nefrНефрологияNephrology (Saint-Petersburg)1561-62742541-9439Pavlov First Saint-Petersburg State Medical University10.24884/1561-6274-2017-21-2-62-72nefr-255Research ArticleОРИГИНАЛЬНЫЕ СТАТЬИ. КЛИНИЧЕСКИЕ ИССЛЕДОВАНИЯORIGINAL ARTICLES. CLINICAL INVESTIGATIONSФАКТОРЫ РИСКА ПОРАЖЕНИЯ СЕРДЕЧНО-СОСУДИСТОЙ СИСТЕМЫ У ДЕТЕЙ С АУТОСОМНО-ДОМИНАНТНОЙ ПОЛИКИСТОЗНОЙ БОЛЕЗНЬЮ ПОЧЕКRISK FACTORS FOR CARDIOVASCULAR COMPLICATIONS IN CHILDREN WITH AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASEПапижС. В.PapizhS. V.

Папиж Светлана Валентиновна, кандидат медицинских наук,  , старший научный сотрудник.

Отдел наследственных и приобретенных болезней почек, научно-исследовательского клинического института педиатрии им. акад. Ю.Е. Вельтищева.

125412, Москва, ул. Талдомская, д. 2.

Prof. Larisa S. Prikhodina, MD, PhD. Research Clinical Institute for Pediatrics n.a. acad. Y.E. Veltishev, Division of Inherited and Acquired Kidney Diseases . 125412 Moscow, Taldomskaya St., 2.

 

papijsveta@mail.ruДлинВ. В.DlinV. V.

Профессор Длин Владимир Викторович, руководитель отдела.  

Отдел наследственных и приобретенных болезней почек Научно-исследовательского клинического института педиатрии им. акад. Ю.Е. Вельтищева. 

125412, Россия, Москва, ул. Талдомская, д. 2. 

Prof. Vladimir V. Dlin, MD, PhD, DMedSci 

Research Clinical Institute for Pediatrics n.a. acad. Y.E. Veltishev, Department of hereditary and acquired diseases of the kidneys

125412 Russia, Moscow, Taldomskaya st., 2

vdlin@pedklin.ruЛеонтьеваИ. В.LeontievaI. V.

Профессор Леонтьева Ирина Викторовна, главный научный сотрудник. 

Отдел детской кардиологии и аритмологии Научно-исследовательского клинического института педиатрии им. акад. Ю.Е. Вельтищева.

125412, Россия, Москва, ул. Талдомская, д. 2. 

Prof. Irina V. Leontieva, MD, PhD, DMedSci 

Research Clinical Institute for Pediatrics n.a. acad. Y.E. Veltishev, Department of pediatric cardiology and arrhythmology

125412 Russia, Moscow, Taldomskaya st., 2

lirina2006@mail.ruТутельманК. М.TutelmanK. M.

Тутельман Константин Моисеевич, кандидат медицинских наукю

Отделение функциональной диагностики Научно-исследовательского клинического института педиатрии им. акад. Ю.Е. Вельтищева.

125412, Россия, Москва, ул. Талдомская, д. 2. 

Konstantin M. Tutelman MD, PhD 

Research Clinical Institute for Pediatrics n.a. acad. Y.E. Veltishev, Department of functional diagnostics

125412 Russia, Moscow, Taldomskaya st., 2

tutelman@mail.ruРоссийский национальный исследовательский медицинский университет им. Н.И. ПироговаРоссияPirogov Russian National Research Medical UniversityRussian Federation2017030320172126272Copyright © Папиж С.В., Длин В.В., Леонтьева И.В., Тутельман К.М., 20172017Папиж С.В., Длин В.В., Леонтьева И.В., Тутельман К.М.Papizh S.V., Dlin V.V., Leontieva I.V., Tutelman K.M.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/255

