| Abstract|| |
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary form of kidney disease. Clinical data on this multisystem disorder are scarce from developing countries. We conducted a prospective observational study of the clinical profile of ADPKD patients at a single center over a period of six years. A total of 208 patients were studied. Majority were male (60.6%) and the mean age was 45.8 ± 14.5 years. About 61.5% had early stage (Stages 1-3) of chronic kidney disease (CKD) and 38.5% had advanced CKD (Stages 4 and 5). Clinical features observed included pain abdomen (46.2%), nocturia (65.9%), hematuria (21.6%), nephrolithiasis (38.9%), urinary tract infection (UTI) (38.9%), hypertension (69.5%), and raised serum creatinine (54.3%). The prevalence of nocturia, hypertension, and renal dysfunction showed a significant increase with age (P = 0.001). Extrarenal manifestations were polycystic liver disease in 77 patients (37%), cysts in pancreas in two (1%), and stroke in three (1.5%) (hemorrhage in 2 and infarct in 1). There was significantly higher prevalence of hypertension (P = 0.027) and nephrolithiasis (P = 0.044) in males compared to females. Ninety-two patients (44.2%) had a positive family history for ADPKD. Fifteen (7.2%) had kidney failure at the diagnosis of ADPKD, were hospitalized, and underwent emergency dialysis. A total of 20 patients (9.6%) developed end-stage kidney disease during the study period. The age at diagnosis was higher, and there was a high prevalence of hypertension, nocturia, abdominal pain, nephrolithiasis, UTI, and renal dysfunction in Indian ADPKD patients.
|How to cite this article:|
Vikrant S, Parashar A. Autosomal dominant polycystic kidney disease: Study of clinical characteristics in an Indian population. Saudi J Kidney Dis Transpl 2017;28:115-24
|How to cite this URL:|
Vikrant S, Parashar A. Autosomal dominant polycystic kidney disease: Study of clinical characteristics in an Indian population. Saudi J Kidney Dis Transpl [serial online] 2017 [cited 2020 Aug 15];28:115-24. Available from: http://www.sjkdt.org/text.asp?2017/28/1/115/198163
| Introduction|| |
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary form of kidney disease that is estimated to affect 12.5 million people worldwide in all ethnic groups. It is responsible for about 10% of patients with end-stage renal disease (ESRD) and constitutes a major health burden. ADPKD is a systemic disorder and affects other organs too with potentially serious complications. The renal and extrarenal manifestations of the disease are quite varied, even in individuals of the same family. The most common abnormalities related to the kidneys are arterial hypertension, abdominal mass, urinary tract infection (UTI), lumbar or abdominal pain, hematuria, nephrolithiasis, and renal failure. The liver is the second most frequently affected organ, with hepatic cysts that cause hepatomegaly and abdominal pain although they may exceptionally cause hepatic dysfunction. Cysts may also occur occasionally in the pancreas, ovaries, and choroid plexus. Other extrarenal manifestations include intracranial aneurysms (ICA) and aortic aneurysms, arachnoid cysts, cerebral artery dolichoectasia, colonic diverticula, and cardiac abnormalities such as mitral valve prolapse, mitral regurgitation, aortic insufficiency, and tricuspid regurgitation.
The epidemiology of ADPKD has rarely been studied in non-Caucasian populations, and the impact on health of this chronic disease has not yet been assessed in developing countries. , , A single study from India recently reported ADPKD to be the etiology of chronic kidney disease (CKD) in 2.6% cases. As far as we know, there are no reports on the evaluation of clinical data in Indian ADPKD patients. Therefore, a clinical study of renal and extrarenal manifestations in Indian patients with ADPKD was undertaken.
| Patients and Methods|| |
This was a prospective observational study of patients with ADPKD attending the Renal Clinic of our hospital over a period of six years (April 2009 to March 2015). The diagnosis of ADPKD was established by renal ultrasonography using unified criteria by Pei et al. As per these criteria, the presence of at least three unilateral or bilateral renal cysts in patients aged 15-39 years, two cysts in each kidney in patients aged 40-59 years, and four cysts or more in each kidney in individuals aged ≥60 years are suggestive of a diagnosis of ADPKD. A positive family history and the presence of hepatic cysts also serve as additional diagnostic criteria. Patients who did not fulfill the unified criteria were excluded from the study. Age, gender, and clinical manifestations related to ADPKD were recorded. The age at diagnosis of ADPKD and at progression to ESRD (characterized as the beginning of dialysis treatment) and duration of hypertension and ADPKD were also recorded.
