n                \n                <p><a class=\"button button-text button-arrow button-arrow-left mps-interactivo-back\" href=\"#\">Voltar<\/a><\/p>\n            <\/div>  \n        <\/div>  \n    <\/div>\n    \n    <div id=\"mps-interactivo-section-not-classic\" class=\"mps-interactivo-section\">\n        <div class=\"wrapper\">\n            <div class=\"section-background\"><\/div>  \n            \n            <div class=\"section-hotspots\">\n                <div class=\"area area-head\">\n                    <a class=\"hotspot\" href=\"#not-classic-section-head\">\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                    <\/a>\n                <\/div>\n                <div class=\"area area-torso\">\n                    <a class=\"hotspot\" href=\"#not-classic-section-torso\">\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                    <\/a>\n                <\/div>\n                <div class=\"area area-legs\">\n                    <a class=\"hotspot\" href=\"#not-classic-section-legs\">\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                    <\/a>\n                <\/div>\n            <\/div>\n            \n            <div class=\"main-content scrolling-container\">\n                <h2>Paciente MPS VI <span>N\u00e3o Cl\u00e1ssico<\/span><\/h2>                      \n                                \n                <div class=\"inline-note\">                   \n                    <p><strong>Nota importante:<\/strong>: Pacientes com MPS VI n\u00e3o cl\u00e1ssica geralmente apresentam sintomas \u00f3sseos e articulares, especialmente dor e rigidez articular, contraturas articulares e dor no quadril e nas costas. No entanto, a maioria desses pacientes desenvolver\u00e1 manifesta\u00e7\u00f5es graves da doen\u00e7a, incluindo degenera\u00e7\u00e3o articular, apneia do sono, diminui\u00e7\u00e3o da fun\u00e7\u00e3o pulmonar, redu\u00e7\u00e3o da resist\u00eancia, valvulopatia e complica\u00e7\u00f5es esquel\u00e9ticas.<\/p>\n                    <p><strong>Refer\u00eancia<\/strong>: Jurecka, A., Zakharova, E., Malinova, V. et al. Attenuated osteoarticular phenotype of type VI mucopolysaccharidosis: a report of four patients and a review of the literature. Clin Rheumatol 33, 725\u2013731 (2014). https:\/\/doi.org\/10.1007\/s10067-013-2423-z<\/p>                  \n                <\/div>\n                \n                <div id=\"not-classic-section-head\" class=\"box section\">\n                    <div class=\"box-options\">\n                        <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/not-classic-head.jpg\" alt=\"Cabe\u00e7a\">\n                        <h3>Cabe\u00e7a<\/h3>\n                        <ul class=\"section-links\">\n                            <li>\n                                <a href=\"#not-classic-head-01\">Baixa estatura<\/a>                               \n                            <\/li>\n                            <li>\n                                <a href=\"#not-classic-head-02\">Opacidade da C\u00f3rnea<\/a>\n                            <\/li>\n                            <li>\n                                <a href=\"#not-classic-head-03\">Mielopatia cervical &#8211; Patologia da coluna cervical<\/a>\n                            <\/li>                           \n                        <\/ul>                                       \n                    <\/div>\n                    \n                    <div id=\"not-classic-head-01\" class=\"box-information\">\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p><strong>Paciente do sexo feminino, 11 anos de idade.<\/strong><\/p>\n                                        <p>Pacientes com s\u00edndrome de <strong>Maroteaux-Lamy<\/strong> (MPS VI) n\u00e3o cl\u00e1ssica (atenuada ou lentamente progressiva) podem apresentar <strong>baixa estatura, s\u00edndrome do t\u00fanel do carpo e displasia do quadril.<\/strong><\/p>\n                                        <p><strong>Refer\u00eancia:<\/strong> 1. Th\u00fcmler A, Miebach E, Lampe C, Pitz S, Kamin W, Kampmann C, Link B, Mengel E. Clinical characteristics of adults with slowly progressing mucopolysaccharidosis VI: a case series. J Inherit Metab Dis. 2012 Nov;35(6):1071-9; 2. Pinto E Vairo F, Conboy E, de Souza CFM, Jones A, Barnett SS, Klee EW, Lanpher BC. Diagnosis of Attenuated Mucopolysaccharidosis VI: Clinical, Biochemical, and Genetic Pitfalls. Pediatrics. 2018 Dec;142(6):e20180658.<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/not-classic01.png\" alt=\"\">\n                                            <\/div>                                          \n                                        <\/figure>\n                                    <\/div>  \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                    <div id=\"not-classic-head-02\" class=\"box-information\">\n                        <div class=\"note\">\n                            <div class=\"note-title\">\n                                Nota importante\n                            <\/div>\n                            <div class=\"note-content\">\n                                <p>O dismorfismo facial pode ser leve ou ausente, a opacidade corneana \u00e9 mais t\u00edpica.<\/p>\n                                <p><strong>Refer\u00eancia<\/strong>: Filippo Pinto e Vairo, Erin Conboy, Carolina Fischinger Moura de Souza, Amie Jones, Sarah S. Barnett, Eric W. Klee, Brendan C. Lanpher; Diagnosis of Attenuated Mucopolysaccharidosis VI: Clinical, Biochemical, and Genetic Pitfalls. Pediatrics December 2018; 142 (6): e20180658. 10.1542\/peds.2018-0658<\/p>\n                            <\/div>\n                        <\/div>\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p>Paciente do sexo feminino com 11 anos e paciente do sexo masculino com 42 anos, ambos com MPS VI. Na paciente do sexo feminino, o primeiro sintoma foi opacidade corneana, que foi progressiva.<\/p>\n                                        <p>Imagem adaptada de Pinto E Vairo F, Conboy E, de Souza CFM, Jones A, Barnett SS, Klee EW, Lanpher BC. Diagnosis of Attenuated Mucopolysaccharidosis VI: Clinical, Biochemical, and Genetic Pitfalls. Pediatrics. 2018 Dec;142(6):e20180658.<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/not-classic02.jpg\" alt=\"\">\n                                            <\/div>\n                                        <\/figure>\n                                    <\/div>  \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                    <div id=\"not-classic-head-03\" class=\"box-information\">\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p><strong>Paciente com MPS VI n\u00e3o cl\u00e1ssica<\/strong><\/p>\n                                        <p>Radiografias laterais da coluna cervical <strong class=\"letter-featured letter-white\">A<\/strong> redu\u00e7\u00e3o da lordose cervical, <strong class=\"letter-featured letter-pink\">B<\/strong> estreitamento do corpo vertebral de C3 e luxa\u00e7\u00e3o do corpo vertebral de C4 (setas) e <strong class=\"letter-featured letter-orange\">C<\/strong> espessamento dos ossos do cr\u00e2nio.<\/p>\n                                        <p>Imagem adaptada de Jurecka A, Zakharova E, Malinova V, Voskoboeva E, Tylki-Szyma\u0144ska A. Attenuated osteoarticular phenotype of type VI mucopolysaccharidosis: a report of four patients and a review of the literature. Clin Rheumatol. 2014 May;33(5):725-31.