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  • 1
    Online Resource
    Online Resource
    Early childhood height-adjusted total kidney volume as a risk marker of kidney survival in ARPKD
    Springer Science and Business Media LLC ; 2021
    In:  Scientific Reports Vol. 11, No. 1 ( 2021-11-04)
    Details
    In: Scientific Reports, Springer Science and Business Media LLC, Vol. 11, No. 1 ( 2021-11-04)
    Abstract: Autosomal recessive polycystic kidney disease (ARPKD) is characterized by bilateral fibrocystic changes resulting in pronounced kidney enlargement. Impairment of kidney function is highly variable and widely available prognostic markers are urgently needed as a base for clinical decision-making and future clinical trials. In this observational study we analyzed the longitudinal development of sonographic kidney measurements in a cohort of 456 ARPKD patients from the international registry study ARegPKD. We furthermore evaluated correlations of sonomorphometric findings and functional kidney disease with the aim to describe the natural disease course and to identify potential prognostic markers. Kidney pole-to-pole (PTP) length and estimated total kidney volume (eTKV) increase with growth throughout childhood and adolescence despite individual variability. Height-adjusted PTP length decreases over time, but such a trend cannot be seen for height-adjusted eTKV (haeTKV) where we even observed a slight mean linear increase of 4.5 ml/m per year during childhood and adolescence for the overall cohort. Patients with two null PKHD1 variants had larger first documented haeTKV values than children with missense variants (median (IQR) haeTKV 793 (450–1098) ml/m in Null/null, 403 (260–538) ml/m in Null/mis, 230 (169–357) ml/m in Mis/mis). In the overall cohort, estimated glomerular filtration rate decreases with increasing haeTKV (median (IQR) haeTKV 210 (150–267) ml/m in CKD stage 1, 472 (266–880) ml/m in stage 5 without kidney replacement therapy). Strikingly, there is a clear correlation between haeTKV in the first eighteen months of life and kidney survival in childhood and adolescence with ten-year kidney survival rates ranging from 20% in patients of the highest to 94% in the lowest quartile. Early childhood haeTKV may become an easily obtainable prognostic marker of kidney disease in ARPKD, e.g. for the identification of patients for clinical studies.
    Type of Medium: Online Resource
    ISSN: 2045-2322
    URL: Article
    DOI: 10.1038/s41598-021-00523-z
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2021
    detail.hit.zdb_id: 2615211-3
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  • 2
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    Online Resource
    Distal renal tubular acidosis: ERKNet/ESPN clinical practice points
    Oxford University Press (OUP) ; 2021
    In:  Nephrology Dialysis Transplantation Vol. 36, No. 9 ( 2021-08-27), p. 1585-1596
    Details
    In: Nephrology Dialysis Transplantation, Oxford University Press (OUP), Vol. 36, No. 9 ( 2021-08-27), p. 1585-1596
    Abstract: Distal renal tubular acidosis (dRTA) is characterized by an impaired ability of the distal tubule to excrete acid, leading to metabolic acidosis. Associated complications include bone disease, growth failure, urolithiasis and hypokalaemia. Due to its rarity, there is limited evidence to guide diagnosis and management; however, available data strongly suggest that metabolic control of the acidosis by alkali supplementation can halt or revert almost all complications. Despite this, cohort studies show that adequate metabolic control is present in only about half of patients, highlighting problems with treatment provision or adherence. With these clinical practice points the authors, part of the working groups tubulopathies in the European Rare Kidney Disease Reference network and inherited kidney diseases of the European Society for Paediatric Nephrology, aim to provide guidance for the management of patients with dRTA to facilitate adequate treatment and establish an initial best practice standard against which treatment of patients can be audited.
