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Darius T, Bertoni S, De Meyer M, Buemi A, Devresse A, Kanaan N, Goffin E, Mourad M. Simultaneous nephrectomy during kidney transplantation for polycystic kidney disease does not detrimentally impact comorbidity and graft survival. World J Transplant 2022; 12:100-111. [PMID: 35663541 PMCID: PMC9136716 DOI: 10.5500/wjt.v12.i5.100] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/31/2021] [Revised: 08/11/2021] [Accepted: 04/09/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND The lack of space, as an indication for a native unilateral nephrectomy for positioning a future kidney graft in the absence of other autosomal dominant polycystic kidney disease-related symptoms, remains controversial. AIM To evaluate the surgical comorbidity and the impact on graft survival of an associated ipsilateral native nephrectomy during isolated kidney transplantation in patients with autosomal dominant polycystic kidney disease. METHODS One hundred and fifty-four kidney transplantations performed between January 2007 and January 2019 of which 77 without (kidney transplant alone (KTA) group) and 77 with associated ipsilateral nephrectomy (KTIN group), were retrospectively reviewed. Demographics and surgical variables were analyzed and their respective impact on surgical comorbidity and graft survival. RESULTS Creation of space for future graft positioning was the main reason (n = 74, 96.1%) for associated ipsilateral nephrectomy. No significant difference in surgical comorbidity (lymphocele, wound infection, incisional hernia, wound hematoma, urinary infection, need for blood transfusion, hospitalization stay, Dindo Clavien classification and readmission rate) was observed between the two study groups. The incidence of primary nonfunction and delayed graft function was comparable in both groups [0% and 2.6% (P = 0.497) and 9.1% and 16.9% (P = 0.230), respectively, in the KTA and KTIN group]. The 1- and 5-year graft survival were 94.8% and 90.3%, and 100% and 93.8%, respectively, in the KTA and KTIN group (P = 0.774). The 1- and 5-year patient survival were 96.1% and 92.9%, and 100% and 100%, respectively, in the KTA and KTIN group (P = 0.168). CONCLUSION Simultaneous ipsilateral native nephrectomy to create space for graft positioning during kidney transplantation in patients with autosomal dominant polycystic kidney disease does not negatively impact surgical comorbidity and short- and long-term graft survival.
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Affiliation(s)
- Tom Darius
- Surgical and Abdominal Transplantation Unit, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Sébastien Bertoni
- Surgical and Abdominal Transplantation Unit, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Martine De Meyer
- Surgical and Abdominal Transplantation Unit, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Antoine Buemi
- Surgical and Abdominal Transplantation Unit, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Arnaud Devresse
- Division of Nephrology, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Nada Kanaan
- Division of Nephrology, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Eric Goffin
- Division of Nephrology, University Clinics Saint Luc, Brussels 1200, Belgium
| | - Michel Mourad
- Surgical and Abdominal Transplantation Unit, University Clinics Saint Luc, Brussels 1200, Belgium
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Hadimeri H, Johansson AC, Haraldsson B, Nyberg G. Capd in Patients with Autosomal Dominant Polycystic Kidney Disease. Perit Dial Int 2020. [DOI: 10.1177/089686089801800414] [Citation(s) in RCA: 33] [Impact Index Per Article: 6.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/17/2022] Open
Abstract
Objective To investigate whether there are specific complications to continuous ambulatory peritoneal dialysis (CAPD) in patients with autosomal dominant polycystic kidney disease (ADPKD) due to defects in various wall structures -causing hernia and diverticulitis -and to enlarged kidneys. Design The clinical experience of CAPD in 26 patients with ADPKD, treated for 11 ± 6 months, was studied in retrospect and compared with that of 26 contemporary controls. Medical records were reviewed with respect to survival in this treatment form and any complication. Peritoneal dialysis capacity (PDC), as measured in 21 ADPKD patients and 20 controls, was also evaluated. Setting University Hospital. Results Before initiation of CAPD, enlarged kidneys necessitated nephrectomy in 2 of 26 ADPKD patients; both cases were registered as preparation tor transplantation, not for CAPD. Survival in CAPD was similar in ADPKD patients and controls. Hernia was present in 4 ADPKD patients and 2 controls, and required transfer to hemodialysis in 1 patient from each group, temporarily. The incidence of peritonitis was 1 per 20 months in ADPKD patients versus 1 in 27 months in the controis, not significantly different. Peritonitis was caused by colonic bacteria in similar numbers. Residual renal function was 1.9 ± 2.1 mL/min per 1.73 m2 in ADPKD patients versus 1.9 ± 1.4 mL/min per 1.73 m2 in the controls. No difference was detected in any of the variables measured by PDC. Conclusion There were no specific problems related to ADPKD.
