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Saudi Journal of Kidney Diseases and Transplantation
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RENAL DATA FROM THE ARAB WORLD  
Year : 2019  |  Volume : 30  |  Issue : 1  |  Page : 175-184
Clinical study on autosomal dominant polycystic kidney disease among North Tunisians


1 Department of Medicine A; Laboratory of Renal Pathology - LR00S001, Charles Nicolle Hospital; Medical School of Tunis, University of Tunis El Manar, Tunis, Tunisia
2 Department of Medicine A, Charles Nicolle Hospital; Medical School of Tunis, University of Tunis El Manar, Tunis, Tunisia
3 Laboratory of Renal Pathology - LR00S001, Charles Nicolle Hospital; Medical School of Tunis, University of Tunis El Manar, Tunis, Tunisia
4 Laboratory of Renal Pathology - LR00S001, Charles Nicolle Hospital, Tunis, Tunisia
5 Department of Medicine A, Charles Nicolle Hospital, Tunis, Tunisia

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Date of Submission20-May-2018
Date of Acceptance10-Jul-2018
Date of Web Publication26-Feb-2019
 

   Abstract 


Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disease, which usually manifests in adulthood. It is characterized by the development of multiple cysts in the kidneys and many other extrarenal manifestations. We aimed to determine the factors that contribute to the progression of ADPKD to end-stage renal disease (ESRD). In a retrospective multicentric study, we reviewed the records of 569 patients with ADPKD, hospitalized at a nephrology department or followed up at the outpatient department of university and regional hospitals, covering the north and center of the country, during the period 1969–2016. The mean age of the study patients was 48.54 ± 13.68 years and 14% were young adults (<40 years). There were 272 female and 297 male patients (sex ratio: male/female = 1.09). A family history of ADPKD was found in 43.7% of cases. Renal symptoms were dominated by loin pain, renal failure, hypertension, and hematuria, seen in, respectively, 51.9%, 48.2%, 29.1%, and 24.6% of the patients. The median serum creatinine level was 459 μmol/L (range: 47–2454), and hypertension had preceded the onset of ADPKD in 28.8% of cases. Extrarenal manifestations consisted of urologic complications (54.6%), liver cysts (43.5%), cardiac involvement (31.9%), cerebral aneurysms (12.9%), and gastrointestinal involvement (9.4%). ESRD occurred in 43.1% after a mean follow-up of 47 months (range: 0–384). Risk factors for poor renal prognosis were age >40 years (P = 0.009), hematuria (P = 0.034), hemoglobin >14 g/dL (P = 0.0013), high uric acid level (P = 0.001), and leukocyturia (P = 0.02). Death occurred in 59 cases (10.3%), mostly caused by infections (44.1%). In our study, ADPKD was lately diagnosed in most cases. Family screening is important, which will enable early detection and management of the complications associated with ADPKD.

How to cite this article:
Hajji M, Barbouch S, Harzallah A, Hedri H, Kaaroud H, Abderrahim E, Goucha R, Hamida FB, Gorsane I, Abdallah TB. Clinical study on autosomal dominant polycystic kidney disease among North Tunisians. Saudi J Kidney Dis Transpl 2019;30:175-84

How to cite this URL:
Hajji M, Barbouch S, Harzallah A, Hedri H, Kaaroud H, Abderrahim E, Goucha R, Hamida FB, Gorsane I, Abdallah TB. Clinical study on autosomal dominant polycystic kidney disease among North Tunisians. Saudi J Kidney Dis Transpl [serial online] 2019 [cited 2019 May 23];30:175-84. Available from: http://www.sjkdt.org/text.asp?2019/30/1/175/252908



   Introduction Top


Autosomal dominant polycystic kidney disease (ADPKD) is a systemic disorder characterized by the development of multiple cysts in the kidney and associated with various extrarenal complications with cystic and non-cystic manifestations. It accounts for 5–10% of patients with end-stage renal disease (ESRD), making it the 4th leading global cause of kidney failure.[1],[2] The incidence rates for ESRD due to ADPKD vary between countries, ranging from 3.9 to 5.3 cases per million population per year in Europe, 4.8 in Japan, and 7.9 in the USA.[3] According to the European Renal Association and European Dialysis and Transplant Association registry data, 21,000 patients with ADPKD were receiving renal replacement therapy in 2010.[4] Diagnosing the disease includes obtaining the family history of ADPKD, age of patient, and number of kidney cysts. We aimed to evaluate the demographics, outcomes, and complications of ADPKD and determine prognostic factors contributing to progression to ESRD.