<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/not-classic03.jpg\" alt=\"\">\n                                            <\/div>                                          \n                                        <\/figure>\n                                    <\/div>  \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                                                                            \n                <\/div>\n                \n                <div id=\"not-classic-section-torso\" class=\"box section\">\n                    <div class=\"box-options\">\n                        <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/not-classic-torso.jpg\" alt=\"Torso e membros\">\n                        <h3>Torso e membros<\/h3>\n                        <ul class=\"section-links\">\n                            <li>\n                                <a  href=\"#not-classic-torso-01\">Restri\u00e7\u00e3o dos arcos de movimento<\/a>\n                            <\/li>\n                            <li>\n                                <a href=\"#not-classic-torso-02\">Valvulopatia card\u00edaca<\/a>\n                            <\/li>\n                            <li>\n                                <a href=\"#not-classic-torso-03\">M\u00e3o em garra, contraturas e rigidez articular<\/a>\n                            <\/li>\n                        <\/ul>                                       \n                    <\/div>\n                    \n                    <div id=\"not-classic-torso-01\" class=\"box-information\">\n                        <div class=\"note\">\n                            <div class=\"note-title\">\n                                Nota importante\n                            <\/div>\n                            <div class=\"note-content\">\n                                <p>A idade de in\u00edcio do primeiro sintoma na MPS VI n\u00e3o cl\u00e1ssica varia de 5 a 8 anos (mediana de 8 anos). O primeiro sinal mais comum que apareceu foi a diminui\u00e7\u00e3o da amplitude de movimento articular das grandes articula\u00e7\u00f5es dos membros superiores e\/ou inferiores. As articula\u00e7\u00f5es mais frequentemente afetadas s\u00e3o os quadris, o que resultou em dor no quadril com dificuldade para andar e subir escadas. Os primeiros erros diagn\u00f3sticos nesses pacientes foram doen\u00e7a de Perthes, displasia coxofemoral ou osteocondrose.<\/p>\n                                <p><strong>Refer\u00eancia:<\/strong> Jurecka A, Zakharova E, Malinova V, Voskoboeva E, Tylki-Szyma\u0144ska A. Attenuated osteoarticular phenotype of type VI mucopolysaccharidosis: a report of four patients and a review of the literature. Clin Rheumatol. 2014 May;33(5):725-31. V\u00eddeo com direitos de utiliza\u00e7\u00e3o atribu\u00eddos a BioMarin <\/p>\n                            <\/div>\n                        <\/div>                      \n                        <div class=\"wrapper\">\n                            <div class=\"inner-wrapper\">\n                                <div class=\"video-poster\">          \n                                    <a class=\"modal-video\" href=\"https:\/\/player.vimeo.com\/video\/1019991651\" style=\"background-image: url(https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/not_classic_torso_video_thumbnail.png)\">Play<\/a>\n                                <\/div>  \n                            <\/div>\n                        <\/div>                      \n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                    <div id=\"not-classic-torso-02\" class=\"box-information\">\n                        <div class=\"note\">\n                            <div class=\"note-title\">\n                                Nota importante\n                            <\/div>\n                            <div class=\"note-content\">\n                                <p>A principal manifesta\u00e7\u00e3o card\u00edaca da MPS VI \u00e9 a degenera\u00e7\u00e3o valvar progressiva com insufici\u00eancia, estenose ou ambas (especialmente da v\u00e1lvula mitral, embora todas as v\u00e1lvulas possam ser afetadas). Dispneia, ortopneia ou dispneia parox\u00edstica noturna com doen\u00e7a valvar com\/sem outras caracter\u00edsticas de MPS, devem sempre levantar a suspeita de MPS, especialmente na presen\u00e7a de sintomas adicionais.<\/p>\n                            <\/div>\n                        <\/div>\n                        <div class=\"split-content mps-interactivo-split-content-carousel content-align-top\">\n                            <div class=\"wrapper\">\n                                <div class=\"owl-carousel mps-interactivo-split-content-carousel-slides\">\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">                                         \n                                           <p>Ecocardiograma e Doppler das v\u00e1lvulas mitral <strong class=\"letter-featured letter-orange\">A<\/strong> e a\u00f3rtica <strong class=\"letter-featured letter-pink\">B<\/strong> em homem de 50 anos com MPS VI A. <strong class=\"letter-featured letter-orange\">A<\/strong> Vis\u00e3o eixo curto da v\u00e1lvula mitral espessada (seta) em di\u00e1stole (esquerda). Demonstra\u00e7\u00e3o com Doppler colorido de insufici\u00eancia mitral (direita). <strong class=\"letter-featured letter-pink\">B<\/strong> Corte eixo curto mostrando espessamento da v\u00e1lvula a\u00f3rtica trivalva (seta) em s\u00edstole (esquerda). Doppler colorido demonstrando fluxo sist\u00f3lico turbulento de estenose valvar a\u00f3rtica (direita).<\/p>\n                                            <p>Refer\u00eancia: Adaptado de Braunlin EA, Harmatz PR, Scarpa M, et al. Cardiac disease in patients with mucopolysaccharidosis: presentation, diagnosis and management. J Inherit Metab Dis. 2011 Dec;34(6):1183-97.<\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/not-classic05.jpg\" alt=\"\">\n                                                <\/div>                                          \n                                            <\/figure>\n                                        <\/div>\n                                    <\/div>\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">                                         \n                                            <p>Aut\u00f3psia de paciente de 27 anos com MPS VI. Sec\u00e7\u00e3o transversal do cora\u00e7\u00e3o. Na foto: v\u00e1lvulas mitral e tric\u00faspide est\u00e3o deformadas, espessadas e fibr\u00f3ticas, fibrose excessiva do endoc\u00e1rdio em ambos os \u00e1trios e ventr\u00edculos, hipertrofia exc\u00eantrica dos \u00e1trios e hipertrofia conc\u00eantrica dos ventr\u00edculos com fibrose do mioc\u00e1rdio.<\/p>\n                                            <p><strong>Refer\u00eancia<\/strong>: Golda A, Jurecka A, Opoka-Winiarska V, Tylki-Szymanska A. Mucopolysaccharidosis type VI: a cardiologist&#8217;s guide to diagnosis and treatment. Int J Cardiol. 2013 Jul 15;167(1):1-10.<\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/not-classic04.jpg\" alt=\"\">\n                                                <\/div>                                              \n                                            <\/figure>\n                                        <\/div>\n                                    <\/div>\n                                    \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                    <div id=\"not-classic-torso-03\" class=\"box-information\">\n                        <div class=\"note\">\n                            <div class=\"note-title\">\n                                Nota importante\n                            <\/div>\n                            <div class=\"note-content\">\n                                <p>A rigidez articular ocorre principalmente nas articula\u00e7\u00f5es do ombro e quadril ou outras grandes articula\u00e7\u00f5es dos membros superiores e\/ou inferiores.Pacientes com MPS VI n\u00e3o cl\u00e1ssica frequentemente apresentam s\u00edndrome do t\u00fanel do carpo, \u00e0s vezes necessitando de cirurgia de descompress\u00e3o. <strong>Refer\u00eancia:<\/strong> Th\u00fcmler A, Miebach E, Lampe C, Pitz S, Kamin W, Kampmann C, Link B, Mengel E. Clinical characteristics of adults with slowly progressing mucopolysaccharidosis VI: a case series. J Inherit Metab Dis. 2012 Nov;35(6):1071-9.