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Pandya BK, Friede T, Williams JD. A Comparison of Peritonitis in Polycystic and Non-Polycystic Patients on Peritoneal Dialysis. Perit Dial Int 2020. [DOI: 10.1177/089686080402400113] [Citation(s) in RCA: 21] [Impact Index Per Article: 4.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022] Open
Affiliation(s)
- Bhavna K. Pandya
- Institute of Nephrology University Hospital of Wales, Cardiff United Kingdom
| | - Tim Friede
- Medical Statistics Unit Lancaster University, Lancaster United Kingdom
| | - John D. Williams
- Institute of Nephrology University Hospital of Wales, Cardiff United Kingdom
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Harris T, Sandford R. European ADPKD Forum multidisciplinary position statement on autosomal dominant polycystic kidney disease care: European ADPKD Forum and Multispecialist Roundtable participants. Nephrol Dial Transplant 2019; 33:563-573. [PMID: 29309655 PMCID: PMC6018982 DOI: 10.1093/ndt/gfx327] [Citation(s) in RCA: 32] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/10/2017] [Indexed: 02/02/2023] Open
Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is a chronic, progressive condition characterized by the development and growth of cysts in the kidneys and other organs and by additional systemic manifestations. Individuals with ADPKD should have access to lifelong, multidisciplinary, specialist and patient-centred care involving: (i) a holistic and comprehensive assessment of the manifestations, complications, prognosis and impact of the disease (in physical, psychological and social terms) on the patient and their family; (ii) access to treatment to relieve symptoms, manage complications, preserve kidney function, lower the risk of cardiovascular disease and maintain quality of life; and (iii) information and support to help patients and their families act as fully informed and active partners in care, i.e. to maintain self-management approaches, deal with the impact of the condition and participate in decision-making regarding healthcare policies, services and research. Building on discussions at an international roundtable of specialists and patient advocates involved in ADPKD care, this article sets out (i) the principles for a patient-centred, holistic approach to the organization and delivery of ADPKD care in practice, with a focus on multispecialist collaboration and shared-decision making, and (ii) the rationale and knowledge base for a route map for ADPKD care intended to help patients navigate the services available to them and to help stakeholders and decision-makers take practical steps to ensure that all patients with ADPKD can access the comprehensive multispecialist care to which they are entitled. Further multispecialty collaboration is encouraged to design and implement these services, and to work with patient organizations to promote awareness building, education and research.
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Affiliation(s)
| | | | - Richard Sandford
- Academic Department of Medical Genetics, University of Cambridge School of Clinical Medicine, Cambridge, UK
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Boonpheng B, Thongprayoon C, Wijarnpreecha K, Medaura J, Chebib FT, Cheungpasitporn W. Outcomes of patients with autosomal‐dominant polycystic kidney disease on peritoneal dialysis: A meta‐analysis. Nephrology (Carlton) 2019; 24:638-646. [DOI: 10.1111/nep.13431] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/20/2018] [Indexed: 01/08/2023]
Affiliation(s)
- Boonphiphop Boonpheng
- Department of Internal MedicineEast Tennessee State University Johnson City Tennessee USA
| | - Charat Thongprayoon
- Department of Internal MedicineBassett Medical Centre Cooperstown New York USA
| | - Karn Wijarnpreecha
- Department of Internal MedicineBassett Medical Centre Cooperstown New York USA
| | - Juan Medaura
- Division of Nephrology, Department of MedicineUniversity of Mississippi Medical Centre Jackson Mississippi USA
| | - Fouad T Chebib
- Division of Nephrology and Hypertension, Department of MedicineMayo Clinic Rochester Minnesota USA
| | - Wisit Cheungpasitporn
- Division of Nephrology, Department of MedicineUniversity of Mississippi Medical Centre Jackson Mississippi USA
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Cheungpasitporn W, Thongprayoon C, Ungprasert P, Wijarnpreecha K, Kaewput W, Leeaphorn N, Bathini T, Chebib FT, Kröner PT. Subarachnoid Hemorrhage in Hospitalized Renal Transplant Recipients with Autosomal Dominant Polycystic Kidney Disease: A Nationwide Analysis. J Clin Med 2019; 8:524. [PMID: 30999564 PMCID: PMC6517948 DOI: 10.3390/jcm8040524] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/21/2019] [Revised: 04/12/2019] [Accepted: 04/15/2019] [Indexed: 12/21/2022] Open
Abstract
BACKGROUND This study aimed to evaluate the hospitalization rates for subarachnoid hemorrhage (SAH) among renal transplant patients with adult polycystic kidney disease (ADPKD) and its outcomes, when compared to non-ADPKD renal transplant patients. METHODS The 2005-2014 National Inpatient Sample databases were used to identify all hospitalized renal transplant patients. The inpatient prevalence of SAH as a discharge diagnosis between ADPKD and non-ADPKD renal transplant patients was compared. Among SAH patients, the in-hospital mortality, use of aneurysm clipping, hospital length of stay, total hospitalization cost and charges between ADPKD and non-ADPKD patients were compared, adjusting for potential confounders. RESULTS The inpatient prevalence of SAH in ADPKD was 3.8/1000 admissions, compared to 0.9/1000 admissions in non-ADPKD patients (p < 0.01). Of 833 renal transplant patients with a diagnosis of SAH, 30 had ADPKD. Five (17%) ADPKD renal patients with SAH died in hospitals compared to 188 (23.4%) non-ADPKD renal patients (p = 0.70). In adjusted analysis, there was no statistically significant difference in mortality, use of aneurysm clipping, hospital length of stay, or total hospitalization costs and charges between ADPKD and non-ADPKD patients with SAH. CONCLUSION Renal transplant patients with ADPKD had a 4-fold higher inpatient prevalence of SAH than those without ADPKD. Further studies are needed to compare the incidence of overall admissions in ADPKD and non-ADPKD patients. When renal transplant patients developed SAH, inpatient mortality rates were high regardless of ADPKD status. The outcomes, as well as resource utilization, were comparable between the two groups.