   Materials and Methods Top


We conducted a retrospective multicentric study including patients with ADPKD hospitalized at a nephrology department and/or followed up at the outpatient department of university and regional hospitals covering the north and the center of the country, between 1969 and 2016 [Figure 1]. Epidemio-clinical, para-clinical, therapeutic, and evolutive data for all cases were compiled electronically into Excel program and analyzed using Statistical Package for the Social Sciences (SPSS) version 11.5 (SPSS Inc., Chicago, IL, USA). The following clinical variables were evaluated: age, sex, geographic origin, family history of ADPKD, clinical presentation at diagnosis, extrarenal complications, ultrasonographic and computed tomography findings, treatment modalities, evolution of hypertension, renal function decline, urologic complications, ESRD, and causes of death. Follow-up time and disease-free survival time were calculated, as were Kaplan–Meier estimates of renal survival. The Cox proportional hazards model was adjusted for time intervals, gender, treatment modalities, and age at ESRD, with time since the onset of ESRD as the underlying time scale. These results were presented as hazard ratios (HR) with corresponding 95% confidence intervals. HR <1 denotes a better survival rate, and HR >1 denotes a poorer survival rate. To compare the rates, we used the Fisher's exact test and to compare durations and delays, we used Student's t-test. Comparison of renal survival according to different parameters was made by log rank test. We also calculated the mortality rate. Statistical significance was defined as P<0.05.
Figure 1: Contributing nephrology and dialysis centers in the north and the center of Tunisia. To the left is the political Algeria border, below is Libya.

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   Results Top


There were 569 patients with ADPKD, representing residents of north and center of Tunisia in the eight administrative countries [Figure 1]. Sixty-four percent of our patients were from North-east Tunisia, 22.7% were from the capital, while 35.3% were from the center of the country. The incidence of patients with ADPKD was estimated at 15.3 patients/year. Age ranged from 8–85 years (mean: 48.5 years). The distribution of patients by age at diagnosis showed that half were between 40 and 60 years of age. Fourteen percent of patients were young (<40 years). There were 297 male and 272 female patients, with a male-to-female ratio of 1.09. A family history for ADPKD was present in 249 of the 569 cases (43.7%). Consanguinity of first, second, and third degrees was noted, respectively, in 26%, 5%, and 5% of cases, while 248 of the 569 (64%) patients had no parental consanguinity. Kidney manifestations are shown in [Table 1]. They were dominated by palpable, bilateral flank masses in 66% of cases and hypertension in 58.8% of cases. The mean age at the onset of hypertension was 53 ± 12.8 years. A total of 248 patients had extrarenal manifestations of ADPKD [Table 1], the most common of which were liver cysts in 213 patients (43.5%) and nodular hepatomegaly in 16.6% of cases. Cardiac involvement was revealed clinically by heart murmur in 7.9% of cases, but transthoracic echocardiography showed abnormalities in 105 cases (18.4%) consisting of hypertrophic cardiomyopathy in 13.5% and valvulopathies in 28.2% of the patients. Aneurysm of atrial septum was detected in two cases. Neurologic involvement (12.9%) consisted of headaches (30.1%), seizures (6.4%), stroke (49.2%), meningeal hemorrhage (3.2%), and coma (3.2%); cerebral aneurysms were found in 1.9% of cases. Gastrointestinal involvement was observed in 9.5% of cases, commonly manifested by abdominal pain and diarrhea and/or constipation; colonic diverticulitis was found in 10 cases (1.75%), while hernias were noted in 11% of cases. One hundred and ninety-nine patients developed urologic complications (34.9%). The most frequent among them was urinary tract infection (24.4%) followed by nephrolithiasis (5%), intracystic hemorrhage (3.2%), and infected cysts (1.8%). The most common organisms isolated were  Escherichia More Details coli (49.2%) and Klebsiella pneumoniae (16.4%). Laboratory findings are also summarized in [Table 1]. Renal failure was noted in 275 patients (48.2%), serum creatinine level ranged from 47 to 2454 μmol/L, and serum urea nitrogen level ranged from 3.4 to 117 mmol/L. Normal renal function was noted in 142 patients (25%). Stage 1 to Stage 5 chronic kidney disease (CKD) was present, respectively, in 2%, 5%, 11%, 34%, and 23% of the patients. Patients initially with Stage 5 CKD (n = 131) were on dialysis (94.6%), and three patients underwent a preemptive kidney transplantation. Anemia was present in 530 patients (93%), whereas hyperglobulinemia was noted in 39 patients (8.8%) and hyperuricemia in 59% of cases. Nephrotic syndrome was found in 14 patients (2.4%); two among them were diabetic and one patient was diagnosed with amyloidosis. Angiotensin-converting enzyme inhibitors (ACEIs) were the most common antihypertensive medications used in our patients (68%) followed by calcium channel blockers (CCBs) in 42% of cases with a good control of blood pressure (BP). The evolution of the renal function after a median follow-up of 47.1 months (range: 0–384) is illustrated in [Table 2] and [Figure 2]. CKD as noted in patients with initial normal renal function in 36 cases (6.3%), after a median duration of one month (range: 0–384). ESRD occurred after a median duration of 25 months (range: 0–457) in 311 cases (54.6%). The mean age at onset of hemodialysis (HD) was 54 ± 11.2 years. Most of the HD patients were males (62%). Death occurred in 59 cases (10.9%) and was related to infections, neurologic, metabolic, cardiovascular, and neoplasic complications in respectively, 44.1, 10.2, 10.2, 6.7 and, 3.5% of patients and it was not known in 25.3% of cases. The outcome of the study patients is shown in [Table 3]. Many statistical correlations were found in our study. To begin with, the incidence of hypertension was higher in males than in females (P = 0.009), and the presence of a family history of hypertension was associated with a higher incidence of hypertension in our patients (P = 0.02). Polycystic liver disease was more prevalent in younger patients (age <30 years) (P = 0.002) and in females (P = 0.006), and it was frequently associated with bigger-sized kidneys (P = 0.01). The mean renal survival was 152 ± 11 months. On univariate analysis [Table 4], five risk factors of poor renal survival were determined as follows: age >40 years (P = 0.009), macros-copic hematuria (P = 0.034), hyperuricemia (P <0.0016), leukocyturia (P = 0.02), and hemoglobulin >14 g/dL (P = 0.0013). In order to identify independent risk factors of progression of renal function, we conducted a multivariate analysis that revealed two risk factors, namely age >40 years (HR: 3.4; P = 0.009) and leukocyturia (HR: 1.9; P = 0.02) [Figure 3], [Figure 4], [Figure 5].
Table 1: Demographic and clinical characteristics of the study patients.

