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17
II. ХИРУРГИЯ
THE CASE OF A COMPLICATED AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE
Isamatov B.K.12, Adenova G.M.2, Gebel D.B.2, Amirov G.K.2, Esenkulov B.Zh.2, Gasanov R.A.2
1JSC "National Scientific Center of Surgery named after A.N. Syzganova";
2State Clinical Hospital " The city emergency hospital of Almaty city" Almaty, Kazakhstan
Abstract
Autosomal dominant polycystic kidney disease is the most common inherited kidney disease found in adults. The urgency of the problem is due to the peculiarity of the development and growth of cysts, the inability of existing methods of treatment to prevent an increase in the size of cysts and kidneys, and progressive impaired renal function. This article describes the general characteristics of ADPP, presents an analysis of the clinical case of a complicated course of ADPP.
УДК: 616-002.191:166-085-07 + 616.61-003.4
ABOUT THEАUTHORS
Issamatov Bekzhan Kalibayevich
- researcher of the JSC «NSCS named after A.N. Syzganovdoctor of computed tomography of the diagnostic department of GBSNP of Almaty. e-mail: [email protected]
Adenova Gulzhan Moldabekovna - head of the diagnostic department of GBSNP of Almaty, doctor of ultrasound diagnostics of the highest category.
Gebel Dmitry Borisovich - doctor of magnetic resonance imaging of the diagnostic department of GBSNP of Almaty.
Amirov Gani Kondybaevich - Head of the Department of Urology, GBSNP of Almaty, urologist of the highest category.
Esenkulov Baglan Zhomalbekovich -
Urologist, Department of Urology, GBSNP of Almaty.
Hasanov Rustam Aliagaoglu - Urologist, Department of Urology, GBSNP of Almaty.
Keywords
duodenal ulcer, bleeding, treatment
Аутосомды-доминантты поликистоздьщ бYЙрек ауруыньщ аскынган жаедайы
Исаматов Б. К.12, Аденова Г.М.2, Гебель Д.Б.2, 0MipoB F-K-2, Есенвдлов Б.Ж.2, Гасанов Р.А.2
1«А.Н. Сь^анов ат. Улттык, ^шыми хирургия орталь™» АК;
2«Алматы калалык жедел шу^ш кемек керсету ауруханасы» МКА, Алматы, Казакстан
АВТОРЛАР ТУРАЛЫ
Исаматов Бекжан Калибайулы-
«A.H.CbtfFaHOB атындагы Улгтык FbrnbiMtt хирургия орталы^ы» FbrnbiMtt кызметкер^ Алматы калалык ЖШККА диагностикалык бел1мшесш!ц компьютерлк томография дэр1гер1. e-mail: [email protected]
Аденова Гулжан Молдабекцызы -
Алматы калалык ЖШККА диагностикалык бел/мшес/н/ц мецгеруш/с/, жоFары санатты ультрадыбыстык диагностика дэр/гер/.
Гебель Дмитрий Борисович - Алматы калалык ЖШККА диагностикалык бел/мшес/н/ц магниттк-резонанстык томография дэр/гер/.
dMipoB fam Цондыбайупы - Алматы калалык ЖШККА урология бел/мшес/н/ц мецгерушсi, жоFары санатты уролог-
двррер.
Есенкулов Батан Жомалбекулы
- Алматы калалык ЖШККА урология белiмшесiнiц уролог-дэргерi.
Гасанов Рустам Алиагаоглы - Алматы калалык ЖШККА урология белiмшесiнiц
уролог-дэрЭгере.
Ацдатпа
Аутосомды-доминантты поликистоздьщ бYЙрек ауруы - ересектерде жи байщалатын тущым щуалайтын бYЙрек ауруы болып табылады. Мэселен'щ взектлю - АДПБА кез'!ндеп кисталардын всу жэне даму ерекшелiктерi, щолданыстагы емдеу эдiстерiнiн бYЙрекжэне киста влшемдерiнiн улгаюынын алдын алуга щаблетаз болуы. Осы мащалада АДПКА-нын жалпы сипаттамасы, АДПБА асщынган агымынын клиникалыщ жагдайынын, талдауы берлген.