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Affiliation(s)
- Wisit Cheungpasitporn
- Division of Nephrology, Department of Medicine, University of Mississippi Medical Center, Jackson, MS 39216, USA.
| | - Charat Thongprayoon
- Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN 55905, USA.
| | - Patompong Ungprasert
- Clinical Epidemiology Unit, Department of Research and Development, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand.
| | - Karn Wijarnpreecha
- Department of Medicine, Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, FL 32224, USA.
| | - Wisit Kaewput
- Department of Military and Community Medicine, Phramongkutklao College of Medicine, Bangkok 10400, Thailand.
| | - Napat Leeaphorn
- Department of Nephrology, Department of Medicine, Saint Luke's Health System, Kansas City, MO 64111, USA.
| | - Tarun Bathini
- Department of Internal Medicine, University of Arizona, Tucson, AZ 85721, USA.
| | - Fouad T Chebib
- Division of Nephrology and Hypertension, Mayo Clinic, Rochester, MN 55905, USA.
| | - Paul T Kröner
- Department of Medicine, Division of Gastroenterology and Hepatology, Mayo Clinic, Jacksonville, FL 32224, USA.
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Effect of Sirolimus on Native Total Kidney Volume After Transplantation in Patients with Autosomal Dominant Polycystic Kidney Disease: A Randomized Controlled Pilot Study. Transplant Proc 2018; 50:1243-1248. [PMID: 29880342 DOI: 10.1016/j.transproceed.2018.02.060] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/31/2017] [Revised: 02/06/2018] [Accepted: 02/19/2018] [Indexed: 12/15/2022]
Abstract
BACKGROUND The mammalian target of rapamycin (mTOR) pathway has been shown to be central to cyst formation and growth in patients with autosomal dominant polycystic kidney disease (ADPKD). Drugs that suppress mTOR signaling are frequently used as antiproliferative agents for maintenance immunosuppression in patients who have undergone kidney transplantation. The aim of this study was to determine the effect of sirolimus, an mTOR inhibitor, on cyst volume regression in patients with ADPKD who have undergone renal transplantation. METHODS In this single-center, prospective, open-label, parallel-group, randomized trial, 23 adult patients with ADPKD who successfully underwent renal transplantation from 2008 to 2012 were subsequently randomized (on a 1:1 basis) to a maintenance immunosuppression regimen with either sirolimus (sirolimus, tacrolimus, prednisone) or mycophenolate (mycophenolate, tacrolimus, prednisone). Total kidney volumes were measured by means of high-resolution magnetic resonance imaging within 2 weeks after transplantation and at 1 year. The primary end point was change in total kidney volume at 1 year. RESULTS Sixteen patients completed the 1-year study (8 patients in each group). There was a decrease in kidney volume in both the sirolimus group (percentage change from baseline, 20.5%; P < .001) and mycophenolate group (percentage change from baseline, 17%; P = .048), but there was no significant difference in percentage change of total kidney volume between the groups (P = .665). CONCLUSIONS In ADPKD patients at 1 year after kidney transplantation, there was a similar decrease in polycystic kidney volume in patients receiving an immunosuppression regimen containing sirolimus compared with patients receiving mycophenolate.