    Type of Medium: Online Resource
    ISSN: 0931-0509 , 1460-2385
    URL: Article
    DOI: 10.1093/ndt/gfab171
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2021
    detail.hit.zdb_id: 1465709-0
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  • 3
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    Arterial stiffness indices in children and adolescents with type 1 diabetes mellitus: A meta‐analysis
    Wiley ; 2022
    In:  Diabetes/Metabolism Research and Reviews Vol. 38, No. 6 ( 2022-09)
    Details
    In: Diabetes/Metabolism Research and Reviews, Wiley, Vol. 38, No. 6 ( 2022-09)
    Abstract: Cardiovascular disease (CVD) represents the most frequent cause of morbidity and mortality among patients with type 1 diabetes mellitus (T1DM). Our aim was to review the evidence and conduct a meta‐analysis assessing measures of arterial stiffness by pulse wave velocity (PWV) and augmentation index (AIx) in children and adolescents with T1DM compared to healthy controls. Methods PubMed and the Cochrane Library were searched for relevant studies published up to 10 May 2021. Results Twenty‐one studies were finally included in the meta‐analysis. The T1DM group had significantly higher carotid to femoral PWV levels than that of the control group (mean difference [d]: 0.53 CI: 0.35–0.71, P   〈  0.00001) but with a fair heterogeneity (I 2:73%). By omitting one study with marked heterogeneity, mean difference in cfPWV remained significantly increased in the T1DM group compared to the control group (mean difference [d]: 0.37 CI: 0.27–0.48, P   〈  0.00001) but with improved heterogeneity ( I 2 = 26%). Regarding Aix, the T1DM group had a significantly higher AI@75 index than that of the control group (mean difference [d]: 0.28 CI: 0.17–0.39, P   〈  0.00001) and with no heterogeneity (I 2 = 8%). Conclusions Youths with T1DM show increased arterial stiffness, either as increased carotid‐femoral pulse wave velocity or increased augmentation index, early in their course of life compared to healthy controls. PROSPERO registration number CRD42021253236
    Type of Medium: Online Resource
    ISSN: 1520-7552 , 1520-7560
    URL: Issue
    URL: Article
    DOI: 10.1002/dmrr.v38.6
    DOI: 10.1002/dmrr.3555
    Language: English
    Publisher: Wiley
    Publication Date: 2022
    detail.hit.zdb_id: 2001565-3
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  • 4
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    Online Resource
    Long-Term Changes in Blood Pressure After Pediatric Kidney Transplantation
    Oxford University Press (OUP) ; 2016
    In:  American Journal of Hypertension Vol. 29, No. 7 ( 2016-07), p. 860-865
    Details
    In: American Journal of Hypertension, Oxford University Press (OUP), Vol. 29, No. 7 ( 2016-07), p. 860-865
    Type of Medium: Online Resource
    ISSN: 0895-7061 , 1941-7225
    URL: Article
    DOI: 10.1093/ajh/hpv192
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2016
    detail.hit.zdb_id: 1479505-X
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  • 5
    Online Resource
    Online Resource
    Pathophysiology of Early Vascular Ageing-Opportunities for Treatment
    Bentham Science Publishers Ltd. ; 2013
    In:  The Open Hypertension Journal Vol. 5, No. 1 ( 2013-12-13), p. 58-62
    Details
    In: The Open Hypertension Journal, Bentham Science Publishers Ltd., Vol. 5, No. 1 ( 2013-12-13), p. 58-62
    Abstract: The story of vascular ageing was first described in the seventeenth century when Thomas Sydenham wrote that ‘A man is as old as his arteries’. This aphorism was returned to publicity lately when Peter Nilsson reintroduced the concept of early vascular ageing. As vascular ageing is described a gradual process involving biochemical, enzymatic, and cellular changes of the vasculature and modification of the signals that modulate them. In susceptible individuals this process appears to be accelerated, leading to features that comprise a condition characterized as early vascular aging (EVA). Early vascular ageing represents the acceleration of the vascular ageing process.
    Type of Medium: Online Resource
    ISSN: 1876-5262
    URL: Article
    DOI: 10.2174/1876526201305010058
    Language: English
    Publisher: Bentham Science Publishers Ltd.
    Publication Date: 2013
    detail.hit.zdb_id: 2677039-8
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  • 6
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    Online Resource
    The role of obesity, salt and exercise on blood pressure in children and adolescents
    Informa UK Limited ; 2011
    In:  Expert Review of Cardiovascular Therapy Vol. 9, No. 6 ( 2011-06), p. 753-761
    Details
    In: Expert Review of Cardiovascular Therapy, Informa UK Limited, Vol. 9, No. 6 ( 2011-06), p. 753-761
    Type of Medium: Online Resource
    ISSN: 1477-9072 , 1744-8344
    URL: Article
    DOI: 10.1586/erc.11.63
    Language: English
    Publisher: Informa UK Limited
    Publication Date: 2011
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  • 7
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    Online Resource
    Familial Hypercholesterolemia in Children and Adolescents: Diagnosis and Treatment
    Bentham Science Publishers Ltd. ; 2019
    In:  Current Pharmaceutical Design Vol. 24, No. 31 ( 2019-01-02), p. 3672-3677
    Details
    In: Current Pharmaceutical Design, Bentham Science Publishers Ltd., Vol. 24, No. 31 ( 2019-01-02), p. 3672-3677
    Abstract: Familial hypercholesterolemia is a hereditary genetic disorder predisposing in premature atherosclerosis and cardiovascular complications. Early diagnosis as well as effective treatment strategies in affected children are challenges among experts. Universal screening and cascade screening among families with familial hypercholesterolemia are being controversially discussed. Diagnosis of familial hypercholesterolemia in children and adolescents is usually based on clinical phenotype upon LDL-C levels and family history of premature cardiovascular and/or elevated LDL-C. Treatment approaches for familial hypercholesterolemia in the pediatric population are multidisciplinary and aim to reduce total cardiovascular risk. The most widely recommended and effective pharmacotherapy in the pediatric age group is currently statins. Ezetimibe and bile acid sequestrants are usually used as second-line agents. New therapeutic approaches, such as mipomersen and PCSK9 inhibitors seem promising. The main gap of evidence remains the lack of longitudinal follow up studies investigating cardiovascular outcomes, side effects, and effectiveness of treatment starting from childhood. Evidence would be expected in the near future by cohort and registry studies.