<\/p>\n                            <\/div>\n                        <\/div>\n                        \n                        <div class=\"split-content mps-interactivo-split-content-carousel content-align-top\">\n                            <div class=\"wrapper\">\n                                <div class=\"owl-carousel mps-interactivo-split-content-carousel-slides\">\n                                        \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">\n                                            <p>Radiografia dorsopalmar de paciente do <strong>sexo feminino, 9 anos de idade,<\/strong> com diagn\u00f3stico confirmado de <strong>MPS VI.<\/strong> A radiografia revela <strong>anormalidades esquel\u00e9ticas<\/strong> sutis na m\u00e3o, por isso \u00e9 sempre necess\u00e1rio <strong>acompanhar<\/strong> com outros exames radiogr\u00e1ficos.<\/p>\n                                            <p>Imagem adaptada de Choy YS, Bhattacharya K, Balasubramaniam S, et al. Identifying the need for a multidisciplinary approach for early recognition of mucopolysaccharidosis VI (MPS VI). Mol Genet Metab. 2015 May;115(1):41-7.<\/p>\n                                            <p><\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/not-classic06.jpg\" alt=\"\">\n                                                <\/div>\n                                            <\/figure>\n                                        <\/div>  \n                                    <\/div>\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">\n                                            <p>Imagens de pacientes do sexo feminino, ambos com <strong>11 anos de idade<\/strong> com <strong>MPS VI<\/storng> n\u00e3o cl\u00e1ssica<\/strong><\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/not-classic07.jpg\" alt=\"\">\n                                                <\/div>\n                                                <figcaption>Imagens adaptadas de Pinto E Vairo F, Conboy E, de Souza CFM, Jones A, Barnett SS, Klee EW, Lanpher BC. Diagnosis of Attenuated Mucopolysaccharidosis VI: Clinical, Biochemical, and Genetic Pitfalls. Pediatrics. 2018 Dec;142(6):e20180658.<\/figcaption>\n                                            <\/figure>\n                                        <\/div>  \n                                    <\/div>\n                                    \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n\n                <\/div>\n                \n                <div id=\"not-classic-section-legs\" class=\"box section\">\n                    <div class=\"box-options\">\n                        <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/not-classic-legs.jpg\" alt=\"Pernas e p\u00e9\">\n                        <h3>Pernas e p\u00e9<\/h3>\n                        <ul class=\"section-links\">\n                            <li>\n                                <a href=\"#not-classic-legs-01\">Dor e displasia no quadril<\/a>\n                            <\/li>\n                            <li>\n                                <a href=\"#not-classic-legs-02\">Dor no p\u00e9<\/a>\n                            <\/li>                           \n                        <\/ul>                                       \n                    <\/div>  \n                    \n                    <div id=\"not-classic-legs-01\" class=\"box-information\">\n                        <div class=\"note\">\n                            <div class=\"note-title\">\n                                Nota importante\n                            <\/div>\n                            <div class=\"note-content\">\n                                <p>A dor no quadril \u00e9 o primeiro sintoma manifestado nesses pacientes. A maioria dos pacientes adultos desenvolver\u00e1 displasia coxofemoral com consequentes complica\u00e7\u00f5es artr\u00edticas e limita\u00e7\u00e3o da mobilidade.<\/p>\n                                <p><strong>Refer\u00eancia:<\/strong> 1. Pinto E Vairo F, Conboy E, de Souza CFM, Jones A, Barnett SS, Klee EW, Lanpher BC. Diagnosis of Attenuated Mucopolysaccharidosis VI: Clinical, Biochemical, and Genetic Pitfalls. Pediatrics. 2018 Dec;142(6):e20180658; 2. Th\u00fcmler A, Miebach E, Lampe C, Pitz S, Kamin W, Kampmann C, Link B, Mengel E. Clinical characteristics of adults with slowly progressing mucopolysaccharidosis VI: a case series. J Inherit Metab Dis. 2012 Nov;35(6):1071-9.<\/p>\n                            <\/div>\n                        <\/div>\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p>Radiografias em AP da pelve de <strong class=\"letter-featured letter-orange\">A<\/strong> paciente do sexo feminino com 11 anos de idade e <strong class=\"letter-featured letter-pink\">B<\/strong> paciente do sexo masculino com 42 anos de idade, ambos com MPS VI.<\/p>\n                                        <p><strong class=\"letter-featured letter-orange\">A<\/strong> A radiografia do primeiro paciente revela displasia coxofemoral bilateral. A segunda radiografia <strong class=\"letter-featured letter-pink\">B<\/strong> do paciente masculino revela aumento da ep\u00edfise do f\u00eamur esquerdo e artrite degenerativa moderadamente avan\u00e7ada. Este paciente tinha diagn\u00f3stico inicial de doen\u00e7a de Legg-Calv\u00e9-Perthes, que necessitou de artroplastia de quadril. A dor no quadril \u00e9 o primeiro sintoma manifestado nesses pacientes. A maioria dos pacientes adultos desenvolver\u00e1 displasia coxofemoral com consequentes complica\u00e7\u00f5es artr\u00edticas e limita\u00e7\u00e3o da mobilidade.<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/not-classic08.jpg\" alt=\"\">\n                                            <\/div>\n                                            <figcaption>Imagen adaptada de: <a href=\"https:\/\/doi.org\/10.1542\/peds.2018-0658\" target=\"_blank\">https:\/\/doi.org\/10.1542\/peds.2018-0658<\/a><\/figcaption>                                            \n                                        <\/figure>\n                                    <\/div>  \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>  \n                    \n                    <div id=\"not-classic-legs-02\" class=\"box-information\">\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p>Fotografias cl\u00ednicas e radiografias de um paciente do sexo masculino, 26 anos, portador de MPS VI.<\/p>\n                                        <p>Contratura fixa em equino, causada por espasticidade gastro-solar secund\u00e1ria \u00e0 compress\u00e3o medular ao n\u00edvel do cone medular. Radiografias do p\u00e9 e tornozelo esquerdos revelam displasia epifis\u00e1ria tibial distal com acunhamento em valgo, metatarsos curtos em forma de &#8220;bala&#8221; e cuneiforme hipopl\u00e1sico.<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/not-classic09.jpg\" alt=\"\">\n                                            <\/div>\n                                            <figcaption>Refer\u00eancia: Adaptado de Stepien KM, Bentley A, Chen C, et al.. Non-cardiac Manifestations in Adult Patients With Mucopolysaccharidosis. Front Cardiovasc Med. 2022 Mar 7;9:839391.