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  1. Article ; Online: Effects of salt and protein intake on polyuria in V2RA-treated ADPKD patients.

    Geertsema, Paul / Koorevaar, Iris W / Ipema, Karin J R / Kramers, Bart J / Casteleijn, Niek F / Gansevoort, Ron T / Meijer, Esther

    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association

    2023  Volume 39, Issue 4, Page(s) 707–716

    Abstract: Background: The only treatment proven to be renoprotective in autosomal dominant polycystic kidney disease (ADPKD) is a vasopressin V2-receptor antagonist (V2RA). However, aquaresis-associated side effects limit tolerability. We investigated whether ... ...

    Abstract Background: The only treatment proven to be renoprotective in autosomal dominant polycystic kidney disease (ADPKD) is a vasopressin V2-receptor antagonist (V2RA). However, aquaresis-associated side effects limit tolerability. We investigated whether salt and/or protein intake influences urine volume and related endpoints in V2RA-treated ADPKD patients.
    Methods: In this randomized, controlled, double-blind, crossover trial, ADPKD patients treated with maximally tolerated dose of a V2RA were included. While on a low salt and low protein diet, patients were given additional salt and protein to mimic regular intake, which was subsequently replaced by placebo in random order during four 2-week periods. Primary endpoint was change in 24-h urine volume. Secondary endpoints were change in quality of life, measured glomerular filtration rate (mGFR), blood pressure and copeptin level.
    Results: Twelve patients (49 ± 8 years, 25.0% male) were included. Baseline salt and protein intake were 10.8 ± 1.3 g/24-h and 1.2 ± 0.2 g/kg/24-h, respectively. During the low salt and low protein treatment periods, intake decreased to 5.8 ± 1.6 g/24-h and 0.8 ± 0.1 g/kg/24-h, respectively. Baseline 24-h urine volume (5.9 ± 1.2 L) decreased to 5.2 ± 1.1 L (-11%, P = .004) on low salt and low protein, and to 5.4 ± 0.9 L (-8%, P = .04) on low salt. Reduction in 24-h urine volume was two times greater in patients with lower urine osmolality (-16% vs -7%). Polyuria quality of life scores improved in concordance with changes in urine volume. mGFR decreased during the low salt and low protein, while mean arterial pressure did not change during study periods. Plasma copeptin decreased significantly during low salt and low protein periods.
    Conclusion: Lowering dietary salt and protein intake has a minor effect on urine volume in V2RA-treated ADPKD patients. Reduced intake of osmoles decreased copeptin concentrations and might thus increase the renoprotective effect of a V2RA in ADPKD patients.
    MeSH term(s) Female ; Humans ; Male ; Antidiuretic Hormone Receptor Antagonists/therapeutic use ; Glomerular Filtration Rate ; Kidney ; Polycystic Kidney, Autosomal Dominant/complications ; Polyuria/chemically induced ; Polyuria/complications ; Polyuria/drug therapy ; Quality of Life ; Sodium Chloride, Dietary ; Tolvaptan/therapeutic use ; Double-Blind Method ; Cross-Over Studies
    Chemical Substances Antidiuretic Hormone Receptor Antagonists ; Sodium Chloride, Dietary ; Tolvaptan (21G72T1950)
    Language English
    Publishing date 2023-10-06
    Publishing country England
    Document type Randomized Controlled Trial ; Journal Article
    ZDB-ID 90594-x
    ISSN 1460-2385 ; 0931-0509
    ISSN (online) 1460-2385
    ISSN 0931-0509
    DOI 10.1093/ndt/gfad218
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  2. Article ; Online: Nefrotoxiciteit van protonpompremmers.

    Klatte, D C F / Wiegersma, J S / Dekker, F W / Gansevoort, R T

    Nederlands tijdschrift voor geneeskunde

    2021  Volume 165

    Abstract: Proton pump inhibitors are widely used, and generally considered safe. In this clinical lesson two cases are presented with a strong suspicion of proton pump inhibitor induced decline of kidney function. This adverse event has only recently been ... ...