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Amro OW, Perrone RD. How Does a Patient's Primary Renal Disease Impact Chronic Dialysis Management?: Patients with Autosomal Dominant Polycystic Kidney Disease. Semin Dial 2015; 28:470-3. [PMID: 26012364 DOI: 10.1111/sdi.12397] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/28/2022]
Affiliation(s)
- Osama W Amro
- Division of Nephrology, Department of Medicine, Tufts Medical Center and Tufts University School of Medicine, Boston, Massachusetts
| | - Ronald D Perrone
- Division of Nephrology, Department of Medicine, Tufts Medical Center and Tufts University School of Medicine, Boston, Massachusetts
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Kanaan N, Devuyst O, Pirson Y. Renal transplantation in autosomal dominant polycystic kidney disease. Nat Rev Nephrol 2014; 10:455-65. [PMID: 24935705 DOI: 10.1038/nrneph.2014.104] [Citation(s) in RCA: 54] [Impact Index Per Article: 4.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
In patients with autosomal dominant polycystic kidney disease (ADPKD) evaluated for kidney transplantation, issues related to native nephrectomy, cystic liver involvement, screening for intracranial aneurysms and living-related kidney donation deserve special consideration. Prophylactic native nephrectomy is restricted to patients with a history of cyst infection or recurrent haemorrhage or to those in whom space must be made to implant the graft. Patients with liver involvement require pretransplant imaging. Selection of patients for pretransplant screening of intracranial aneurysms should follow the general recommendations for patients with ADPKD. In living related-donor candidates aged <30 years and at-risk of ADPKD, molecular genetic testing should be carried out when ultrasonography and MRI findings are normal or equivocal. After kidney transplantation, patient and graft survival rates are excellent and the volume of native kidneys decreases. However, liver cysts continue to grow and treatment with a somatostatin analogue should be considered in patients with massive cyst involvement. Cerebrovascular events have a marginal effect on post-transplant morbidity and mortality. An increased risk of new-onset diabetes mellitus and nonmelanoma skin cancers has been reported, but several studies have challenged these findings. Finally, no data currently support the preferential use of mammalian target of rapamycin inhibitors as immunosuppressive agents in transplant recipients with ADPKD.
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Affiliation(s)
- Nada Kanaan
- Division of Nephrology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, 10 Avenue Hippocrate, B-1200 Brussels, Belgium
| | - Olivier Devuyst
- Division of Nephrology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, 10 Avenue Hippocrate, B-1200 Brussels, Belgium
| | - Yves Pirson
- Division of Nephrology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, 10 Avenue Hippocrate, B-1200 Brussels, Belgium
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He Y, Wang Q, Zhang M, Wang B, Xiong Z, Luo Q, Wu S. Abdominal Aortic Dissection in a Patient With Autosomal Dominant Polycystic Kidney Disease After Starting Peritoneal Dialysis. Urol Case Rep 2014; 2:123-5. [PMID: 26839787 PMCID: PMC4735488 DOI: 10.1016/j.eucr.2014.04.004] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/12/2014] [Accepted: 04/16/2014] [Indexed: 11/30/2022] Open
Abstract
Autosomal dominant polycystic kidney disease (ADPKD), one of the most common genetic disorders, is caused by mutations in the PKD1 or PKD2 gene. ADPKD primarily affects the kidneys, causing the development of multiple bilateral cysts that are characteristic of this condition. Besides renal abnormalities, other manifestations of ADPKD include hepatic, pancreatic, and splenic cysts, intracranial aneurysms, aortic aneurysms, and mitral valve prolapse. Reports of ADPKD-associated abdominal aortic dissections are not rare, but there have been no reports of an ADPKD patient developing intestinal obstruction and abdominal aortic dissection after initiating peritoneal dialysis. Herein, we present one such case.
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Affiliation(s)
- Yingying He
- Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, China
| | - Qin Wang
- Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, China
| | - Meng Zhang
- Department of Urological Surgery, Shenzhen Second People's Hospital, The First Affiliated Hospital of Shenzhen University, Shenzhen, China
| | - Bo Wang
- Department of Urological Surgery, Shenzhen Second People's Hospital, The First Affiliated Hospital of Shenzhen University, Shenzhen, China
| | - Zuying Xiong
- Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, China
| | - Qiong Luo
- Department of Nephrology, Peking University Shenzhen Hospital, Shenzhen, China
| | - Song Wu
- Department of Urological Surgery, Shenzhen Second People's Hospital, The First Affiliated Hospital of Shenzhen University, Shenzhen, China; Department of Tumor Immunology, Zhongshan School of Medicine, Sun Yat-sen University, Guangzhou, China
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11
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Yoo DJ, Agodoa L, Yuan CM, Abbott KC, Nee R. Risk of intracranial hemorrhage associated with autosomal dominant polycystic kidney disease in patients with end stage renal disease. BMC Nephrol 2014; 15:39. [PMID: 24571546 PMCID: PMC3939494 DOI: 10.1186/1471-2369-15-39] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/23/2013] [Accepted: 02/12/2014] [Indexed: 11/10/2022] Open
Abstract
BACKGROUND An analysis of intracranial hemorrhage (ICH) in a national sample of autosomal dominant polycystic kidney disease (ADPKD) patients receiving long-term dialysis has not been reported. It is often assumed that patients with ADPKD are not at increased risk of ICH after starting dialysis. We hypothesized that patients with ADPKD would have a higher subsequent risk of ICH even after the start of chronic dialysis. METHODS Retrospective cohort study of Medicare primary patients with and without ADPKD in the United States Renal Data System (USRDS), initiated on chronic dialysis or transplanted between 1 January 1999 and 3 July 2009, and followed until 31 December 2009. Covariates included age, gender, race, prior stroke, diabetes mellitus, dialysis modality, body mass index, serum albumin and other co-morbid conditions from the Medical Evidence Form. Primary outcome was ICH, based on inpatient and outpatient Medicare claims, and all-cause mortality. Kaplan-Meier analysis was used for unadjusted assessment of time to events. Cox regression was used for assessment of factors associated with ICH and mortality. We performed competing risk regression using kidney transplant and death as competing risks. Kidney transplant was also modeled as a time-dependent covariate in Cox regression. RESULTS Competing risk regression demonstrated that ADPKD had a subhazard ratio 2.97 for ICH (95% CI 2.27-3.89). Adjusted Cox analysis showed that ADPKD patients had an AHR for death of 0.59 vs. non-ADPKD patients (95% CI 0.57-0.61). CONCLUSIONS ADPKD is a significant risk factor for ICH among patients on maintenance dialysis. Our Medicare primary cohort was older than in previous studies of intracranial aneurysm rupture among ADPKD patients. There are also limitations inherent to using the USRDS database.