ЦЕЛЬ: выявить частоту и факторы риска поражения сердечно-сосудистой системы у детей с аутосомно-доминантной поликистозной болезнью почек (АДПБП). ПАЦИЕНТЫ И МЕТОДЫ: Было обследовано 54 ребенка (27 м/27 д) с АДПБП, медиана возраста составила 12,0 (8,0;15,0) лет. Всем детям было проведено ЭХО-кардиографическое исследование по стандартной методике с последующим расчетом массы миокарда левого желудочка (ММЛЖ), индекса массы миокарда левого желудочка (ИММЛЖ) (отношение ММЛЖ к росту 2,7), относительной толщины стенок левого желудочка (ОТСЛЖ). Нормальной считали геометрию левого желудочка при величине ОТСЛЖ<0,42-го и ИММЛЖ<95-го перцентиля. При ИММЛЖ≥95-го перцентиля и ОТЗСЛЖ>0,42 диагностировалась концентрическая ГМЛЖ. Эксцентрическую ГМЛЖ определяли при ИММЛЖ≥95-го перцентиля и ОТСЛЖ>0,42. Концентрическое ремоделирование определяли при ИММЛЖ<95-го перцентиля и ОТСЛЖ>0,42. В зависимости от результатов СМАД выделялась АГ (АД≥95-го перцентиля), высокое нормальное АД (90-й перцентиль ≤АД>95 перцентиль) и нормальный уровень АД (<90-го перцентиля). Суммарный объем почек (см3) определяли по результатам УЗИ с коррекцией на стандартную поверхность тела и оценкой по центильным таблицам. Статическую нефросцинтиграфию с внутривенным введением ТС-99м-Технемек (ДМСА) и расчетом индекса интегрального захвата (ИИЗ) проводили 25 детям. РЕЗУЛЬТАТЫ. У детей с АДПБП АГ была диагностирована в 42,5% случаев, высокое нормальное АД имело место в 18,5% случаев. Систолическая АГ в течение суток статистически значимо чаще выявлялась в группе детей с суммарным объемом почек, более чем на 50% превышающим 97-й перцентиль, по сравнению с группой детей с суммарным объемом почек менее 97-го перцентиля (p=0,03, ОР=2,9 (95% ДИ:1,4–4,9). Структурно-функциональные перестройки сердца в виде гипертрофии и ремоделирования миокарда ЛЖ были выявлены в 14,8% случаев у детей с АДПБП (концентрическая ГМЛЖ – 7,4%; эксцентрическая ГМЛЖ – 3,7%; ремоделирование по концентрическому типу – 3,7%). У детей с ИММЛЖ>90-го перцентиля частота повышения систолического АД в дневное время и диастолического АД в ночное время более 95-го перцентиля была статистически значимо выше, чем у детей с ИММЛЖ<90-го перцентиля: 54% против 18% (p=0,04), ОР=1,81 (95% ДИ:0,93–3,5) и 55% против 16% (p=0,008), ОР=2,2 (95% ДИ:0,98–4,6 соответственно). При систолодиастолической АГ отмечается более высокий уровень ИММЛЖ по сравнению с изолированной диастолической АГ [34,15 (30,7; 39) г/м2 против 22,77 (22,04; 23,5) г/м2 (p = 0,03)]. У детей с ИММЛЖ>90‰ статически значимо чаще выявляли увеличение объема почек и снижение ИИЗ по сравнению с детьми с ИММЛЖ<90-го перцентиля [(p=0,04), ОР=1,7 (95% ДИ:1,1-2,6) и (р=0,04), ОР=1,8 (95% ДИ: 1,1–3,07)]. ЗАКЛЮЧЕНИЕ. Фактором риска развития АГ у детей с АДПБП является увеличение объема почек. Факторами риска развития ГМЛЖ являются систолическая АГ в дневное время, диастолическая АГ в ночное время, увеличение суммарного объема почек и снижение ИИЗ по результатам статической нефросцинтиграфии. 

THE AIM: to identify the frequency and risk factors of cardiovascular lesions in children with ADPKD. PATIENTS AND METHODS: 54 children (27M/27F) with ADPKD were examined. The median age was 12 (IQR: 8.0;15.0) years. Standard two-dimensional echocardiogram was performed. LV mass (LVM) was calculated, normalized to height2.7 and estimated by centile tables. Relative wall thickness (RWT) was calculated. Patterns of abnormal LV geometry were defined as follows: LV concentric remodelling by normal LVMI and RWT ≥0.42; eccentric LV hypertrophy (LVH) by increased LVMI and RWT <0.42; concentric LVH by increased LVMI and RWT ≥0.42. We checked blood pressure with ABPM. Patients were divided into 3 groups according to three levels of BP: hypertension (HBP; greater than the 95th percentile for sex, age, and height), high normal blood pressure (HNBP; 90–95th percentile), and normotension (NBP; less than the 90th percentile). Total kidney volume (cm3) was assessed by ultrasound, corrected for standard body surface and estimated by centile tables. Renal scintigraphy with 99mTc-dimercaptosuccinic acid (DMSA) with the calculation of the integral index of capture (IIC) was performed. RESULTS: Hypertension was found in 42,5 % of cases, HNBP was in 18,5% of cases. HBP were detected more frequently in children with increased renal volume (cm3/1,73m2) more than 97‰+≥50% compared with children with renal volume less than 97‰: (p=0.03), RR=2.9 (95% CI:1.4-4.9). Changes in the structure and geometry of the LV were identified in 14.8% of cases (concentric LVH – 7,4%; eccentric LVH -3,7%; LV concentric remodeling -3,7%). Children with LVMI >90 percentile were more frequently detected systolic hypertension in the daytime, diastolic hypertension at night compared with children with LVMI <90 percentile: 54% vs. 18% (p=0.04), RR=1.81 (95% CI:0.93-3.5) and 55% vs. 16% (p=0.008), RR=2.2 (95% CI:0.98-4.6). LVMI in children with systolic and diastolic hypertension was significantly higher than in children with isolated diastolic hypertension: 34.15 (30.7; 39) vs. 22.77 (22.04; 23.5) (p = 0.03). Increased renal volume (cm3/1,73m2) more than 97‰ and decreased IIC by DMSA were detected more frequently in children with LVMI>90 percentile compared with children with LVMI <90 percentile: (p=0.04), RR=1.7 (95% CI:1.1-2.6) and (р=0.04), RR=1.8 (95% CI:1.1-3.07). CONCLUSION: Risk factor for hypertension in children with ADPKD is increased renal volume. Risk factors for the development of left ventricular hypertrophy are systolic hypertension in the daytime, diastolic hypertension at night, increased kidney volume, and decreased IIC by DMSA. 

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