Туйш сездер
аутосомды-доминантты поликистоздыщ буйрек ауруы, буйрек поликистозы, асщынулар
Случай осложненной аутосомно-доминантной поликистозной болезни почек
ОБ АВТОРАХ
Исаматов Бекжан Калибаевич -
научный сотрудник АО «Национальный научный центр хирургии им. А.Н. Сызганова»; врач компьютерной томографии диагностического отделения ГБСНП г.Алматы. е-таИ: [email protected]
Аденова Гульжан Молдабековна -
заведующая диагностическим отделением ГБСНП г.Алматы, врач ультразвуковой диагностики высшей категории.
Гебель Дмитрий Борисович - врач магнитно-резонансной томографии диагностического отделения ГБСНП г.
Алматы.
Амиров Гани Кондыбаевич -
заведующий отделением урологии ГБСНП г.Алматы, врач-уролог высшей категории.
Есенкулов Баглан Жомалбекович -
врач-уролог отделения урологии ГБСНП г.Алматы.
Гасанов Рустам Алиагаоглы - врач-уролог отделения урологии ГБСНП г.
Алматы.
Ключевые слова
аутосомно-доминантная по-ликистозная болезнь почек, поликистоз почек, осложнения
Исаматов Б. К.12, Аденова Г.М.2, Гебель Д.Б.2, Амиров Г.К.2, Есенкулов Б.Ж.2, Гасанов Р.А.2
1АО «Национальный научный центр хирургии им. А.Н. Сызганова»;
2ГКБ «Городская больница скорой неотложной помощи г.Алматы», Алматы, Казахстан
Аннотация
Аутосомно-доминантная поликистозная болезнь почек - наиболее распространенное наследственное заболевание почек, выявляющийся у взрослых. Актуальность проблемы обусловлена особенностью развития и роста кист, неспособностью существующих методов лечения предотвратить увеличение размеров кист и почек, прогрессирующее нарушение функции почек. В настоящей статье описана общая характеристика АДПБП, представлен анализ клинического случая осложненного течения АДПБП.
Introduction
The urgency of the problem of autosomal dominant polycystic kidney disease (ADPBP) is the development and growth of cysts, the inability of existing treatment methods to prevent an increase in the size of cysts and kidneys, and progressive impairment of renal function.
ADPBP is the most common hereditary kidney disease, with a prevalence rate in the general population of 1: 1000-2500, often detected in adulthood (30-40 years) [1, 2].
ADPBP is caused by mutations in the PKD1 (chromosome 16p13.3) and PKD2 (chromosome 4q21) mutations (abbreviation for Polycystic Kidney Disease). These genes encode proteins called poly-cystins 1 and 2 (PC1 and PC2). PKD1 mutation is responsible for 78% of cases of ADPD; PKD2 mutation is found in 15% of patients [3]. Also, the PKD 3 gene (11q12.3) was recently discovered, which is responsible for the interaction of PC1 and PC2. This mutation contributes to 0.3% of cases [4, 5].
The PKD 1 mutation is associated with a more severe course of the disease, faster progression to the terminal stage of chronic kidney disease, and early death. So, in patients with PKD 1, by the age of 50, chronic renal failure develops in 35% of cases, by the age of 60 - in 70%, and by the age of 70 - exceeds 95% [6]. Today there is no way to completely cure ADPP.
Cysts can be localized in the medulla, in the cortical layers of the kidney; in the pelvis of the kidney, in the perinefral region. Kidney cyst in ADPBP is an extended segment of the nephron or collecting tube. The wall of the cyst is lined with one layer of altered tubular epithelium (cubic, flattened) or a thin layer of connective tissue, it is a partition between them. The size of the cysts depends on the amount of content and can vary within various limits: from small (<2 mm in diameter, containing not > 3 ml of fluid) to giant. Areas of cystic tissue are combined with areas of healthy renal transplant, the amount of which, as the first grows, rapidly decreases. Cyst fluid communicates with tubular contents, blood vessels of the kidneys, and contents of the renal pelvis [7].
ADPBP is characterized by the development and growth of multiple cysts in the parenchyma of both kidneys. Progressive loss of kidney function occurs over several decades and often leads to the terminal stage of chronic kidney disease. Hypertension is a common manifestation of ADPD and is found in most people with enlarged kidneys or a reduced glomerular filtration rate.
