Email this article 
Autosomal-recessive polycystic kidney disease in children
- Authors: Andreeva E.F1, Savenkova N.D1, Tilouche M.A1, Natochina N.Y1, Dug I.V1
-
Affiliations:
- St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
- Issue: Vol 7, No 4 (2016)
- Pages: 45-49
- Section: Articles
- URL: https://journals.eco-vector.com/pediatr/article/view/5966
- DOI: https://doi.org/10.17816/PED7445-49
- ID: 5966
Cite item
Open Access
Access granted
Abstract
The aim of the study was to assess the features of development of renal and extrarenal cysts, arterial hypertension, syndrome of portal hypertension in autosomal recessive polycystic kidney disease (ARPKD) in children. Patients and methods. With the aim of establishing the type of inheritance of polycystic kidney disease the genealogical analysis of 12 families, clinical ultrasound of the kidneys and abdominal organs, computed tomography. The study included 14 children with ARPKD. Conducted follow-up study of 14 children with ARPKD to determine the age by the detection of cysts based on ultrasound, the features of the initial clinical manifestations and course, complications and outcome.
Results: the Age of the children back to the time of detection of the cysts in the kidneys based on ultrasound when ARPKD was 2.3 ± 0.4 month. Identified a high incidence of arterial hypertension in neonates and infants with ARPKD at 92.9%. Extrarenal location of the cysts is set at 71.4%. Syndrome of portal hypertension, bleeding from varicose veins of esophagus and stomach, melanau installed in 5 (35,7%) children. Of the 14 in 5 (35,7%) patients diagnosed ARPKD children with liver fibrosis, which has a favorable prognosis without the formation of renal failure in infants and early childhood, 9 (64,3%) diagnosed with classic ARPKD in neonates and infants that is characterized by progression to end-stage renal disease in the first year of life.
Full Text
About the authors
Elvira F Andreeva
St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
Author for correspondence.
Email: A-Elvira@yandex.ru
MD, PhD, Assistant Dept. of Faculty Pediatric Russian Federation
Nadejda D Savenkova
St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
Email: savenkova.n.spb@mail.ru
MD, PhD, Dr. Med. Sci., prof. Dept. of Faculty Pediatric Russian Federation
Mohamed A Tilouche
St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
Email: amin_amt@hotmail.com
Postgraduate Student, Department of Faculty Pediatric Russian Federation
Natalya Y Natochina
St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
Email: smallnataly@gmail.com
Associate, Dept. of Faculty Pediatric Russian Federation
Igor V Dug
St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
Email: Dyug72@mail.ru
surgery the Department of Microsurgery Russian Federation
References
- Андреева Э.Ф. Катамнез детей и подростков с поликистозом почек // Нефрология. – 2016. – Т . 20. –
- № 3. – С. 60–68. [Andreeva EF. Katamnez detey i podrostkov s polikistozom pochek. Nefrologiya. 2016:20(3):60-68. (In Russ.)]
- Андреева Э.Ф., Папаян А.В., Савенкова Н.Д. Поликистоз почек // Папаян А.В., Савенкова Н.Д. Клиническая нефрология детского возраста. — СП б.: Левша, 2008. – С. 121–143. [Andreeva EF, Papayan AV, Savenkova ND. Polikistoz pochek. In: Papayan AV, Savenkova ND. Klinicheskaya nefrologiya detskogo vozrasta. Saint Petersburg: Levsha; 2008. 121-143 p. (In Russ.)]
- Андреева Э.Ф., Савенкова Н.Д. Кистозные болезни почек у детей: Учебное пособие для студентов. – СП б.: СП бГПМУ, 2012. – 40 с. [Andreeva EF, Savenkova ND. Kistoznyie bolezni pochek u detey. Uchebnoe posobie dlya studentov. Saint Petersburg: SpbGPMU; 2012. 40 p. (In Russ.)]
- Андреева Э.Ф. Течение и исход аутосомно-рецессивного поликистоза почек у детей. Материалы конференции педиатров-нефрологов Северо-Западного федерального округа // Педиатр. – 2011. – Т. 2. – № 1. – С. М3. [Andreeva EF. Techenie i ishod autosomno-retsessivnogo polikistoza pochek u Severo-Zapadnogo Federalnogo okruga (conference proceedings). Pediatr. 2011;2(1):M3. (In Russ.)]
- Bergmann C. ARPKD and early manifestations of ADPKD: the original polycystic kidney disease and phenocopies. Pediatr Nephrol. 2015;30:15-30. doi: 10.1007/s00467-013-2706-2.
- Bonsib SM. The classification of renal cystic diseases and other congenital malformations of the kidney and urinary tract. Arch Pathol Lab Med. 2010;134 (4):554-568.
- Zerres K. New options for prenatal diagnosis in autosomal recessive polycystic kidney disease by mutation analysis of the PKHD1 gene / K. Zerres, J. Senderek, S. Rudnik-Schoneborn, et al. Clinical Genetics.2004;66:53-57. doi: 10.1111/j.0009-9163.2004.00259.x.
Supplementary files
