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Saudi Journal of Kidney Diseases and Transplantation
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ORIGINAL ARTICLE Table of Contents   
Year : 2009  |  Volume : 20  |  Issue : 5  |  Page : 806-810
Analysis of causes of mortality in patients with autosomal dominant polycystic kidney disease: A single center study


Department of Medicine, King Khalid University Hospital, Riyadh, Saudi Arabia

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Date of Web Publication2-Sep-2009
 

   Abstract 

This study was aimed at determining the median survival and most frequent causes of death in patients with the Autosomal Dominant Polycystic Kidney Disease (ADPKD). A retrospective, observational analysis was made on patients registered with a diagnosis of ADPKD, in the computer records of the Sheffield Kidney Institute (SKI), United Kingdom, during the years 1981 to 1999. Data on 363 patients were analyzed from these computer records and further infor­mation, if any, was obtained from the patients' clinical notes. During this period, 88 patients died. The median age of the patients who died was 60.5 years, with the youngest being 37 years old and the oldest being 82 years. The major causes of death in this study group were cardiovascular (46.6%), infection (15.9%), central nervous system (CNS) disorders (11.36%), and miscellaneous causes (11.36%). Our study suggests that the major cause of death in patients with ADPKD was cardiovascular followed by infection, of which 42% of the deaths were due to septicemia. CNS causes of death comprised 11.36% of whom 60% had cerebrovascular events including sub­arachnoid hemorrhage in 20% of the patients. Uremia was the cause of death in only 2.2% of the patients in this series.

How to cite this article:
Rahman E, Niaz FA, Al-Suwaida A, Nahrir S, Bashir M, Rahman H, Hammad D. Analysis of causes of mortality in patients with autosomal dominant polycystic kidney disease: A single center study. Saudi J Kidney Dis Transpl 2009;20:806-10

How to cite this URL:
Rahman E, Niaz FA, Al-Suwaida A, Nahrir S, Bashir M, Rahman H, Hammad D. Analysis of causes of mortality in patients with autosomal dominant polycystic kidney disease: A single center study. Saudi J Kidney Dis Transpl [serial online] 2009 [cited 2019 Jun 24];20:806-10. Available from: http://www.sjkdt.org/text.asp?2009/20/5/806/55366

   Introduction Top


Autosomal Dominant Polycystic Kidney Disease (ADPKD)) is the most common hereditary kidney disease, [1] characterized by replacement of functioning renal parenchyma with cysts and increase in the volume of the kidney. Patients with ADPKD are at high risk for renal and extra-renal complications, resulting in high morbidity and mortality, especially due to car­diovascular complications. [2]

The reported mortality rate in patients with ADPKD ranges between 1.6 and 2.88 folds (95% confidence interval, R/R 1.3-2.0) in com­parison with the general population. [3] ADPKD patients accounts for nearly 10% of all cases of Chronic Renal Failure (CRF) requiring dialysis or renal transplantation. [1],[4] They are prone to end-stage renal disease (ESRD) and carry a poor survival rate. [5] Hypertension occurs at an early age in such patients. [6]

Cysts in the liver and hepatic failure are among the common extra-renal manifestations adding to morbidity and mortality. [7] Cardiovascular complications are common, progressive and often fatal; thus, ADPKD patients have an unfavorable risk profile. [8] There is a progre­ssive increase in ventricular mass index, left ventricular hypertrophy (LVH), valvular pro­lapse and hypertension. [9],[10] Coronary and intra­cranial aneurysms and their rupture contribute further to mortality. [11] Complications are more commonly seen in whites. [12],[13] Acute coronary dissection, although rare, is a dreaded compli­cation. [14] Infections also add to fatality in pa­tients with ADPKD.


   Materials and Methods Top


This is a retrospective, observational study using computer records of the Sheffield Kidney Institute (SKI), the United Kingdom. A total of 363 patients with a diagnosis of ADPKD were registered between 1981 and 1999. Further in­formation on these patients, if required, was obtained from their clinical notes. The total number of patients who died of ADPKD during the study period was 88. The diagnosis of ADPKD was made by clinical examination, urography, and ultrasound or CT scan of abdo­men. The dominant inheritance of polycystic kidney disease was diagnosed from positive family history. However, the gene distinguishing the two variants of ADPKD was not looked for.