<\/figcaption>                                            \n                                        <\/figure>\n                                    <\/div>  \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>                                  \n                    \n                <\/div>\n                \n                <p><a class=\"button button-text button-arrow button-arrow-left mps-interactivo-back\" href=\"#\">Voltar<\/a><\/p>\n            <\/div>  \n        <\/div>  \n    <\/div>\n    \n    <div id=\"mps-interactivo-section-classic\" class=\"mps-interactivo-section\">\n        <div class=\"wrapper\">\n            <div class=\"section-background\"><\/div>  \n            \n            <div class=\"section-hotspots\">\n                <div class=\"area area-head\">\n                    <a class=\"hotspot\" href=\"#classic-section-head\">\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                    <\/a>\n                <\/div>\n                <div class=\"area area-torso\">\n                    <a class=\"hotspot\" href=\"#classic-section-torso\">\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                    <\/a>\n                <\/div>\n                <div class=\"area area-legs\">\n                    <a class=\"hotspot\" href=\"#classic-section-legs\">\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                        <div><\/div>\n                    <\/a>\n                <\/div>\n            <\/div>                      \n            \n            <div class=\"main-content scrolling-container\">\n                <h2>Paciente MPS IVA <span>Cl\u00e1ssico<\/span><\/h2>                     \n                                \n                <div id=\"classic-section-head\" class=\"box section\">\n                    <div class=\"box-options\">\n                        <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic-head.jpg\" alt=\"Cabe\u00e7a\">\n                        <h3>Cabe\u00e7a<\/h3>\n                        <ul class=\"section-links\">\n                            <li>\n                                <a href=\"#classic-head-01\">Baixa estutura<\/a>                               \n                            <\/li>\n                            <li>\n                                <a href=\"#classic-head-02\">Opacidade da C\u00f3rnea<\/a>\n                            <\/li>\n                            <li>\n                                <a href=\"#classic-head-03\">Pesco\u00e7o curto<\/a>\n                            <\/li>\n                            <li>\n                                <a href=\"#classic-head-04\">Compress\u00e3o de medula espinhal<\/a>\n                            <\/li>\n                        <\/ul>                                       \n                    <\/div>\n                    \n                    <div id=\"classic-head-01\" class=\"box-information\">\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p>Pacientes com s\u00edndrome de Morquio de fen\u00f3tipo cl\u00e1ssico geralmente t\u00eam estatura muito baixa. Embora o crescimento seja tipicamente normal durante a inf\u00e2ncia, a velocidade de crescimento come\u00e7a a declinar frequentemente ap\u00f3s um ano de idade. Aos 3 anos de idade, a baixa estatura \u00e9 evidente. No fen\u00f3tipo n\u00e3o cl\u00e1ssico, a altura dos pacientes permanece dentro dos limites normais at\u00e9 o estir\u00e3o de crescimento pr\u00e9-adolescente, quando esse d\u00e9ficit de crescimento se torna evidente.<\/p>\n                                        <p>Refer\u00eancia: White KK, Jester A, Bache CE, Harmatz PR, Shediac R, Thacker MM, Mackenzie WG. Orthopedic management of the extremities in patients with Morquio A syndrome. J Child Orthop. 2014 Aug;8(4):295-304<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic01.jpg\" alt=\"\">\n                                            <\/div>\n                                            <figcaption>Fen\u00f3tipo cl\u00e1ssico e n\u00e3o cl\u00e1ssico de pacientes do sexo feminino com MPS IVA<\/figcaption>\n                                        <\/figure>\n                                    <\/div>  \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                    <div id=\"classic-head-02\" class=\"box-information\">\n                        <div class=\"note\">\n                            <div class=\"note-title\">\n                                Nota importante\n                            <\/div>\n                            <div class=\"note-content\">\n                                <p>As manifesta\u00e7\u00f5es oculares est\u00e3o presentes em todos os tipos de MPS, mas s\u00e3o particularmente comuns nas MPS I, VI e VII. Refer\u00eancia: Del Longo et al. Italian Journal of Pediatrics 2018, 44(Suppl 2):125; https:\/\/doi.org\/10.1186\/s13052-018-0559-9<\/p>\n                            <\/div>\n                        <\/div>\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p>A opacidade corneana \u00e9 causada pelo ac\u00famulo de gr\u00e2nulos amarelo-acinzentados formados por glicosaminoglicanos (GAGs) que se depositam em todas as camadas da c\u00f3rnea. O ac\u00famulo excessivo de GAGs na c\u00f3rnea afeta o tamanho dos querat\u00f3citos, comprometendo a integridade da rede de fibrilas col\u00e1genas no estroma, causando opacidade corneana. A opacifica\u00e7\u00e3o da c\u00f3rnea \u00e9 difusa e lentamente progressiva, com consequente perda da acuidade visual.<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <div class=\"video-poster\">          \n                                            <a class=\"modal-video\" href=\"https:\/\/player.vimeo.com\/video\/1019991832\" style=\"background-image: url(https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic_head_video_thumbnail.png)\">Play<\/a>\n                                        <\/div>\n                                        <p class=\"small\">Paciente do sexo feminino com fen\u00f3tipo cl\u00e1ssico MPS IVA com discreta opacidade corneana<\/p>\n                                    <\/div>  \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                    <div id=\"classic-head-03\" class=\"box-information\">\n                        <div class=\"split-content mps-interactivo-split-content-carousel content-align-top\">\n                            <div class=\"wrapper\">\n                                <div class=\"owl-carousel mps-interactivo-split-content-carousel-slides\">\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">\n                                            <p>Manifesta\u00e7\u00f5es cl\u00ednicas comuns que aumentam o risco anest\u00e9sico em pacientes com MPS<\/p>\n                                            <p>Imagem adaptada de Moretto, A., Bosatra, M.G., Marchesini, L. <em>et al.<\/em> Anesthesiological risks in mucopolysaccharidoses. <em>Ital J Pediatr<\/em> 44 (Suppl 2), 116 (2018). https:\/\/doi.org\/10.1186\/s13052-018-0554-1<\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic02.jpg\" alt=\"\">\n                                                <\/div>\n                                            <\/figure>\n                                        <\/div>\n                                    <\/div>\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">\n                                            <p>Manifesta\u00e7\u00f5es cl\u00ednicas comuns que aumentam o risco anest\u00e9sico em pacientes com MPS<\/p>\n                                            <p>Imagem adaptada de Moretto, A., Bosatra, M.G., Marchesini, L. et al. Anesthesiological risks in mucopolysaccharidoses. Ital J Pediatr 44 (Suppl 2), 116 (2018).<\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic03.jpg\" alt=\"\">\n                                                <\/div>\n                                            <\/figure>\n                                        <\/div>\n                                    <\/div>\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">\n                                            <p>Broncoscopia em paciente com MPS demonstrando infiltra\u00e7\u00e3o de glicosaminoglicanos (GAGs) nas vias a\u00e9reas superiores, bem como estreitamento traqueal.