Non-genetic factors also influence the clinical manifestations and the rate of progression of ADPD. This is evidenced, for example, by the fact that cystic lesion of the liver is more severe in women, especially in those who took combined oral con-
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ВЕСТНИК ХИРУРГИИ КАЗАХСТАНА № 4-2019
traceptives, replacing estrogen therapy, and who have a history of multiple pregnancies. It is believed that in men with ADPD, the rate of increase in cyst size is higher than in women, and the terminal stage of chronic renal failure with ADPD occurs earlier in men, which indicates the role of sex hormones that can change the course of the disease [8].
Complications of the disease can be macrohe-maturia, infection and suppuration of cysts, nephrolithiasis, which are manifested by acute or chronic pain in the lumbar region [9].
Polycystic liver disease (PLD) is one of the most common extrarenal manifestations (in over 83% of cases) of autosomal dominant polycystic kidney disease. Although in many patients PLD is asymptomatic, nevertheless, in some cases, patients develop hepatomegaly, which is accompanied by abdominal pain, a feeling of fullness, shortness of breath, and a decrease in the quality of life. ADPBP can also be accompanied with pancreatic and / or spleen cysts, intestinal diverticulosis (65%), heart valve pathology (24%), ovarian cyst lesion (40%), intracranial vascular aneurysms (10%), inguinal hernia (15%) [10].
Treatment
For ADPB symptomatic therapy is performed. When detecting arterial hypertension, an exception is currently being made to renal pathology. It is necessary to exclude stay in adverse weather conditions, heavy physical exertion. It is important to follow a diet in the early stages with a moderate restriction of animal proteins, as kidney failure progresses, more strict adherence to a low-protein diet [11].
Antihypertensive therapy should include inhibitors of angiotensin-converting enzyme, calcium antagonists (lerkamen), and beta-blockers (concor, betalok-ZOK, moxonidine) [12].
In patients with severe polyuria for the prevention of dehydration, electrolyte loss, it is advisable to use thiazides to distally reduce the release of electrolytes and water. Therapy of urinary tract infection should be carried out taking into account the results of bacteriological examination of urine. Periodically carry out herbal medicine. In patients with frequent relapses of macrohematuria, a coagu-logram study is necessary.
With the development of anemia - erythropoi-etin preparations, intravenous administration of iron are useful. With the development of terminal chronic renal failure to perform programmed hemo-dialysis. It must be borne in mind that the introduction of heparin during hemodialysis sessions can cause the appearance of macrohematuria, hemorrhages in the cysts, and especially severe consequences can be observed with hemorrhages in the brain cysts. A radical treatment for ADPD is kidney transplantation [11].
The purpose of this article was to demonstrate the clinical, laboratory, and instrumental features of a case of complicated ADPP, as well as the tactics of treating a patient.
Clinical case
Patient O., a woman born in 1975, 44 years old, was admitted to the clinic on December 7, 2019, with complaints of pain in the lumbar region on both sides, more on the right, an fever to 38.0°C for 2 days, weakness, malaise.
Patient considers ill for three days before admission. According to her own treatment did not accept. In connection with the deterioration of the condition and the growth of the above complaints, she called an ambulance team and was taken to the admission department of the GBSNP in Almaty, examined by the urologist. Laboratory and instrumental research methods were prescribed. Spasmolytic and analgesic therapy was carried out, after which the pain syndrome was partially stopped. Then the patient was hospitalized in the Department of Urology for further examination and determination of treatment tactics.
From the anamnesis, polycystic kidney and liver disease was detected about 10 years ago. Inspected annually by the therapist at the place of residence. The last time she was examined was in November 2018, outpatient treatment was recommended. Kidney transplantation not suggested.
The patient's condition is serious, due to pain and intoxication syndromes. External pain scale - 2 points. The patient's consciousness is clear, adequate.
On palpation, the abdomen is soft, painless. The lumbar region is symmetrical, with superficial palpation in the projection of the right kidney pain is noted. With deep palpation: the kidneys are mobile, pain on the right. Symptom striking (+) on both sides, more on the right. Urination is frequent, painless.
Laboratory methods: The full blood count determined: leukocytosis, moderate erythropenia and anemia, thrombocytopenia. In dynamics, the number of leukocytes decreased (table 1).
