scholarly journals Incoercible Vomiting in a Polycystic (ADPKD) Patient on Peritoneal Dialysis

2021 ◽  
pp. 321-326
Author(s):  
Victor Burguera Vion ◽  
R. Haridian Sosa Barrios ◽  
Maria Delgado Yagüe ◽  
Milagros Fernández Lucas ◽  
Maite E. Rivera Gorrín
Keyword(s):  
Autosomal Dominant ◽  
Gastrointestinal Symptoms ◽  
Liver Cyst ◽  
Polycystic Kidney ◽  
Abdominal Ct ◽  
Extrinsic Compression ◽  
Autosomal Dominant Polycystic Kidney ◽  
Stage Renal Disease ◽  
End Stage ◽  
Cava Vein

Although gastrointestinal symptoms are not uncommon in PD patients due to several causes, such as infusion volume with early satiety, constipation, or peritonitis, sometimes the differential diagnosis is more challenging for nephrologists. We present the case of a woman with end-stage renal disease due to autosomal dominant polycystic kidney disease on PD who presented with swollen legs and incoercible vomiting. After ruling out constipation and infection, an abdominal CT was done, revealing extrinsic compression of the intrahepatic inferior cava vein (ICV) and massive venous thrombosis from ICV to bilateral iliofemoral deep veins. In addition, CT also showed displacement and extrinsic compression of the stomach, pylorus, and duodenum due to an enlarged liver cyst. Percutaneous drainage and sclerosis of the cyst compressing the stomach was performed, anticoagulation was started, and the patient clinically improved with complete resolution of symptoms.

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.

Autosomal dominant polycystic kidney disease

2018 ◽  
Author(s):  
Albert C. M. Ong ◽  
Richard Sandford
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Health Care Systems ◽  
The United States ◽  
Polycystic Kidney ◽  
Care Systems ◽  
Autosomal Dominant Polycystic Kidney ◽  
Stage Renal Disease ◽  
End Stage

Based on an estimated population prevalence of between 1 in 400 and 1 in 1000, there are over 60,000 individuals with or at risk of developing complications associated with autosomal dominant polycystic kidney disease (ADPKD) in the United Kingdom. This equates to over 300,000 people in the United States and 7 million worldwide. Once diagnosed, an individual with ADPKD will require long-term medical follow-up and treatment with an unknown cost to national health care systems. A major proportion, probably two-thirds, will develop end-stage renal disease (ESRD) requiring renal replacement therapy—dialysis or transplantation. ADPKD is therefore the commonest genetic cause of ESRD. Most centres worldwide report that approximately one in ten patients receiving dialysis therapy have a diagnosis of ADPKD. Improvements in healthcare for individuals with ADPKD will therefore impact directly on patients, their families, and healthcare resources.

scholarly journals An Unusual Cardiac Manifestation in Autosomal Dominant Polycystic Kidney Disease

2012 ◽  
Vol 2012 ◽  
pp. 1-4
Author(s):  
Fausta Catapano ◽  
Stefano Pancaldi ◽  
Carlo Pace Napoleone ◽  
Lucia Barbara De Sanctis ◽  
Gaetano Gargiulo ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Polycystic Kidney ◽  
Autosomal Dominant Polycystic Kidney ◽  
Atrial Septum Aneurysm ◽  
Cardiac Manifestation ◽  
Stage Renal Disease ◽  
End Stage ◽  
Tissue Defects

Autosomal dominant polycystic kidney disease is a common hereditary disorder characterized by renal and extrarenal, cystic and noncystic manifestations. Connective tissue defects, including cerebral aneurysm, meningeal diverticula, abdominal wall hernias, intestinal diverticula, and cardiac valvular abnormalities, are widely known manifestations. Instead intracardiac aneurysms have never been reported in adults with autosomal dominant polycystic kidney disease. We describe a 65-year-old patient with end-stage renal disease due to autosomal dominant polycystic kidney disease and an atrial septum aneurysm associated with platypnoea-orthodeoxia syndrome.

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