scholarly journals Bilateral Nephrectomy in Patients with Autosomal Dominant Polycystic Kidney Disease and End-Stage Chronic Renal Failure

Nephron ◽  
2021 ◽  
pp. 1-7
Author(s):  
Alexander E. Lubennikov ◽  
Nicolay V. Petrovskii ◽  
German E. Krupinov ◽  
Evgeniy M. Shilov ◽  
Roman N. Trushkin ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Large Bowel ◽  
Elective Surgery ◽  
Length Of Hospital Stay ◽  
Bowel Injury ◽  
Polycystic Kidney ◽  
Group I ◽  
Group Ii ◽  
Autosomal Dominant Polycystic Kidney ◽  
End Stage

<b><i>Background:</i></b> In patients with autosomal dominant polycystic kidney disease (ADPKD) and end-stage kidney disease, bilateral nephrectomy (BN) is currently performed predominantly via the laparoscopic approach. We analysed the results of BN depending on the approach and preoperative and perioperative factors. <b><i>Patients and Methods:</i></b> This was a single-centre retrospective study carried out from April 2010 to March 2020, including a total of 142 patients presenting with ADPKD who were treated by BN. Of these, 108 patients meeting the inclusion criteria were selected to analyse the results. We compared therapeutic outcomes depending on the surgical approach (laparotomy or laparoscopy) and the type of the operation (emergent or elective). <b><i>Results:</i></b> Of the 108 eligible patients, 36 (group I) underwent laparoscopic BN and the remaining 72 patients (group II) were subjected to midline laparotomy. Sixty-nine patients underwent elective surgery and 39 endured emergent operations. The most frequent indications (87 patients, 80.6%) for surgical treatment were urinary tract infection and infected cysts. The median length of hospital stay for group I and group II patients amounted to 8 days (IQR: 7.5–9) and 12.5 days (IQR: 9–16.5), respectively (<i>p</i> &#x3c; 0.001). However, comparing the patients operated on electively, the actual difference in the length of hospital stay was inconsiderable: median 8 days (IQR: 7–9) in group I and 9 days (IQR: 9–11.5) in group II. The median duration of the operation was significantly (<i>p</i> &#x3c; 0.001) longer in group I amounting to 217.5 min (IQR: 197.5–305) than in group II equalling 115 min (IQR: 107.5–145). The frequency of postoperative complications, lethal outcomes, and blood loss volume did not statistically significantly differ depending on the surgical approach. Only patients operated on emergency underwent releparotomy due to intraoperative large bowel injury. Lethal outcomes (<i>n</i> = 18, 16.7%) after surgery were observed only in emergent patients. Sepsis prior to surgery, systemic inflammation response syndrome (SIRS) with the CRP level above 173 mg/mL, prolonged preoperative antibacterial therapy, and undiagnosed large bowel injury were associated with a lethal outcome after BN. <b><i>Conclusion:</i></b> The results of open and laparoscopic BN in elective surgery were comparable. Emergency operations for infected renal cysts and SIRS were associated with increased incidence of large bowel injury and lethal outcomes.

scholarly journals Identification of patients with autosomal dominant polycystic kidney disease at highest risk for end-stage renal disease.

1997 ◽  
Vol 8 (10) ◽  
pp. 1560-1567 ◽  
Author(s):  
A M Johnson ◽  
P A Gabow
Keyword(s):  
Kidney Disease ◽  
Risk Ratio ◽  
End Stage Renal Disease ◽  
Autosomal Dominant ◽  
Renal Survival ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney ◽  
Stage Renal Disease ◽  
End Stage ◽  
Risk Ratios

To identify those potential factors that, early in the course of disease, mark a population of patients with autosomal dominant polycystic kidney disease (ADPKD) who have worse renal survival, survival analysis and risk ratio calculation for 1215 ADPKD patients were performed. Survival times were calculated as time to dialysis, transplantation, or death. Risk ratios were calculated using the Cox proportional hazards model. Three hundred eighty-eight patients entered end-stage renal disease and 205 patients died. ADPKD2 subjects had longer renal survival than ADPKD1 subjects (median survival, 68 versus 53 yr; P < 0.0005; risk ratio, 2.5). Women had significantly better renal survival than men (56 versus 52 yr; P < 0.0001; risk ratio, 1.6). Subjects who were diagnosed before age 30 and those who developed hypertension before age 35 had worse renal survival than those subjects who were diagnosed after age 30 or those who remained normotensive after age 35, respectively (age of diagnosis: 49 versus 59 yr; P < 0.0001; risk ratio, 3.2; hypertension: 51 versus 65 yr; P < 0.0001; risk ratio, 4.4). Similarly, those who had an episode of gross hematuria before age 30 had a worse renal outcome than those who did not (49 versus 59 yr; P < 0.0001; risk ratio, 2.6). We have also calculated risk ratios for a combined model. When therapeutic interventions become available for this disease, these populations with high risk ratios should be considered for such interventions.

