perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”10.21508/1027-4065-2019-64-2-22-29perinatology-852Research ArticleОБЗОР ЛИТЕРАТУРЫREVIEWSЛечение аутосомно-рецессивного и аутосомно-доминантного поликистоза почекTreatment of autosomal recessive and autosomal dominant polycystic kidney diseasehttps://orcid.org/0000-0002-8753-1415АндрееваЭ. Ф.AndreevaE. F.

Андреева Эльвира Фаатовна – к.м.н., ассистент кафедры факультетской педиатрии.

194100 С.-Петербург, ул. Литовская, д. 2. 

A-Elvira@yandex.ruhttps://orcid.org/0000-0002-9415-4785СавенковаН. Д.SavenkovaN. D.

Савенкова Надежда Дмитриевна – д.м.н., проф., зав. кафедрой факультетской педиатрии.

194100 С.-Петербург, ул. Литовская, д. 2.

Saint-Petersburg

ФГБОУ ВО «Санкт-Петербургский государственный педиатрический медицинский университет» Минздрава РоссииРоссияSaint-Petersburg State Pediatric Medical UniversityRussian Federation2019140520196422229Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20192019Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/852

В статье отражены генетические варианты поликистозной болезни почек, описана современная стратегия лечения поликистоза почек у детей и взрослых. Представлены результаты клинических испытаний антагонистов рецептора вазопрессина V2 (толваптан, ликсиваптан), ингибитора мультикиназы (тесеватиниб), аналогов соматостатина (ланкреотид, октреотид), статинов (правастатин), ингибиторов mТОР (эверолимус, сиролимус), метформина у пациентов с аутосомно-рецессивной и аутосомно-доминантной поликистозной болезнью почек. Обсуждены факторы, определяющие прогноз и исход при данных заболеваниях.

The article reflects the genetic variants of polycystic kidney disease, describes the modern strategy for the treatment of polycystic kidney disease in children and adults. The authors present the results of clinical trials of vasopressin V2 receptor antagonists (tolvaptan, liksivaptan), a multi-kinase inhibitor (tezevatinib), somatostatin analogues (lankreotide, octreotide), statins (pravastatin), mTOR inhibitors (everolimus, sirolimus), metformin in patients with autosomal recessive and autosomal polycystic kidney disease. The authors discuss the factors determining the prognosis and outcome of these diseases.