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Affiliation(s)
| | | | | | - Kevin C Abbott
- Nephrology, Walter Reed National Military Medical Center, 8901 Wisconsin Ave, Bethesda, MD, USA.
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Potluri K, Holt D, Hou S. Neurologic complications in renal transplantation. HANDBOOK OF CLINICAL NEUROLOGY 2014; 121:1245-1255. [PMID: 24365416 DOI: 10.1016/b978-0-7020-4088-7.00084-5] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 06/03/2023]
Abstract
Neurologic complications following kidney transplant are more common than in the general population with the reported incidence around 10-21%. Need for multiple drugs, decreased cellular immunity, accelerated atherosclerotic disease, and frequency of metabolic abnormalities are the most common predisposing factors for neurologic abnormalities. Neurologic side-effects of calcineurin inhibitors range from mild tremors to paraplegia or posterior reversible encephalopathy syndrome (PRES) and are generally reversible by lowering the dose or complete discontinuation of the drug when possible. Clinical presentation of central nervous system infection in transplant recipients can be different from the normal population as the anti-inflammatory effects of immunosuppressive therapy may obscure signs of meningeal inflammation and changes in the level of consciousness may be subtle. Bacterial infections remain the most common infections but unusual pathogens figure prominently in the differential diagnosis. The most frequent malignancies of the brain are lymphomas and metastatic tumors which are for the most part, de novo malignancies from immunosuppression. Decreasing immunosuppression is almost always a part of treating malignancy. The prevalence of stroke is reported to be around 8% with age>40 years, diabetic nephropathy as the underlying cause of end-stage kidney disease, and peripheral vascular disease being the strongest predictors.
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Affiliation(s)
- Kavitha Potluri
- Department of Medicine, Division of Nephrology and Hypertension, Loyola University Medical Center, Maywood, IL, USA.
| | - David Holt
- Department of Surgery, Loyola University Medical Center, Maywood, IL, USA
| | - Susan Hou
- Department of Medicine, Division of Nephrology and Hypertension, Loyola University Medical Center, Maywood, IL, USA
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Helal I, Reed B, Mettler P, Mc Fann K, Tkachenko O, Yan XD, Schrier RW. Prevalence of cardiovascular events in patients with autosomal dominant polycystic kidney disease. Am J Nephrol 2012; 36:362-70. [PMID: 23038404 DOI: 10.1159/000343281] [Citation(s) in RCA: 56] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/11/2012] [Accepted: 09/10/2012] [Indexed: 11/19/2022]
Abstract
BACKGROUND This study evaluates the prevalence of cardiovascular events in autosomal dominant polycystic kidney disease (ADPKD) patients. METHODS We distributed surveys to 1,439 subjects from our ADPKD research database. In total, 426 subjects completed and returned surveys; 7 of these were from children and were excluded from the study. RESULTS The patients who responded were female (63.2%), nonHispanic (88.1%) and white (93.6%). The mean age of the total group was 53.2 ± 13.7 years; 82.8% had a family history of ADPKD and 32.5% had reached end-stage renal disease (ESRD). With respect to cardiovascular risk factors, 86.6% were hypertensive with a mean age at diagnosis of 36.9 ± 12.9 years and hypertension was significantly more prevalent in males. In addition, 19.6% of the subjects were obese, 20.8% were smokers, 8.7% had diabetes, 45.7% had high cholesterol and 17.8% were sedentary. The most prevalent self-reported cardiovascular events were arrhythmias (25.9%), evidence of peripheral vascular disease (16.5%), heart valve problems (14.4%), cardiac enlargement (9.5%), stroke or cerebral bleeding (7.5%), myocardial infarction (6%) and brain aneurysm (5.0%). The most commonly used antihypertensive medications were renin-angiotensin inhibitors used by 75% of ADPKD patients. Older ADPKD patients and those at ESRD had a significantly higher incidence of cardiovascular events. CONCLUSION These findings support the high prevalence of cardiovascular risk factors and events in ADPKD patients which contribute to a greater mortality risk. Due to the prevalence of cardiovascular risk factors in the ADPKD population, early diagnosis and clinical intervention are recommended.
