{"id":166,"date":"2018-01-06T20:13:28","date_gmt":"2018-01-06T20:13:28","guid":{"rendered":"https:\/\/ctxawareness.com\/symptoms\/"},"modified":"2020-10-06T21:20:32","modified_gmt":"2020-10-06T21:20:32","slug":"symptome","status":"publish","type":"page","link":"https:\/\/ctxawareness.com\/de\/symptome\/","title":{"rendered":"Symptome"},"content":{"rendered":"[vc_row type=&#8220;in_container&#8220; full_screen_row_position=&#8220;middle&#8220; column_margin=&#8220;default&#8220; scene_position=&#8220;center&#8220; text_color=&#8220;dark&#8220; text_align=&#8220;left&#8220; class=&#8220;column-block&#8220; overlay_strength=&#8220;0.3&#8243; shape_divider_position=&#8220;bottom&#8220; bg_image_animation=&#8220;none&#8220; shape_type=&#8220;&#8220;][vc_column column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][vc_column_text]\n<h2>Wie erkennt man die Symptome von CTX?<\/h2>\n<p>Es sollte nicht vergessen werden, dass sich die Anzeichen und Symptome von CTX von Patienten zu Patienten unterscheiden und sich neurologische Symptome im Verlauf der Krankheit verschlimmern k\u00f6nnen.<\/p>\n<h3>Leitsymptome<\/h3>\n<ul>\n<li>Eines der Leitsymptome ist ein bereits bei Neugeborenen einsetzender chronischer Durchfall.<\/li>\n<li>Linsentr\u00fcbungen in beiden Augen treten h\u00e4ufig bei Kindern und Jugendlichen auf, auch wenn sich diese eventuell nicht gleichzeitig entwickeln und auch erst sp\u00e4ter auftreten k\u00f6nnen.<\/li>\n<li>Xanthome oder Fettablagerungen an den Sehnen und anderen Organen k\u00f6nnen bei Jugendlichen und jungen Erwachsenen auftreten und betreffen meistens die Knie, Achillessehnen und Ellbogen.<\/li>\n<li>Schwerwiegende neurologische Anzeichen der Erkrankung (Krampfanf\u00e4lle, Demenz, Dystonie) treten h\u00e4ufig zuerst im jungen Erwachsenenalter auf. In manchen F\u00e4llen k\u00f6nnen neurologische Symptome wie eine reduzierte Muskelkoordination, abnorme Reflexe und Abnahme der Intelligenz bereits in der Jugend und kognitive Beeintr\u00e4chtigungen, Entwicklungsverz\u00f6gerungen und Epilepsie bereits in der Kindheit auftreten.<sup>1,2<\/sup><\/li>\n<\/ul>\n[\/vc_column_text][\/vc_column][\/vc_row][vc_row type=&#8220;in_container&#8220; full_screen_row_position=&#8220;middle&#8220; column_margin=&#8220;default&#8220; scene_position=&#8220;center&#8220; text_color=&#8220;dark&#8220; text_align=&#8220;left&#8220; class=&#8220;column-block&#8220; overlay_strength=&#8220;0.3&#8243; shape_divider_position=&#8220;bottom&#8220; bg_image_animation=&#8220;none&#8220; shape_type=&#8220;&#8220;][vc_column column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; top_margin=&#8220;25&#8243; bottom_margin=&#8220;25&#8243; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;2px&#8220; column_border_color=&#8220;#004b87&#8243; column_border_style=&#8220;solid&#8220; enable_border_animation=&#8220;true&#8220; bg_image_animation=&#8220;none&#8220;][vc_column_text css=&#8220;.vc_custom_1573140785208{padding-top: 30px !important;padding-bottom: 25px !important;}&#8220;]\n<h3 style=\"text-align: center;\">Ohne ad\u00e4quate Behandlung von CTX sterben die meisten Patienten zwischen der 4. und 6. Lebensdekade.<sup>3<\/sup><\/h3>\n[\/vc_column_text][\/vc_column][\/vc_row][vc_row type=&#8220;full_width_background&#8220; full_screen_row_position=&#8220;middle&#8220; column_margin=&#8220;default&#8220; bg_color=&#8220;#e1e7eb&#8220; scene_position=&#8220;center&#8220; text_color=&#8220;dark&#8220; text_align=&#8220;left&#8220; overlay_strength=&#8220;0.3&#8243; shape_divider_position=&#8220;bottom&#8220; bg_image_animation=&#8220;none&#8220; shape_type=&#8220;&#8220;][vc_column column_padding=&#8220;padding-10-percent&#8220; column_padding_position=&#8220;left-right&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; top_margin=&#8220;25&#8243; bottom_margin=&#8220;25&#8243; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;35&#8243;][vc_column_text]\n<h2>Was ist wichtig, zu wissen?