    Title translation Proton pump inhibitor-induced nephrotoxicity.
    Abstract Proton pump inhibitors are widely used, and generally considered safe. In this clinical lesson two cases are presented with a strong suspicion of proton pump inhibitor induced decline of kidney function. This adverse event has only recently been identified in epidemiological studies. Our cases illustrate that chronic proton pump inhibitor nephrotoxicity can manifest subtle and may therefore be difficult to recognize. We discuss the current epidemiological evidence to support these observations, and the pathophysiology and clinical manifestations of proton pump inhibitor nephrotoxicity. In case a subject using a proton pump inhibitor shows kidney function decline, without a clear cause, withdrawal of this medication is advised. Although for an individual patient the risk may not be high, the large number of proton pump users makes that this adverse event is important on a population level.
    MeSH term(s) Humans ; Proton Pump Inhibitors/adverse effects
    Chemical Substances Proton Pump Inhibitors
    Language Dutch
    Publishing date 2021-09-09
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 82073-8
    ISSN 1876-8784 ; 0028-2162
    ISSN (online) 1876-8784
    ISSN 0028-2162
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  3. Article ; Online: Kidney phosphate wasting predicts poor outcome in polycystic kidney disease.

    Xue, Laixi / Geurts, Frank / Meijer, Esther / de Borst, Martin H / Gansevoort, Ron T / Zietse, Robert / Hoorn, Ewout J / Salih, Mahdi

    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association

    2023  

    Abstract: Background and hypothesis: Patients with ADPKD have disproportionately high levels of fibroblast growth factor-23 (FGF-23) for their CKD-stage with only a subgroup that develops kidney phosphate wasting. We assessed factors associated with phosphate ... ...

    Abstract Background and hypothesis: Patients with ADPKD have disproportionately high levels of fibroblast growth factor-23 (FGF-23) for their CKD-stage with only a subgroup that develops kidney phosphate wasting. We assessed factors associated with phosphate wasting and hypothesize that it identifies patients with more severe disease and predicts disease progression.
    Methods: We included 604 patients with ADPKD from a multi-center prospective observational (DIPAK) cohort in 4 university medical centers in the Netherlands. We measured parathyroid hormone (PTH), total plasma FGF-23 levels and calculated the ratio of tubular maximum reabsorption rate of phosphate to glomerular filtration rate (TmP/GFR) with < 0.8 mmol/L defined as kidney phosphate wasting. We analysed the association of TmP/GFR with eGFR decline over time and the risk for a composite kidney outcome (≥ 30% eGFR decline, kidney failure or kidney replacement therapy).
    Results: In our cohort (age 48 ± 12 years, 39% male, eGFR 63 ± 28 mL/min/1.73m2), 59% of patients had phosphate wasting. Male sex (coefficient -0.2, 95% confidence interval [CI] -0.2; -0.1), eGFR (0.002, 0.001-0.004), FGF-23 (0.1, 0.03-0.2), PTH(-0.2, -0.3; -0.06) and Copeptin(-0.08, -0.1; -0.08) were associated with TmP/GFR. Corrected for PTH, FGF-23 and eGFR, every 0.1 mmol/L decrease in TmP/GFR was associated with a greater eGFR decline of 0.2 ml/min/1.73m2/year (95% CI 0.01-0.3) and an increased hazard ratio of 1.09 (95% CI 1.01-1.18) of the composite kidney outcome.
    Conclusion: Our study shows that in patients with ADPKD phosphate wasting is prevalent and associated with more rapid disease progression. Phosphate wasting may be a consequence of early proximal tubular dysfunction and insufficient suppression of PTH.
    Language English
    Publishing date 2023-11-20
    Publishing country England
    Document type Journal Article
    ZDB-ID 90594-x
    ISSN 1460-2385 ; 0931-0509
    ISSN (online) 1460-2385
    ISSN 0931-0509
    DOI 10.1093/ndt/gfad247
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  4. Article ; Online: Prostaglandin E2, Osmoregulation, and Disease Progression in Autosomal Dominant Polycystic Kidney Disease.