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Affiliation(s)
- Imed Helal
- Division of Renal Diseases and Hypertension, University of Colorado Denver, Aurora, Colo. 80045, USA
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14
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Lukowsky LR, Molnar MZ, Zaritsky JJ, Sim JJ, Mucsi I, Kovesdy CP, Kalantar-Zadeh K. Mineral and bone disorders and survival in hemodialysis patients with and without polycystic kidney disease. Nephrol Dial Transplant 2011; 27:2899-907. [PMID: 22207323 DOI: 10.1093/ndt/gfr747] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/28/2023] Open
Abstract
BACKGROUND Maintenance hemodialysis (MHD) patients with polycystic kidney disease (PKD) have better survival than non-PKD patients. Mineral and bone disorders (MBD) are associated with accelerated atherosclerosis and cardiovascular death in MHD patients. It is unknown whether the different MBD mortality association between MHD populations with and without PKD can explain the survival differential. METHODS Survival models were examined to assess the association between different laboratory markers of MBD [such as serum phosphorous, parathyroid hormone (PTH), calcium and alkaline phosphatase] and mortality in a 6-year cohort of 60,089 non-PKD and 1501 PKD MHD patients. RESULTS PKD and non-PKD patients were 57±13 and 62±15 years old and included 46 and 45% women and 14 and 32% Blacks, respectively. Whereas PKD individuals with PTH 150 to <300 pg/mL (reference) had the lowest risk for mortality, the death risk was higher in patients with PTH<150 [hazard ratio (HR): 2.16 (95% confidence interval 1.53-3.06)], 300 to <600 [HR: 1.30 (0.97-1.74)] and ≥600 pg/mL [HR: 1.46 (1.02-2.08)], respectively. Similar patterns were found in non-PKD patients. Fully adjusted death HRs of time-averaged serum phosphorous increments<3.5, 5.5 to <7.5 and ≥7.5 mg/dL (reference: 3.5 to <5.5 mg/dL) for PKD patients were 2.82 (1.50-5.29), 1.40 (1.12-1.75) and 2.25 (1.57-3.22). The associations of alkaline phosphatase and calcium with mortality were similar in PKD and non-PKD patients. CONCLUSION Bone-mineral disorder markers exhibit similar mortality trends between PKD and non-PKD MHD patients, although some differences are observed in particular in low PTH and phosphorus ranges.
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Affiliation(s)
- Lilia R Lukowsky
- Harold Simmons Center for Chronic Disease Research and Epidemiology, Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center, Torrance, CA, USA
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15
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Molnar MZ, Lukowsky LR, Streja E, Dukkipati R, Jing J, Nissenson AR, Kovesdy CP, Kalantar-Zadeh K. Blood pressure and survival in long-term hemodialysis patients with and without polycystic kidney disease. J Hypertens 2010; 28:2475-84. [PMID: 20720499 PMCID: PMC3169709 DOI: 10.1097/hjh.0b013e32833e4fd8] [Citation(s) in RCA: 38] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/27/2022]
Abstract
BACKGROUND In maintenance dialysis patients, low blood pressure (BP) values are associated with higher death rates when compared with normal to moderately high values. This 'hypertension paradox' may be related to comorbid conditions. Dialysis patients with polycystic kidney disease (PKD) usually have a lower comorbidity burden and greater survival. We hypothesized that in PKD dialysis patients, a representative of a healthier dialysis patient population, high BP is associated with higher mortality. METHODS Time-dependent survival models including after multivariate adjustment were examined to assess the association between prehemodialysis and posthemodialysis BP and all-cause mortality in a 5-year cohort of 67 085 non-PKD and 1579 PKD hemodialysis patients. RESULTS In PKD patients, low prehemodialysis and posthemodialysis SBPs were associated with increased mortality, whereas high prehemodialysis DBP was associated with greater survival. Fully adjusted death hazard ratios (and 95% confidence levels) for prehemodialysis and posthemodialysis BP of less than 120 mmHg (reference 140 to <160 mmHg) were 1.30 (1.06-1.92) and 1.45 (1.04-2.02), respectively, and for prehemodialysis DBP of 80 mmHg or more (reference 70 to <80 mmHg) was 0.68 (0.49-0.93, all P values <0.05). Similar associations were observed in non-PKD patients. In pooled analyses, within each commensurate BP stratum, PKD patients exhibited superior survival to non-PKD patients. CONCLUSION Among hemodialysis patients, those with PKD display a similar BP paradox as those without PKD, even though within each BP category PKD patients maintain superior survival. Randomized clinical trials are needed to define optimal blood pressure targets in the hemodialysis population.