Patients were considered to be hypertensive at the first consultation if they had previously been medicated with antihypertensive drugs or their blood pressure measured in a seated position after 5 min' rest was ≥140/90 and this value was confirmed by measurements taken during the period before the next consultation. Renal function was assessed by serum creatinine level, with endogenous creatinine clearance being calculated using the CockroftGault formula. The patients were stratified according to the five stages of CKD listed in the National Kidney Foundation guidelines. Diagnosis of nephrolithiasis was made by a history of stone passage, removal of stone, or calcific foci/nephrocalcinosis seen on imaging. Ultrasonography or other imaging reports were also reviewed to identify involvement of other abdominal viscera.
After obtaining informed consent, a standardized family history questionnaire was used to screen for a history of ADPKD in first-degree relatives. For each patient, a family tree consisting of parents, siblings, and offspring was prepared. History of polycystic kidney disease (PKD) was explored in these family members. The diagnosis of positive history of PKD in a family member was made on the basis of a known diagnosis of ADPKD or kidney failure in that family member. Institute Ethical Committee approved this study.
| Statistical Analysis|| |
Descriptive statistics including means, standard deviation, and percentages were used to describe the demographic and clinical data. Comparison between groups was performed by Chi-square or Fisher's exact test for categorical data and Student's t-test or Mann- Whitney U-test as appropriate for continuous data. Multivariate binary logistic regression analysis was performed for factors predictive of ESRD. P <0.05 was considered statistically significant. All statistics were carried out using Statistical Program for Social Sciences (SPSS) software version 16.0 (SPSS, Chicago, IL, USA).
| Results|| |
Demographic and clinical characteristics
A total of 208 patients were studied; 126 patients (60.6%) were males and 82 (39.4%) were females. The mean age of the patients was 45.8 ± 14.5 years; 68.3% belonged to the age group of 30-59 years, 18.8% to the age group ≥60 years, and 12% to the 15-30 years group and 1% were <15 years ([Figure 1]). Sixty-nine (33.2%) were known cases of ADPKD for a mean duration of 5.5 ± 5.6 years and 86 (41.3%) were known to be hypertensive for a mean duration of 5.9 ± 5.8 years. Mean age at the diagnosis of PKD was 43.9 ±14.5 years with a range of 4-80 years. Mean serum creatinine was 3.3 ± 3.6 mg/dL and mean hemoglobin was 10.5 ± 2.4 g/dL. Almost 61.5% had early CKD (Stages 1-3) and 38.5% had advanced CKD (Stages 4 and 5).
Clinical features seen in the study patients included pain abdomen (46.2%), nocturia (65.9%), hematuria (21.6%), nephrolithiasis (38.9%), UTI (38.9%), hypertension (69.5%), and raised serum creatinine (54.3%). Four patients (1.9%) had other associated renal diseases including amyloidosis, chronic sclerosing glomerulopathy, diabetic nephropathy and renal cell carcinoma (RCC), in one patient each. Two patients (1%) had features of the nephrotic syndrome ([Table 1]). The prevalence of nocturia, hypertension, and renal dysfunction had a significant increase with age (P = 0.001). Nephrolithiasis (P = 0.025) and polycystic liver (P = 0.036) differed in the various age-groups ([Table 2]).
|Table 1. Demographic and clinical characteristics of the study patients (n=208).|
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|Table 2. Comparison of clinical characteristics in different age groups (n=208).|
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There was a significant increase in the prevalence of hypertension with age and stage of CKD. Hypertension was seen in the following groups: age group <30 years (29.6%), 30-59 years (74.6%), and ≥60 years (79.5%) (P = 0.001). The prevalence of hypertension in different CKD stages was CKD Stage 1 (45.9%), Stage 2 (75%), Stage 3 (76.7%), Stage 4 (80%), and Stage 5 (84%) (P = 0.001).
