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  • 1
    Online Resource
    Online Resource
    100 Gb/s all-optical clock recovery based on a monolithic dual-mode DBR laser
    Shanghai Institute of Optics and Fine Mechanics ; 2016
    In:  Chinese Optics Letters Vol. 14, No. 3 ( 2016), p. 030604-30607
    Details
    In: Chinese Optics Letters, Shanghai Institute of Optics and Fine Mechanics, Vol. 14, No. 3 ( 2016), p. 030604-30607
    Type of Medium: Online Resource
    ISSN: 1671-7694
    Uniform Title: 100 Gb/s all-optical clock recovery based on a monolithic dual-mode DBR laser
    URL: Issue
    URL: Journal
    URL: Article
    DOI: 10.3788/COL/2016/14/3
    DOI: 10.3788/COL
    DOI: 10.3788/COL201614.030604
    Language: English , Chinese
    Publisher: Shanghai Institute of Optics and Fine Mechanics
    Publication Date: 2016
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  • 2
    Online Resource
    Online Resource
    Number of Daily Peritoneal Dialysis Exchanges and Mortality Risk in a Chinese Population
    SAGE Publications ; 2018
    In:  Peritoneal Dialysis International: Journal of the International Society for Peritoneal Dialysis Vol. 38, No. 2_suppl ( 2018-12), p. 53-63
    Details
    In: Peritoneal Dialysis International: Journal of the International Society for Peritoneal Dialysis, SAGE Publications, Vol. 38, No. 2_suppl ( 2018-12), p. 53-63
    Abstract: We report outcomes on ≥ 4 compared with 〈 4 exchanges/day in a Chinese cohort on continuous ambulatory peritoneal dialysis (CAPD). Methods Data were sourced from the Baxter (China) Investment Co. Ltd Patient Support Program database, comprising an inception cohort commencing CAPD between 1 January 2005 and 13 August 2015. We used cause-specific Cox proportional hazards and Fine-Gray competing risks (kidney transplantation, change to hemodialysis) models to estimate mortality risk on ≥ 4 compared with 〈 4 exchanges/day. We matched or adjusted for age, gender, employment, insurance, primary renal disease, size of CAPD program, year of dialysis inception, and treatment center. Results We modeled 100,022 subjects from 1,177 centers over 239,876 patient-years. Of these subjects, 43,185 received 〈 4 exchanges/day and 56,837 ≥ 4 exchanges/day. The proportion of patients on 〈 4 exchanges/day varied widely between centers. Those on 〈 4 exchanges/day were significantly older, more often female, of unknown employment, and from rural China. In the various models, ≥ 4 exchanges/day was associated with a significantly lower risk of death by 30% – 35% compared with 〈 4 exchanges/day. This beneficial effect was greatest in younger and rural patients. Conclusions In this Chinese CAPD cohort, ≥ 4 exchanges/day was associated with significantly lower mortality risk than 〈 4 exchanges/day. Analyses are limited by residual confounding from unavailability of important prognostic covariates (e.g., comorbidity, socioeconomic factors) and data on residual renal function, peritoneal clearance, and transport status with which to judge the clinical appropriateness of CAPD prescription. Nonetheless, our study indicates this area as a high priority for further detailed study.