детиполикистоз почеклечениепрогнозchildrenpolycystic kidney diseasetreatmentprognosisReferencesИгнатова М.С., Длин В.В. Роль генетики в развитии детской нефрологии. Российский вестник перинатологии и педиатрии 2015; 60(3): 6–9.Ignatova M.S., Dlin V.V. Role of genetics in the development of pediatric nephrology. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics) 2015; 60(3): 6–9. (in Russ)Николаева Е.А., Семячкина А.Н. Современные возможности лечения наследственных заболеваний у детей. Российский вестник перинатологии и педиатрии 2018; 63(4): 6–14. DOI: 10.21508/1027-4065-2018-63-4-6-14Nikolaeva E.A., Semyachkina A.N. Modern possibilities of hereditary diseases treatment in children. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics) 2018; 63(4): 6–14. (in Russ) DOI: 10.21508/1027-4065-2018-63-4-6-14Bergmann C. Genetics of autosomal recessive polycystic kidney disease and its differential diagnoses. Front Pediatr 2018; 5:221–234. DOI: 10.3389/fped.2017.00221Bergmann C. Genetics of autosomal recessive polycystic kidney disease and its differential diagnoses. Front Pediatr 2018; 5:221–234. DOI: 10.3389/fped.2017.00221Bergmann C., Guay-Woodford L.M., Harris P.C., Horie S., Peters D.J. M., Torres V.E. Polycystic kidney disease. Nature Reviews Disease Primers 2018; 4(1): 50–74. DOI: 10.1038/s41572-018-0047-yBergmann C., Guay-Woodford L.M., Harris P.C., Horie S., Peters D.J. M., Torres V.E. Polycystic kidney disease. Nature Reviews Disease Primers 2018; 4(1): 50–74. DOI: 10.1038/s41572-018-0047-yAymé S., Bauerenhauer D., Day S., Devuyst O., Guay-Woodford L. M., Ingelfinger J.R. et al. Common Elements in Rare Kidney Diseases: Conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Intern 2017; 92(4): 796–808. DOI: 10.1016/j.kint.2017.06.018Aymé S., Bauerenhauer D., Day S., Devuyst O., Guay-Woodford L. M., Ingelfinger J.R. et al. Common Elements in Rare Kidney Diseases: Conclusions from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Intern 2017; 92(4): 796–808. DOI: 10.1016/j.kint.2017.06.018The National Library of Medicine (NLM). ClinicalTrials.gov. https://clinicaltrials.gov (ссылка активна на 20.12.2018).The National Library of Medicine (NLM). ClinicalTrials.gov. https://clinicaltrials.gov (ссылка активна на 20.12.2018).Polycystic Kidney Disease foundation. ttps://pkdcure.org. Ссылка активна на 20.12.2018.Polycystic Kidney Disease foundation. ttps://pkdcure.org. Ссылка активна на 20.12.2018.Patil A., Sweeney Jr. W.E., Avner E.D., Pan C. Childhood Polycystic Kidney Disease. In: X. Li (ed.). Polycystic Kidney Disease. Codon Publications, Brisbane, Australia, 2015; 21–60. DOI: 10.15586/codon.pkd.2015Patil A., Sweeney Jr. W.E., Avner E.D., Pan C. Childhood Polycystic Kidney Disease. In: X. Li (ed.). Polycystic Kidney Disease. Codon Publications, Brisbane, Australia, 2015; 21–60. DOI: 10.15586/codon.pkd.2015Harutyunyan S., Andreeva E., Savenkova N., Larionova V., Leviashvili G. Extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). Pediatr Nephrol 2011; 26(9): 1691.Harutyunyan S., Andreeva E., Savenkova N., Larionova V., Leviashvili G. Extrarenal manifestation of autosomal dominant polycystic kidney disease (ADPKD). Pediatr Nephrol 2011; 26(9): 1691.Bonsib S.M. The Classification of Renal Cystic Diseases and Other Congenital Malformations of the Kidney and Urinary Tract. Arch Pathol Lab Med 2010; 134 (4): 554–567. DOI: 10.1043/1543-2165-134.4.554Bonsib S.M. The Classification of Renal Cystic Diseases and Other Congenital Malformations of the Kidney and Urinary Tract. Arch Pathol Lab Med 2010; 134 (4): 554–567. DOI: 10.1043/1543-2165-134.4.554Андреева Э.Ф., Савенкова Н.Д. Кистозные болезни почек у детей (обзор литературы). Нефрология 2012; 16(3/2): 34–47. DOI: 10.24884/1561-6274-2012-16-3/2-34-47Andreeva E.F., Savenkova N.D. Cystic kidney disease in children (literature review). Nephrology 2012; 16 (3/2): 34–47. (in Russ) DOI: 10.24884/1561-6274-2012-16-3/2-34-47Андреева Э.