Выраженность клинических проявлений МПС I в разном возрасте
Система
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Симптом
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Дети
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Подростки/взрослые
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Сердечно-сосудистая
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Кардиомиопатия
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+
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+
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Утолщение створок клапанов
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+++
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+++
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ЦНС
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Нарушение поведения
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++
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++
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Цервикальная миелопатия
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++
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++
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Гидроцефалия
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++
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++
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Задержка и отставание в развитии
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+++
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++
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Судороги
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+
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++
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Нарушение глотания
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+
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![00000010.wmz](data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAABIAAAAMCAAAAABKebJgAAAABGdBTUEAALGPC/xhBQAAACBjSFJNAAB6JgAAgIQAAPoAAACA6AAAdTAAAOpgAAA6mAAAF3CculE8AAAAAmJLR0QA/4ePzL8AAAAHdElNRQfoAx4SMiOREDsuAAAAEGNhTnYAAAMiAAAEZQAAAYkAAAIt4ymn6QAAAEZJREFUCNdj/M+ADpjgrO+YQq6YQgx4hBj/MzAwnM5lYGC4psXAwHCCgYGB4T8MWMMYeM2SRDELFbAwMDAwnCmAcY9gVwUAX54bkJ+C54QAAAAldEVYdGRhdGU6Y3JlYXRlADIwMjQtMDMtMzBUMTU6NTA6MzUrMDM6MDCfhFFSAAAAJXRFWHRkYXRlOm1vZGlmeQAyMDI0LTAzLTMwVDE1OjUwOjM1KzAzOjAw7tnp7gAAAABJRU5ErkJggg==)
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Пищеварительная система
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Диарея
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+
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![00000011.wmz](data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAABIAAAAMCAAAAABKebJgAAAABGdBTUEAALGPC/xhBQAAACBjSFJNAAB6JgAAgIQAAPoAAACA6AAAdTAAAOpgAAA6mAAAF3CculE8AAAAAmJLR0QA/4ePzL8AAAAHdElNRQfoAx4SMiOREDsuAAAAEGNhTnYAAAMiAAAEZQAAAYkAAAIt4ymn6QAAAEZJREFUCNdj/M+ADpjgrO+YQq6YQgx4hBj/MzAwnM5lYGC4psXAwHCCgYGB4T8MWMMYeM2SRDELFbAwMDAwnCmAcY9gVwUAX54bkJ+C54QAAAAldEVYdGRhdGU6Y3JlYXRlADIwMjQtMDMtMzBUMTU6NTA6MzUrMDM6MDCfhFFSAAAAJXRFWHRkYXRlOm1vZGlmeQAyMDI0LTAzLTMwVDE1OjUwOjM1KzAzOjAw7tnp7gAAAABJRU5ErkJggg==)
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Гепатоспленомегалия
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+++
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+++
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Орган слуха
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Потеря слуха
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++
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++
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Рецидивирующий средний отит
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++
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++
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Орган зрения
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Дистрофия сетчатки
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![00000012.wmz](data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAABIAAAAMCAAAAABKebJgAAAABGdBTUEAALGPC/xhBQAAACBjSFJNAAB6JgAAgIQAAPoAAACA6AAAdTAAAOpgAAA6mAAAF3CculE8AAAAAmJLR0QA/4ePzL8AAAAHdElNRQfoAx4SMiOREDsuAAAAEGNhTnYAAAMiAAAEZQAAAYkAAAIt4ymn6QAAAEZJREFUCNdj/M+ADpjgrO+YQq6YQgx4hBj/MzAwnM5lYGC4psXAwHCCgYGB4T8MWMMYeM2SRDELFbAwMDAwnCmAcY9gVwUAX54bkJ+C54QAAAAldEVYdGRhdGU6Y3JlYXRlADIwMjQtMDMtMzBUMTU6NTA6MzUrMDM6MDCfhFFSAAAAJXRFWHRkYXRlOm1vZGlmeQAyMDI0LTAzLTMwVDE1OjUwOjM1KzAzOjAw7tnp7gAAAABJRU5ErkJggg==)
