nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical University10.24884/1561-6274-2018-22-3-51-57nefr-365Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИ. КЛИНИЧЕСКИЕ ИССЛЕДОВАНИЯORIGINAL ARTICLES. CLINICAL INVESTIGATIONSЧАСТОТА И ХАРАКТЕР СОЧЕТАННОЙ ВРОЖДЕННОЙ АНОМАЛИИ ПОЧЕК И МОЧЕВЫВОДЯЩИХ ПУТЕЙ В СТРУКТУРЕ CAKUT- СИНДРОМА У ДЕТЕЙFREQUENCY AND CHARACTER OF COMBINED CONGENITAL ANOMALY OF KIDNEYS AND URINARY TRACT IN STRUCTURE CAKUT-SYNDROME IN CHILDRENКутырлоИ. Э.KutyrloI.kutyrloirina@gmail.comСавенковаН. Д.SavenkovaN.

д-р мед., зав. каф. факультетской педиатрии

Savenkova.n.spb@mail.ruСанкт-Петербургский государственный педиатрический медицинский университетРоссияSaint-Petersburg Pediatric State Medical UniversityRussian Federation2018120520182235157Copyright © Кутырло И.Э., Савенкова Н.Д., 20182018Кутырло И.Э., Савенкова Н.Д.Kutyrlo I., Savenkova N.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/365

ЦЕЛЬ. Выявить частоту и характер сочетанной врожденной аномалии развития почек и мочевых путей в структуре изолированного и синдромального CAKUT-синдрома у 100 детей. ПАЦИЕНТЫ И МЕТОДЫ. Обследовано 100 детей (57 мальчиков и 43 девочки) с CAKUT-синдромом в возрасте от 2 мес до 17 лет. РЕЗУЛЬТАТЫ. У 100 пациентов в 94% выявлен изолированный и в 6% выявлен синдромальный CAKUT-синдром. Стратификация стадий ХБП С1–5, проведенная с учётом скорости клубочковой фильтрации, у 85 детей с CAKUT (в возрасте 2–17 лет) показала преобладание С1 (74%).

THE AIM. Identify the frequency and nature of the combined congenital anomaly of kidney and urinary tract development in the structure of isolated and syndromic CAKUT syndrome in 100 children. PATIENTS AND METHODS. 100 children (57 boys and 43 girls) with CAKUT syndrome, aged 2 months to 17 years, were examined. RESULTS. Out of 100 patients, 94% were isolated and 6% had syndrome CAKUT syndrome. Stratification of stage of CKD C1–5 was performed taking into account the glomerular filtration rate in 85 children with CAKUT (aged 2–17 years), the predominance С1 (74%).