Ф. Катамнез детей и подростков с поликистозом почек. Нефрология 2016; 20(3): 60–68.Andreeva E.F. The follow-up of children and adolescents with polycystic kidney disease. Nephrology 2016; 20(3): 60–68. (in Russ)Online Mendelian Inheritance in Man. http://omim.org (ссылка активна на 20.12.2018).Online Mendelian Inheritance in Man. http://omim.org (ссылка активна на 20.12.2018).Андреева Э.Ф., Савенкова Н.Д., Тилуш М.А, Наточина Н.Ю., Дюг И.В. Особенности течения аутосомнорецессивного поликистоза почек у детей. Педиатр 2016; 7(4): 45–49.Andreeva E.F., Savenkova N.D., Tilouche M.A., Natochina N.Y., Dug I.V. Features of the course of autosomal recessive polycystic kidney disease in children. Pediatr 2016; 7(4): 45–49. (in Russ)Lu H., Galeano M.C.R., Ott E., Kaeslin G., Kausalya P.J., Kramer C. et al. Mutations in DZIP1L, which encodes a ciliary-transition-zone protein, cause autosomal recessive polycystic kidney disease. Nature Genet 2017; 49(7): 1025–1034. DOI: 10.1038/ng.3871Lu H., Galeano M.C.R., Ott E., Kaeslin G., Kausalya P.J., Kramer C. et al. Mutations in DZIP1L, which encodes a ciliary-transition-zone protein, cause autosomal recessive polycystic kidney disease. Nature Genet 2017; 49(7): 1025–1034. DOI: 10.1038/ng.3871Арутюнян С.С., Савенкова Н.Д. Характеристика почечных и внепочечных проявлений аутосомно-доминантного поликистоза почек у детей. Нефрология 2013; 17(3): 60–67. DOI: 10.24884/1561-6274-2013-17-3-60-67Arutyunyan S.S., Savenkova N.D. Characteristics of renal and extrarenal manifestations of autosomal dominant polycystic kidney disease in children. Nephrology 2013; 17(3): 60–67. (in Russ) DOI: 10.24884/1561-6274-2013-17-3-60-67Sweeney Jr.W.E., Gunay-Aygun M., Patil A., Avner E.D. Childhood Polycystic Kidney Disease In: E.D. Avner, W.E. Harmon, P. Niaudet, N. Yoshikawa, F. Emma, S.L. Goldstein (eds). Pediatric Nephrology, 7nd ed. Springer-Verlag, Berlin Heidelberg, 2016; 1103–1153. DOI: 10.1007/978-3-662-43596-0Sweeney Jr.W.E., Gunay-Aygun M., Patil A., Avner E.D. Childhood Polycystic Kidney Disease In: E.D. Avner, W.E. Harmon, P. Niaudet, N. Yoshikawa, F. Emma, S.L. Goldstein (eds). Pediatric Nephrology, 7nd ed. Springer-Verlag, Berlin Heidelberg, 2016; 1103–1153. DOI: 10.1007/978-3-662-43596-0Polycystic Kidney Disease Outcomes Consortium. Qualification of Total Kidney Volume as a Prognostic Biomarker for use in Clinical Trials Evaluating Patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD). Final Briefing Book, 2014. http://www.ema.europa.eu. Ссылка активна на 20.12.2018.Polycystic Kidney Disease Outcomes Consortium. Qualification of Total Kidney Volume as a Prognostic Biomarker for use in Clinical Trials Evaluating Patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD). Final Briefing Book, 2014. http://www.ema.europa.eu. Ссылка активна на 20.12.2018.Riella С., Czarnecki P.G., Steinman T.I. Therapeutic Advances in the Treatment of Polycystic Kidney Disease. Nephron Clin Pract 2014; 128: 297–302. DOI: 10.1159/000368244Riella С., Czarnecki P.G., Steinman T.I. Therapeutic Advances in the Treatment of Polycystic Kidney Disease. Nephron Clin Pract 2014; 128: 297–302. DOI: 10.1159/000368244Xue C., Zhou C.-C., Wu M., Mei C.-L. The Clinical Manifestation and Management of Autosomal Dominant Polycystic Kidney Disease in China. Kidney Dis 2016; 2(3): 111–119. DOI: 10.1159/000449030Xue C., Zhou C.-C., Wu M., Mei C.-L. The Clinical Manifestation and Management of Autosomal Dominant Polycystic Kidney Disease in China. Kidney Dis 2016; 2(3): 111–119. DOI: 10.1159/000449030Reddy B.V., Chapman A.B. The spectrum of autosomal dominant polycystic kidney disease in children and adolescents. Pediatr Nephrol 2016; 32(1): 31–42. DOI: 10.1007/s00467-016-3364-yReddy B.V., Chapman A.B. The spectrum of autosomal dominant polycystic kidney disease in children and adolescents. Pediatr Nephrol 2016; 32(1): 31–42. DOI: 10.1007/s00467-016-3364-yСавенкова Н.