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Table 2: The evolution of renal function in autosomal dominant kidney disease patients.

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Figure 2: The incidence of autosomal dominant kidney disease over the years.

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Table 3: Outcome of patients with autosomal dominant kidney disease.

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Table 4: Risk factors of progression to end-stage renal disease in patients with autosomal dominant kidney disease on univariate analysis.

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Figure 3: Renal survival among the study patients.

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Figure 4: Young age (<40 years) as an independent risk factor of end-stage renal disease (P = 0.009).

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Figure 5: Leukocyturia as an independent risk factor of end-stage renal disease (P = 0.02).

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   Discussion Top


ADPKD is the most common hereditary kidney disease, with approximately half of the patients experiencing ESRD by the age of 60 years.[5] It is caused by mutations in PKD1 in 85% of patients or in PKD2 in 15% of patients.[6],[7] However, genetic study was not performed in our series. The prevalence of ADPKD in different countries has been studied. It is estimated to be 1/2684 in England, 1/3058 in Germany, 1/1100 in France, and 1/4033 in Japan.[8],[9],[10],[11] There is a lack of statistical data on the prevalence of ADPKD in Tunisia and Africa. In Tunisia, ADPKD accounts for 6.9% of patients who receive HD.[12],[13] Unfortunately, a national register for ADPKD is not available in our country. In our study, the mean age at diag-nosis of the disease was 48.5 years, whereas in other series, it varied between 21 and 46 years.[14],[15] This could be attributed to the delay in the diagnosis of ADPKD in our country. However, it should be mentioned that, although an increasing number of patients with ADPKD reached ESRD in Tunisia, their age at onset of ESRD also increased significantly throughout the period. Family history of ADPKD was found only in 43.7% of our population, which was less than that in other series.[14],[15] It has been reported that ADPKD is slightly more severe in males than that in females, but the difference was not statistically significant and that symptoms generally increase with age.[14],[16] Hypertension occurs in more than 60% of patients with ADPKD before even a significant loss of renal function, with an average age at onset of 30 years.[3] In our series, it accounted for 58.8% of patients, with a mean age at onset of 53 years. There are multifactorial reasons, but the most important cause is the activation of renin–angiotensin aldos-terone system caused by enlargement of the renal cysts.[17] A prospective study found a significant reduction in left ventricular mass index in hypertensive ADPKD patients after BP control <120/80 mmHg.[18] Another study showed that proteinuria decreased significantly only on treatment with ACEIs, when compared with treatment with CCB.[19] Thus, it seems that, with ACEIs, more patients achieved good control of BP,[20] which may influence the age of onset of ESRD.[21] This finding was concordant with our results. Given that ADPKD is a systemic disease, hepatic cysts, cerebral aneurysms, and cardiac valvular abnormalities were well described in literature.[22],[23] In our series, ADPKD was associated with liver cysts in 43.5%, whereas valvular abnormalities and cerebral aneurysms were observed only in, respectively, 28.2% and 1.9% of the patients. The most frequent sign that revealed the disease in our study was renal failure (48.2%), which was seen in a larger number of patients compared to other studies.[7],[14],[15] This may be due to the delay in diagnosis and mostly to a selection bias, given that all patients were admitted at a nephrology department or seen by a nephrologist. The prevalence of hyperuricemia increases with the decline of renal function.[24] Our study confirmed that high levels of uric acid were associated with an increased risk of progression to ESRD (P <0.0001). Hyperglobulinemia was noted only in 8.8% of cases in our study. This may be explained by the higher prevalence of the advanced stages of CKD. The diagnosis of ADPKD relies essentially on imaging. Ultra-sonography is the gold standard imaging modality, given its availability, safety, and low cost. Typical imaging findings reveal large kidneys with multiple bilateral cysts.