Autosomal-recessive polycystic kidney disease in children

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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.


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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

Russian Federation MD, PhD, Assistant Dept. of Faculty Pediatric

Nadejda D Savenkova

St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation

Email: savenkova.n.spb@mail.ru

Russian Federation MD, PhD, Dr. Med. Sci., prof. Dept. of Faculty Pediatric

Mohamed A Tilouche

St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation

Email: amin_amt@hotmail.com

Russian Federation Postgraduate Student, Department of Faculty Pediatric

Natalya Y Natochina

St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation

Email: smallnataly@gmail.com

Russian Federation Associate, Dept. of Faculty Pediatric

Igor V Dug

St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation

Email: Dyug72@mail.ru

Russian Federation surgery the Department of Microsurgery

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Copyright (c) 2016 Andreeva E.F., Savenkova N.D., Tilouche M.A., Natochina N.Y., Dug I.V.

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