    Type of Medium: Online Resource
    ISSN: 1381-6128
    URL: Article
    DOI: 10.2174/1381612824666181010145807
    Language: English
    Publisher: Bentham Science Publishers Ltd.
    Publication Date: 2019
    SSG: 15,3
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  • 8
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    Unmasking hypertension in children and adolescents with sickle/beta‐thalassemia
    Wiley ; 2020
    In:  The Journal of Clinical Hypertension Vol. 22, No. 8 ( 2020-08), p. 1444-1449
    Details
    In: The Journal of Clinical Hypertension, Wiley, Vol. 22, No. 8 ( 2020-08), p. 1444-1449
    Abstract: Sickle cell disease (SCD) is associated with increased risk of cardiovascular disease, although blood pressure (BP) levels have been reported to be lower in SCD patients compared to general population. Aims of the present study were to investigate the prevalence of BP phenotypes and levels of arterial stiffness in pediatric patients with SCD and to assess the differences with children at risk for hypertension. We included in the study 16 pediatric SCD (HbS/β‐thalassemia, S/β‐thal) patients and 16 consecutive children at risk for hypertension referred to our hypertension clinic that served as high‐risk controls. All patients underwent ambulatory BP monitoring and measurement of carotid‐femoral pulse wave velocity (PWV). S/β‐thal patients had lower office systolic BP than the high‐risk control group (115.43 ± 10.03 vs 123.37 ± 11.92, P  = .05) but presented similar levels of day and night ambulatory BP. Office hypertension was found in 12.5% of the S/β‐thal patients and in 43.8% of the high‐risk controls ( P  = .06), while 18.8% of the S/β‐thal patients and 25% of the high‐risk controls presented hypertension by ambulatory BP levels ( P  = .21). All of the S/β‐thal patients with ambulatory hypertension had night hypertension (one combined night and day hypertension) with office normotension (masked hypertension). S/β‐thal patients and high‐risk controls presented equal prevalence of masked hypertension (18.8%). Children and adolescents with S/β‐thal present similar prevalence of BP phenotypes and levels of PWV with children at risk for hypertension. A significant number of children and adolescents with S/β‐thal may have masked nighttime hypertension despite normal office BP levels.
    Type of Medium: Online Resource
    ISSN: 1524-6175 , 1751-7176
    URL: Issue
    URL: Article
    DOI: 10.1111/jch.v22.8
    DOI: 10.1111/jch.13957
    Language: English
    Publisher: Wiley
    Publication Date: 2020
    detail.hit.zdb_id: 2058690-5
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  • 9
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    Correction to: Masked Hypertension in Healthy Children and Adolescents: Who Should Be Screened?
    Springer Science and Business Media LLC ; 2023
    In:  Current Hypertension Reports
    Details
    In: Current Hypertension Reports, Springer Science and Business Media LLC
    Type of Medium: Online Resource
    ISSN: 1522-6417 , 1534-3111
    URL: Article
    DOI: 10.1007/s11906-023-01271-3
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2023
    detail.hit.zdb_id: 2094165-1
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  • 10
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    Online Resource
    Masked Hypertension in Healthy Children and Adolescents: Who Should Be Screened?
    Springer Science and Business Media LLC ; 2023
    In:  Current Hypertension Reports Vol. 25, No. 9 ( 2023-09), p. 231-242
    Details
    In: Current Hypertension Reports, Springer Science and Business Media LLC, Vol. 25, No. 9 ( 2023-09), p. 231-242
    Abstract: The goal is to review masked hypertension (MH) as a relatively new phenomenon when patients have normal office BP but elevated out-of-office BP. Firstly, it was described in children in 2004. It has received increased attention in the past decade. Recent Findings The prevalence of MH in different pediatric populations differs widely between 0 and 60% based on the population studied, definition of MH, or method of out-of-office BP measurement. The highest prevalence of MH has been demonstrated in children with chronic kidney disease (CKD), obesity, diabetes, and after heart transplantation. In healthy children but with risk factors for hypertension such as prematurity, overweight/obesity, diabetes, chronic kidney disease, or positive family history of hypertension, the prevalence of MH is 9%. In healthy children without risk factors for hypertension, the prevalence of MH is very low ranging 0–3%. Summary In healthy children, only patients with the following clinical conditions should be screened for MH: high-normal/elevated office BP, positive family history of hypertension, and those referred for suspected hypertension who have normal office BP in the secondary/tertiary center.
    Type of Medium: Online Resource
    ISSN: 1522-6417 , 1534-3111
    URL: Article
    DOI: 10.1007/s11906-023-01260-6
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2023
    detail.hit.zdb_id: 2094165-1
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