NATURAL HISTORY OF AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE

Introduction. In recent years, the number of patients with autosomal dominant polycystic kidney disease (ADPKD) who undergo kidney transplantation without nephrectomy has increased. The most frequent and adverse complication from your own kidneys is infection of cysts (IC). This dictates the need to predict the probability of IC and determine diagnostic and therapeutic approaches in this category of patients. Materials and methods. The results of observation and treatment of 55 patients with ADPKD who underwent kidney transplantation from 2000 to 2019 without prior nephrectomy were evaluated. Results. Bilateral nephrectomy in connection with IC was performed in 10 (18.1%) patients, and one patient died from sepsis progression. Burdened urological history (kidney operations for suppuration of cysts and recurrent urinary tract infection (UTI)) significantly increased the chances of nephrectomy for IC by 6.8 times (AOR 6.83; 95% CI 1.34-34.8; p=0.021). The median time from kidney transplantation to nephrectomy was 7 months (Q1-Q3: 2-8). Acute graf pyelonephritis was associated with IR (p=0.045) in single-factor analysis. Forty-five patients are under observation, with a median follow-up of 41 months (Q1-Q3: 19-76). Seventeen patients underwent magnetic resonance imaging using diffusely weighted image protocols (MRI-DWI). MR-signs of infection were detected in 5 patients. Given the absence of clinical and laboratory manifestations of inflammation, nephrectomy was not performed. Further followup did not indicate the development of clinical and laboratory signs of UTI in any case. Discussion. As our study and a number of other studies have shown, in patients with ADPKD after kidney transplantation, the most frequent indication for nephrectomy is inflammatory changes in their own kidneys. Patients with a history of severe pyelonephritis or IC who previously had pyelonephritis or IC before kidney transplantation are at risk for developing inflammatory changes after kidney transplantation, and it does not matter how long ago they had a history of pyelonephritis attacks or kidney surgery. This fact should be taken into account before kidney transplantation and offer patients a nephrectomy before kidney transplantation. Our work is consistent with a number of non-numerous publications that have shown that in the diagnosis of IC, the most informative non-invasive, imaging method is MRI of the kidneys, but this method has low specificity, which can lead to an increase in the number of false positive conclusions and an increase in the number of unjustified nephrectomies. Conclusion. The predictor of infection of own kidney cysts after transplantation is a burdened urological history. MRI DWI has high sensitivity and low specificity in the diagnosis of IR in ADPKD. When identifying single cysts with MR-signs of infection in the absence of clinical, laboratory manifestations of UTI, nephrectomy is not indicated.

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More adverse renal prognosis of autosomal dominant polycystic kidney disease in families with primary hypertension.

Journal of the American Society of Nephrology ◽

10.1681/asn.v661643 ◽

1995 ◽

Vol 6 (6) ◽

pp. 1643-1648

Author(s):

S Geberth ◽

E Stier ◽

M Zeier ◽

G Mayer ◽

M Rambausek ◽

...

Keyword(s):

Blood Pressure ◽

Kidney Disease ◽

Polycystic Kidney Disease ◽

Autosomal Dominant ◽

Primary Hypertension ◽

Polycystic Kidney ◽

Marked Variability ◽

History Of ◽

Unaffected Parent ◽

Autosomal Dominant Polycystic Kidney

Marked variability of age at renal death is noted in autosomal dominant polycystic kidney disease (ADPKD). The hypothesis that the coexistence of primary hypertension and ADPKD within families is associated with earlier renal death was tested. Of a total of 162 ADPKD patients treated in one Austrian and three German centers, 57 propositi were identified whose families provided (1) information concerning blood pressure; (2) documented presence of ADPKD (by sonography or autopsy) in one parent; and (3) age at renal death in the propositus. Hypertension of the unaffected parent was defined as blood pressure above 140/90 mm Hg or antihypertensive treatment before age 60 yr. Age at renal death in the propositus was defined as the start of renal replacement therapy. Median age at renal death of 23 offspring (11 male, 12 female) from families with a history of primary hypertension of the nonaffected parent was lower than that of 34 offspring (16 male, 18 female) from families without a known history of primary hypertension of the nonaffected parent, i.e., 49 yr (26 to 64) versus 54 yr (28 to 82) (P < 0.03). The data are consistent with the notion that genetic predisposition to primary hypertension is associated with an earlier onset of terminal renal failure in families with ADPKD.