CAKUT-синдромхроническая болезнь почекдетиCAKUT-syndromechronic kidney diseasechildrenReferencesИгнатова МС. Дизэмбиогенез органов мочевой системы и нефропатии. Детская нефрология. М.С. Игнатова, ред. 2011; 10–15Ignatova M. S. Dizehmbiogenez organov mochevoj sistemy i nefropatii. Detskaya nefrologiya. Pod red. M. S. Ignatovoj. 2011; 10–15Папаян АВ, Савенкова НД. Клиническая нефрология детского возраста. «Левша. Санкт-Петербург», СПб., 2008; 600Papayan AV, Savenkova ND. Klinicheskaya nefrologiya detskogo vozrasta. «Levsha. Sankt-Peterburg», SPb., 2008; 600Игнатова МС. CAKUT-синдром у детей. Педиатрия 2012; 91(6):141–144Ignatova M.S. CAKUT-syndrom u detei. Pedaitria 2012; 91(6):141–144Кутырло ИЭ, Савенкова НД. CAKUT-синдром у детей. Нефрология 2017;21(3):18–24Kutyrlo IE, Savenkova ND. CAKUT-syndrom u detei. Nephrologia 2017;21(3):18–24Vivante A, Kohl S, Hwang DY et al. Single-gene causes of congenital anomalies of the kidney and urinary tract (CAKUT) in humans. Pediatr Nephrol 2014; (29): 695–704Vivante A, Kohl S, Hwang DY et al. Single-gene causes of congenital anomalies of the kidney and urinary tract (CAKUT) in humans. Pediatr Nephrol 2014; (29): 695–704Лопаткин НА. Аномалии развития почек, мочевых путей и мужских половых органов. В: Урология. «Медицина», М., 2013; 147–210Lopatkin N. A. Anomalii razvitiya pochek, mochevyih putey i muzhskih polovyih organov. V: Urologiya. «Meditsina», М., 2013; 147–210Renkema KJ, Winyard PY, Skovorodkin IN, et al. Congenital anomalies of the kidney and urinary tract (CAKUT). Nephrol Dual Transplant 2012; 8 (12): 3842–3851Renkema KJ, Winyard PY, Skovorodkin IN, et al. Congenital anomalies of the kidney and urinary tract (CAKUT). Nephrol Dual Transplant 2012; 8 (12): 3842–3851Nicolaou N, Renkema KY, Bongers EM et all. Genetic, environmental, and epigenetic factors involved in CAKUT. Nat Rev Nephrol 2015;11(12):720–731Nicolaou N, Renkema KY, Bongers EM et all. Genetic, environmental, and epigenetic factors involved in CAKUT. Nat Rev Nephrol 2015;11(12):720–731Danziger P, Berman DR, Luckritz K et al. Severe congenital anomalies of the kidney and urinary tract: epidemiology can inform ethical decision-making. J Perinatology 2016; 36: 954–959Danziger P, Berman DR, Luckritz K et al. Severe congenital anomalies of the kidney and urinary tract: epidemiology can inform ethical decision-making. J Perinatology 2016; 36: 954–959Длин ВВ, Османов ИМ, Чугунова ОЛ. Инфекция мочевой системы у детей. «Оверлей», М., 2017; 422Dlin V.V., Osmanov I.M., Chugunova O.L. Infekciya mochevoy sistemyi u detei. «Overley», М., 2017; 422K/DOOQ, Clinical Practice Guidelines for Chronic Kidney Disease: Evaluation Classification Stratification. Am J Kidney Dis 2002; 39 (2 Suppl. 1): 1–266K/DOOQ, Clinical Practice Guidelines for Chronic Kidney Disease: Evaluation Classification Stratification. Am J Kidney Dis 2002; 39 (2 Suppl. 1): 1–266Hogg RJ, Furth S, Lemley KV et al. National Kidney Foundation’s KidneyDisease Outcomes Quality Initiative clinical practice guidelines for chronic kidney disease in children and adolescents: evaluation, classification, and stratification. Pediatrics 2003;111:1416–1421Hogg RJ, Furth S, Lemley KV et al. National Kidney Foundation’s KidneyDisease Outcomes Quality Initiative clinical practice guidelines for chronic kidney disease in children and adolescents: evaluation, classification, and stratification. Pediatrics 2003;111:1416–1421Schnaper H.W/ Patophysiology of prigressive Renal Diasease in Children. Pediatric Nephrology. Eds: Avner E.D., Harmon W.E., Niaudet P, Yoshikawa N. 7 Completely Revised, Update and Enlerge Edition, Vol2, Springer-Verlag Berlin Heidelberg. 