<\/h2>\n<p><strong>Erfahren Sie mehr von CTX-Experten.<\/strong>[\/vc_column_text][vc_row_inner column_margin=&#8220;default&#8220; text_align=&#8220;left&#8220;][vc_column_inner column_padding=&#8220;padding-10-percent&#8220; column_padding_position=&#8220;left-right&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; column_link_target=&#8220;_self&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220; offset=&#8220;vc_hidden-md vc_hidden-sm vc_hidden-xs&#8220;][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;35&#8243;][vc_column_text]<iframe loading=\"lazy\" src=\"https:\/\/player.vimeo.com\/video\/464998905\" width=\"640\" height=\"480\" frameborder=\"0\" allowfullscreen=\"allowfullscreen\"><\/iframe>[\/vc_column_text][\/vc_column_inner][\/vc_row_inner][vc_row_inner column_margin=&#8220;default&#8220; text_align=&#8220;left&#8220;][vc_column_inner column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;left-right&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; column_link_target=&#8220;_self&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220; offset=&#8220;vc_hidden-lg&#8220;][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;35&#8243;][vc_column_text]<iframe loading=\"lazy\" src=\"https:\/\/player.vimeo.com\/video\/464998905\" width=\"640\" height=\"480\" frameborder=\"0\" allowfullscreen=\"allowfullscreen\"><\/iframe>[\/vc_column_text][\/vc_column_inner][\/vc_row_inner][\/vc_column][\/vc_row][vc_row type=&#8220;in_container&#8220; full_screen_row_position=&#8220;middle&#8220; column_margin=&#8220;default&#8220; scene_position=&#8220;center&#8220; text_color=&#8220;dark&#8220; text_align=&#8220;left&#8220; class=&#8220;column-block&#8220; overlay_strength=&#8220;0.3&#8243; shape_divider_position=&#8220;bottom&#8220; bg_image_animation=&#8220;none&#8220; shape_type=&#8220;&#8220;][vc_column column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][vc_row_inner column_margin=&#8220;default&#8220; text_align=&#8220;left&#8220;][vc_column_inner column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; column_link_target=&#8220;_self&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][divider line_type=&#8220;No Line&#8220;][\/vc_column_inner][\/vc_row_inner][vc_column_text]\n<h3>CTX-Symptome bei Kindern<sup>4,5<\/sup><\/h3>\n[\/vc_column_text][vc_row_inner column_margin=&#8220;default&#8220; text_align=&#8220;left&#8220;][vc_column_inner column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; column_link_target=&#8220;_self&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][vc_column_text]\n<h4>Hauptsymptome:<\/h4>\n<ul>\n<li>Chronischer Durchfall<\/li>\n<li>Beidseitiger Grauer Star (nicht angeboren)<\/li>\n<\/ul>\n[\/vc_column_text][vc_column_text]\n<h4>Weitere Symptome:<\/h4>\n<ul>\n<li>Verl\u00e4ngerte Neugeborenengelbsucht<\/li>\n<li>Entwicklungsverz\u00f6gerungen und neurologische St\u00f6rungen wie ungew\u00f6hnliches Verhalten und Lernst\u00f6rungen<\/li>\n<li>Sehbeeintr\u00e4chtigungen wie verschwommenes Sehen, blinde Flecken oder Sehverlust<\/li>\n<li>Autismus-Spektrum-St\u00f6rung<\/li>\n<li>Epilepsie<\/li>\n<\/ul>\n[\/vc_column_text][\/vc_column_inner][\/vc_row_inner][vc_row_inner column_margin=&#8220;default&#8220; text_align=&#8220;left&#8220;][vc_column_inner column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; column_link_target=&#8220;_self&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][divider line_type=&#8220;No Line&#8220;][\/vc_column_inner][\/vc_row_inner][vc_column_text]\n<h3>CTX-Symptome bei Jugendlichen und Erwachsenen<sup>4<\/sup><\/h3>\n[\/vc_column_text][vc_column_text]\n<h4>Hauptsymptome:<\/h4>\n<ul>\n<li>Neurologische Probleme einschlie\u00dflich Lernst\u00f6rungen, Krampfanf\u00e4lle, Demenz und atypisches Parkinson-Syndrom<\/li>\n<li>Xanthome<\/li>\n<li>Psychiatrische Symptome