电邮这篇文章 Autosomal-recessive polycystic kidney disease in children
- 作者: Andreeva E.F1, Savenkova N.D1, Tilouche M.A1, Natochina N.Y1, Dug I.V1
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隶属关系:
- St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
- 期: 卷 7, 编号 4 (2016)
- 页面: 45-49
- 栏目: Articles
- URL: https://journals.eco-vector.com/pediatr/article/view/5966
- DOI: https://doi.org/10.17816/PED7445-49
- ID: 5966
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详细
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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作者简介
Elvira Andreeva
St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
编辑信件的主要联系方式.
Email: A-Elvira@yandex.ru
MD, PhD, Assistant Dept. of Faculty Pediatric 俄罗斯联邦
Nadejda 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 俄罗斯联邦
Mohamed 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 俄罗斯联邦
Natalya Natochina
St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
Email: smallnataly@gmail.com
Associate, Dept. of Faculty Pediatric 俄罗斯联邦
Igor Dug
St Petersburg State Pediatric Medical University, Ministry of Healthcare of the Russian Federation
Email: Dyug72@mail.ru
surgery the Department of Microsurgery 俄罗斯联邦
参考
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