Д. Нефрогенная артериальная гипертензия у детей и подростков: причины, классификация, диагностика. Российский вестник перинатологии и педиатрии 2017; 62(4): 43–48. DOI: 10.21508/1027–4065–2017–62–4–43–48Savenkova N.D. Renal Arterial Hypertension in children and adolescents: causes, classification, diagnosis. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics) 2017; 62(4): 43–48. (in Russ) DOI: 10.21508/1027–4065–2017–62–4–43–48Sweeney Jr.W.E., Avner E.D. Emerging Therapies for Childhood Polycystic Kidney Disease. Front Pediatr 2017; 5: 77. DOI: 10.3389/fped.2017.00077Sweeney Jr.W.E., Avner E.D. Emerging Therapies for Childhood Polycystic Kidney Disease. Front Pediatr 2017; 5: 77. DOI: 10.3389/fped.2017.00077Mangolini A., Stephanis L., Aguiari G. Role of calcium in polycystic kidney disease: From signaling to pathology. World J Nephrol 2016; 5(1): 76–83. DOI: 10.5527/wjn.v5.i1.76Mangolini A., Stephanis L., Aguiari G. Role of calcium in polycystic kidney disease: From signaling to pathology. World J Nephrol 2016; 5(1): 76–83. DOI: 10.5527/wjn.v5.i1.76Папиж С.В., Длин В.В., Виноградова Т.В., Леонтьева И.В., Тутельман К.М., Фомин Д.К. и др. Роль профиброгенных цитокинов в прогрессировании почечного и сердечнососудистого повреждения у детей с аутосомно-доминантной поликистозной болезнью почек. Российский вестник перинатологии и педиатрии 2014; 59(3): 91–98.Papizh S.V., Dlin V.V., Vinogradova T.V., Leont`eva I.V., Tutel`man K.M., Fomin D.K. et al. The role of profibrogenic cytokines in the progression of renal and cardiovascular damage in children with autosomal dominant polycystic kidney disease. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics) 2014; 59(3): 91–98. (in Russ)Network for Early Onset Cystic Kidney Diseases-A Comprehensive Multidisciplinary Approach to Hereditary Cystic Kidney Diseases in Childhood. Network for Early Onset Cystic Kidney Diseases-A Comprehensive Multidisciplinary Approach to Hereditary Cystic Kidney Diseases in Childhood. Front Pediatr 2018; 6: 24. DOI: 10.3389/fped.2018.00024Network for Early Onset Cystic Kidney Diseases-A Comprehensive Multidisciplinary Approach to Hereditary Cystic Kidney Diseases in Childhood. Network for Early Onset Cystic Kidney Diseases-A Comprehensive Multidisciplinary Approach to Hereditary Cystic Kidney Diseases in Childhood. Front Pediatr 2018; 6: 24. DOI: 10.3389/fped.2018.00024Gimpel C., Avni F.E., Bergmann C., Cetiner M., Habbig S., Haffner D. et al. Perinatal diagnosis, management, and follow-up of cystic renal diseases. JAMA Pediatr 2018; 172(1): 74–86. DOI: 10.1001/jamapediatrics.2017.3938Gimpel C., Avni F.E., Bergmann C., Cetiner M., Habbig S., Haffner D. et al. Perinatal diagnosis, management, and follow-up of cystic renal diseases. JAMA Pediatr 2018; 172(1): 74–86. DOI: 10.1001/jamapediatrics.2017.3938Ebner K., Schaefer F., Liebau M.Ch., the ARegPKD Consortium. Recent Progress of the ARegPKD Registry Study on Autosomal Recessive Polycystic Kidney Disease. Front Pediatr 2017; 5: 18–23. DOI: 10.3389/fped.2017.00018Ebner K., Schaefer F., Liebau M.Ch., the ARegPKD Consortium. Recent Progress of the ARegPKD Registry Study on Autosomal Recessive Polycystic Kidney Disease. Front Pediatr 2017; 5: 18–23. DOI: 10.3389/fped.2017.00018Игнатова М.С., Кирилина С.А., Длин В.В. Агапов Е.Г., Нижегородцева Т.В. Способ лечения детей с поликистозной болезнью почек. Патентный поиск в РФ: новые патенты, заявки на патент библиотека патентов на изобретения. http://www.freepatent.ru/patents/2316322. (Ссылка активна на 20.12.2018).Ignatova M.S., Kirilina S.A., Dlin V.V. Agapov E.G., Nizhegorodceva T.V. A method of treating children with polycystic kidney disease. Patent search in Russia: new patents, patent applications library patents for inventions. (in Russ) http://www.freepatent.ru/patents/2316322. (Ссылка активна на 20.12.2018).Padovano V., Podrini C., Boletta A., Caplan M.J. Metabolism and mitochondria in polycystic kidney disease research and therapy. Nat Rev Nephrol 2018; 14(11): 678–687. DOI: 