<\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <div class=\"video-poster\">\n                                                <a class=\"modal-video\" href=\"https:\/\/player.vimeo.com\/video\/1019991904\" style=\"background-image: url(https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic_head_02_video_thumbnail.png)\">Play<\/a>\n                                            <\/div>\n                                            <p class=\"small\">V\u00eddeo com direitos de uso cedidos \u00e0 BioMarin<\/p>\n                                        <\/div>\n                                    <\/div>\n                                    \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                    <div id=\"classic-head-04\" class=\"box-information\">\n                        <div class=\"note\">\n                            <div class=\"note-title\">\n                                Nota importante\n                            <\/div>\n                            <div class=\"note-content\">\n                                <p>Hipoplasia do odontoide e instabilidade da coluna cervical foram documentadas em 65% e 49% dos pacientes com MPS IVA, respectivamente. A cirurgia profil\u00e1tica de descompress\u00e3o e fus\u00e3o occipitocervical ou C1-C2 para estabilizar a coluna cervical superior \u00e9 considerada uma interven\u00e7\u00e3o que salva vidas em casos graves, particularmente antes dos 4 anos de idade. A cirurgia de descompress\u00e3o espinhal \u00e9 necess\u00e1ria em 20% dos pacientes com S\u00edndrome de Morquio (MPS IVA).<\/p>\n                                <p>Refer\u00eancia: Padash, S., Obaid, H., Henderson, R.D.E. et al. A pictorial review of the radiographic skeletal findings in Morquio syndrome (mucopolysaccharidosis type IV). Pediatr Radiol (2023). https:\/\/doi.org\/10.1007\/s00247-022-05585-3<\/p>\n                            <\/div>\n                        <\/div>\n                        <div class=\"split-content mps-interactivo-split-content-carousel content-align-top\">\n                            <div class=\"wrapper\">\n                                <div class=\"owl-carousel mps-interactivo-split-content-carousel-slides\">\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">\n                                            <p>Radiografia lateral do pesco\u00e7o em flexo-extens\u00e3o em paciente de 3 anos de idade com <strong>MPS VI<\/strong><\/p>\n                                            <p>Paciente com mielopatia cl\u00ednica e achados de imagem com instabilidade cervical, estenose e mielomal\u00e1cia. A radiografia mostra instabilidade atlanto-axial com <strong class=\"letter-featured letter-white\">A<\/strong> estreitamento do canal \u00f3sseo espinhal de 17 mm durante a extens\u00e3o para 10 mm durante a flex\u00e3o. As medidas s\u00e3o feitas no canal vertebral, e n\u00e3o no espa\u00e7o pr\u00e9-dental.<\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic04.jpg\" alt=\"\">\n                                                <\/div>  \n                                                <figcaption>Imagens adaptadas de G.A. Solanki et al. Molecular Genetics and Metabolism 118 (2016) 310\u2013318. http:\/\/dx.doi.org\/10.1016\/j.ymgme.2016.06.001<\/figcaption>\n                                            <\/figure>\n                                        <\/div>\n                                    <\/div>\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">\n                                            <p>Radiografia lateral da coluna cervical de uma paciente de 6 anos de idade com MPS IVA <\/p>\n                                            <p><strong class=\"letter-featured letter-pink\">B<\/strong> Hipoplasia dos odontoides (linha tracejada enfatiza que o odontoide n\u00e3o se estende superiormente dentro do anel C1); <\/p>\n                                            <p><strong class=\"letter-featured letter-orange\">C<\/strong> Platispondilia (achatamento vertebral) indicada pelas setas duplas e subluxa\u00e7\u00e3o anterior de C7 em T1 (seta indica transla\u00e7\u00e3o anterior). <\/p>\n                                            <p>Imagem adaptada de Regier DS, Oetgen M, Tanpaiboon P. Mucopolysaccharidosis Type IVA. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Amemiya A, eds. GeneReviews\u00ae. Seattle (WA): University of Washington, Seattle; July 11, 2013.<\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic05.jpg\" alt=\"\">\n                                                <\/div>                                              \n                                            <\/figure>\n                                        <\/div>\n                                    <\/div>                                                                      \n                                    \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                <\/div>\n                \n                <div id=\"classic-section-torso\" class=\"box section\">\n                    <div class=\"box-options\">\n                        <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic-torso.jpg\" alt=\"Torso e membros\">\n                        <h3>Torso e membros<\/h3>\n                        <ul class=\"section-links\">\n                            <li>\n                                <a  href=\"#classic-torso-01\">Pectus Carinatum<\/a>\n                            <\/li>\n                            <li>\n                                <a href=\"#classic-torso-02\">Cifoescoliose<\/a>\n                            <\/li>\n                            <li>\n                                <a href=\"#classic-torso-03\">H\u00e9rnias umbilicais e\/ou inguinais<\/a>\n                            <\/li>\n                            <li>\n                                <a href=\"#classic-torso-04\">Hipermobilidade articular<\/a>\n                            <\/li>\n                        <\/ul>                                       \n                    <\/div>\n                    \n                    <div id=\"classic-torso-01\" class=\"box-information\">\n                        <div class=\"note\">\n                            <div class=\"note-title\">\n                                Nota importante\n                            <\/div>\n                            <div class=\"note-content\">\n                                <p>Em pacientes com S\u00edndrome de Morquio (MPS IVA) o pectus carinatum \u00e9 uma caracter\u00edstica dominante, presente em 97% dos casos, embora casos com pectus excavatum tamb\u00e9m tenham sido relatados<\/p> \n                                <p>Refer\u00eancia: Padash, S., Obaid, H., Henderson, R.D.E. et al. A pictorial review of the radiographic skeletal findings in Morquio syndrome (mucopolysaccharidosis type IV). Pediatr Radiol (2023). https:\/\/doi.org\/10.1007\/s00247-022-05585-3<\/p>\n                            <\/div>\n                        <\/div>\n                        <div class=\"split-content mps-interactivo-split-content-carousel content-align-top\">\n                            <div class=\"wrapper\">\n                                <div class=\"owl-carousel mps-interactivo-split-content-carousel-slides\">\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">\n                                            <p>Radiografia simples de t\u00f3rax anteroposterior comparativa: <strong class=\"letter-featured letter-black\">a<\/strong> Radiografia normal de paciente do sexo feminino com 10 anos de idade. <strong class=\"letter-featured letter-black\">b c<\/strong> Radiografia de t\u00f3rax AP de paciente do sexo feminino, 10 anos de idade, portadora de MPS IV.