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MO051EFFECT OF TOLVAPTAN ON THE SUPPRESSION OF TOTAL KIDNEY VOLUME INCREASE IN PATIENTS WITH MODERATE-TO-SEVERE RENAL DYSFUNCTION IN AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfab080.0023 ◽

2021 ◽

Vol 36 (Supplement_1) ◽

Author(s):

Hiroki Nomi ◽

Daisuke Mori ◽

Shinjiro Tamai ◽

Maho Tokuchi ◽

Natsumi Inoue ◽

...

Keyword(s):

Kidney Disease ◽

Renal Dysfunction ◽

Polycystic Kidney Disease ◽

Autosomal Dominant ◽

Kidney Volume ◽

Polycystic Kidney ◽

Total Kidney Volume ◽

History Of ◽

Pre Treatment ◽

Autosomal Dominant Polycystic Kidney

Abstract Background and Aims Tolvaptan (TV) slows down the increase in total kidney volume (TKV) in patients with autosomal dominant polycystic kidney disease (ADPKD). The efficacy of TV in patients with moderate-to-severe renal dysfunction (RD) in ADPKD remains unknown. Method This was a single-centre retrospective study involving 27 patients with ADPKD who took TV and visited our hospital in the past six years. The participants were divided into two groups: the normal-to-mild RD (estimated glomerular filtration rate (eGFR) ≥ 45mL/min/1.73m2) group and the moderate-to-severe RD (eGFR < 45mL/min/1.73m2) group. Treatment effects were evaluated using ΔTKV, which was calculated as post-/pre-treatment annual TKV change. Continuous variables are presented using the median [interquartile range]. Results The moderate-to-severe RD group comprised 11 patients. Baseline characteristics of the normal-to-mild vs. moderate-to-severe RD group were as follows: eGFR, 56 [50–69] vs. 29 [24–38] mL/min/1.73m2; age, 48 [39–55] vs. 49 [43–58] years; male gender, 57% vs. 36%; body mass index (BMI) , 26 [23–28] vs. 24 [22–27] kg/m2; TKV 1700 [1084–2574] vs. 1827 [1331–2424] mL; family history of ADPKD, 100% vs. 82%; history of cerebral aneurysm, 19% vs. 36%; hypertension, 81% vs. 82%; hyperuricemia, 13% vs. 27%; dyslipidaemia, 19% vs. 18%; diabetes, 6.1% vs. 9.1% and systolic blood pressure (sBP) on admission 138 [129–144] vs. 131 [128-137] mmHg. No significant differences were noted in all these parameters, except for renal function. The starting dose of TV was 60 mg/day in all cases (0.9 [0.7–1.0] vs. 0.9 [0.8–1.1] mg/kg; P = 0.35). Urine volume (7.5 [5.7–9.6] vs. 4.0 [3.3–4.7] L/day; P = 0.006) and urinary sodium excretion (163 [126–226] vs. 89 [81–120] mEq/day; P = 0.003) were higher in the normal-to-mild RD group. Between the groups, there were no differences in urine protein (0.12 [0.0–0.3] vs. 0.16 [0.08–0.29] g/day; P = 0.31) and ΔeGFR (98% [88–123] vs. 106% [102–112]; P = 0.45), which was calculated as post-/pre-treatment annual eGFR change. Although both groups experienced the therapeutic effects of TV, the efficacy was poorer in the moderate-to-severe RD group (ΔTKV, 82% [76–85] vs. 96% [86–97]; P = 0.001). Conclusion The efficacy of TV patients with moderate-to-severe RD in ADPKD might be modest.