    Geurts, Frank / Xue, Laixi / Kramers, Bart J / Zietse, Robert / Gansevoort, Ron T / Fenton, Robert A / Meijer, Esther / Salih, Mahdi / Hoorn, Ewout J

    Clinical journal of the American Society of Nephrology : CJASN

    2023  Volume 18, Issue 11, Page(s) 1426–1434

    Abstract: Background: Prostaglandin E2 (PGE2) plays a physiological role in osmoregulation, a process that is affected early in autosomal dominant polycystic kidney disease (ADPKD). PGE2 has also been implicated in the pathogenesis of ADPKD in preclinical models, ...

    Abstract Background: Prostaglandin E2 (PGE2) plays a physiological role in osmoregulation, a process that is affected early in autosomal dominant polycystic kidney disease (ADPKD). PGE2 has also been implicated in the pathogenesis of ADPKD in preclinical models, but human data are limited. Here, we hypothesized that urinary PGE2 excretion is associated with impaired osmoregulation, disease severity, and disease progression in human ADPKD.
    Methods: Urinary excretions of PGE2 and its metabolite (PGEM) were measured in a prospective cohort of patients with ADPKD. The associations between urinary PGE2 and PGEM excretions, markers of osmoregulation, eGFR and height-adjusted total kidney volume were assessed using linear regression models. Cox regression and linear mixed models were used for the longitudinal analysis of the associations between urinary PGE2 and PGEM excretions and disease progression defined as 40% eGFR loss or kidney failure, and change in eGFR over time. In two intervention studies, we quantified the effect of starting tolvaptan and adding hydrochlorothiazide to tolvaptan on urinary PGE2 and PGEM excretions.
    Results: In 562 patients with ADPKD (61% female, eGFR 63±28 ml/min per 1.73 m 2 ), higher urinary PGE2 or PGEM excretions were independently associated with higher plasma copeptin, lower urine osmolality, lower eGFR, and greater total kidney volume. Participants with higher baseline urinary PGE2 and PGEM excretions had a higher risk of 40% eGFR loss or kidney failure (hazard ratio, 1.28; 95% confidence interval [CI], 1.13 to 1.46 and hazard ratio, 1.50; 95% CI, 1.26 to 1.80 per two-fold higher urinary PGE2 or PGEM excretions) and a faster change in eGFR over time (-0.39 [95% CI, -0.59 to -0.20] and -0.53 [95% CI, -0.75 to -0.31] ml/min per 1.73 m 2 per year). In the intervention studies, urinary PGEM excretion was higher after starting tolvaptan, while urinary PGE2 excretion was higher after adding hydrochlorothiazide to tolvaptan.
    Conclusions: Higher urinary PGE2 and PGEM excretions in patients with ADPKD are associated with impaired osmoregulation, disease severity, and progression.
    MeSH term(s) Humans ; Female ; Male ; Polycystic Kidney, Autosomal Dominant/complications ; Polycystic Kidney, Autosomal Dominant/drug therapy ; Tolvaptan/therapeutic use ; Dinoprostone/pharmacology ; Prospective Studies ; Osmoregulation ; Disease Progression ; Kidney/pathology ; Renal Insufficiency/complications ; Hydrochlorothiazide/pharmacology ; Glomerular Filtration Rate ; Antidiuretic Hormone Receptor Antagonists
    Chemical Substances Tolvaptan (21G72T1950) ; Dinoprostone (K7Q1JQR04M) ; Hydrochlorothiazide (0J48LPH2TH) ; Antidiuretic Hormone Receptor Antagonists
    Language English
    Publishing date 2023-08-14
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 2226665-3
    ISSN 1555-905X ; 1555-9041
    ISSN (online) 1555-905X
    ISSN 1555-9041
    DOI 10.2215/CJN.0000000000000269
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  5. Article ; Online: Plasma calprotectin and new-onset type 2 diabetes in the general population: a prospective cohort study.