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![00000013.wmz](data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAABIAAAAMCAAAAABKebJgAAAABGdBTUEAALGPC/xhBQAAACBjSFJNAAB6JgAAgIQAAPoAAACA6AAAdTAAAOpgAAA6mAAAF3CculE8AAAAAmJLR0QA/4ePzL8AAAAHdElNRQfoAx4SMiOREDsuAAAAEGNhTnYAAAMiAAAEZQAAAYkAAAIt4ymn6QAAAEZJREFUCNdj/M+ADpjgrO+YQq6YQgx4hBj/MzAwnM5lYGC4psXAwHCCgYGB4T8MWMMYeM2SRDELFbAwMDAwnCmAcY9gVwUAX54bkJ+C54QAAAAldEVYdGRhdGU6Y3JlYXRlADIwMjQtMDMtMzBUMTU6NTA6MzUrMDM6MDCfhFFSAAAAJXRFWHRkYXRlOm1vZGlmeQAyMDI0LTAzLTMwVDE1OjUwOjM1KzAzOjAw7tnp7gAAAABJRU5ErkJggg==)
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Помутнение роговицы
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+
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+++
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Скелетно-мышечная
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Нестабильность атланто-окципитального сочленения
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+
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+
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Туннельный карпальный синдром
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++
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++
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Грубые черты лица
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+++
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++
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Дисплазия ТБС
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++
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++
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Множественный дизостоз
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+
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+
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Грыжи
|
+++
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++
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Контрактуры суставов
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+++
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+++
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Кифоз
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++
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+
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Макроцефалия
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+++
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++
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Дыхательная
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Обструктивное апноэ во сне
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+++
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+++
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Рестриктивное заболевание легких
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+
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+
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Обструкция верхних дыхательных путей
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+++
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+++
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Специальные лабораторные тесты
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Дерматан сульфат в моче
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![00000014.wmz](data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAABkAAAANCAAAAAB5xZoyAAAABGdBTUEAALGPC/xhBQAAACBjSFJNAAB6JgAAgIQAAPoAAACA6AAAdTAAAOpgAAA6mAAAF3CculE8AAAAAmJLR0QA/4ePzL8AAAAHdElNRQfoAx4SMiOREDsuAAAAEGNhTnYAAAMiAAAEZQAAAYMAAAItqZm/SAAAAFdJREFUGNNj/M+AAzBBqCftvxnQWf///////6CxmONrNBbD/////6/NeR98KvI+Cgsi8+///+D///+hsP4zMTAwMDAywEgEC+YC3G6jm4wsXATGYsQZbgAUcEWy+o0abQAAACV0RVh0ZGF0ZTpjcmVhdGUAMjAyNC0wMy0zMFQxNTo1MDozNSswMzowMJ+EUVIAAAAldEVYdGRhdGU6bW9kaWZ5ADIwMjQtMDMtMzBUMTU6NTA6MzUrMDM6MDDu2enuAAAAAElFTkSuQmCC)