<\/p>\n                                            <p>A radiografia <strong class=\"letter-featured letter-black\">b<\/strong> mostra <strong class=\"letter-featured letter-white\">A<\/strong> costelas em forma de remo, proximalmente c\u00f4nicas e distalmente mais largas (cabe\u00e7as de setas brancas) e <strong class=\"letter-featured letter-pink\">B<\/strong> escoliose \u00f3bvia. Raio-X com vis\u00e3o ampliada das clav\u00edculas, evid\u00eancia <strong class=\"letter-featured letter-orange\">C<\/strong>  clav\u00edculas largas e curtas (seta branca).<\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic06.jpg\" alt=\"\">\n                                                <\/div>\n                                                <figcaption>Imagens adaptadas de: Palmucci, S., Attin\u00e0, G., Lanza, M.L. et al. Imaging findings of mucopolysaccharidoses: a pictorial review. Insights Imaging 4, 443\u2013459 (2013). https:\/\/doi.org\/10.1007\/s13244-013-0246-8<\/figcaption>                                           \n                                            <\/figure>\n                                        <\/div>\n                                    <\/div>\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">                                         \n                                            <p>Paciente do sexo masculino com MPS IVA com incapacidade de elevar os bra\u00e7os acima da cabe\u00e7a e estender totalmente os cotovelos, pectus carinatum e pesco\u00e7o curto.<\/p>\n                                            <p>Imagem adapatada de Colmenares-Bonilla D, Esquitin-Gardu\u00f1o N. Diagnosis of Morquio-A patients in Mexico: How far are we from prompt diagnosis?. <em>Intractable Rare Dis Res.<\/em> 2017;6(2):119-123. doi:10.5582\/irdr.2017.01016<\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic07.jpg\" alt=\"\">\n                                                <\/div>                                              \n                                            <\/figure>\n                                        <\/div>\n                                    <\/div>                                                                      \n                                    \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                    <div id=\"classic-torso-02\" class=\"box-information\">\n                        <div class=\"note\">\n                            <div class=\"note-title\">\n                                Nota importante\n                            <\/div>\n                            <div class=\"note-content\">\n                                <p>A cifose \u00e9 uma das caracter\u00edsticas mais comuns na S\u00edndrome de Morquio, ocorrendo em 85% dos casos e muitas vezes pode ser evidente desde os primeiros 6 meses de vida.<\/p>\n                                <p>Refer\u00eancia: Padash, S., Obaid, H., Henderson, R.D.E. et al. Pediatr Radiol (2023). https:\/\/doi.org\/10.1007\/s00247-022-05585-3, Pediatr J Child Orthop (2014) 8:295\u2013304 DOI 10.1007\/s11832-014-0601-4.<\/p>\n                            <\/div>\n                        <\/div>\n                        <div class=\"split-content mps-interactivo-split-content-carousel content-align-top\">\n                            <div class=\"wrapper\">\n                                <div class=\"owl-carousel mps-interactivo-split-content-carousel-slides\">\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">                                         \n                                            <p>Radiografia lateral e em AP da coluna vertebral em pacientes com <strong>MPS IVA.<\/strong><\/p>\n                                            <p>Radiografia lateral esquerda: revela <strong class=\"letter-featured letter-white\">A<\/strong> bico anterior (imagem de proje\u00e7\u00e3o anterior da l\u00edngua\/incisura anterossuperior)<\/p>\n                                            <p><strong class=\"letter-featured letter-pink\">B<\/strong> <strong>Platispondilia<\/strong> (achatamento dos corpos vertebrais)<\/p> <p><strong class=\"letter-featured letter-orange\">C<\/strong> <strong>Irregularidade<\/strong> na plataforma do corpo vertebral<\/p> <p><strong class=\"letter-featured letter-red\">D<\/strong> <strong>Alargamento das costelas<\/strong> (costelas em forma de remo)<\/p>\n                                            <p><strong class=\"letter-featured letter-blue\">E<\/strong> <strong>Hiperlordose lombar.<\/strong><\/p>     \n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic08.jpg\" alt=\"\">\n                                                <\/div>  \n                                                <figcaption>Imagens cortesia de Dr. Hugo Flores<\/figcaption>                                            \n                                            <\/figure>\n                                        <\/div>\n                                    <\/div>\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">\n                                            <p>Revela uma morfologia anormal das v\u00e9rtebras na jun\u00e7\u00e3o toracolombar, mais proeminente em L1 e L2 <strong class=\"letter-featured letter-white\">A<\/strong> associada \u00e0 hipercifose na faceta da jun\u00e7\u00e3o toracolombar <strong class=\"letter-featured letter-red\">F<\/strong> ; desloca\u00e7\u00e3o facet\u00e1ria em T12, L1-L2 e L2-L3, e espondilolistese de T12 e L2. \u00c9 visto um bico anterior dos corpos vertebrais em L1 e L2 (pontas de flecha), semelhante a uma l\u00edngua ou bala. <strong class=\"letter-featured letter-blue\">E<\/strong> Hiperlordose lombar.<\/p>\n                                            <p>Imagem adaptada de Landen, Michiel &#038; Eyskens, Francois &#038; Vanhoenacker, Filip. (2021). Vertebral Tongue-Like Deformity in Mucopolysaccharidosis VI. Journal of the Belgian Society of Radiology. 105. 10.5334\/jbsr.2611.<\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic09.jpg\" alt=\"\">\n                                                <\/div>                                              \n                                            <\/figure>\n                                        <\/div>\n                                    <\/div>\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">                                         \n                                            <p>Paciente com <strong>MPS IVA<\/strong> demonstrando <strong>cifoescoliose<\/strong><\/p>\n                                            <p>Imagem adaptada de Frigeni, M., Rodriguez-Buritica, D. F., Saavedra, H., Gunther, K. A., Hillman, P. R., Balaguru, D., &#038; Northrup, H. (2021). The youngest pair of siblings with Mucopolysaccharidosis type IVA to receive enzyme replacement therapy to date: A case report. American Journal of Medical Genetics Part A, 185A: 3510\u20133516. https:\/\/doi.org\/10.1002\/ajmg.a.62469<\/p>                                        \n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic10.jpg\" alt=\"\">\n                                                <\/div>                                              \n                                            <\/figure>\n                                        <\/div>\n                                    <\/div>                                                                      \n                                    \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                    <div id=\"classic-torso-03\" class=\"box-information\">\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p>Paciente com MPS IVA com pectus carinatum e h\u00e9rnia umbilical<\/p>\n                                        <p>Na maioria das MPS, as h\u00e9rnias aparecem frequentemente nos primeiros 6 meses de vida, juntamente com infec\u00e7\u00f5es respirat\u00f3rias anormalmente frequentes, otites e organomegalias.