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Affiliation(s)
- Miklos Z Molnar
- Harold Simmons Center for Chronic Disease Research & Epidemiology, Los Angeles Biomedical Research Institute at Harbor-UCLA Medical Center, Torrance, California, USA
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Halvorson CR, Bremmer MS, Jacobs SC. Polycystic kidney disease: inheritance, pathophysiology, prognosis, and treatment. Int J Nephrol Renovasc Dis 2010; 3:69-83. [PMID: 21694932 PMCID: PMC3108786 DOI: 10.2147/ijnrd.s6939] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/22/2010] [Indexed: 01/09/2023] Open
Abstract
Both autosomal dominant and recessive polycystic kidney disease are conditions with severe associated morbidity and mortality. Recent advances in the understanding of the genetic and molecular pathogenesis of both ADPKD and ARPKD have resulted in new, targeted therapies designed to disrupt cell signaling pathways responsible for the abnormal cell proliferation, dedifferentiation, apoptosis, and fluid secretion characteristic of the disease. Herein we review the current understanding of the pathophysiology of these conditions, as well as the current treatments derived from our understanding of the mechanisms of these diseases.
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Affiliation(s)
- Christian R Halvorson
- Department of Surgery, University of Maryland School of Medicine, Baltimore, MD, USA.
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Alam A, Perrone RD. Management of ESRD in patients with autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis 2010; 17:164-72. [PMID: 20219619 DOI: 10.1053/j.ackd.2009.12.006] [Citation(s) in RCA: 38] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/15/2009] [Revised: 12/24/2009] [Accepted: 12/26/2009] [Indexed: 01/13/2023]
Abstract
Autosomal dominant polycystic kidney disease (ADPKD) is the leading hereditary cause of ESRD in the United States. Because of the renal and extrarenal manifestations of ADPKD, specific challenges exist caring for these patients once they reach ESRD. In this article, we report the overall outcomes of individuals with ADPKD after ESRD as compared with non-ADPKD patients. We also review the available literature concerning issues specific to dialysis or kidney transplantation. For the ADPKD patient on dialysis, we address the use of peritoneal dialysis, the management of renal cystic, and extrarenal complications, and we discuss the significance of the relative polycythemia often observed in this population. For the ADPKD patient undergoing kidney transplantation, we highlight issues of anemia management and aneurysm screening pretransplant, the indications for nephrectomy of the native ADPKD kidneys, the potential benefits of select immunosuppressive agents, the role for combined kidney-liver transplantation, and renal and extrarenal complications of ADPKD postkidney transplantation. In general, patients with ADPKD have more favorable outcomes after ESRD as compared with those with other causes of kidney failure. Most of our knowledge, however, is based on case series and observational studies. Although these reports have certainly been valuable to our understanding, there still remains considerable uncertainty and ambiguity in many aspects of ADPKD patient care as it relates to ESRD. Particular focus needs to be placed on performing clinical trials with the goal of enhancing outcomes and quality of life of patients with ADPKD.
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Autosomal-Dominant Polycystic Kidney Disease and Kidney Transplantation: Experience of a Single Center. Transplant Proc 2009; 41:887-90. [DOI: 10.1016/j.transproceed.2009.01.069] [Citation(s) in RCA: 14] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
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Kumar S, Fan SLS, Raftery MJ, Yaqoob MM. Long term outcome of patients with autosomal dominant polycystic kidney diseases receiving peritoneal dialysis. Kidney Int 2008; 74:946-51. [DOI: 10.1038/ki.2008.352] [Citation(s) in RCA: 45] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
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Transplantation rénale pour polykystose rénale autosomique dominante: spécificités de la préparation et du suivi des patients. Nephrol Ther 2007; 3:449-55. [DOI: 10.1016/j.nephro.2007.07.002] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/12/2007] [Revised: 07/08/2007] [Accepted: 07/09/2007] [Indexed: 11/17/2022]
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Oliveras A, Roquer J, Puig JM, Rodríguez A, Mir M, Orfila MA, Masramon J, Lloveras J. Stroke in renal transplant recipients: epidemiology, predictive risk factors and outcome. Clin Transplant 2003; 17:1-8. [PMID: 12588314 DOI: 10.1034/j.1399-0012.2003.02042.x] [Citation(s) in RCA: 75] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/23/2022]
Abstract
