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Saudi Journal of Kidney Diseases and Transplantation
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ORIGINAL ARTICLE Table of Contents   
Year : 2008  |  Volume : 19  |  Issue : 1  |  Page : 72-75
Outcome of Kidney Transplantation in Patients with Polycystic Kidney Disease: A Single Center Study


Shiraz Organ Transplant Center, Namazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran

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   Abstract 

Autosomal dominant polycystic kidney disease (ADPKD) is a common cause of end­stage renal disease and a common indication for renal transplantation. Patients with ADPKD show some differences in graft outcome and complications following renal transplantation. This study was undertaken to evaluate the demographics, outcome and complications of renal transplantation in patients with ADPKD. In a retrospective case-control design, 51 patients with ADPKD were recognized amongst a total of 1200 renal transplant patients. For each case, a matched control based on sex, age (± 5 years) and type of kidney donor, was selected. All relevant data were gathered using patients' records and PNOT software. There were 34 males (66.7%) and 17 females (33.3%) with ADPKD. Mean age at transplantation was 42.6 ± 14.3 years and source of donor organ was predominantly live unrelated (72.5%). Forty patients (78.4%) had extra-renal manifestations of ADPKD, the most common of which were cardiac valvular disease (24 cases, 47.1%), and liver cysts (10 cases, 19.6%). Rejection occurred in 12 patients in the case-group (23.5%) in comparison to nine patients (17.6%) in the control group (p > 0.05). Twenty-nine cases (56.9%) did not develop any complications. The common complications noted after transplantation included infections (15.7% in cases vs 19.6% in controls), and cerebrovascular accidents (13.7% in cases vs 16.6% in controls). Patient outcome after short- and long-term follow-up was slightly better in the ADPKD population than the control group; however, it was not statistically significant. Also, no complication was found to occur more frequently in ADPKD patients.

How to cite this article:
Roozbeh J, Razmkon AR, Jalaeian H, Raiss-Jalali GA, Behzadi S, Sagheb MM, Salahi H, Bahador A, Nikeghbalian S, Davari HR, Salehipour M, Malek-Hosseini SA. Outcome of Kidney Transplantation in Patients with Polycystic Kidney Disease: A Single Center Study. Saudi J Kidney Dis Transpl 2008;19:72-5

How to cite this URL:
Roozbeh J, Razmkon AR, Jalaeian H, Raiss-Jalali GA, Behzadi S, Sagheb MM, Salahi H, Bahador A, Nikeghbalian S, Davari HR, Salehipour M, Malek-Hosseini SA. Outcome of Kidney Transplantation in Patients with Polycystic Kidney Disease: A Single Center Study. Saudi J Kidney Dis Transpl [serial online] 2008 [cited 2019 Aug 25];19:72-5. Available from: http://www.sjkdt.org/text.asp?2008/19/1/72/37437

   Introduction Top


Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic kidney disease with a prevalence of one case per 1000. ADPKD accounts for 2 to 9% of patients with end-stage renal failure. [1],[2]

The main feature of kidney involvement in ADPKD is tubular dysmorphogenesis with formation of cysts secreting liquid. Renal cysts develop as a consequence of altered function and structure of tubular cells. Increasing size and number of cysts disrupt renal architecture, leading to terminal renal failure in 50% of patients over 60 years. [1] ADPKD is a multi-organ disorder; the extra­renal manifestations include hepatic cysts, colon diverticuli, intracranial aneurysms, aortic aneurysms, and valve abnormalities, which substantially increase cardiovascular risk.

Patients with ADPKD show some diffe­rences in graft outcome and complications following renal transplantation. The aim of the present retrospective case-control study was to describe the demographics, outcome and complications of renal transplantation in patients with ADPKD and compare them with other recipients at our center.


   Material and Methods Top


All study patients underwent kidney trans­plantation at the Shiraz Organ Transplan­tation Center, the only transplant center in Southern Iran, between December 1988 and December 2003. A total of 1200 kidneys were transplanted during this period. Detailed analysis of the patients' underlying renal diagnosis was performed, based on medical records from the transplant unit. Of the total, 51 transplantations were performed on patients diagnosed to have ADPKD. The diagnosis was based on the demonstration of enlarged polycystic kidneys by various radiologic methods before transplantation, and in indi­vidual patients it was further supported by a family history of the disease and/or the demonstration of cysts in the liver.

For each patient with ADPKD, a matched control was picked from the files of trans­plant recipients. Matching was made for sex, age (± 5 years) and type of kidney donor, cadaveric (CD) or living (LD). [Table - 1] shows the demographic data for ADPKD patients and controls.

The immunosuppressive protocol and general procedures used in our transplant unit consisted of cyclosporine A (CsA), mycophenolate mofetil (CellCept®) (MMF), azathioprine (Immuran®) (AZA), and pred­nisolone (Pred). MMF was first used in October 29, 2001. The operation technique included placing the graft in iliac fossa extraperitoneally.

Detailed clinical information was given to the patients by the nephrologists when they were referred for transplantation. After transplantation, patients with functioning grafts were seen in the transplant unit at regular follow-up visits indefinitely. Mean follow-up duration in the present study was 67 months (range 22-107 months).