einschlie\u00dflich Verhaltens\u00e4nderungen, Halluzinationen, Agitiertheit, Aggression und Depression<\/li>\n<li>Erh\u00f6hte Steifigkeit, langsame Bewegungen, abnormer Gang<\/li>\n<\/ul>\n[\/vc_column_text][vc_column_text]\n<h4>Weitere Symptome<\/h4>\n<ul>\n<li>Atherosklerose<\/li>\n<li>Osteoporose und Frakturen<\/li>\n<li>Andere Sehprobleme<\/li>\n<\/ul>\n[\/vc_column_text][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;35&#8243;][vc_row_inner equal_height=&#8220;yes&#8220; content_placement=&#8220;middle&#8220; column_margin=&#8220;default&#8220; text_align=&#8220;left&#8220;][vc_column_inner column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; column_link_target=&#8220;_self&#8220; width=&#8220;1\/3&#8243; tablet_width_inherit=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][image_with_animation image_url=&#8220;98&#8243; alignment=&#8220;center&#8220; animation=&#8220;None&#8220; hover_animation=&#8220;none&#8220; border_radius=&#8220;none&#8220; box_shadow=&#8220;none&#8220; image_loading=&#8220;default&#8220; max_width=&#8220;100%&#8220; max_width_mobile=&#8220;default&#8220;][\/vc_column_inner][vc_column_inner column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;left-right&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; column_link_target=&#8220;_self&#8220; width=&#8220;2\/3&#8243; tablet_width_inherit=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][vc_column_text]Xanthome, also Fettablagerungen, die meistens an der Achillessehne auftreten, sind ein h\u00e4ufiges Symptom bei Jugendlichen und Erwachsenen mit CTX.<sup>1<\/sup> Foto mit freundlicher Genehmigung von William Connor, MD, Oregon Health and Science University.<sup>3<\/sup>[\/vc_column_text][\/vc_column_inner][\/vc_row_inner][\/vc_column][\/vc_row][vc_row type=&#8220;full_width_background&#8220; full_screen_row_position=&#8220;middle&#8220; equal_height=&#8220;yes&#8220; content_placement=&#8220;middle&#8220; column_margin=&#8220;default&#8220; bg_color=&#8220;#e1e7eb&#8220; scene_position=&#8220;center&#8220; text_color=&#8220;dark&#8220; text_align=&#8220;left&#8220; top_padding=&#8220;50&#8243; bottom_padding=&#8220;50&#8243; overlay_strength=&#8220;0.3&#8243; shape_divider_position=&#8220;bottom&#8220; bg_image_animation=&#8220;none&#8220; shape_type=&#8220;&#8220;][vc_column centered_text=&#8220;true&#8220; column_padding=&#8220;padding-10-percent&#8220; column_padding_position=&#8220;left&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; width=&#8220;1\/4&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][image_with_animation image_url=&#8220;57&#8243; alignment=&#8220;center&#8220; animation=&#8220;None&#8220; hover_animation=&#8220;none&#8220; border_radius=&#8220;none&#8220; box_shadow=&#8220;none&#8220; image_loading=&#8220;default&#8220; max_width=&#8220;100%&#8220; max_width_mobile=&#8220;default&#8220; el_class=&#8220;alert&#8220;][\/vc_column][vc_column column_padding=&#8220;padding-10-percent&#8220; column_padding_position=&#8220;right&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; width=&#8220;3\/4&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][vc_column_text]\n<h2>Achtung<\/h2>\n<h2>Eine fr\u00fche Diagnose ist entscheidend!<\/h2>\n<p>Eine Studie von 55 Patienten zeigte, dass das Durchschnittsalter beim ersten Auftreten der Symptome bei 9,5\u00b19,0 Jahren und das durchschnittliche Alter bei der Diagnose bei 35,5\u00b111,8 Jahren lag, mit einer gro\u00dfen Diagnoseverz\u00f6gerung von 20-25 Jahren<sup>6<\/sup>. Beim Zeitpunkt der Diagnose k\u00f6nnen bereits neurologische und psychiatrische Probleme vorgelegen haben. Da die Symptome und das Fortschreiten von CTX von Patienten zu Patienten stark variieren k\u00f6nnen, ist eine fr\u00fchzeitige Diagnose entscheidend. Eine ad\u00e4quate Behandlung kann den Verlauf der Erkrankung positiv beeinflussen, insbesondere wenn in den fr\u00fchen Phasen der Erkrankung damit begonnen wird.