<\/p>\n                                        <p>Imagem adaptada de Frigeni, M., Rodriguez-Buritica, D. F., Saavedra, H., Gunther, K. A., Hillman, P. R., Balaguru, D., &#038; Northrup, H. (2021). The youngest pair of siblings with Mucopolysaccharidosis type IVA to receive enzyme replacement therapy to date: A case report. American Journal of Medical Genetics Part A, 185A: 3510\u20133516. https:\/\/doi.org\/10.1002\/ajmg.a.62469<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic11.jpg\" alt=\"\">\n                                            <\/div>                                              \n                                        <\/figure>\n                                    <\/div>  \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                    <div id=\"classic-torso-04\" class=\"box-information\">                     \n                        <div class=\"split-content mps-interactivo-split-content-carousel content-align-top\">\n                            <div class=\"wrapper\">\n                                <div class=\"owl-carousel mps-interactivo-split-content-carousel-slides\">\n                                    \n                                    <div class=\"inner-wrapper\">                                     \n                                        <div class=\"content-block\">\n                                            <div class=\"note\">\n                                                <div class=\"note-title\">\n                                                    Nota importante\n                                                <\/div>\n                                                <div class=\"note-content\">\n                                                    <p>As m\u00e3os s\u00e3o frequentemente comprometidas com deteriora\u00e7\u00e3o gradual da for\u00e7a de preens\u00e3o, estabilidade do punho e habilidades motoras finas, levando a um comprometimento funcional significativo.<\/p>\n                                                    <p><strong>Refer\u00eancia:<\/strong> White KK, Jester A, Bache CE, et al. Orthopedic management of the extremities in patients with Morquio A syndrome. J Child Orthop. 2014 Aug;8(4):295-304<\/p>\n                                                <\/div>\n                                            <\/div>\n                                            <p>Radiografia dorso-palmar de paciente com MPS IVA. A radiografa mostra <strong class=\"letter-featured letter-white\">A<\/strong> falanges distais curtas (em forma de bala), <strong class=\"letter-featured letter-pink\">B<\/strong> metacarpos curtos com estreitamento proximal e hipoplasia do carpo <strong class=\"letter-featured letter-orange\">C<\/strong>. Al\u00e9m disso, as extremidades radial distal e ulnar s\u00e3o irregulares e inclinados uma para a outra <strong class=\"letter-featured letter-red\">D<\/strong>.<\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic12.jpg\" alt=\"\">\n                                                <\/div>  \n                                                <figcaption>Imagem cortesia do Dr. Hugo Flores<\/figcaption>                                         \n                                            <\/figure>\n                                        <\/div>\n                                    <\/div>\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <div class=\"content-block\">                                         \n                                            <p>O exame cl\u00ednico das m\u00e3os em pacientes com MPS IVA frequentemente revela hipermobilidade da articula\u00e7\u00e3o do punho com seu desvio ulnar caracter\u00edstico que afeta ativa e passivamente a amplitude de movimento. Os pacientes tipicamente apresentam diferen\u00e7as significativas nas amplitudes de movimento ativa e passiva, refletindo a perda da estabilidade dessa articula\u00e7\u00e3o. O resultado da hipermobilidade \u00e9 a redu\u00e7\u00e3o da for\u00e7a do punho e das articula\u00e7\u00f5es metacarpofalangeanas e interfalangeanas proximais. Os pacientes t\u00eam uma diminui\u00e7\u00e3o pronunciada na for\u00e7a de preens\u00e3o e pin\u00e7a e t\u00eam dificuldade em desenvolver suas atividades de vida di\u00e1ria, exigindo for\u00e7a para abrir uma garrafa, carregar objetos e at\u00e9 mesmo para o uso de garfo e faca por exemplo.<\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <div class=\"video-poster\">          \n                                                <a class=\"modal-video\" href=\"https:\/\/player.vimeo.com\/video\/1019991578\" style=\"background-image: url(https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic_torso_video_thumbnail.png)\">Play<\/a>\n                                            <\/div>\n                                            <p class=\"small\">Paciente com fen\u00f3tipo cl\u00e1ssico MPS IVA com hipermobilidade articular<\/p>\n                                        <\/div>\n                                    <\/div>\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic13.jpg\" alt=\"\">\n                                            <\/div>  \n                                            <figcaption>Paciente com fen\u00f3tipo cl\u00e1ssico MPS IVA com frouxid\u00e3o dos ligamentos<\/figcaption>           \n                                        <\/figure>\n                                    <\/div>                                                                      \n                                    \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                <\/div>\n                \n                <div id=\"classic-section-legs\" class=\"box section\">\n                    <div class=\"box-options\">\n                        <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic-legs.jpg\" alt=\"Pernas e p\u00e9\">\n                        <h3>Pernas e p\u00e9<\/h3>\n                        <ul class=\"section-links\">\n                            <li>\n                                <a href=\"#classic-legs-01\">Displasia no quadril<\/a>\n                            <\/li>\n                            <li>\n                                <a href=\"#classic-legs-02\">Geno valgo<\/a>\n                            <\/li>\n                            <li>\n                                <a href=\"#classic-legs-03\">Dist\u00farbio da marcha<\/a>\n                            <\/li>\n                        <\/ul>                                       \n                    <\/div>\n                    \n                    <div id=\"classic-legs-01\" class=\"box-information\">\n                        <div class=\"note\">\n                            <div class=\"note-title\">\n                                Nota importante\n                            <\/div>\n                            <div class=\"note-content\">\n                                <p>Displasia acetabular ocorre em 70% dos pacientes com MPS IVA. As articula\u00e7\u00f5es do quadril podem desenvolver uma amplitude de movimento restrita devido \u00e0 osteoartrite de in\u00edcio precoce, os pacientes frequentemente t\u00eam uma incapacidade de andar. 5,5% dos pacientes com s\u00edndrome de Morquio necessitam de cirurgia de quadril em torno de 8,4 \u00b1 3,6 anos de idade.