The available records were reviewed and data on the course of the disease before and after transplantation were analyzed. Trans­plant data analyzed were: age at the time of diagnosis and transplantation, duration on dialysis before transplantation, age of donor, time and cause of graft loss and death, extra-renal manifestations of the ADPKD, source of graft, patient's survival at one, three, and five years post-transplantation and post-operative complications. The records of the control group were also reviewed by the same investigator using the same criteria and relevant data were noted.

Statistical analyses were performed using SPSS 11 (SPSS, Chicago, IL) package. Unless otherwise stated, values are mentioned as mean ± SD. Cumulative one-year, three-year, and five-year survival rates were calculated according to Kaplan-Meier analysis; Chi­square and T-tests were used to test the significance. P-values > 0.05 were considered not significant (NS).


   Results Top


The Demographic data of the 51 ADPKD patients and their matched controls are depicted in [Table - 1]. Forty (78.4%) of them had associated extra-renal manifestations, including: cardiac valvular disease (24 cases, 47.1%), and liver cysts (10 cases, 19.6%). Other less common co-morbid conditions were diabetes mellitus, congestive heart failure, ischemic heart disease, renal stones, urethral stricture, ulcerative colitis, hepatitis C infection, convulsions, and diverticulosis.

[Figure - 1] shows the one-, three-, and five­year survival rates among case and control subjects. Rejection occurred in 12 ADPKD patients (23.5%), in contrast to nine (17.6%) controls (P > 0.05). Twenty-nine ADPKD cases (56.9%) did not develop any com­plications. The most frequently encountered complications (other than rejection) were infections (cases: 15.7%, controls: 19.6%, P > 0.05), and cardiovascular accidents (cases: 13.7%, controls: 16.6%, P > 0.05).

Furthermore, hematoma, convulsions, basal cell carcinoma of skin, gastrointestinal obs­truction, peritonitis, rise in liver enzymes, pericardial effusion, anuria, and urine leakage were noted as post-transplant complications. No significant difference was found in the incidence of different complications among case and control subjects (P = 0.2).


   Discussion Top


ADPKD was seen in 51 of 1200 consecutive renal transplant recipients (8.3%), and was a common indication for renal transplantation in our study. Although ADPKD is inherited as an autosomal dominant disease, women were in the minority in our survey. This may be explained by a more benign course of the disease in women. [3] The source of the organ in the study group was predominantly live unrelated (72.5%); however, live unre­lated donors constitute only 41% of the total donor population in our center. [4] This diffe­rence seems to be due to the inheritable pattern of ADPKD, in which, live related kidneys might be ignored due to the fear of future appearance of polycystic disease.

Occurrence of extra-renal manifestations was not different from other studies. [3],[5],[6] However, cerebrovascular accidents were not encountered in our study, which may be due to the short period of follow-up for the majority of cases.

Short- and long-term survivals were slightly better in the ADPKD population than other subjects; however, it was not statistically significant. Similar studies have shown a good but not a better outcome for ADPKD patients. [3],[5]

No post-operative complication was found to occur more frequently in ADPKD patients. However, other studies have reported cardio­vascular, cerebrovascular, or malignancies to occur more commonly among these patients. [3],[5] Further studies with longer periods of follow-up and greater number of ADPKD participants are recommended to compare the complications and results of transplan­tation among ADPKD subjects with other recipients more precisely.

 
   References Top

1.Rozanski J, Kozlowska I, Myslak M, et al. Pretransplant nephrectomy in patients with autosomal dominant polycystic kidney disease. Transplant Proc 2005;37(2):666-8.  Back to cited text no. 1    
2.Singh S, Hariharan S. Renal replacement therapy in autosomal dominant polycystic kidney disease, Nephron 1991;57(1):40.  Back to cited text no. 2    
3.Hadimeri H, Norden G, Friman S, Nyberg G. Autosomal dominant polycystic kidney disease in a kidney transplant population. Nephrol Dial Transplant 1997;12(7):1431-6.  Back to cited text no. 3    
4.Malek-Hosseini S, Razmkon A, Mehdizadeh A, et al. Long-term results of renal transplantation: A single-center analysis of 1200 transplants. Transplant Proc 2006;38 (2):454-6.  Back to cited text no. 4    
5.Errasti P, Manrique J, Lavilla J, et al. Autosomal-dominant polycystic kidney disease: High prevalence of graft loss for death-related malignancies and cardiovascular risk factors. Transplant Proc 2003;35(5): 1717-9.  Back to cited text no. 5    
6.HTAuranen MH, Ala-Mello S, Turunen JA, Jarvela I. Further evidence for linkage of autosomal-dominant medullary cystic kidney disease on chromosome 1q21. Kidney Int 2001;60(4):1225-32  Back to cited text no. 6    

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Correspondence Address:
Jamshid Roozbeh
Associate Professor of Nephrology, Namazee Hospital, Shiraz University of Medical Sciences, Shiraz
Iran
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PMID: 18087127

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