<sup>3<\/sup>[\/vc_column_text][\/vc_column][\/vc_row][vc_row type=&#8220;full_width_background&#8220; full_screen_row_position=&#8220;middle&#8220; equal_height=&#8220;yes&#8220; content_placement=&#8220;bottom&#8220; column_margin=&#8220;default&#8220; bg_color=&#8220;#e1e7eb&#8220; scene_position=&#8220;center&#8220; text_color=&#8220;dark&#8220; text_align=&#8220;left&#8220; overlay_strength=&#8220;0.3&#8243; shape_divider_position=&#8220;bottom&#8220; bg_image_animation=&#8220;none&#8220; shape_type=&#8220;&#8220;][vc_column column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][vc_row_inner equal_height=&#8220;yes&#8220; content_placement=&#8220;bottom&#8220; column_margin=&#8220;default&#8220; text_align=&#8220;left&#8220;][vc_column_inner column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; column_link_target=&#8220;_self&#8220; width=&#8220;1\/3&#8243; tablet_width_inherit=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;35&#8243;][image_with_animation image_url=&#8220;216&#8243; alignment=&#8220;center&#8220; animation=&#8220;Fade In&#8220; hover_animation=&#8220;none&#8220; border_radius=&#8220;none&#8220; box_shadow=&#8220;none&#8220; image_loading=&#8220;default&#8220; max_width=&#8220;100%&#8220; max_width_mobile=&#8220;default&#8220;][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;50&#8243;][\/vc_column_inner][vc_column_inner column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; column_link_target=&#8220;_self&#8220; width=&#8220;1\/3&#8243; tablet_width_inherit=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;35&#8243;][image_with_animation image_url=&#8220;201&#8243; alignment=&#8220;center&#8220; animation=&#8220;Fade In&#8220; hover_animation=&#8220;none&#8220; border_radius=&#8220;none&#8220; box_shadow=&#8220;none&#8220; image_loading=&#8220;default&#8220; max_width=&#8220;100%&#8220; max_width_mobile=&#8220;default&#8220;][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;50&#8243;][\/vc_column_inner][vc_column_inner column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; column_link_target=&#8220;_self&#8220; width=&#8220;1\/3&#8243; tablet_width_inherit=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;35&#8243;][image_with_animation image_url=&#8220;207&#8243; alignment=&#8220;center&#8220; animation=&#8220;Fade In&#8220; hover_animation=&#8220;none&#8220; border_radius=&#8220;none&#8220; box_shadow=&#8220;none&#8220; image_loading=&#8220;default&#8220; max_width=&#8220;100%&#8220; max_width_mobile=&#8220;default&#8220;][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;50&#8243;][\/vc_column_inner][\/vc_row_inner][\/vc_column][\/vc_row][vc_row type=&#8220;full_width_background&#8220; full_screen_row_position=&#8220;middle&#8220; column_margin=&#8220;default&#8220; scene_position=&#8220;center&#8220; text_color=&#8220;dark&#8220; text_align=&#8220;left&#8220; overlay_strength=&#8220;0.3&#8243; shape_divider_position=&#8220;bottom&#8220; bg_image_animation=&#8220;none&#8220; shape_type=&#8220;&#8220;][vc_column column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; el_class=&#8220;column-block&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;75&#8243;][vc_column_text]\n<h2>Was verursacht CTX?<\/h2>\n<h3>CTX tritt auf, wenn ein defektes Gen ein wichtiges Enzym nicht produzieren kann.