Diagnosis of infected kidney cysts in patients with autosomal dominant polycystic kidney disease and end-stage renal disease

Urologiia ◽  
2021 ◽  
Vol 3_2021 ◽  
pp. 50-55
Author(s):  
A.E. Lubennikov Lubennikov ◽  
A.A. Shishimorov Shishimorov ◽  
R.N. Trushkin Trushkin ◽  
T.K. Isaev T ◽  
O.N. Kotenko Kotenko ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Renal Disease ◽  
Polycystic Kidney Disease ◽  
End Stage Renal Disease ◽  
Autosomal Dominant ◽  
Polycystic Kidney ◽  
Kidney Cysts ◽  
Autosomal Dominant Polycystic Kidney ◽  
Stage Renal Disease ◽  
End Stage

scholarly journals An Overview of In Vivo and In Vitro Models for Autosomal Dominant Polycystic Kidney Disease: A Journey from 3D-Cysts to Mini-Pigs

2020 ◽  
Vol 21 (12) ◽  
pp. 4537
Author(s):  
Svenja Koslowski ◽  
Camille Latapy ◽  
Pierrïck Auvray ◽  
Marc Blondel ◽  
Laurent Meijer
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
In Vitro Models ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney ◽  
Stage Renal Disease ◽  
End Stage

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inheritable cause of end stage renal disease and, as of today, only a single moderately effective treatment is available for patients. Even though ADPKD research has made huge progress over the last decades, the precise disease mechanisms remain elusive. However, a wide variety of cellular and animal models have been developed to decipher the pathophysiological mechanisms and related pathways underlying the disease. As none of these models perfectly recapitulates the complexity of the human disease, the aim of this review is to give an overview of the main tools currently available to ADPKD researchers, as well as their main advantages and limitations.

Peritoneal Dialysis for Patients with Autosomal Dominant Polycystic Kidney Disease

2017 ◽  
Vol 37 (4) ◽  
pp. 384-388 ◽  
Author(s):  
Sana Khan ◽  
Anna Giuliani ◽  
Carlo Crepaldi ◽  
Claudio Ronco ◽  
Mitchell H. Rosner
Keyword(s):  
Peritoneal Dialysis ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Disease Patient ◽  
Cystic Kidney ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney ◽  
Stage Renal Disease ◽  
End Stage

End-stage renal disease secondary to autosomal dominant poly-cystic kidney (ADPKD) is a common issue worldwide. Peritoneal dialysis (PD) is a reasonable option for renal replacement therapy for these patients and should not be withheld due to concerns that the patient may not tolerate the fluid volumes in the peritoneal cavity. This review covers the existing data on the outcomes and complications associated with the use of PD in the polycystic kidney disease patient. In general, PD is well tolerated and outcomes in ADPKD patients are equivalent to or better than other patient groups.

scholarly journals Interrupted Aortic Arch in an Adult with Polycystic Kidney Disease

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Ayşe Şeker Koçkara ◽  
Mansur Kayataş ◽  
Can Huzmeli ◽  
Ferhan Candan ◽  
Cesur Gümüş
Keyword(s):  
Kidney Disease ◽  
Aortic Arch ◽  
Polycystic Kidney Disease ◽  
Rare Case ◽  
Autosomal Dominant ◽  
Interrupted Aortic Arch ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney ◽  
Extrarenal Complications ◽  
End Stage

Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease and is responsible for 8–10% of patients with end-stage renal failure. The major extrarenal complications of ADPKD are cardiovascular abnormalities. Interrupted aortic arch (IAA) is a lethal congenital cardiac abnormality seen with a frequency of 3/1000000 births and is defined as a segment of the arcus aorta being atresic. In the literature, there are no any reports showing that polycystic kidney disease and interrupted aortic arch occur together. In this study, we present a rare case in which the patient has polycystic kidney disease and IAA together and discuss whether IAA is a complication of ADPKD.

scholarly journals Hypertension in adult polycystic kidney disease: a narrative review

2019 ◽  
Vol 8 (2) ◽  
pp. 122-126
Author(s):  
Sarah Mian ◽  
Yogesh Acharya ◽  
Ranjan Dahal
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Focal Point ◽  
Polycystic Kidney ◽  
Adult Polycystic Kidney Disease ◽  
Renal Disorder ◽  
Autosomal Dominant Polycystic Kidney ◽  
Stage Renal Disease ◽  
End Stage ◽  
Review Current

Autosomal dominant polycystic kidney disease (ADPKD) is an inherited renal disorder that impacts approximately 12 million worldwide. It is characterized by bilateral kidney enlargement and cystic growth. Hypertension (HTN) is a focal point in the management of ADPKD and is linked to a faster progression to end stage renal disease. Current novel therapies have proven to reduce the progression of renal damage. The ideal goal is to minimize risk through preventative studies and pharmacology to further increase life expectancy and quality. The purpose of this article is to highlight the importance of blood pressure management in ADPKD and review current literature to determine the most effective preventative pharmacotherapy.

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