nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical University10.24884/1561-6274-2013-17-3-60-67nefr-498Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИ. КЛИНИЧЕСКИЕ ИССЛЕДОВАНИЯORIGINAL ARTICLES. CLINICAL INVESTIGATIONSХАРАКТЕРИСТИКА ПОЧЕЧНЫХ И ВНЕПОЧЕЧНЫХ ПРОВЛЕНИЙ АУТОСОМНО-ДОМИНАНТНОГО ПОЛИКИСТОЗА ПОЧЕК У ДЕТЕЙCHARACTERISTICS OF RENAL END EXTRARENAL MANIFESTATIONS OF AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE IN CHILDRENАрутюнянС. С.АrutyunyanS. S.

Кафедра факультетской педиатрии

СавенковаН. Д.SavenkovaN. D.

Кафедра факультетской педиатрии 

194100, Санкт-Петербург, Литовская ул., д. 2. Тел. +78124165286

Savenkova@NS12254.spb.eduСанкт-Петербургский государственный педиатрический медицинский университетRussian Federation2013100320131736067Copyright © Арутюнян С.С., Савенкова Н.Д., 20132013Арутюнян С.С., Савенкова Н.Д.Аrutyunyan S.S., Savenkova N.D.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://journal.nephrolog.ru/jour/article/view/498

ЦЕЛЬ ИССЛЕДОВАНИЯ: оценить особенности почечных и внепочечных проявлений аутосомно-доминантного поликистоза почек (АДПП) у детей. ПАЦИЕНТЫ И МЕТОДЫ: в исследование включено 67 детей из 60 семей с АДПП. Использованы катамнестический, клинико-лабораторные и инструментальные методы исследования. Проведена стратификация тяжести хронической болезни почек (ХБП) согласно классификаций NKF-K/DOQI (2002) и национальных рекомендаций (2012). РЕЗУЛЬТАТЫ: выявлены возрастные особенности диагностики почечных кист по УЗИ у детей с АДПП: 0–15 лет в 91%, из них 0–18 мес (очень раннее выявление) в 19,4%; 15–18 лет в 9% случаев. Преобладает двустороннее расположение почечных кист: при первом выявлении (8,24±0,64 года) в 59,7%, на момент катамнеза (13,2±0,54 года) в 95,5%. Регрессионный анализ установил ежегодное нарастание максимального диаметра кист на 0,21±0,03 см, средней длины почек на 0,42±0,05 см у детей с АДПП по данным УЗИ. Артериальная гипертензия (АГ) при первом выявлении кист диагностирована в 4,5% случаев, на момент катамнеза в 21%. Средняя длина почек и максимальный диаметр кист у детей с АДПП, имеющих АГ, достоверно больше, чем у детей без АГ. Достоверных различий между максимальным диаметром кист, средней длиной почек и частотой АГ у детей с очень ранним и более поздним выявлением кист не установлено. Внепочечные проявления АДПП у детей в катамнезе (5,1±0,6 года) выявлены достоверно чаще (31,3%), чем при первом выявлении кист в почках (13,5%). Показано, что КТ и МРТ являются более информативными методами выявления внепочечных кист у детей, чем УЗИ. У детей с АДПП преобладает I стадия ХБП в 94% случаев. ЗАКЛЮЧЕНИЕ: выявлены особенности почечных и внепочечных проявлений, течения и исхода АДПП у детей.

AIM OF STUDY. To estimate the characteristics of renal and extrarenal manifestations of autosomal dominant polycystic kidney disease (ADPKD) in children. PATIENTS AND METHODS. 67 children from 60 families with ADPKD were involved in research. Data of follow-up, clinical, laboratory and instrumental examinations were used. Stratification of chronic kidney disease (CKD) according to NKF-K/DOQI classification (2002) and national recommendations (2012) was carried out. RESULTS. The agespecific features of renal cysts diagnostics by ultrasound were detected: 0-15 years in 91%, including 0-18 month (very early onset) in 19,4%; 15-18 years in 9%. Bilateral location of renal cysts dominate: at first detection (mean age 8,24±0,64 years) in 59,7%, at follow-up moment (mean age 13,2±0,54 years) in 95,5%. Regression analysis revealed that in ADPKD children annual increase of maximal diameter of renal cysts by ultrasound (US) scan was 0,21±0,03 cm, annual increase of average renal length by US scan was 0,42±0,05 cm. Arterial hypertension (AH) was diagnosed at first detection of cysts in 4,5%, at follow-up in 21%. Average renal length and maximal diameter of renal cysts in ADPKD children with AH is significantly larger than in ADPKD children without AH. There are no significant differences between average renal length, maximal diameter of renal cysts and AH frequency in children with very early and later detection of cysts. Extrarenal manifestations of ADPKD in children at followup (5,1±0,6 years) are significantly more often (31,3%), than at first renal cysts detection (13,5%). It was demonstrated that computer tomography and magnetic resonance imaging are significantly more informative methods for detection of extrarenal cysts than US scan. In ADPKD children I stage of CKD dominates in 94%. CONCLUSION. Characteristics of renal and extrarenal manifestations, course and outcome of ADPKD in children were revealed.

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