10.1038/s41581-018-0051-1Padovano V., Podrini C., Boletta A., Caplan M.J. Metabolism and mitochondria in polycystic kidney disease research and therapy. Nat Rev Nephrol 2018; 14(11): 678–687. DOI: 10.1038/s41581-018-0051-1Janssens P., Weydert C., De Rechter S., Wissing K.M., Liebau M.C., Mekahli D. Expanding the role of vasopressin antagonism in polycystic kidney diseases: From adults to children? Pediatr Nephrol 2018; 33(3): 395–408. DOI: 10.1007/s00467-017-3672-xJanssens P., Weydert C., De Rechter S., Wissing K.M., Liebau M.C., Mekahli D. Expanding the role of vasopressin antagonism in polycystic kidney diseases: From adults to children? Pediatr Nephrol 2018; 33(3): 395–408. DOI: 10.1007/s00467-017-3672-xCasteleijn N.F., Blais J.D., Chapman A.B., Czerwiec F.S., Devuyst O., Higashihara E. et al. Tolvaptan and kidney pain in patients with autosomal dominant polycystic kidney disease: secondary analysis from a randomized controlled trial. Am J Kidney Dis 2017; 69(2): 210–219. DOI: 10.1053/j.ajkd.2016.08.028Casteleijn N.F., Blais J.D., Chapman A.B., Czerwiec F.S., Devuyst O., Higashihara E. et al. Tolvaptan and kidney pain in patients with autosomal dominant polycystic kidney disease: secondary analysis from a randomized controlled trial. Am J Kidney Dis 2017; 69(2): 210–219. DOI: 10.1053/j.ajkd.2016.08.028Morimoto K., Akai Y., Matsui M., Yano H., Tagawa M., Samejima K. et al. Acute pulmonary thromboembolism occurring during treatment with tolvaptan in a patient with autosomaldominant polycystic kidney disease. CEN Case Reports 2017; 6(1): 61–65. DOI: 10.1007/s13730-016-0245-yMorimoto K., Akai Y., Matsui M., Yano H., Tagawa M., Samejima K. et al. Acute pulmonary thromboembolism occurring during treatment with tolvaptan in a patient with autosomaldominant polycystic kidney disease. CEN Case Reports 2017; 6(1): 61–65. DOI: 10.1007/s13730-016-0245-yIrazabal M.V., Rangel L.J., Bergstralh E.J., Osborn S.L., Harmon A.J., Sundsbak J. L. et al. Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials. J Am Soc Nephrol 2014; 26(1): 160–172. DOI: 10.1681/asn.2013101138Irazabal M.V., Rangel L.J., Bergstralh E.J., Osborn S.L., Harmon A.J., Sundsbak J. L. et al. Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials. J Am Soc Nephrol 2014; 26(1): 160–172. DOI: 10.1681/asn.2013101138Gansevoort R.T., Arici M., Benzing T., Birn H., Capasso G., Covic A. еt al. Recommendations for the use of tolvaptan in autosomal dominant polycystic kidney disease: a position statement on behalf of the ERA-EDTA working groups on inherited kidney disorders and European renal best practice. Nephrol Dial Transplant 2016; 31: 337–348. DOI: 10.1093/ndt/gfv456Gansevoort R.T., Arici M., Benzing T., Birn H., Capasso G., Covic A. еt al. Recommendations for the use of tolvaptan in autosomal dominant polycystic kidney disease: a position statement on behalf of the ERA-EDTA working groups on inherited kidney disorders and European renal best practice. Nephrol Dial Transplant 2016; 31: 337–348. DOI: 10.1093/ndt/gfv456Gevers T.J.G., Hol J.C., Monshouwer R., Dekker H.M., Wetzels J.F.M., Drenth J.P.H. Effect of lanreotide on polycystic liver and kidneys in autosomal dominant polycystic kidney disease: an observational trial. Liver Int 2014; 35(5): 1607–1614. DOI: 10.1111/liv.12726Gevers T.J.G., Hol J.C., Monshouwer R., Dekker H.M., Wetzels J.F.M., Drenth J.P.H. Effect of lanreotide on polycystic liver and kidneys in autosomal dominant polycystic kidney disease: an observational trial. Liver Int 2014; 35(5): 1607–1614. DOI: 10.1111/liv.12726Hogan M.C., Masyuk T.V., Page L.J., Kubly V.J., Bergstralh E.J., Li X. et al. Randomized Clinical Trial of LongActing Somatostatin for Autosomal Dominant Polycystic Kidney and Liver Disease. J Am Soc Nephrol 2010; 21(6): 1052–1061. DOI: 10.1681/asn.2009121291Hogan M.C., Masyuk T.V., Page L.J., Kubly V.J., Bergstralh E.J., Li X. et al. Randomized Clinical Trial of LongActing Somatostatin for Autosomal Dominant Polycystic