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![00000015.wmz](data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAABkAAAANCAAAAAB5xZoyAAAABGdBTUEAALGPC/xhBQAAACBjSFJNAAB6JgAAgIQAAPoAAACA6AAAdTAAAOpgAAA6mAAAF3CculE8AAAAAmJLR0QA/4ePzL8AAAAHdElNRQfoAx4SMiOREDsuAAAAEGNhTnYAAAMiAAAEZQAAAYMAAAItqZm/SAAAAFdJREFUGNNj/M+AAzBBqCftvxnQWf///////6CxmONrNBbD/////6/NeR98KvI+Cgsi8+///+D///+hsP4zMTAwMDAywEgEC+YC3G6jm4wsXATGYsQZbgAUcEWy+o0abQAAACV0RVh0ZGF0ZTpjcmVhdGUAMjAyNC0wMy0zMFQxNTo1MDozNSswMzowMJ+EUVIAAAAldEVYdGRhdGU6bW9kaWZ5ADIwMjQtMDMtMzBUMTU6NTA6MzUrMDM6MDDu2enuAAAAAElFTkSuQmCC)
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Гепаран сульфат в моче
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![00000016.wmz](data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAABkAAAANCAAAAAB5xZoyAAAABGdBTUEAALGPC/xhBQAAACBjSFJNAAB6JgAAgIQAAPoAAACA6AAAdTAAAOpgAAA6mAAAF3CculE8AAAAAmJLR0QA/4ePzL8AAAAHdElNRQfoAx4SMiQPdK6NAAAAEGNhTnYAAAMiAAAEZQAAAYMAAAItqZm/SAAAAFdJREFUGNNj/M+AAzBBqCftvxnQWf///////6CxmONrNBbD/////6/NeR98KvI+Cgsi8+///+D///+hsP4zMTAwMDAywEgEC+YC3G6jm4wsXATGYsQZbgAUcEWy+o0abQAAACV0RVh0ZGF0ZTpjcmVhdGUAMjAyNC0wMy0zMFQxNTo1MDozNiswMzowMK5sS88AAAAldEVYdGRhdGU6bW9kaWZ5ADIwMjQtMDMtMzBUMTU6NTA6MzYrMDM6MDDfMfNzAAAAAElFTkSuQmCC)
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![00000017.wmz](data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAABkAAAANCAAAAAB5xZoyAAAABGdBTUEAALGPC/xhBQAAACBjSFJNAAB6JgAAgIQAAPoAAACA6AAAdTAAAOpgAAA6mAAAF3CculE8AAAAAmJLR0QA/4ePzL8AAAAHdElNRQfoAx4SMiQPdK6NAAAAEGNhTnYAAAMiAAAEZQAAAYMAAAItqZm/SAAAAFdJREFUGNNj/M+AAzBBqCftvxnQWf///////6CxmONrNBbD/////6/NeR98KvI+Cgsi8+///+D///+hsP4zMTAwMDAywEgEC+YC3G6jm4wsXATGYsQZbgAUcEWy+o0abQAAACV0RVh0ZGF0ZTpjcmVhdGUAMjAyNC0wMy0zMFQxNTo1MDozNiswMzowMK5sS88AAAAldEVYdGRhdGU6bW9kaWZ5ADIwMjQtMDMtMzBUMTU6NTA6MzYrMDM6MDDfMfNzAAAAAElFTkSuQmCC)
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Идуронидаза
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![00000018.wmz](data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAACIAAAANCAAAAADp1uP9AAAABGdBTUEAALGPC/xhBQAAACBjSFJNAAB6JgAAgIQAAPoAAACA6AAAdTAAAOpgAAA6mAAAF3CculE8AAAAAmJLR0QA/4ePzL8AAAAHdElNRQfoAx4SMiQPdK6NAAAAEGNhTnYAAAMiAAAEZQAAAX4AAAItGT0rpAAAAFtJREFUGNNj/M9ACDBB6SlwkSnoDJiSXXAlu9AZMCX/4Er+oTPoruQvXMlfdAaxpqyd9f/fx4Z7yCyEEAPD///////v9ZY3OYLKQghBlPzf7vboPxoLzmAkHEcAoAFfzVTjVLoAAAAldEVYdGRhdGU6Y3JlYXRlADIwMjQtMDMtMzBUMTU6NTA6MzYrMDM6MDCubEvPAAAAJXRFWHRkYXRlOm1vZGlmeQAyMDI0LTAzLTMwVDE1OjUwOjM2KzAzOjAw3zHzcwAAAABJRU5ErkJggg==)
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![00000019.wmz](data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAACIAAAANCAAAAADp1uP9AAAABGdBTUEAALGPC/xhBQAAACBjSFJNAAB6JgAAgIQAAPoAAACA6AAAdTAAAOpgAAA6mAAAF3CculE8AAAAAmJLR0QA/4ePzL8AAAAHdElNRQfoAx4SMiQPdK6NAAAAEGNhTnYAAAMiAAAEZQAAAX4AAAItGT0rpAAAAFtJREFUGNNj/M9ACDBB6SlwkSnoDJiSXXAlu9AZMCX/4Er+oTPoruQvXMlfdAaxpqyd9f/fx4Z7yCyEEAPD///////v9ZY3OYLKQghBlPzf7vboPxoLzmAkHEcAoAFfzVTjVLoAAAAldEVYdGRhdGU6Y3JlYXRlADIwMjQtMDMtMzBUMTU6NTA6MzYrMDM6MDCubEvPAAAAJXRFWHRkYXRlOm1vZGlmeQAyMDI0LTAzLTMwVDE1OjUwOjM2KzAzOjAw3zHzcwAAAABJRU5ErkJggg==)
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Общие ГАГ в моче
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![00000020.wmz](data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAABkAAAANCAAAAAB5xZoyAAAABGdBTUEAALGPC/xhBQAAACBjSFJNAAB6JgAAgIQAAPoAAACA6AAAdTAAAOpgAAA6mAAAF3CculE8AAAAAmJLR0QA/4ePzL8AAAAHdElNRQfoAx4SMiQPdK6NAAAAEGNhTnYAAAMiAAAEZQAAAYMAAAItqZm/SAAAAFdJREFUGNNj/M+AAzBBqCftvxnQWf///////6CxmONrNBbD/////6/NeR98KvI+Cgsi8+///+D///+hsP4zMTAwMDAywEgEC+YC3G6jm4wsXATGYsQZbgAUcEWy+o0abQAAACV0RVh0ZGF0ZTpjcmVhdGUAMjAyNC0wMy0zMFQxNTo1MDozNiswMzowMK5sS88AAAAldEVYdGRhdGU6bW9kaWZ5ADIwMjQtMDMtMzBUMTU6NTA6MzYrMDM6MDDfMfNzAAAAAElFTkSuQmCC)
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![00000021.wmz](data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAABkAAAANCAAAAAB5xZoyAAAABGdBTUEAALGPC/xhBQAAACBjSFJNAAB6JgAAgIQAAPoAAACA6AAAdTAAAOpgAAA6mAAAF3CculE8AAAAAmJLR0QA/4ePzL8AAAAHdElNRQfoAx4SMiQPdK6NAAAAEGNhTnYAAAMiAAAEZQAAAYMAAAItqZm/SAAAAFdJREFUGNNj/M+AAzBBqCftvxnQWf///////6CxmONrNBbD/////6/NeR98KvI+Cgsi8+///+D///+hsP4zMTAwMDAywEgEC+YC3G6jm4wsXATGYsQZbgAUcEWy+o0abQAAACV0RVh0ZGF0ZTpjcmVhdGUAMjAyNC0wMy0zMFQxNTo1MDozNiswMzowMK5sS88AAAAldEVYdGRhdGU6bW9kaWZ5ADIwMjQtMDMtMzBUMTU6NTA6MzYrMDM6MDDfMfNzAAAAAElFTkSuQmCC)
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