    Bourgonje, Arno R / Bourgonje, Martin F / Sokooti, Sara / la Bastide-van Gemert, Sacha / Nilsen, Tom / Hidden, Clara / Gansevoort, Ron T / Mulder, Douwe J / Hillebrands, Jan-Luuk / Bakker, Stephan J L / van Beek, André / Dullaart, Robin P F / van Goor, Harry / Abdulle, Amaal E

    The Journal of clinical endocrinology and metabolism

    2024  

    Abstract: Background: Systemic inflammation plays a pivotal role in the development of type 2 diabetes (T2D). Here we hypothesized that circulating levels of calprotectin, a myeloid cell-derived biomarker of inflammation, is associated with the development of new- ...

    Abstract Background: Systemic inflammation plays a pivotal role in the development of type 2 diabetes (T2D). Here we hypothesized that circulating levels of calprotectin, a myeloid cell-derived biomarker of inflammation, is associated with the development of new-onset T2D in the general population.
    Methods: A total of 4,815 initially non-diabetic participants of the Prevention of Renal and Vascular ENd-stage Disease (PREVEND), a prospective population-based cohort study, were assessed for plasma levels of calprotectin at baseline. Circulating levels of calprotectin were investigated for potential associations with the risk of new-onset T2D, defined as a fasting plasma glucose level ≥7.0 mmol/l, a random plasma glucose level ≥11.1 mmol/l, a self-reported physician-based diagnosis of T2D, the use of glucose-lowering drugs, or any combinations thereof.
    Results: Median plasma calprotectin levels were 0.49 [0.35-0.69] mg/l. Plasma calprotectin levels were significantly associated with the risk of new-onset T2D (hazard ratio [HR] per doubling 1.42 [95% confidence interval: 1.22-1.66], P<0.001). The association remained independent of adjustment for age and sex (HR 1.34 [95%CI: 1.14-1.57], P<0.001), but not after further adjustment for potentially confounding factors (HR 1.11 [95% CI: 0.90-1.37], P=0.326), with adjustment for hyperlipidemia and high-sensitivity C-reactive protein explaining the loss of significance. Stratified analyses showed significant effect modification by hypertension, history of cardiovascular disease and HOMA-IR (Pinteraction≤0.001 for each), with higher HRs in individuals without hypertension, without history of cardiovascular disease and with below-median HOMA-IR.
    Conclusions: Elevated plasma levels of calprotectin are associated with a higher risk of developing T2D in the general population and may represent a moveable inflammatory biomarker. This association, however, does not represent a direct effect, and seems dependent on hyperlipidemia and systemic inflammation.
    Language English
    Publishing date 2024-03-04
    Publishing country United States
    Document type Journal Article
    ZDB-ID 3029-6
    ISSN 1945-7197 ; 0021-972X
    ISSN (online) 1945-7197
    ISSN 0021-972X
    DOI 10.1210/clinem/dgae130
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  6. Article ; Online: Plasma Calprotectin Levels Associate With New-Onset Hypertension in the General Population: A Prospective Cohort Study.

    Bourgonje, Arno R / Bourgonje, Martin F / la Bastide-van Gemert, Sacha / Nilsen, Tom / Hidden, Clara / Gansevoort, Ron T / Bakker, Stephan J L / Mulder, Douwe J / Dullaart, Robin P F / Abdulle, Amaal E / van Goor, Harry

    Journal of the American Heart Association

    2023  Volume 13, Issue 1, Page(s) e031458

    Abstract: Background: Low-grade systemic inflammation is a relevant pathogenic mechanism underlying the development of hypertension. In this study, we hypothesized that plasma calprotectin levels, as a biomarker of neutrophil-mediated inflammation, is associated ... ...

