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  • 4. Реабилитация

  • 5. Профилактика и диспансерное наблюдение Рекомендуется

  • Рекомендуется

  • 6. Дополнительная информация, влияющая на течение и исход заболевания Критерии оценки качества медицинской помощи

  • Критерии качества

  • Уровень убедительности рекомендаций

  • Список литературы

  • Приложение А1. Состав рабочей группы

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    Клинические рекомендации ППР 20.04.2020. Преждевременное половое развитие


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    3.2 Хирургическое лечение


    • Хирургическое или лучевое лечение объемных образований головного мозга у детей с ППР проводится только по неврологическим показаниям, определяемым нейрохирургом. Гонадотропин-зависимое преждевременное половое развитие хорошо поддается терапии пролонгированными аналогами ГнРГ вне зависимости от нозологического варианта и не является показанием к хирургическому лечению [3] УДД -3 УУР-B.

    • Все варианты ППР, обусловленные объемными образованиями надпочечников и гонад лечатся хирургическим методом (см. протокол ведения пациентов с опухолями надпочечников и гонад) [121, 122, 123, 124] УДД -3 УУР-B.

    • Не рекомендуется хирургическое удаление кист яичников при синдроме МакКьюна-Олбрайта-Брайцева, учитывая потенциальный негативный эффект на фертильности и высокую вероятность рецидива кист в оставшейся ткани яичника. Исключение составляют случаи длительной персистенции кист и подозрение на новообразование (GPP).

    • Девочкам с врожденной дисфункцией коры надпочечников при наличии вирилизации проводится феминизирующая пластика [125] УДД -3 УУР-B.

    3.3 Иное лечение


    • При синдроме МакКьюна-Олбрайта-Брайцева проводится терапия каждого компонента заболевания в зависимости от представленности проявлений заболевания в каждом конкретном случае [100] УДД -3 УУР-B.

    • Мальчики с ХГЧ-секретирующими герминативно-клеточными опухолями получают лечение у онкологов по соответствующим протоколам [126] УДД -3 УУР-B.

    4. Реабилитация

    При отсутствии своевременного назначения лечения при преждевременном половом развитии отмечается ускоренное закрытие зон роста и негативный прогноз конечного роста, что может потребовать психологической поддержки для адаптации пациента.

    5. Профилактика и диспансерное наблюдение

    • Рекомендуется для раннего выявления признаков преждевременного полового развития и своевременного назначения лечения у всех детей проводить антропометрию, регулярный подсчет скорости роста за предшествующие 6-12 мес., клиническую оценку полового развития на основании шкалы Таннер (см. физикальное обследование) [4, 54] УДД – 3 УУР – B

    • Рекомендуется проводить регулярное обследование с оценкой параметров эффективности на фоне назначенного лечения по поводу преждевременного полового развития (см. Лечение) [3, 101] УДД – 3 УУР – B.


    6. Дополнительная информация, влияющая на течение и исход

    заболевания

    Критерии оценки качества медицинской помощи

    №
    Критерии качества
    Уровень достоверности доказательств
    Уровень убедительности рекомендаций















    Выполнено измерение роста и оценка SDS роста
    УДД – 3 УУР – B.
    УДД – 3 УУР – B.



    Проведен подсчет скорости роста за предшествующие 6-12 месяцев (при наличии данных роста)
    УДД – 3 УУР – B.
    УДД – 3 УУР – B.



    Выполнена клиническая оценка полового развития на основании шкалы Таннер
    GPP
    GPP



    Выполнена оценка костного возраста по атласу Greulich-Pale или системе Tanner-Whitehouse или методом Бухмана
    GPP
    GPP



    Выполнено определение в крови уровня ЛГ
    УДД – 3 УУР – B.
    УДД – 3 УУР – B



    У детей с периферическим ППР или изолированным адренархе выполнено определение в крови 17ОПГ, ДГЭА-С
    УДД – 3 УУР – B.
    УДД – 3 УУР – B.



    Выполнено МРТ головного мозга у мальчиков до 9 лет с центральным ППР и у девочек до 6 лет с центральным ППР
    УДД – 3 УУР – B.
    УДД – 3 УУР – B.



    Выполнено УЗИ органов малого таза у девочек и УЗИ тестикул у мальчиков с периферическим ППР
    УДД – 3 УУР – B.
    УДД – 3 УУР – B.



    Выполнено УЗИ/МКСТ надпочечников при адренархе и повышенном уровне ДГЭА-С
    УДД – 3 УУР – B.
    УДД – 3 УУР – B.



    Девочкам младше 6 лет и мальчикам младше 9 лет назначен аналог Гн-РГ пролонгированного действия при доказанном центральном ППР

    УДД – 5 УУР – А
    УДД – 5 УУР – А



    Детям с центральным ППР, получающим лечение аналогом Гн-РГ пролонгированного действия, проводится регулярное (1 раз в 6 -12 мес.) обследование для оценки эффективности терапии
    GPP
    GPP



    Детям с центральным ППР, получающим лечение аналогом Гн-РГ пролонгированного действия, при выявлении неэффективности проводимого лечения, проведено увеличение дозы препарата и/или сокращение интервала между инъекциями.
    GPP
    GPP

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