nidНефрология и диализNephrology and Dialysis1680-44222618-9801Российское диализное обществоnid-1572Research ArticleШКОЛА НЕФРОЛОГАEDUCATIONAL MATERIALSГематурия при наследственных нефропатияхHematuria in hereditary nephropathiesИгнатоваМ. С.IgnatovaM. S.nephrolog@pedklin.ruФГУ МНИИ педиатрии и ДХ Росздрава, г. МоскваRussian Federation20062306202584363369Copyright © Игнатова М.С., 20252025Игнатова М.С.Ignatova M.S.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephro.ru/jour/article/view/1572

На основании собственных наблюдений и литературных данных представлена характеристика гематурии при наследственных нефропатиях: синдроме Альпорта и болезни тонких базальных мембран (БТБМ). Обращено внимание на необходимость дифференцирования указанных заболеваний в связи с их различным прогнозом. Указывается и на клиническом примере показано, что БТБМ может быть предрасполагающим фактором к развитию иммунной нефропатии (мезангиопролиферативного гломерулонефрита). Представлен первый опыт использования ингибиторов ангиотензин-превращающего фермента при наследственном нефрите.

Isolated hematuria in hereditary nephropathies is characterized on the basis of literature data and our own experience. An attention was focused on the thin basement membrane disease (TBMD) and the Alport syndrome because these diseases have different prognosis. It is pointed out that TBMD could be a predisposition factor in the development of immune glomerulopathies. Preliminary results of therapy of the Alport syndrome with ACE-inhibitors are presented

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The authors declare that there are no conflicts of interest present.