Extrarenal manifestations were polycystic liver disease (PLD) in 77 (37%), cysts in pancreas in two (1%), and three patients (1.5%) developed stroke (hemorrhagic stroke in 2 and 1 had infarct). PLD was seen in 59 patients (42.8%) above 40 years as compared to that in 18 patients (25.7%) aged <40 years (P = 0.022).
Thirty-five (42.7%) female patients had PLD as compared to 42 (33.3%) male patients (P = 0.188). The mean age of the patients developing stroke was 47.3 ± 5 years. There was a history of stroke at age below 55 years in first-degree relative in four patients (2%).
Twenty patients (9.6%) had undergone surgery for stone removal before the diagnosis of PKD. Fifteen patients (7.2%) had kidney failure at the diagnosis of PKD and were hospitalized and underwent emergency dialysis. A total of 20 patients (9.6%) developed ESRD during the study period. Fifteen (11.9%) were male and five (6.1%) were female (P = 0.229). The mean age of patients developing ESRD was
50.6 ± 9.3 years, and the mean age at diagnosis of ADPKD in these patients was 48.8 ± 10.1 years. The mean serum creatinine at diagnosis was 8.1 ± 4.1 mg/dL, which was a predictor of ESRD on multivariate logistic regression analysis including age, creatinine at diagnosis, hypertension, hematuria, nephrolithiasis, and UTI (odds ratio: 1.2, 95% confidence interval 1.1-1.4, P = 0.001). Three (1.4%) were initiated on chronic peritoneal dialysis and two (1%) patients on maintenance hemodialysis.
Comparison of demographic and clinical characteristics between male and female patients
Nearly 47.6% of the male patients were known to have hypertensive as compared to 28% of the female patients (P = 0.023). Details are shown in [Table 3]. There was no significant difference between the two genders in the other demographic and clinical characteristics.
|Table 3. Comparison of demographic and clinical characteristics between male and female patients (n=208).|
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Family history of polycystic kidney disease
Details of the family history of PKD in the study patients are shown in [Table 4]. Ninetytwo patients (44.2%) had a positive family history for PKD. Nineteen patients (9.1%) had history of PKD in their offspring. Twelve patients (13%) had a history of PKD in their sons and seven (7.6%) in their daughters. Ten patients had a history of PKD in one son and six had PKD in one daughter.
| Discussion|| |
As far as we know, this is the first study that evaluated the renal and extrarenal clinical manifestations in a cohort of Indian ADPKD population. The study showed a higher prevalence (60.6%) of this condition among males, whereas a higher prevalence of 51.4% and 63%, respectively, in female European and Brazilian populations has been reported. Mean age at diagnosis was higher in our patients as compared to that in studies by Romγo et al and Thong and Ong. Even the serum creatinine at diagnosis was higher in our study as compared to that in a study by Romγo et al. A high prevalence of hypertension, nocturia, abdominal pain, nephrolithiasis, UTI, and renal dysfunction was found in Indian ADPKD patients.
Pain is a common manifestation of ADPKD. , , Potential etiologies include cyst hemorrhage, nephrolithiasis, cyst infection, and rarely, tumor. Discomfort, ranging from a sensation of fullness to severe pain, can also result from renal enlargement and distortion by cysts. Passage of clots can also be a source of pain. It may develop after an episode of acute pain and is likely maintained by aberrant activity of sensory and autonomic neurons innervating the kidney. Diffuse abdominal or unilateral or bilateral lumbar pain affects 25.7%-62% ADPKD patients , , , and may be the initial presenting symptom leading to diagnosis of PKD. Symptoms of lumbar or abdominal pain were reported by 46.2% of our study patients.
An impaired concentrating ability leads to nocturia. It was present in two-third (65.9%) of our study patients. A defect in urine concentrating ability with onset in childhood is common in patients with ADPKD, many of whom also report polydipsia and polyuria at an early stage of the disease. These symptoms have been linked to an altered osmolality- vasopressin regulatory axis. Recent studies suggest that the urinary concentrating defect and elevated serum concentration of vasopressin may contribute to cystogenesis. They may also contribute to the glomerular hyperfiltration seen in children and young adults, development of hypertension, and progression of CKD.