In the biochemical analysis of blood, an increase in nitrogenous toxins was revealed, which indicated a decrease of renal function; moderate increase glucose in blood (table 2).
Coagulological blood tests were within normal limits.
In the general analysis of urine, signs of renal failure, inflammation (proteinuria, hematuria, leuko-cyturia) were revealed (table 3).
When analyzing urine according to Nechipo-renko, a large number of leukocytes in the urine was detected - 20,250 Leu / ul (normal indicators 2000-4000 in 1).
Table 1.
Dynamic indicators of a full blood count.
Table 2.
Modified components of a biochemical blood test.
Table 3.
Modified components of the urine test.
Figure 1.
Sonograms of the right (A) and left (B) lobes of the liver during longitudinal scanning: 1 - multiple cysts, 2 - liver parenchyma.
Components, Elements Standard values Date, F Results Values of SI Units
07.12.2019 08.12.2019 13.12.2019 18.12.2019
White blood cells (WBC) 4,0-9,0 17,2 11.70 14.30 10.40 109/l
Red blood cells (RBC) 4,3-5,5 4,09 3.47 2.54 2.51 10№l/l
Hemoglobin (HGB) 120-140 118 101.00 74.00 72.00 g/l
Hematocrit (HCT) 0,39-0,49 38,25 32.40 23.80 23.70 %
Platelets (PLT) 150-400 198 205.00 466.00 819.00 109/l
Components, Elements Results Standard values Values of SI Units
Urea 12.49 2,7 - 8,3 mmol/L
Creatinine 200.05 men (44 - 115), women (45 - 97) umol/L
Glucose 7.17 3,50 - 6,00 mmol/L
Components, Elements Results Standard values Values of SI Units
Color Light yellow
Transparency muddy
Number 50,0 ml
Protein (PRO) 2+ Negative %0
pH 6,0 5-7
Specific gravity (S.G.) 1.010 1,005-1,025
Blood (BLD) 2+ Negative mg/L
Ketones (KET) +- Negative mmol/L
Leukocyte (LEU) 500 to 25 Leu/uL
Blood type: B (III) Third, Rh + positive.
Instrumental methods of research: Ultrasound examination of the liver. The size of the liver is increased, the oblique vertical size of the right lobe is 20.5 cm, the oblique vertical size of the left lobe is 13.9 cm. The echostructure is even, the echo density is increased, the intrahepatic ducts are not dilated. In the structure of the liver, multiple an-echogenic thin-walled cystic formations are determined, with sizes from 0.7 cm to 9.2 cm, homoge-
neous liquid contents. Conclusion: Polycystic liver disease. Diffuse changes in the liver parenchyma (figure 1).
Ultrasound examination of the kidneys. The location of the kidneys is typical. The right kidney is 15.3 x 7.8 cm in size, the parenchyma is thinned. In the structure of multiple anechogenic masses in diameter from 0.5 cm to 5.6 cm. Pyelocaliceal system - major calyxes expanded to 1.7 cm. The left kidney is 16.5x8.2 cm in size, the parenchyma is thinned. In the structure, anechogenic masses are in diameter from 0.5 cm to 6.3 cm. The pyelocaliceal system is not expanded. Conclusion: Polycystic kidney disease. Calicoecta-sia on the right. Acute pyelonephritis on the right (Figure 2).
Magnetic resonance imaging of the abdominal cavity and retroperitoneal space. On a series
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BECTHMK XMPYPrMM KA3AXCTAHA № 4-2019
of MRI tomograms, the kidneys are relatively the same, significantly increased to 20.0x10.0x1010 cm. The usual structure of the kidneys is not traced against the background of many cystic lesions diffusely occupying the entire volume of the renal parenchyma, so that pyelocaliceal system is not traced on both sides. There is also a heterogeneous composition of the contents of the cysts, some of which have sedimented hemorrhagic contents, some are filled with a liquid with a high protein content, which intensively limits diffusion. A larger number of cysts with a changed composition of contents is located in the right kidney. Paranephric fiber is not changed. Adrenal glands without features.
The liver is sharply enlarged (maximum cranio-caudal size - 24.0 cm, maximum transverse size
Figure 2.