<\/p>\n                                <p>Refer\u00eancia: Padash, S., Obaid, H., Henderson, R.D.E. et al. A pictorial review of the radiographic skeletal findings in Morquio syndrome (mucopolysaccharidosis type IV). Pediatr Radiol (2023). https:\/\/doi.org\/10.1007\/s00247-022-05585-3<\/p>\n                            <\/div>\n                        <\/div>\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p>Radiografia simples anteroposterior da pelve em paciente com MPS IVA. A radiografia mostra displasia acetabular bilateral associada \u00e0 irregularidade do teto cartilaginoso acetabular <strong class=\"letter-featured letter-white\">A<\/strong>, il\u00edaca arredondada <strong class=\"letter-featured letter-pink\">B<\/strong>, coxa valga <strong class=\"letter-featured letter-orange\">C<\/strong>, ep\u00edfise femoral proximal <strong class=\"letter-featured letter-red\">D<\/strong> achatada e irregular bilateralmente, necrose avascular (ep\u00edfise femoral irregular) ou imagem dentada.<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic14.jpg\" alt=\"\">\n                                            <\/div>\n                                            <figcaption>Imagem cortesia de Dr. Hugo Flores<\/figcaption>\n                                        <\/figure>\n                                    <\/div>  \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                    <div id=\"classic-legs-02\" class=\"box-information\">                      \n                        <div class=\"split-content mps-interactivo-split-content-carousel content-align-top\">\n                            <div class=\"wrapper\">\n                                <div class=\"owl-carousel mps-interactivo-split-content-carousel-slides\">\n                                    \n                                    <div class=\"inner-wrapper\">                                     \n                                        <div class=\"content-block\">                                         \n                                            <p>Radiografia simples anteroposterior de ambos joelhos em paciente do sexo feminino, 6 anos de idade, portadora de MPS IVA.<\/p>\n                                            <p>A radiografia mostra <strong class=\"letter-featured letter-white\">A<\/strong> geno valgo bilateral. Envolvimento da l\u00e2mina tibial lateral das ep\u00edfises proximais e hipoplasia das ep\u00edfises. O espa\u00e7o articular dos joelhos \u00e9 amplo.<\/p>\n                                            <p>Imagem adaptada de Padash, S., Obaid, H., Henderson, R.D.E. et al. A pictorial review of the radiographic skeletal findings in Morquio syndrome (mucopolysaccharidosis type IV). Pediatr Radiol (2023). https:\/\/doi.org\/10.1007\/s00247-022-05585-3<\/p>\n                                        <\/div>\n                                        <div class=\"content-block\">\n                                            <figure>\n                                                <div class=\"image\">\n                                                    <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic15.jpg\" alt=\"\">\n                                                <\/div>                                                  \n                                            <\/figure>\n                                        <\/div>\n                                    <\/div>\n                                    \n                                    <div class=\"inner-wrapper\">\n                                        <figure>\n                                            <div class=\"image\">\n                                                <img decoding=\"async\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic16.jpg\" alt=\"\">\n                                            <\/div>  \n                                            <figcaption>Paciente masculino con MPS IVA fen\u00f3tipo cl\u00e1ssico con geno valgo severo<\/figcaption>\n                                            <p>Imagem adaptada de Colmenares-Bonilla D, Esquitin-Gardu\u00f1o N. Diagnosis of Morquio-A patients in Mexico: How far are we from prompt diagnosis?. Intractable Rare Dis Res. 2017;6(2):119-123. doi:10.5582\/irdr.2017.01016<\/p>\n                                        <\/figure>\n                                    <\/div>                                                                                                                                      \n                                    \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                    <div id=\"classic-legs-03\" class=\"box-information\">\n                        <div class=\"note\">\n                            <div class=\"note-title\">\n                                Nota importante\n                            <\/div>\n                            <div class=\"note-content\">\n                                <p>Dist\u00farbios da marcha, juntamente com baixa estatura, geno valgo, cifoescoliose, pectus carinatum e frouxid\u00e3o articular, s\u00e3o geralmente evidentes at\u00e9 o terceiro ou quarto ano de vida do paciente.<\/p>\n                                <p>Refer\u00eancia: White KK, Jester A, Bache CE, et al. Orthopedic management of the extremities in patients with Morquio A syndrome. J Child Orthop. 2014 Aug;8(4):295-304.<\/p>\n                            <\/div>\n                        <\/div>\n                        <div class=\"split-content\">\n                            <div class=\"wrapper\">\n                                <div class=\"inner-wrapper\">\n                                    <div class=\"content-block\">\n                                        <p>O envolvimento musculoesquel\u00e9tico progressivo de pacientes com Morquio faz com que os pacientes necessitem de m\u00faltiplas interven\u00e7\u00f5es ortop\u00e9dicas para prevenir deformidades e melhorar a fun\u00e7\u00e3o. O envolvimento progressivo do membro inferior e o aumento da fraqueza muscular comprometem a mobilidade e, consequentemente, muitos pacientes necessitam de dispositivos auxiliares ou cadeiras de rodas desde a adolesc\u00eancia. Velocidade reduzida, cad\u00eancia e dist\u00e2ncia percorrida s\u00e3o as principais anormalidades da marcha associadas \u00e0 s\u00edndrome de Morquio. O desalinhamento do membro inferior, a fraqueza muscular e a frouxid\u00e3o articular contribuem para o padr\u00e3o patol\u00f3gico da marcha.<\/p>\n                                    <\/div>\n                                    <div class=\"content-block\">\n                                        <div class=\"video-poster\">          \n                                            <a class=\"modal-video\" href=\"https:\/\/player.vimeo.com\/video\/1019991509\" style=\"background-image: url(https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/img\/components\/mps-interactivo\/classic_legs_video_thumbnail.png)\">Play<\/a>\n                                        <\/div>\n                                        <p class=\"small\">Fen\u00f3tipo paciente com fen\u00f3tipo cl\u00e1ssico MPS IVA realizando teste de caminhada de 6 minutos (6MWT- 6 Minute Walk Test).<\/p>\n                                    <\/div>  \n                                <\/div>\n                            <\/div>\n                        <\/div>\n                        <a class=\"box-close\" href=\"#\">Close<\/a>\n                    <\/div>\n                    \n                <\/div>\n                \n                <p><a class=\"button button-text button-arrow button-arrow-left mps-interactivo-back\" href=\"#\">Voltar<\/a><\/p>\n            <\/div>              \n        <\/div>\n    <\/div>\n    \n<\/div>\n<script type=\"module\" src=\"https:\/\/hcp.biomarin.com\/pt-br\/mps\/mpsinteractivo\/wp-content\/themes\/biomarin-master\/js\/model-viewer.min.js\"><\/script>","protected":false},"excerpt":{"rendered":"","protected":false},"author":2,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"_acf_changed":false,"inline_featured_image":false,"footnotes":""},"class_list":["post-28","page","type-page","status-publish","hentry"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.1.1 - 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