    Abstract Background: Low-grade systemic inflammation is a relevant pathogenic mechanism underlying the development of hypertension. In this study, we hypothesized that plasma calprotectin levels, as a biomarker of neutrophil-mediated inflammation, is associated with developing new-onset hypertension in the general population.
    Methods and results: Plasma calprotectin levels were determined in 3524 participants who participated in the PREVEND (Prevention of Renal and Vascular End-Stage Disease) study, a prospective population-based cohort study. Plasma calprotectin levels were studied for associations with the risk of new-onset hypertension, defined as systolic blood pressure of at least 140 mm Hg, diastolic blood pressure of at least 90 mm Hg, or the first recorded use of antihypertensives. Participants with hypertension at baseline were excluded. Median plasma calprotectin levels were 0.48 (0.34-0.66) mg/L, and median systolic blood pressure was 117 (109-126) mm Hg. Plasma calprotectin levels were significantly associated with the risk of new-onset hypertension (hazard ratio [HR], per doubling 1.30 [95% CI, 1.21-1.41];
    Conclusions: Higher plasma calprotectin levels are associated with an increased risk of new-onset hypertension in the general population. This association is dependent on baseline systolic blood pressure and is particularly prominent in men compared with women.
    MeSH term(s) Male ; Humans ; Female ; Prospective Studies ; Cohort Studies ; Leukocyte L1 Antigen Complex ; Risk Factors ; Hypertension/diagnosis ; Hypertension/epidemiology ; Hypertension/complications ; Blood Pressure/physiology ; Inflammation/complications ; Albumins
    Chemical Substances Leukocyte L1 Antigen Complex ; Albumins
    Language English
    Publishing date 2023-12-29
    Publishing country England
    Document type Journal Article
    ZDB-ID 2653953-6
    ISSN 2047-9980 ; 2047-9980
    ISSN (online) 2047-9980
    ISSN 2047-9980
    DOI 10.1161/JAHA.123.031458
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  7. Article: A Sex-Specific Comparative Analysis of Oxidative Stress Biomarkers Predicting the Risk of Cardiovascular Events and All-Cause Mortality in the General Population: A Prospective Cohort Study.

    Bourgonje, Martin F / Abdulle, Amaal E / Kieneker, Lyanne M / la Bastide-van Gemert, Sacha / Bakker, Stephan J L / Gansevoort, Ron T / Gordijn, Sanne J / van Goor, Harry / Bourgonje, Arno R

    Antioxidants (Basel, Switzerland)

    2023  Volume 12, Issue 3

    Abstract: Oxidative stress plays a pivotal role in cardiovascular (CV) disease, but current biomarkers used to predict CV events are still insufficient. In this study, we comparatively assessed the utility of redox-related biomarkers in predicting the risk of CV ... ...

    Abstract Oxidative stress plays a pivotal role in cardiovascular (CV) disease, but current biomarkers used to predict CV events are still insufficient. In this study, we comparatively assessed the utility of redox-related biomarkers in predicting the risk of CV events and all-cause mortality in male and female subjects from the general population. Subjects (
    Language English
    Publishing date 2023-03-10
    Publishing country Switzerland
    Document type Journal Article
    ZDB-ID 2704216-9
    ISSN 2076-3921
    ISSN 2076-3921
    DOI 10.3390/antiox12030690
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  8. Article ; Online: The association of urinary epidermal growth factors with ADPKD disease severity and progression.

    Harskamp, Laura R / Perez-Gomez, Maria Vanessa / Heida, Judith E / Engels, Gerwin E / van Goor, Harry / van den Heuvel, Marius C / Streets, Andrew J / Ong, Albert C M / Ortiz, Alberto / Gansevoort, Ron T

    Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association

    2023  Volume 38, Issue 10, Page(s) 2266–2275

    Abstract: ... with baseline eGFR (R = 0.54, P < .001) and a lower EGF was strongly associated with a more rapid GFR decline ...