Sonograms of the right (A) and left (B) kidneys during longitudinal transluminal scanning: 1 - multiple cysts, 2 - renal parenchyma.
- 24.6 cm). In the structure of the liver, multiple cystic lesions ranging in size from 0.5 to 10.0 cm in diameter, filled with a homogeneous fluid without impurities. The hepatic ducts are not dilated. The portal vein is not dilated, up to 0.9 cm in caliber (Figure 3, 4).
Conclusion of MRI: Multicystic lesions of the liver and kidneys, hemorrhages and inflammatory changes in several cystic formations of the right kidney. Inflammatory changes in single cysts of the left kidney. Severe hepatomegaly and bilateral nephromegaly.
MR picture of complicated polycystic kidney disease with diffuse extrarenal liver damage.
Chest x-ray. On the survey x-ray of the chest organs, pulmonary fields without fresh focal and infiltrative shadows. Pulmonary pattern reinforced,
Figure 3.
MRI - tomograms in the coronal view. A, B, C - in T2; D, E, F - Diffusion Weighted Imaging (DWI): 1 - multiple cysts in the liver, 2 - multiple cysts in the kidneys, 3 - cysts with purulent contents, 4 - kidney parenchyma with inflammatory changes, 5 - single cysts with hemorrhagic contents.
Figure 4.
MRI - tomograms in axial view. A, B, C - in T2 WI; D, E, F - Diffusion Weighted Imaging (DWI): 1 - multiple cysts in the liver, 2 - multiple cysts in the kidneys, 3 - cysts with purulent contents, 4 - renal parenchyma with inflammatory changes, 5 - cysts with hemorrhagic contents.
Figure 5.
Intraoperative image of a polycystic right kidney with purulent-necrotic contents of cysts.
deformed. The roots are somewhat denser. The diaphragm is smooth, the sinuses are free. The shadow of the heart corresponds to age. Conclusion: Chronic bronchitis.
Based on the foregoing, a diagnosis was made: Polycystic kidney and liver, type adult. Acute tub-ulo-interstitial nephritis. Suppurative cysts of the right kidney. Chronic bilateral pyelonephritis, exacerbation on the right. Chronic renal failure of the I degree.
Complex treatment was prescribed: Mode II, diet table No. 7. Antibacterial, detoxification, analgesic therapy. Preparing for the operation.
On December 10, 2019, an operation was performed: Lumbotomy on the right. Autopsy and excision of suppurative cysts of the right kidney. Lum-bostomy.
Protocol of operation: The position of the patient on the left side. After processing the surgical field three times with a solution of povidone and once with a solution of spirit, a lumbotomic skin incision was made according to Fedorov about 10.0 cm long. Hemostasis. The wound was opened in layers (subcutaneous tissue, aponeurosis, mus-
cles) to the peritoneum. The peritoneum is pushed back. Hemostasis. Paranephric fiber was opened, the latter is gibbed, there is vitreous edema. With technical difficulties, the right kidney is highlighted. The right kidney is increased in size to 16.0 x 8.0 cm. During the audit, the structure of the right kidney is represented by multiple thin-walled cystic formations, sizes from 0.5 cm to 6.0 cm, with turbid fluid contents in many of them, as well as the presence of a hemorrhagic component in some cysts (Figure 5).
Excision and drainage of inflamed cysts was performed, purulent hemorrhagic contents were obtained. A small amount of biological material was taken for bacteriological studies in order to identify an infectious agent and sensitivity to antibiotics. Pieces of tissue were taken from several cyst walls for histological examination. Conducted he-mostasis. The wound cavity and kidney are actively washed with solutions of chlorhexidine, furatsillin. A lumbostomy is established. The wound cavity was washed with furatsillin solution, drained. Control for hemostasis is dry. The wound is sutured in layers. Stitches are applied to the skin. Spirit. Aseptic dressing.
Postoperative diagnosis: Polycystic kidney disease, type adult. Suppurative cysts of the right kidney. Chronic bilateral pyelonephritis, exacerbation on the right. Chronic renal failure of the I degree.
Conclusion of bacteriological research: Entero-bacter Aerogenes. This is an optional anaerobic, gram-negative, rod-shaped bacterium of the En-terobacteriaceae family, which is a conditionally pathogenic microflora of the gastrointestinal tract. It is resistant to ampicillin, sensitive to meropenem, cefuroxime, cefotaxime, amikacin, ceftazidime.
