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

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.

Autosomal dominant polycystic kidney disease management

2018 ◽  
Author(s):  
Young-Hwan Hwang ◽  
York Pei
Keyword(s):  
Blood Pressure ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Kidney Volume ◽  
Polycystic Kidney ◽  
Renal Disease Progression ◽  
Kidney Size ◽  
Autosomal Dominant Polycystic Kidney ◽  
Cyst Growth

Management of patients with autosomal dominant polycystic kidney disease (ADPKD) currently comprises non-specific measures including promotion of healthy lifestyle, optimization of blood pressure control, and modification of cardiovascular risk factors. A high water intake of 3–4 L per day in patients with glomerular filtration rate greater than 30 mL/min/1.73 m2 may decrease the risk of kidney stones, but its potential benefit in reducing renal cyst growth is presently unproven. Maintenance of a target blood pressure of 130/80 mmHg is recommended by expert clinical guidelines though this is unlikely to slow cyst growth. It is unclear whether pharmacological blockade of the renin–angiotensin axis confers an extrarenal protective effect. Recognition of the variable clinical presentations of cyst infection, cyst haemorrhage, or nephrolithiasis is important for early diagnosis and optimal management of these complications. Most patients with ADPKD do well on dialysis and after transplantation. Nephrectomy may be needed to make space for a donor kidney, or if kidney size or infection is an issue after end-stage renal failure is reached. Recent advances in ADPKD have led to the identification of multiple potential therapeutic targets with more than 10 clinical trials completed or currently in progress. Given the promising results of the TEMPO trial, tolvaptan may well be the first disease-modifying drug to be approved for clinical use. Several other classes of drugs (e.g. somatostatin analogues, triptolide, metformin, and glucosylceramide synthase inhibitors) with good long-term safety profiles are promising candidates which may be repurposed for this disease. In the future, identifying patients with different risks of renal disease progression by their genotype and/or kidney volume will likely assume an important role for the clinical management of ADPKD.

Natural history of intracranial aneurysms in autosomal dominant polycystic kidney disease

2017 ◽  
Vol 51 (6) ◽  
pp. 476-480 ◽  
Author(s):  
Mariusz Niemczyk ◽  
Monika Gradzik ◽  
Magda Fliszkiewicz ◽  
Andrzej Kulesza ◽  
Marek Gołębiowski ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Natural History ◽  
Polycystic Kidney Disease ◽  
Intracranial Aneurysms ◽  
Autosomal Dominant ◽  
Polycystic Kidney ◽  
History Of ◽  
Autosomal Dominant Polycystic Kidney

scholarly journals Nephrectomy in patients with autosomal dominant polycystic kidney disease after kidney transplantation

2020 ◽  
Vol 13 (5) ◽  
pp. 126-131
Author(s):  
A.E. Lubennikov ◽  
◽  
R.N. Trushkin ◽  
D.F. Kantimerov ◽  
L.Yu. Artyukhina ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Dominant ◽  
Imaging Method ◽  
Polycystic Kidney ◽  
Number Of Patients ◽  
History Of ◽  
Autosomal Dominant Polycystic Kidney ◽  
Inflammatory Changes

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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