Kidney and Liver Disease. J Am Soc Nephrol 2010; 21(6): 1052–1061. DOI: 10.1681/asn.2009121291Pisani A., Sabbatini M., Imbriaco M., Riccio E., Rubis N., Prinster A. et al. Long-term effects of octreotide on liver volume in patients with polycystic kidney and liver disease. Clin Gastroenterol Hepatol 2016; 14(7): 1022–1030. DOI: 10.1016/j.cgh.2015.12.049Pisani A., Sabbatini M., Imbriaco M., Riccio E., Rubis N., Prinster A. et al. Long-term effects of octreotide on liver volume in patients with polycystic kidney and liver disease. Clin Gastroenterol Hepatol 2016; 14(7): 1022–1030. DOI: 10.1016/j.cgh.2015.12.049Guay-Woodford L.M., Bissler J.J., Braun M.C., Bockenhauer D., Cadnapaphornchai M.A., Dell K.M. et al. Consensus Expert Recommendations for the Diagnosis and Management of Autosomal Recessive Polycystic Kidney Disease: Report of an International Conference. J Pediatr 2014; 165(3): 611–617. DOI: 10.1016/j.jpeds.2014.06.015Guay-Woodford L.M., Bissler J.J., Braun M.C., Bockenhauer D., Cadnapaphornchai M.A., Dell K.M. et al. Consensus Expert Recommendations for the Diagnosis and Management of Autosomal Recessive Polycystic Kidney Disease: Report of an International Conference. J Pediatr 2014; 165(3): 611–617. DOI: 10.1016/j.jpeds.2014.06.015Deep A., Saxena R., Jose B. Acute kidney injury in children with chronic liver disease. Pediatr Nephrol 2019; 34: 45–59. DOI: 10.1007/s00467-018-3893-7Deep A., Saxena R., Jose B. Acute kidney injury in children with chronic liver disease. Pediatr Nephrol 2019; 34: 45–59. DOI: 10.1007/s00467-018-3893-7Bockenhauer D. Hyponatremia and cyst growth in neonatal polycystic kidney disease: a case for aquaretics? Pediatr Nephrol 2017; 32: 721–723. DOI: 10.1007/s00467-017-3578-7Bockenhauer D. Hyponatremia and cyst growth in neonatal polycystic kidney disease: a case for aquaretics? Pediatr Nephrol 2017; 32: 721–723. DOI: 10.1007/s00467-017-3578-7Абеуова Б.А., Нигматуллина Н.Б., Алтынова В.Х., Ахмадьяр Н.С. Клинический протокол диагностики и лечения. Кистозная болезнь почек у детей. МЗ Республики Казахстан, протокол №10 от 4 июля 2014 года. https://docplayer.ru/36612925-Klinicheskiy-protokol-diagnostiki-ilecheniya-kistoznaya-bolezn-pochek-u-detey. (Ссылка активна на 20.12.2018).Abeuova B.A., Nigmatullina N.B., Alty`nova V.X., Axmad`yar N.S. Clinical diagnosis and treatment protocol. Cystic kidney disease in children. Ministry of Health of the Republic of Kazakhstan, protocol № 10 dated July 4, 2014. (in Russ) https://docplayer.ru/36612925-Klinicheskiy-protokol-diagnostiki-ilecheniya-kistoznaya-bolezn-pochek-u-detey. (Ссылка активна на 20.12.2018).Grenda R., Kaliciński P. Combined and sequential liver–kidney transplantation in children. Pediatr Nephrol 2018; 33(12): 2227–2237. DOI: 10.1007/s00467-017-3880-4Grenda R., Kaliciński P. Combined and sequential liver–kidney transplantation in children. Pediatr Nephrol 2018; 33(12): 2227–2237. DOI: 10.1007/s00467-017-3880-4Gilbert R.D., Evans H., Olalekan K. Tolvaptan treatment for severe neonatal autosomal-dominant polycystic kidney disease. Pediatr Nephrol 2017; 32: 893–896. DOI: 10.1007/s00467-017-3584-9Gilbert R.D., Evans H., Olalekan K. Tolvaptan treatment for severe neonatal autosomal-dominant polycystic kidney disease. Pediatr Nephrol 2017; 32: 893–896. DOI: 10.1007/s00467-017-3584-9Cadnapaphornchai M.A., George D.M., McFann K., Wang W., Gitomer B., Strain J.D. et al. Effect of pravastatin on total kidney volume, left ventricular mass index, and microalbuminuria in pediatric autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol 2014; 9(5): 889–896. DOI: 10.2215/cjn.08350813Cadnapaphornchai M.A., George D.M., McFann K., Wang W., Gitomer B., Strain J.D. et al. Effect of pravastatin on total kidney volume, left ventricular mass index, and microalbuminuria in pediatric autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol 2014; 9(5): 889–896. DOI: 10.2215/cjn.08350813

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