BACKGROUND Cerebrovascular and cardiovascular diseases are the most important causes of increased morbidity and mortality in patients with end-stage renal disease. Stroke has been widely reported in chronic dialysis patients, but there is scarce information about stroke in renal transplant recipients (RTR), although cerebrovascular events are the most common and potentially life-threatening neurological complications in them. Our aim is to analyze the prevalence, risk factors, etiopathogenia, clinical aspects and outcome of stroke in RTR. METHODS We analyzed 403 patients who received one or more renal grafts between 1979 and 2000: group A = patients who had stroke (n = 19); group B = those who did not (n = 384). Medical records and pertinent data were compiled. The risk of stroke was studied using univariate and multivariate Cox regression models. RESULTS prevalence of stroke in RTR was 7.97% at 10 yr. Time elapsed between renal transplantation (RT) and stroke: 49.3 months. Possible risk factors based on the univariate analyses were: diabetic nephropathy (DN) (p < 0.001) and autosomal-dominant-polycystic-kidney-disease (p = 0.049) as original nephropathies, peripheral vascular disease (PVD) (p < 0.001), diabetes mellitus prior to RT (p = 0.005), age older than 40 yr (p = 0.037) and hypertension (p = 0.049). Other analysed risk factors such as gender, renal function, cytomegalovirus infection, hyperlipidemia, hyperuricemia, erythrocytosis or hypertensive donor failed to show any significant predictive value for stroke in these patients. When multivariate analyses were carried out, we found that DN (OR = 4.8; p = 0.010), PVD (OR = 8.2; p < 0.001) and age > 40 yr (OR = 3.3; p = 0.019) were predictive risk factors for stroke. For group A, hypertension was present in all patients, 68.4% had hyperlipidemia and 42.1% reported previous stroke. Cerebral hemorrhage occurred in seven of 19 (36.84%) of the stroke patients, but no subarachnoid hemorrhage occurred in them. Seven of 12 ischemic strokes were atherotrombotic. Considering all strokes, basal ganglia was the predominant localization. The outcome was poor, as nearly half of the patients died in the 3 months following stroke. CONCLUSIONS Prevalence of stroke in our RTR population was 7.97%. Cerebral hemorrhage appears to be more prevalent in RTR than in general population. More than that, the cerebral hemorrhage rate we found is higher than that reported elsewhere in RTR. The main predictors of stroke were DN, PVD and age. No patient with interstitial nephropathy suffered stroke. Mortality is high in RTR with stroke.
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Affiliation(s)
- Anna Oliveras
- Department of Nephrology, Hospital Universitari del Mar, Barcelona, Spain.
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Nicolau C, Torra R, Bianchi L, Vilana R, Gilabert R, Darnell A, Brú C. Abdominal sonographic study of autosomal dominant polycystic kidney disease. JOURNAL OF CLINICAL ULTRASOUND : JCU 2000; 28:277-282. [PMID: 10867665 DOI: 10.1002/1097-0096(200007/08)28:6<277::aid-jcu2>3.0.co;2-l] [Citation(s) in RCA: 39] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/23/2023]
Abstract
PURPOSE The purpose of this study was to determine whether kidney size in patients who have autosomal dominant polycystic kidney disease (ADPKD) is related to renal function, hypertension, or extrarenal manifestations of the disease and to sonographically evaluate the abdominal manifestations of ADPKD. METHODS Between 1994 and 1998, 400 individuals from 85 families with a history of ADPKD were examined. There were 213 persons with ADPKD and 187 unaffected family members; there were 182 males and 218 females, 1-82 years old (mean, 39.3 years). We obtained a complete medical history, performed a physical examination, measured the arterial blood pressure and serum creatinine levels, and performed abdominal sonography on each subject. The sonographic features that were studied were renal length and the presence and number of cysts on the kidneys, liver, and pancreas. RESULTS There was a relationship between kidney size and age (p < 0.05), kidney size and renal function (p < 0.001), and kidney size and hypertension (p < 0.001). The overall prevalence of hepatic cysts in patients with ADPKD was 67%, and the prevalence increased with age. The presence of hepatic cysts was related to the severity of renal disease. Females had more severe polycystic liver disease, and massive polycystic liver disease (ie, hepatomegaly with innumerable cysts) was seen only in females. The prevalence of pancreatic cysts in the 187 persons in whom the pancreas was well evaluated sonographically was 5%. CONCLUSIONS Kidney size in patients with ADPKD is related to renal function, hypertension, and extrarenal involvement and can be used to predict the outcome of the disease. Hepatic cysts are very common in patients with ADPKD and are related to age and renal function; pancreatic cysts are infrequent in these patients.
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Affiliation(s)
- C Nicolau
- Imaging Diagnosis Center, Ultrasound Unit, Hospital Clínic, University of Barcelona, Spain.
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Norby SM, Torres VE. Complications of autosomal dominant polycystic kidney disease in hemodialysis patients. Semin Dial 2000; 13:30-5. [PMID: 10740669 DOI: 10.1046/j.1525-139x.2000.00010.x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Affiliation(s)
- S M Norby
- Department of Nephrology/Internal Medicine, Mayo Clinic, Rochester, MN 55905, USA
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