Hemorrhage into the cyst and gross hematuria are frequent complications of ADPKD. Gross hematuria can result from cyst hemorrhage, nephrolithiasis, infection, and rarely, from renal cell or urothelial carcinoma. Cyst hemorrhage occurs in about 60% of individuals; gross or microscopic hematuria, if the cyst connects to the collecting system. Excessive angiogenesis results in fragile blood vessels stretched across walls of enlarging cysts, which are susceptible to minor trauma with resultant hemorrhage. Patients with recurrent episodes of gross hematuria have the largest kidneys and progress more quickly to kidney failure. Gross or microscopic hematuria was reported in 21.6% patients in our study. The prevalence of nephrolithiasis is considerably greater in patients with ADPKD than in the general population. ADPKD patients develop nephrolithiasis because of increased intrarenal anatomic obstruction, as well as lower levels of urinary inhibitors of stones such as magnesium and citrate. Nephrolithiasis has been reported in 20%-30% of patients, of whom 50% are symptomatic for stone disease and 20% require definite urologic intervention. , , Nephrolithiasis is more common in male ADPKD patients. Nephrolithiasis was observed in 38.9% of our patients; males had a significantly higher prevalence of nephrolithiasis compared to females (0.044).
Symptomatic lower UTI affects 50%-75% of all polycystic patients at some time. Nearly 30% to 50% of patients with ADPKD will have a UTI, either pyelonephritis or cyst infection, during their lifetime. UTI is more common in women with ADPKD. In the current study, 38.9% of the patients had suffered from at least one episode of UTI. Expansion of renal cysts causes distortion and compression of normal renal tissues, intrarenal ischemia, hyperactivation of the renin- angiotensin-aldosterone system, and the development of sustained hypertension. Hypertension results in cardiovascular disease, the leading cause of death in ADPKD, renal function decline, and may also further accelerate cyst growth. The average age at onset of hypertension is between 30 and 34 years and usually precedes renal impairment with men being more commonly affected than women, and there is increasing prevalence of hypertension with decreasing renal function. Hypertension has been known to occur in 70%-80% of adults with ADPKD before loss of kidney function, possibly affecting 80% of the ADPKD patients with renal failure. Hypertension is probably the most remediable and serious complication of ADPKD. Hypertension was the most common manifestation of ADPKD patients with 69.7% being hypertensive. There was a significant increase of hypertension with age and CKD stage (P = 0.001). The prevalence of hypertension was significantly higher in male as compared to female patients.
The new onset of hypertension in a patient at risk for PKD should prompt aggressive treatment and diagnostic studies. Patients with hypertension during the course of ADPKD have early and more severe left ventricular hypertrophy. Early diagnosis and follow-up at a presymptomatic stage of the disease are important since this enables early initiation of antihypertensive therapy, which could reduce the rate of cardiovascular events in this population.
Another relevant issue related to hypertension is stroke. In the present report, stroke was detected in three patients, with two of them being cases of confirmed hemorrhagic stroke. ICAs are perhaps the most severe extrarenal manifestation of ADPKD although aneurysms are only present in approximately 5% of all patients with ADPKD. Those with a family history of early death due to stroke or prior ruptured intracranial aneurysms are much more likely to have these aneurysms. Generally, renal function is maintained until the fourth to sixth decade of life. However, once the compensatory mechanism of the kidneys fails, a rapid decline in renal function occurs. The progressive disease ultimately leads to ESRD, and chronic renal failure presents in about 50% of patients by the age of 60 years. Majority of our study patients had elevated serum creatinine level and 38.5% had late CKD Stage (4 and 5) at diagnosis of ADPKD. The mean creatinine at diagnosis was found to be a significant predictor of ESRD. Almost 9.6% of the study patients developed ESRD at the mean age of 50.6 ± 9.3 years during the study period and majority were male. APDKD has a more progressive disease course in male patients who are more likely to reach ESRD than females. Risk factors for disease progression include gross hematuria before 30 years of age, degree of proteinuria, and early onset hypertension. Hypertension and proteinuria are the major treatable risk factors for the progression of CKD in ADPKD patients. Proteinuria (>300 mg/day) occurs in about 25% of adults diagnosed with ADPKD but typically does not exceed 1 g/day. Proteinuria is associated with faster decline of renal function, and earlier onset of ESRD. In patients with nephroticrange proteinuria, the presence of an additive disorder should be considered. In our patient, only two had features of nephrotic syndrome and one had RCC. RCC does not occur more frequently in individuals with ADPKD than in the general population. However, when RCC develops in individuals with ADPKD, it has a different biologic behavior including earlier age at presentation, frequent constitutional symptoms, and a higher proportion of sarcomatoid, bilateral, multicentric, and metastatic tumors. An increased risk for RCC in individuals with ADPKD who are on dialysis for ESRD can be explained by the increased overall risk of RCC in patients with advanced kidney disease and may not be specific for ADPKD.