    Abstract Background: The epidermal growth factor receptor (EGFR) pathway is involved in kidney tissue repair and growth. Preclinical interventional data and scarce human data have suggested a role for this pathway in the pathophysiology of autosomal dominant polycystic kidney disease (ADPKD), while other data have suggested that its activation is causally linked to repair of damaged kidney tissue. We hypothesize that urinary EGFR ligands, as a reflection of EGFR activity, are associated with kidney function decline in ADPKD in the context of tissue repair following injury, and as the disease progresses as a sign of insufficient repair.
    Methods: In the present study, we measured the EGFR ligands, EGF and heparin binding-EGF (HB-EGF), in 24-h urine samples of 301 ADPKD patients and 72 age- and sex-matched living kidney donors to dissect the role of the EGFR pathway in ADPKD. During a median follow-up of 2.5 years, the association of urinary EGFR ligand excretion with annual change in estimated glomerular filtration rate (eGFR) and height-adjusted total kidney volume in ADPKD patients was analyzed using mixed-models methods, and the expression of three closely related EGFR family receptors in ADPKD kidney tissue was investigated by immunohistochemistry. Additionally, the effect of reducing renal mass (after kidney donation), was assessed to investigate whether urinary EGF matches this reduction and thus reflects the amount of remaining healthy kidney tissue.
    Results: At baseline, urinary HB-EGF did not differ between ADPKD patients and healthy controls (P = .6), whereas a lower urinary EGF excretion was observed in ADPKD patients [18.6 (11.8-27.8)] compared with healthy controls [51.0 (34.9-65.4) μg/24 h, P < .001]. Urinary EGF was positively associated with baseline eGFR (R = 0.54, P < .001) and a lower EGF was strongly associated with a more rapid GFR decline, even when adjusted for ADPKD severity markers (β = 1.96, P < .001), whereas HB-EGF was not. Expression of the EGFR, but not other EGFR-related receptors, was observed in renal cysts but was absent in non-ADPKD kidney tissue. Finally, unilateral nephrectomy resulted in a decrease of 46.4 (-63.3 to -17.6) % in urinary EGF excretion, alongside a decrease of 35.2 ± 7.2% in eGFR and 36.8 ± 6.9% in measured GFR (mGFR), whereas maximal mGFR (measured after dopamine induced hyperperfusion) decreased by 46.1 ± 7.8% (all P < .001).
    Conclusions: Our data suggest that lower urinary EGF excretion may be a valuable novel predictor for kidney function decline in patients with ADPKD.
    MeSH term(s) Humans ; Polycystic Kidney, Autosomal Dominant/complications ; Heparin-binding EGF-like Growth Factor ; Epidermal Growth Factor ; Disease Progression ; Kidney ; Glomerular Filtration Rate ; Patient Acuity
    Chemical Substances Heparin-binding EGF-like Growth Factor ; Epidermal Growth Factor (62229-50-9)
    Language English
    Publishing date 2023-03-14
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 90594-x
    ISSN 1460-2385 ; 0931-0509
    ISSN (online) 1460-2385
    ISSN 0931-0509
    DOI 10.1093/ndt/gfad050
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  9. Article ; Online: Validation of a semi-automatic method to measure total liver volumes in polycystic liver disease on computed tomography - high speed and accuracy.

    Aapkes, Sophie E / Barten, Thijs R M / Coudyzer, Walter / Drenth, Joost P H / Geijselaers, Ivo M A / Ter Grote, Sterre A M / Gansevoort, Ron T / Nevens, Frederik / van Gastel, Maatje D A

    European radiology

    2023  Volume 33, Issue 5, Page(s) 3222–3231

    Abstract: Objectives: Polycystic liver disease (PLD) is characterized by growth of hepatic cysts, causing hepatomegaly. Disease severity is determined using total liver volume (TLV), which can be measured from computed tomography (CT). The gold standard is manual ...