   Statistical Analysis Top


The median age of survival in this group and distribution of causes of death was calculated using Microsoft Excel. The incidence of diffe­rent associations and complications were also calculated using the same program.


   Results Top


The median age at death of the study patients was 60.5 years, with the youngest being 37 years and the oldest, 82 years. The major causes of death in this group of patients included car­diovascular in 46.6% of the patients, infection in 16%, central nervous system (CNS) dis­orders in 11.36% and miscellaneous causes in 11.36% of the patients [Figure 1]. The misce­llaneous group included one patient who was found not suitable for dialysis, one patient committed suicide, one patient died of gastro­intestinal (GI) bleeding and one other patient died of rheumatoid arthritis. Uremia caused death in only 2.2% of the patients in this series. In both cases, death occurred due to voluntary refusal to undergo dialysis.

Cardiovascular causes of death

The principal cardiovascular causes of death included Ischemic Heart Disease (IHD) in 46.3%, Hypertensive Heart Disease (HHT) in 8.8% and Congestive Cardiac Failure (CCF) in 17.1% [Figure 2]. Two patients died of retro­peritoneal hemorrhage secondary to aortic dissection.

CNS causes of death

In order of decreasing frequency, the CNS causes of mortality included cerebrovascular accidents (CVA) in 60%, sub-arachnoid hemo­rrhage (SAH) in 20% and brain-stem hemo­rrhage and intracerebral hemorrhage in 10% each. The distribution of different CNS causes of death is shown in [Figure 3].

Infectious causes of death

Infections accounted for 15.9% of the causes of death in this series. The leading cause was septicemia (42.9%) followed by pneumonia (35.7%), peritonitis (14.3%) and infective endo­carditis in 7.1% of the patients.

Miscellaneous causes of death

This series comprised 11.36% of the study patients. Their distribution is summarized in [Table 1].


   Discussion Top


Mortality in patients with ADPKD is higher than in the general population. The present study reveals that the median age at death was 60 years while life span as low as 37 years was observed. The most frequent cause of death was cardiovascular events, accounting for 46.6% of the total mortality. Amongst them, IHD seen in 46.3% and CHF, seen in 17.1%, were the common causes. Similar results have been reported by Fick GM et al from Colorado, USA. In their study, cardiovascular diseases were responsible for 36% of deaths; followed by infection in 24%. [13] Cardiac hypertrophy was found in 89% of patients in their study.

In another study from the Netherlands, Florijin KW et al demonstrated a relative risk of 2.88 (95% CI: 1.41-5.9) for cardiovascular mortality after adjustment for age. In a report from Canada in 2006, Handa SP described an un­favorable cardiovascular risk profile respon­sible for increased risk for cardiovascular mor­tality. [9]

Infection was another important cause of death in the present study, accounting for 42.9% of total deaths; pneumonia was seen in 35.7%, peritonitis in 14.3% and infective endo­carditis in 7.1% of the patients. Previous studies have also reported infection as a major cause of mortality, being responsible for 24-30% of deaths. [4]

Deaths due to CNS causes accounted for 11.36% in this series. Patients with ADPKD are at increased risk of cranial and extra­cranial aneurysms and rupture of these aneu­rysms are responsible for increased mortality in these patients. [12] Rivera M, [13] reported the occurrence of acute cerebrovascular events in 9.8% of their patients with ADPKD including ischemia, ruptured aneurysms, cerebral and intracranial hemorrhage.

A high incidence of ESRD is associated with ADPKD patients (57.9%). [6] Uremia accounted for 2.2% of deaths in our study.

A total of 11.3% of the deaths were due to miscellaneous causes. Malignancies and respi­ratory causes for death accounted for 70% of the miscellaneous group.