The histological conclusion: fibrous adipose tissue with extensive fields of hemorrhage, purulent inflammation.
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ВЕСТНИК ХИРУРГИИ КАЗАХСТАНА № 4-2019
After complex therapy, body temperature decreased from 38.0°C to normal values, with episodes of febrile temperature (37.1-37.5°C) in the following days after surgery.
On the tenth day after the operation (20/12/2019), the general condition of the patient is relatively satisfactory, stable in dynamics. Consciousness is clear. Complaints of a periodic increase in body temperature to 37.5°C. Urination independent, urine light. Swathe is clean, removed. The wound is calm, with no signs of inflammation, the seams lie evenly. Toilet wounds. Aseptic swathe. Continues treatment.
After complex treatment (antibiotic therapy, surgery, symptomatic therapy), the patient was discharged in satisfactory condition on day 16 from the hospital under the supervision of a urologist in the clinic at the place of residence.
References
1. Lanktree MB, Haghighi A, Guiard E, et al. Prevalence estimates of polycystic kidney and liver disease by population sequencing. J Am Soc Nephrol 2018; 29: 2593-600.
2. Willey CJ, Blais JD, Hall AK, Krasa HB, Makin AJ, Czerwiec FS. Prevalence of autosomal dominant polycystic kidney disease in the European Union. Nephrol Dial Transplant 2017; 32: 1356-63.
3. Cornec-Le Gall E, Torres VE, Harris PC. Genetic complexity of autosomal dominant polycystic kidney and liver diseases. J Am Soc Nephrol 2018; 29: 13-23.
4. Heyer, C.M., Sundsbak, J.L., Abebe, K.Z., Chapman, A.B., Torres, V.E., Grantham, J.J., Bae, K.T., Schrier, R.W., Perrone, R.D., Braun, W.E., et al.; HALT PKD and CRISP Investigators (2016). Predicted mutation strength of nontruncating PKD1 mutations aids genotype-phenotype correlations in autosomal dominant polycystic kidney disease. J. Am. Soc. Nephrol. Published online January 28, 2016. http://dx.doi. org/10.1681/ ASN.2015050583.
5. Porath, B., Gainullin, V. G., Cornec-Le Gall, E., Dillinger, E. K., Heyer, C. M., Hopp, K., Edwards, M. E., Madsen, C. D., Mauritz, S. R., Banks, C. J., Baheti, S., Reddy, B., and 16 others. Mutations in GANAB, encoding the glucosidase II-alpha subunit, cause autosomal-dominant polycystic kidney and liver disease. Am. J. Hum. Genet. 98: 11931207, 2016.
Conclusion
Thus, in this clinical case, the importance of understanding and timely revealing the complications of ADPKD, the features of clinical, laboratory and instrumental research methods, as well as the tactics of treating a patient with purulent-necrotic inflammation of the kidney cysts, is shown. It is necessary to remember the possibility of suppuration of cysts not only of the kidneys, but also of other organs, which can cause the development of a purulent-necrotic process of cystic transformed kidneys, which will further aggravate the morphological and functional state of the kidneys. A brief review of ADPKD will inform practitioners, especially young professionals, about the etiology, clinical, and instrumental manifestations of the disease and the complication of ADPKD.
6. Basics Pathologists of diseases according to Rob-binson and Kotran, translated from English by Vi-nay Kumar, Abul K. Abbas, Nelson Rausto] [Published in Russian]. Publishing house "Logosphere" Moscow 2016 Volume 2.
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10. [N. Yu. Kostenkov, S. S. Elgaitarova, M. O. Belush-enko, L. V. Borodina, P. V. Koroy]. Modern aspects of autosomal dominant polycystic kidney disease in adults. [Published in Russian] Bulletin of a young scientist, 2019.V. 8. No. 2.
11. A.V. Smirnov, V.A. Dobronravov et al., Clinical recommendations for the treatment and diagnosis of polycystic kidney disease] [Published in Russian] Research Institute of Nephrology, St. Petersburg State Medical University. Acad. I.P. Pavlova. -2015. - T. 19, No. 1. - S. 67-76.
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