Transplantation is the optimal choice of renal replacement therapy in appropriate patients with ADPKD. When transplantation is not an option, or for those waiting for transplantation, either hemodialysis or peritoneal dialysis is a suitable modality. Although intra-abdominal space restrictions, increased risk for abdominal wall hernias, and increased prevalence of colonic diverticula may pose challenges, ADPKD is not a contraindication for peritoneal dialysis.
Polycystic liver disease is the most common extrarenal manifestation of ADPKD. Thirtyseven percent of patients in our study had PLD, and pancreatic cysts were detected in only 1% cases. The prevalence of PLD was significantly higher in patients aged above 40 years and nonsignificantly higher in female patients in our study. The prevalence of hepatic cysts in ADPKD patients has been estimated to be as high as 80%. , Hepatic cysts are more commonly seen in women and in patients over the age of 40 years. Quite often, these cysts are incidental findings and not clinically significant. Cyst size and number increase with age. Although hepatic failure is uncommon, symptoms related to a massively enlarged cystic liver include loss of appetite, weight loss, esophageal reflux, and abdominal discomfort, which interfere with the activities of daily living. ,
Cystic involvement of the pancreas has been estimated to be about 10% in patients with ADPKD. Pancreatic cysts were associated with increasing age, female sex, and PKD1 mutation. Pancreatic involvement is usually benign, with no reports of endocrine or exocrine dysfunction. However, cystic compression of the main pancreatic duct has resulted in chronic pancreatitis.
Family history was positive for ADPKD in 44.2% of our study cohort. Due to the autosomal dominant type of inheritance, the morbidity risk for siblings and offspring of an ADPKD patient is 50%. Family history should serve to determine the existence or nonexistence of that risk. It should be a standard procedure to perform further tests on each person with a 50% risk of developing ADPKD. As a rule, ADPKD is diagnosed in one of the parents. In case of people with no family history, the risk of ADPKD development is the same as in the general population, i.e., 1/1000.
Family history analysis is a simple and inexpensive approach to identifying individuals at risk for ADPKD. The increased use of family history analysis as a tool for diagnosing ADPKD has been found to be useful for diagnosis at an earlier age and has important implications for altering the renal and cardiovascular complications of the disease.
In approximately 25%-40% of cases, ADPKD occurs in people without a family history of the disease. New mutations and more frequently, particularly in families without PKD1, it is a disease that progresses slowly and never causes symptoms. In our study, a high proportion (55.8%) had a negative family history because the only instrument used was known history of the disease, and ultrasonography was not used to screen the first-degree relatives for the presence of ADPKD.
This is one of the first studies of ADPKD among Indian population of a fairly large size showing a spectrum of renal and extrarenal manifestations. Limitations of the study are observational nature of the study, imaging was limited to abdominal or for other symptomatic manifestations and systemic evaluation for other manifestations such as cardiac or intracranial aneurysms was not performed. Further, mutational analysis was not performed, and ultrasonographic screening of families of the ADPKD patients was not carried out.
Conflict of interest: None declared.
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Department of Nephrology, Indira Gandhi Medical College, Shimla - 171 001, Himachal Pradesh
[Table 1], [Table 2], [Table 3], [Table 4]