   Conclusion Top


Our study suggests that cardiovascular di­sease, infection and cerebrovasular diseases are the common causes of mortality in patients with ADPKD. Constant vigilance and aggre­ssive management of blood pressure, prompt control of infection if it occurs, and frequent screening for malignancies can improve the outcome of these patients.[17]

 
   References Top

1.Schrier RW. Optimal care of autosomal dominant polycystic kidney disease patients. Nephrology (Carlton) 2006;11(2):124-30.  Back to cited text no. 1    
2.Florijin KW, Chang PC, Van der WoudeFJ, van Bockel JH, van Saase JL. Long-term cardiovascular morbidity and mortality in auto­somal dominant polycystic kidney disease patients after transplantation. Transplantation 1994;57(1):73-81.  Back to cited text no. 2    
3.Florijin KW, Noteboom WM, Van Saase JL, et al. A century of mortality in five large families with polycystic kidney disease. Am J Kidney Dis 1995;25(3):370-4.  Back to cited text no. 3    
4.Fick GM, Johnson AM, Hammond WS, et al. Causes of death in autosomal dominant poly­cystic kidney disease. J Am Soc Nephrol 1995; 5(12):2048-56.  Back to cited text no. 4    
5.Sotirakopoulos N, Tsitsios T, Stambolidou M, Cristodoulidou C, Spaia S, Mavromatidis K. Anticipation of end stage renal disease in patients with autosomal dominant polycystic kidney disease in successive generations. Ren Fail 2001;23(5):715-20.  Back to cited text no. 5    
6.Fourtounas C, Panteris V, Valis D. Survival after end stage renal disease in autosomal dominant polycystic kidney disease. Am J Kidney Dis 2002;39(3):660.  Back to cited text no. 6    
7.Schrier R, Kimberly M, Johnson A, et al. Cardiac and renal effects of standard versus rigorous blood pressure control in autosomal dominant polycystic kidney disease: Results of a seven year prospective randomized study. J Am Soc Nephrol 2002;13:1733-9.  Back to cited text no. 7    
8.Elias TJ, Bnnister KM, Clarkson AR, et al. Progressive hepatic failure secondary to adult polycystic kidney disease. Aust NZJ Med 1999;29(2):282-3.  Back to cited text no. 8    
9.Handa SP. Cardiovascular manifestations of autosomal dominant polycystic kidney disease in young adults. Clin Invest Med 2006;29(6): 339-46.  Back to cited text no. 9    
10.Bardaji A, Martinez-Vea A, Valero A. Cardiac involvement in autosomal dominant polycystic kidney disease: A hypertensive heart disease. Clin Nephrol 2001;56(3):211-20.  Back to cited text no. 10    
11.Chapman AB, Johnson AM, Rainguet S, et al. Left ventricular hypertrophy in autosomal dominant polycystic kidney disease. J Am Soc Nephrol 1997;8(8):1292-7.  Back to cited text no. 11    
12.Hadimeri H, Lamm C, Nyberg G. Coronary aneurysms in patients with autosomal domi­nant polycystic kidney disease. J Am Soc Nephrol 1998;9(5):837-41.  Back to cited text no. 12    
13.Riviera M, GonzaloA, Gobernado JM, et al. Stroke in Adult polycystic kidney disease. Postgrad Med J 1992;68(803):735-8.  Back to cited text no. 13    
14.Fredman BI, Souci JM, Chapman A, et al Racial variation in autosomal dominant poly­cystic kidney disease. Am J Kidney Dis 2000;35(1):35-9.  Back to cited text no. 14    
15.Kaehny WD, Everson GT. Extra renal mani­festations of autosomal dominant polycystic kidney disease. Semin Nephrol 1991;11(6): 661-70.  Back to cited text no. 15    
16.Yiyum J, Gabow P, Johnson A. autosomal dominant polycystic kidney disease in blacks. J Am Soc Nephrol 1994;4(9):1670-4.  Back to cited text no. 16    
17.Pecczkowska MJ, Anuszewiez A, Grzeszczak W, et al. The coexistence of acute aortic di­ssection with autosomal dominant polycystic kidney disease. Blood Press 2004;13(5):283-6.  Back to cited text no. 17    

Top
Correspondence Address:
Ebadur Rahman
Department of Medicine (38) Division of Nephrology, King Khalid University Hospital P.O. Box 2925, Riyadh 11461
Saudi Arabia
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PMID: 19736478

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    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]

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    Introduction
    Materials and Me...
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