2016; 2171–2207Schnaper H.W/ Patophysiology of prigressive Renal Diasease in Children. Pediatric Nephrology. Eds: Avner E.D., Harmon W.E., Niaudet P, Yoshikawa N. 7 Completely Revised, Update and Enlerge Edition, Vol2, Springer-Verlag Berlin Heidelberg. 2016; 2171–2207Смирнов АВ, Шилов ЕМ, Добронравов ВА и др. На-циональные рекомендации. Хроническая болезнь почек: основные принципы скрининга, диагностики, профилактики и подходы к лечению. «Левша», СПб., 2012; 54Smirnov AV, SHilov EM, Dobronravov VA i dr. Nacional’nye rekomendacii. Hronicheskaya bolezn’ pochek: osnovnye principy skrininga, diagnostiki, profilaktiki i podhody k lecheniyu. «Levsha», Sankt-Peterburg, 2012; 54Laglois V. Laboratory Evalution at Different Ages. In: Comprehensive pediatric nephrology. Eds. D.F. Geary, F. Schaefer Mosby Elsevier, Philadelphia. 2008; 39–54Laglois V. Laboratory Evalution at Different Ages. In: Comprehensive pediatric nephrology. Eds. D.F. Geary, F. Schaefer Mosby Elsevier, Philadelphia. 2008; 39–54Stoll C, Dott B, Alembik Y, Roth MP. Associated nonurinary congenital anomalies among infants with congenital anomalies of kidney and urinary tract (CAKUT). Eur J Med Genet 2014; 57(7):322–328Stoll C, Dott B, Alembik Y, Roth MP. Associated nonurinary congenital anomalies among infants with congenital anomalies of kidney and urinary tract (CAKUT). Eur J Med Genet 2014; 57(7):322–328Левиашвили ЖГ, Савенкова НД, Аничкова ИВ. Особенности патологии почек у детей с Lowe синдромом. Нефрология 2015; 19(6): 53–60Leviashvili ZhG, Savenkova ND, Anichkova IV. Оsobennosti patologii pochek u detey s Lowe sindromom. Nefrologiya 2015; 19 (6): 53–60Renkema KJ, Winpred PJ, Scovorodkin IN et al. Nonel Perspectives for undestending congenital anomalies of the kidney and urinary tract (CAKUT). Nephrol dial Transplant 2011; 26(14): 3841–3851Renkema KJ, Winpred PJ, Scovorodkin IN et al. Nonel Perspectives for undestending congenital anomalies of the kidney and urinary tract (CAKUT). Nephrol dial Transplant 2011; 26(14): 3841–3851Ishikura К, Uemura O, Hamasaki Y et al. Insignificant impact of VUR on the progression of CKD in children with CAKUT. Pediatr Nephrol 2016; 31:105–112Ishikura К, Uemura O, Hamasaki Y et al. Insignificant impact of VUR on the progression of CKD in children with CAKUT. Pediatr Nephrol 2016; 31:105–112Harambat J, van Stalen KJ, Kim JJ et al. Epidemiology of chronic kidney diseases. Pediatr Nephrol 2012; 27(3): 363–373Harambat J, van Stalen KJ, Kim JJ et al. Epidemiology of chronic kidney diseases. Pediatr Nephrol 2012; 27(3): 363–373Лысова ЕВ, Савенкова НД. Частота CAKUT-синдрома в этиологической структуре хронической болезни почек у детей и подростков. Росс вестн перинатол педиатр 2016; 61: 212–213Lysova EV, Savenkova ND. Chastota CAKUT-sindroma v ehtiologicheskoj strukture hronicheskoj bolezni pochek u detej i podrostkov. Ross vest perinatol pediatr 2016; 61: 212–213Cordell H, Darlay R, Charoen P et al. Whole-Genome Linkage and Association Scan in Primary, Non synndromatic Vesicoureteral Reflux. J Am Soc Nephrol 2010; 21(1):113–123Cordell H, Darlay R, Charoen P et al. Whole-Genome Linkage and Association Scan in Primary, Non synndromatic Vesicoureteral Reflux. J Am Soc Nephrol 2010; 21(1):113–123Вялкова АА. Хроническая болезнь почек. Оренбургский медицинский вестник 2015; 42–51Vailkova AA. Chronicheskay bolezn pochek. Orenburgskiy med vestnik 2015; 42–51Вялкова АА. Современное представление о тубулоинтерстициальных нефропатиях и концепция хронической болезни почек в практической нефрологии. Педиатрия 2008; 129–131Vjalkova AA. Sovremennoe predstavlenie o tubulointersticial’nyh nefropatijah i koncepcija hronicheskoj bolezni pochek v prakticheskoj nefrologii. Pediatrija 2008; 129–131

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