A clinical case of surgical treatment of a large kidney cyst in a 9-year-old girl with autosomal dominant polycystic kidney disease

封面

如何引用文章

全文:

开放存取 开放存取
受限制的访问 ##reader.subscriptionAccessGranted##
受限制的访问 订阅或者付费存取

详细

BACKGROUND: Autosomal dominant polycystic kidney disease is currently the most common inherited form of cystic kidney disease, the frequency of which is about 1 per 400–1000 newborns, what makes the problem relevant. Its progressive course in patients with polycystic disease is primarily associated with the ischemic component, the severity of which depends on the number, location and size of cysts. Arterial hypertension, which is an autosomal dominant polycystic kidney disease complication, plays a leading role in renal failure progression. Given the current lack of specific treatment, the main therapeutic goal is to prevent complications, postpone the development of renal failure. Therefore, if there are large and giant cysts, surgical treatment is an effective modality and should be performed in a timely manner.

CLINICAL CASE DESCRIPTION: The authors present a clinical case of a successful endovideosurgical cyst excision in a -year-old patient with a giant cyst in the right kidney, autosomal dominant polycystic disease, and hypertension of renal genesis.

Endovideosurgical cystectomy in the patient with autosomal dominant polycystic kidney disease has become an important pathogenetic link in the treatment of this pathology. Currently, the patient is under dynamic follow-up for more than one year. During the follow-up period, the patient demonstrates relief of urinary syndrome in the form of disappeared haematuria, stabilization of albuminuria indices, improvement of blood flow parameters in the renal parenchyma, as well as stabilization of arterial pressure.

CONCLUSION: The given clinical example demonstrates that reasonable surgical intervention with the choice of a rational method of access, taking into account the anatomical localization and size of the cystic formation, allowed to obtain a good result in the form of stabilization of renal hemodynamics in a patient with autosomal dominant polycystic kidney disease.

全文:

受限制的访问

作者简介

Anna Oganisyan

V.F. Voyno-Yasenetsky Scientific and Practical Center of Specialized Medical Care for Children

编辑信件的主要联系方式.
Email: oganisyanaa@yandex.ru
ORCID iD: 0000-0002-5495-4315
SPIN 代码: 3707-2382
俄罗斯联邦, Moscow

Artem Vrublevskiy

V.F. Voyno-Yasenetsky Scientific and Practical Center of Specialized Medical Care for Children

Email: a.s.vrublevskij@yandex.ru
ORCID iD: 0000-0001-8550-8636
SPIN 代码: 3041-3173
俄罗斯联邦, Moscow

Revaz Valiev

V.F. Voyno-Yasenetsky Scientific and Practical Center of Specialized Medical Care for Children

Email: rvaliev.dr@yandex.ru
ORCID iD: 0000-0003-1805-9169
SPIN 代码: 9460-0920
俄罗斯联邦, Moscow

Filipp Turov

V.F. Voyno-Yasenetsky Scientific and Practical Center of Specialized Medical Care for Children

Email: filipp_100@mail.ru
ORCID iD: 0000-0002-9301-7586
SPIN 代码: 6683-1547
俄罗斯联邦, Moscow

Mamay Khanov

V.F. Voyno-Yasenetsky Scientific and Practical Center of Specialized Medical Care for Children

Email: 8_00@mail.ru
ORCID iD: 0000-0003-3354-5018
SPIN 代码: 8968-3164
俄罗斯联邦, Moscow

Sergey Vrublevskiy

V.F. Voyno-Yasenetsky Scientific and Practical Center of Specialized Medical Care for Children; Russian National Research Medical University named after N.I. Pirogov

Email: s.g.vrublevskiy@yandex.ru
ORCID iD: 0000-0001-9400-7673
SPIN 代码: 6535-2570
俄罗斯联邦, Moscow; Moscow

Elena Vrublevskaya

V.F. Voyno-Yasenetsky Scientific and Practical Center of Specialized Medical Care for Children; Russian National Research Medical University named after N.I. Pirogov

Email: vrublevskaj@mail.ru
ORCID iD: 0000-0001-7312-5945
SPIN 代码: 4003-1783
俄罗斯联邦, Moscow; Moscow

参考

  1. Ignatova MS, Veltischev YE. Paediatric nephrology: A guide for doctors. Leningrad: Meditsina; 1989. (In Russ).
  2. Živná M, Kidd KO. Autosomal dominant tubulointerstitial kidney disease: A review. Am J Med Genet C Semin Med Genet. 2022;190(3):309-324. EDN: JOPGCM doi: 10.1002/ajmg.c.32008
  3. Papayan AV, Styazhkina IS. Cystic renal diseases. Polycystic kidney disease. Neonatal nephrology. Manual. Saint Petersburg: Peter; 2002. (In Russ).
  4. Cadnapaphornchai MA. Autosomal dominant polycystic kidney disease in children. Curr Opin Pediatr. 2015;27(2):193-200. doi: 10.1097/MOP.0000000000000195
  5. Kozlova SI, Semanova E, Demikova NS, Blinnikova OE. Hereditary syndromes and medical and genetic counselling. Moscow: Praktika; 1996. (In Russ).
  6. Kim DY, Park JH. Genetic Mechanisms of ADPKD. Adv Exp Med Biol. 2016;(933):13-22. doi: 10.1007/978-981-10-2041-4_2
  7. Grantham JJ. Clinical practice. Autosomal dominant polycystic kidney disease. N Engl J Med. 2008;359(14):1477-1485. doi: 10.1056/NEJMcp0804458
  8. Ong AC, Wheatley DN. Policystic kidney disease: The ciliary connection. Lancet. 2003;1(361):774-776. doi: 10.1016/S0140-6736(03)12662-1
  9. Guay-Woodford L. Murine models of polycystic kidney disease: Molecular and therapeutic insights. Am J Physiol Renal Physiol. 2003;285(6):1034-1049. doi: 10.1152/ajprenal.00195.2003
  10. Guay-Woodford L. RIP-ed and ready to dance: New mechanisms for polycysin-1 signaling. J Clin.Invest. 2004;114(10):1404-1406. doi: 10.1172/JCI23544
  11. Guay-Woodford LM, Galliani CM. Diffuse renal cystic disease in children: Morphologic and genetic correlations. Pediatr Nephrol. 1988;12(3):173-182. doi: 10.1007/s004670050431
  12. Vrublevsky SG. Differentiated approach to the treatment of children with solitary cystic renal masses. In: Proceedings of the V Anniversary All-Russian School on paediatric urology andrology “Paediatric urology: present and future”, 7-9 April 2016. Moscow; 2016. Р: 33.

补充文件

附件文件
动作
1. JATS XML
2. Fig. 1. Ultrasound examination of patient K., 10 years old: a — autosomal dominant polycystic kidney disease in B-mode; b — cyst of the middle segment of the right kidney.

下载 (184KB)
3. Fig. 2. Patient K., laparoscopic picture: a — cyst of the right kidney is visualized; b — puncture of the cyst with evacuation of its content and subsequent sclerosing.

下载 (374KB)
4. Fig. 3. Patient K. Laparoscopic picture, plasma ablation of the cyst inner lining.

下载 (159KB)
5. Fig. 4. Ultrasound examination of patient K., autosomal dominant polycystic kidney disease, result one month after endosurgical fenestration of the cyst: a — B-mode with dopplerography; b — В-mode.

下载 (240KB)

版权所有 © Oganisyan A.A., Vrublevskiy A.S., Valiev R.Y., Turov F.O., Khanov M.M., Vrublevskiy S.G., Vrublevskaya E.N., 2023

##common.cookie##