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Neonatal presentation of autosomal dominant polycystic kidney disease with a maternal history of tuberous sclerosis

Nephrology Dialysis Transplantation ◽

10.1093/ndt/12.11.2284 ◽

1997 ◽

Vol 12 (11) ◽

pp. 2284-2288 ◽

Cited By ~ 2

Author(s):

M Griffin

Keyword(s):

Kidney Disease ◽

Tuberous Sclerosis ◽

Polycystic Kidney Disease ◽

Autosomal Dominant ◽

Polycystic Kidney ◽

Maternal History ◽

History Of ◽

Autosomal Dominant Polycystic Kidney ◽

Neonatal Presentation

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P0037POPULATION CHARACTERISTICS ACROSS AN EXPANDED POOLED DATABASE OF MULTIPLE AUTOSOMAL DOMINANT POLYCYSTIC KIDNEY DISEASE CLINICAL STUDIES

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p0037 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Xiaolei Zhou ◽

Eric Davenport ◽

John Ouyang ◽

Molly Hoke ◽

Diana Garbinsky ◽

...

Keyword(s):

Chronic Kidney Disease ◽

Kidney Disease ◽

Disease Progression ◽

Polycystic Kidney Disease ◽

Autosomal Dominant ◽

Extension Study ◽

Polycystic Kidney ◽

History Of ◽

Baseline Characteristics ◽

Autosomal Dominant Polycystic Kidney

Abstract Background and Aims Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease and the fourth-leading cause of kidney failure. Over the past two decades, various studies have been conducted to characterize the natural history of ADPKD and investigate impacts of potential treatments on disease progression. Previously, we created a pooled longitudinal database of unique subjects from nine studies to evaluate and analyze outcomes. The database was expanded to include data from two recent tolvaptan (TOL) trials (156-13-210 and 156-13-211). Here, we describe the baseline characteristics of the expanded pooled population. Method Data from 11 ADPKD studies (from 2001 to 2018, sponsored by Otsuka or National Institutes of Health) were combined and divided into two groups: TOL and standard of care (SOC). TOL consisted of trial subjects initiating treatment in one of seven trials (156-04-250, 156-04-251, 156-06-260, 156-09-284, 156-09-290, 156-08-271, and 156-13-210); SOC included subjects from placebo arms of two TOL randomized trials (156-04-251 and 156-09-290), all standard blood pressure control arms in the HALT-PKD trials, and subjects from two observational studies (156-10-291 and CRISP). Subjects in the placebo arm of study 156-13-210 received TOL for 5 weeks before randomization and were therefore included in the TOL group. Eligible subjects who completed an early TOL study continued TOL treatment in the extension study 156-08-271 and/or in a second extension study 156-13-211. Estimated glomerular filtration rate (eGFR) was calculated in all studies using the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI) equation. Total kidney volume (TKV) was measured by magnetic resonance imaging and available in all studies except 156-13-210, 156-13-211, and HALT-PKD study B. Results The pooled analysis included 7,117 eligible subjects (TOL: 2,928; SOC: 4,189) from the United States (47.5%) and other countries. The two cohorts had similar age (mean age, 43.6 vs. 44.1 years) and sex distribution (50.5% male vs. 45.2% male). The TOL group had more white subjects (90.5% vs. 80.7%) and fewer Hispanic subjects (4.0% vs. 12.6%), a lower baseline mean eGFR (60 vs. 70 mL/min/1.73 m2), more in chronic kidney disease (CKD) stage 3 or above (58.1% vs. 41.0%), and more frequent history of signs of rapid disease progression (e.g., nephrolithiasis, hematuria, urinary tract infection). Among 4,917 subjects with TKV assessments, mean baseline TKV was higher in the TOL group (1,817 mL) compared with SOC (1,627 mL). Conclusion In this large, longitudinal database of unique subjects with ADPKD, distinct differences exist in some baseline characteristics of the TOL and SOC groups. Compared with the previous database, the expanded database doubled the size of the TOL group and included more subjects who were older and with advanced chronic kidney disease stage. This database provides a diverse ADPKD population to assess outcomes.

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