    Abstract Objectives: Polycystic liver disease (PLD) is characterized by growth of hepatic cysts, causing hepatomegaly. Disease severity is determined using total liver volume (TLV), which can be measured from computed tomography (CT). The gold standard is manual segmentation which is time-consuming and requires expert knowledge of the anatomy. This study aims to validate the commercially available semi-automatic MMWP (Multimodality Workplace) Volume tool for CT scans of PLD patients.
    Methods: We included adult patients with one (n = 60) or two (n = 46) abdominal CT scans. Semi-automatic contouring was compared with manual segmentation, using comparison of observed volumes (cross-sectional) and growth (longitudinal), correlation coefficients (CC), and Bland-Altman analyses with bias and precision, defined as the mean difference and SD from this difference. Inter- and intra-reader variability were assessed using coefficients of variation (CV) and we assessed the time to perform both procedures.
    Results: Median TLV was 5292.2 mL (IQR 3141.4-7862.2 mL) at baseline. Cross-sectional analysis showed high correlation and low bias and precision between both methods (CC 0.998, bias 1.62%, precision 2.75%). Absolute volumes were slightly higher for semi-automatic segmentation (manual 5292.2 (3141.4-7862.2) versus semi-automatic 5432.8 (3071.9-7960.2) mL, difference 2.7%, p < 0.001). Longitudinal analysis demonstrated that semi-automatic segmentation accurately measures liver growth (CC 0.908, bias 0.23%, precision 4.04%). Inter- and intra-reader variability were small (2.19% and 0.66%) and comparable to manual segmentation (1.21% and 0.63%) (p = 0.26 and p = 0.37). Semi-automatic segmentation was faster than manual tracing (19 min versus 50 min, p = 0.009).
    Conclusions: Semi-automatic liver segmentation is a fast and accurate method to determine TLV and liver growth in PLD patients.
    Key points: • Semi-automatic liver segmentation using the commercially available MMWP volume tool accurately determines total liver volume as well as liver growth over time in polycystic liver disease patients. • This method is considerably faster than manual segmentation through the use of Hounsfield unit settings. • We used a real-life CT set for the validation and showed that the semi-automatic tool measures accurately regardless of contrast used for the CT scan or not, presence of polycystic kidneys, liver volume, and previous invasive treatment for polycystic liver disease.
    MeSH term(s) Adult ; Humans ; Cross-Sectional Studies ; Tomography, X-Ray Computed/methods ; Liver Diseases/diagnostic imaging ; Reproducibility of Results
    Language English
    Publishing date 2023-01-14
    Publishing country Germany
    Document type Journal Article
    ZDB-ID 1085366-2
    ISSN 1432-1084 ; 0938-7994 ; 1613-3749
    ISSN (online) 1432-1084
    ISSN 0938-7994 ; 1613-3749
    DOI 10.1007/s00330-022-09346-6
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: Familiaire cystenieren: jongvolwassen familieleden screenen of niet?

    Kramers, B J / Storm, M / Gansevoort, R T

    Nederlands tijdschrift voor geneeskunde

    2017  Volume 161, Page(s) D1942

    Abstract: Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disease, with a global prevalence of 10 per 10,000. It is characterized by the formation of numerous cysts in both kidneys, and leads to renal function loss; the ... ...

    Title translation Autosomal dominant polycystic kidney disease: should patients' young adult relatives be screened or not?
    Abstract Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disease, with a global prevalence of 10 per 10,000. It is characterized by the formation of numerous cysts in both kidneys, and leads to renal function loss; the majority of patients will eventually need renal replacement therapy. It is possible to screen patients' presymptomatic family members from a young age, but this has not historically been recommended as until recently there were no treatment options. This year, the vasopressin V2 receptor antagonist tolvaptan was approved for prescription in ADPKD, to slow the rate of renal function decline. The availability of this new treatment option, along with other factors such as the possible use of IVF procedures with pre-implantation genetic diagnosis, imply that we have to rethink the issue of presymptomatic screening. Young adults at-risk should be screened, to give them the chance to opt for treatment.
    MeSH term(s) Adult ; Antidiuretic Hormone Receptor Antagonists ; Genetic Testing ; Humans ; Polycystic Kidney, Autosomal Dominant/diagnosis ; Polycystic Kidney, Autosomal Dominant/genetics ; Young Adult
    Chemical Substances Antidiuretic Hormone Receptor Antagonists
    Language Dutch
    Publishing date 2017
    Publishing country Netherlands
    Document type Journal Article
    ZDB-ID 82073-8
    ISSN 1876-8784 ; 0028-2162
    ISSN (online) 1876-8784
    ISSN 0028-2162
    Database MEDical Literature Analysis and Retrieval System OnLINE

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