[25] In our series, abdominal ultrasound and computed tomography allowed screening for the disease in, respectively, 64% and 21% of the patients. In patients with ADPKD, pharmacologic therapy includes controlling BP, abnormalities related to renal failure, urologic complications, and stopping enlargement of kidney cysts. However, one of the limitations of treating early stages of ADPKD in our country was the nonavailability of tolvaptan. Tolvaptan demonstrated its effectiveness in slowing disease progression to ESRD.[26] However, it is indicated only in patients with age <50 years, Stage 1 to 3A of CKD, and with a rapid progression of renal failure.[27] Cardiovascular pathology and infectious complications account for approximately 90% of deaths of ADPKD patients.[28],[29] Another cause of mortality in ADPKD is subarachnoid hemorrhage from intracranial aneurysms,[30] which were rare and severe. In the literature, the major factors predicting CKD progression in ADPKD were genotype, younger age, male sex, Black race, initial renal function, and total kidney volume.[25],[31],[32],[33] Several cohort studies demonstrated that the genotype can predict the age at onset of ESRD,[6],[34] which was not studied in this analysis. Strikingly, older age was identified as a predictive factor of progression of renal failure. This finding can be explained by the fact that, the majority of our patients were diagnosed late, at an advanced age. No studies have shown that male gender was in fact a risk factor of progression.[6],[35] On the other hand, the lower incidence rate of female patients with the onset of ESRD could reflect better pre- dialysis survival. However, one of the limitations of this study was the lack of survival data on all patients with ADPKD before the onset of ESRD. Therefore, the association between predialysis mortality rate in female patients and lower ESRD remains unclear. Risk factors of severe renal disease included hypertension, proteinuria >1 g/day, hematuria,[32] and left ventricular hypertrophy. In our study, hypertension did not seem to be correlated with a poorer renal survival. However, treatment with either an ACEI or angiotensin receptor blocker seemed to be more effective in the control of BP in our population, although this finding has not been clearly demonstrated.[36],[37] Since echocardiography was not performed in all of our patients, left ventricular hypertrophy was not studied in this context. Proteinuria was not significantly associated with poor renal survival in our series, whereas hematuria, leukocyturia, and hyper-uricemia have been, indeed, predictive factors of progression of CKD.


   Conclusion Top


In our study, ADPKD was diagnosed in most cases after the onset of renal failure. Therefore, we emphasize the importance of family screening, for early detection and treatment of complications associated with ADPKD and for the assessment of antihypertensive medications and renal replacement therapy needed in our country. Tolvaptan has been approved to slow the progression of ADPKD; we hope that our patients will benefit from its prescription in the years to come. A national multicentric study is necessary for determination of the current prevalence of ADPKD in Tunisia, the geographic distribution, and the possible areas of predilection for ADPKD.


   Acknowledgments: Top


Collaborating authors names.


   Appendix I: Tunisian Study on Autosomal Dominant Polycystic Kidney Disease Group Top


Bechir Zouari1, Mohammed Karim Zouaghi2, Rania Kheder2, Jannet Laabidi3, Yosra Ben Ariba3, Cyrine Karoui4, Hanène El Kateb5, Mohammed Chermiti6, Saida Hajri6, Salah Yahyaoui7, El Ati Zohra8, Sassi Zohra9, Khaled Mkaouer10, Walid Hedhili11, Monia Abbess12, Sami Ben Khalifa13.

1Medical School of Tunis, 2La Rabta University Hospital, Tunis, 3Military Hospital, Tunis, 4Mahmoud El Martri Hospital-L'Ariana, 5Nephrology and Hemodialysis Center, La Mannouba, 6Regional Hospital-El Kef, 7Regional Hospital, Siliana, 8Regional Hospital, Mahdia, 9Regional Hospital, Sidi Bouzid, 10Regional Hospital, Kairouan; 11Regional Hospital, Jendouba, 12Regional Hospital, Bizerte; 13Regional Hospital, Tabarka.

Conflict of interest:

None declared.



 
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[PUBMED]  [Full text]  
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Correspondence Address:
Meriam Hajji
Department of Medicine A, Charles Nicolle Hospital, Tunis
Tunisia
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DOI: 10.4103/1319-2442.252908

PMID: 30804279

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