<\/h3>\n[\/vc_column_text][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;50&#8243;][image_with_animation image_url=&#8220;195&#8243; alignment=&#8220;center&#8220; animation=&#8220;Fade In&#8220; hover_animation=&#8220;none&#8220; border_radius=&#8220;none&#8220; box_shadow=&#8220;none&#8220; image_loading=&#8220;default&#8220; max_width=&#8220;100%&#8220; max_width_mobile=&#8220;default&#8220;][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;50&#8243;][vc_column_text]CTX ist eine autosomal-rezessive Erkrankung. Dies bedeutet, dass von der Krankheit betroffene Personen mit zwei defekten Kopien des CYP27A1-Gens geboren werden. Dieses Gen enth\u00e4lt normalerweise die Anweisungen f\u00fcr die Produktion eines Enzyms mit dem Namen Sterol-27-Hydroxylase. Personen, die mit nur einer Kopie des besch\u00e4digten Gens geboren werden, werden als Tr\u00e4ger bezeichnet und entwickeln normalerweise keine CTX. Das Sterol-27-Hydroxylase-Enzym ist in der Leber aktiv und baut Cholesterin ab, um es in die prim\u00e4ren Gallens\u00e4uren Chenodesoxychols\u00e4ure (CDCA) und Chols\u00e4ure (CA) umzuwandeln.<sup>7<\/sup><\/p>\n<p>Mutationen des CYP27A1-Gens f\u00fchren zu einer Funktionsst\u00f6rung des Sterol-27-Hydroxylase-Enzyms und st\u00f6ren die Bildung von prim\u00e4ren Gallens\u00e4uren. Die Unterbrechung dieser Stoffwechselwege f\u00fchrt im Laufe der Zeit au\u00dferdem zur Bildung und Ablagerung von Cholestanol (einem Cholesterin-\u00e4hnlichen Lipid) in verschiedenen K\u00f6rpergeweben, was zu CTX-Symptomen f\u00fchrt.<sup>8<\/sup>[\/vc_column_text][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;30&#8243;][\/vc_column][\/vc_row][vc_row type=&#8220;in_container&#8220; full_screen_row_position=&#8220;middle&#8220; column_margin=&#8220;default&#8220; scene_position=&#8220;center&#8220; text_color=&#8220;dark&#8220; text_align=&#8220;left&#8220; overlay_strength=&#8220;0.3&#8243; shape_divider_position=&#8220;bottom&#8220; bg_image_animation=&#8220;none&#8220;][vc_column column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][divider line_type=&#8220;No Line&#8220;][\/vc_column][\/vc_row][vc_row type=&#8220;full_width_background&#8220; full_screen_row_position=&#8220;middle&#8220; column_margin=&#8220;default&#8220; bg_color=&#8220;#e1e7eb&#8220; scene_position=&#8220;center&#8220; text_color=&#8220;dark&#8220; text_align=&#8220;left&#8220; top_padding=&#8220;25&#8243; bottom_padding=&#8220;25&#8243; overlay_strength=&#8220;0.3&#8243; shape_divider_position=&#8220;bottom&#8220; bg_image_animation=&#8220;none&#8220; shape_type=&#8220;&#8220;][vc_column column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; el_class=&#8220;column-block&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;35&#8243;][vc_column_text]\n<h2>Bef\u00fcrchten Sie, an CTX zu leiden?<\/h2>\n<p>Sollten Sie den Verdacht haben unter CTX zu leiden oder eine CTX-Diagnose erhalten haben, wenden Sie sich bitte f\u00fcr eine professionelle Beratung an Ihren behandelnden Arzt.[\/vc_column_text][divider line_type=&#8220;No Line&#8220; custom_height=&#8220;35&#8243;][\/vc_column][\/vc_row][vc_row type=&#8220;in_container&#8220; full_screen_row_position=&#8220;middle&#8220; column_margin=&#8220;default&#8220; scene_position=&#8220;center&#8220; text_color=&#8220;dark&#8220; text_align=&#8220;left&#8220; overlay_strength=&#8220;0.3&#8243; shape_divider_position=&#8220;bottom&#8220; bg_image_animation=&#8220;none&#8220;][vc_column column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][divider line_type=&#8220;No Line&#8220;][\/vc_column][\/vc_row][vc_row type=&#8220;in_container&#8220; full_screen_row_position=&#8220;middle&#8220; column_margin=&#8220;default&#8220; scene_position=&#8220;center&#8220; text_color=&#8220;dark&#8220; text_align=&#8220;left&#8220; class=&#8220;column-block&#8220; overlay_strength=&#8220;0.3&#8243; shape_divider_position=&#8220;bottom&#8220; bg_image_animation=&#8220;none&#8220; shape_type=&#8220;&#8220;][vc_column column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][vc_column_text]\n<h3>Quellen:<\/h3>\n<ol>\n<li class=\"references\">Federico A, Dotti MT, Gallus GN. Cerebrotendinous xanthomatosis. In: Adam MP, Ardinger HH, Pagon RA et al, Hrsg. Gene Reviews [Internet]. Seattle: University of Washington; 2013; aktualisiert 2016. <a href=\"https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK1409\/\" target=\"_blank\" rel=\"noopener noreferrer\">https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK1409\/<\/a>. Zugriff am 25. Januar 2018<\/li>\n<li class=\"references\">Verrips A. Cerebrotendinous xenthomatosis. In: Hollak CEM, Lachmann R. Hrsg. Inherited Metabolic Disease in Adults: A Clinical Guide. Oxford University Press; 2016: Kap. 40.<\/li>\n<li class=\"references\">Steiner RD. Cerebrotendinous xanthomatosis. Medscape; 2015; aktualisiert 2017. <a href=\"https:\/\/emedicine.medscape.com\/article\/1418820-overview#showall\" target=\"_blank\" rel=\"noopener noreferrer\">https:\/\/emedicine.medscape.com\/article\/1418820-overview#showall<\/a>. Zugriff am 29. Januar 2018<\/li>\n<li class=\"references\">Berginer VM, Gross B, Morad K, Kfir N. Chronic diarrhoea and juvenile cataracts: Think Cerebrotendinous Xanthomatosis and treat. Pediatrics. 2009;123:143-7.<\/li>\n<li class=\"references\">Stelten BML, Bonnot O, Huidekoper HH, et al. Autism spectrum disorder: an early and frequent feature in cerebrotendinous xanthomatosis. Journal of Inherited Metabolic Disease. 2017. doi: 10.1007\/s10545-017-0086-7. [elektronische Ver\u00f6ffentlichung vor dem Druck]<\/li>\n<li class=\"references\">Mignarri A, Gallus GN, Dotti MT, Federico A. A suspicion index for early diagnosis and treatment of cerebrotendinous xanthomatosis. Journal of Inherited Metabolic Disease. 2014;37:421-9.<\/li>\n<li class=\"references\">Verrips A, Cruysberg JRM, Wevers RA. Cerebrotendinous xanthomatosis. In: Roach ES, Miller VS, Hrsg. Neurocutaneous Disorders. Cambridge University Press; 2004. Kap. 30.<\/li>\n<li class=\"references\">Moghadasian M. Cerebrotendinous xanthomatosis: clinical course, genotypes and metabolic backgrounds. Clinical and Investigative Medicine. 2004;27(1):42-50.<\/li>\n<\/ol>\n[\/vc_column_text][\/vc_column][\/vc_row][vc_row type=&#8220;in_container&#8220; full_screen_row_position=&#8220;middle&#8220; column_margin=&#8220;default&#8220; scene_position=&#8220;center&#8220; text_color=&#8220;dark&#8220; text_align=&#8220;left&#8220; overlay_strength=&#8220;0.3&#8243; shape_divider_position=&#8220;bottom&#8220; bg_image_animation=&#8220;none&#8220;][vc_column column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][divider line_type=&#8220;No Line&#8220;][\/vc_column][\/vc_row]\n","protected":false},"excerpt":{"rendered":"<p>[vc_row type=&#8220;in_container&#8220; full_screen_row_position=&#8220;middle&#8220; column_margin=&#8220;default&#8220; scene_position=&#8220;center&#8220; text_color=&#8220;dark&#8220; text_align=&#8220;left&#8220; class=&#8220;column-block&#8220; overlay_strength=&#8220;0.3&#8243; shape_divider_position=&#8220;bottom&#8220; bg_image_animation=&#8220;none&#8220; shape_type=&#8220;&#8220;][vc_column column_padding=&#8220;no-extra-padding&#8220; column_padding_position=&#8220;all&#8220; background_color_opacity=&#8220;1&#8243; background_hover_color_opacity=&#8220;1&#8243; column_link_target=&#8220;_self&#8220; column_shadow=&#8220;none&#8220; column_border_radius=&#8220;none&#8220; width=&#8220;1\/1&#8243; tablet_width_inherit=&#8220;default&#8220; tablet_text_alignment=&#8220;default&#8220; phone_text_alignment=&#8220;default&#8220; overlay_strength=&#8220;0.3&#8243; column_border_width=&#8220;none&#8220; column_border_style=&#8220;solid&#8220; bg_image_animation=&#8220;none&#8220;][vc_column_text] Wie erkennt man&#8230;<\/p>\n","protected":false},"author":2,"featured_media":0,"parent":0,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"class_list":["post-166","page","type-page","status-publish"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.3 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>Symptoms of CTX - CTX Awareness | Leadiant Biosciences, Ltd.<\/title>\n<meta name=\"description\" content=\"Signs and symptoms of CTX can vary, change, and worsen over time. 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