    Type of Medium: Online Resource
    ISSN: 0896-8608 , 1718-4304
    URL: Article
    DOI: 10.3747/pdi.2017.00283
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2018
    detail.hit.zdb_id: 2075957-5
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  • 3
    Online Resource
    Online Resource
    The Effect of Automated versus Continuous Ambulatory Peritoneal Dialysis on Mortality Risk in China
    SAGE Publications ; 2018
    In:  Peritoneal Dialysis International: Journal of the International Society for Peritoneal Dialysis Vol. 38, No. 2_suppl ( 2018-12), p. 25-35
    Details
    In: Peritoneal Dialysis International: Journal of the International Society for Peritoneal Dialysis, SAGE Publications, Vol. 38, No. 2_suppl ( 2018-12), p. 25-35
    Abstract: There is an emerging practice pattern of automated peritoneal dialysis (APD) in China. We report on outcomes compared to continuous ambulatory peritoneal dialysis (CAPD) in a Chinese cohort. Methods Data were sourced from the Baxter Healthcare (China) Investment Co. Ltd Patient Support Program database, comprising an inception cohort commencing PD between 1 January 2005 and 13 August 2015. We used time-dependent cause-specific Cox proportional hazards and Fine-Gray competing risks (kidney transplantation, change to hemodialysis) models to estimate relative mortality risk between APD and CAPD. We adjusted or matched for age, gender, employment, insurance, primary renal disease, size of PD program, and year of dialysis inception. We used cluster robust regression to account for center effect. Results We modeled 100,351 subjects from 1,178 centers over 240,803 patient-years. Of these, 368 received APD at some time. Compared with patients on CAPD, those on APD were significantly younger, more likely to be male, employed, self-paying, and from larger programs. Overall, APD was associated with a hazard ratio (HR) for death of 0.79 (95% confidence interval [CI] 0.64 – 0.97) compared with CAPD in Cox proportional hazards models, and 0.76 (0.62 – 0.95) in Fine-Gray competing risks regression models. There was prominent effect modification by follow-up time: benefit was observed only up to 4 years follow-up, after which risk of death was similar. Conclusion Automated peritoneal dialysis is associated with an overall lower adjusted risk of death compared with CAPD in China. Analyses are limited by the likelihood of important selection bias arising from group imbalance, and residual confounding from unavailability of important clinical covariates such as comorbidity and Kt/V.
    Type of Medium: Online Resource
    ISSN: 0896-8608 , 1718-4304
    URL: Article
    DOI: 10.3747/pdi.2017.00235
    Language: English
    Publisher: SAGE Publications
    Publication Date: 2018
    detail.hit.zdb_id: 2075957-5
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  • 4
    Online Resource
    Online Resource
    The first successful expanded compassionate use of Iptacopan in a patient with paroxysmal nocturnal hemoglobinuria
    Springer Science and Business Media LLC ; 2022
    In:  Annals of Hematology Vol. 101, No. 11 ( 2022-11), p. 2537-2539
    Details
    In: Annals of Hematology, Springer Science and Business Media LLC, Vol. 101, No. 11 ( 2022-11), p. 2537-2539
    Type of Medium: Online Resource
    ISSN: 0939-5555 , 1432-0584
    URL: Article
    DOI: 10.1007/s00277-022-04933-5
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2022
    detail.hit.zdb_id: 1458429-3
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  • 5
    Online Resource
    Online Resource
    Gray matter atrophy patterns within the cerebellum-neostriatum-cortical network in SCA3
    Ovid Technologies (Wolters Kluwer Health) ; 2020
    In:  Neurology Vol. 95, No. 22 ( 2020-12-01), p. e3036-e3044
    Details
    In: Neurology, Ovid Technologies (Wolters Kluwer Health), Vol. 95, No. 22 ( 2020-12-01), p. e3036-e3044
    Abstract: To investigate the spatial patterns and the probable sequences of gray matter atrophy in spinocerebellar ataxia type 3 (SCA3). Methods A total of 47 patients with SCA3 and 49 age- and sex-matched healthy controls participated in the study. High-resolution T1-weighted MRI were examined in all participants. We used the causal network of structural covariance (CasCN) to identify the sequence of gray matter atrophy patterns. This was achieved by applying Granger causality analysis to a gray matter atrophy staging scheme performed by voxel-based morphometry from the network level. Results Participants in the premanifest stage of the disease showed the presence of focal gray matter atrophy in the vermis. As the disease duration increased, there was progressive gray matter atrophy in the cerebellar, neostriatum, frontal lobe, and parietal lobe. The patients with SCA3 also showed proximal and distal cortical atrophy sequences exerting from the vermis to the regions mainly located in the cerebellum-neostriatum-cortical network. Conclusion Our results, although preliminary in nature, indicate that the gray matter atrophy in SCA3 lies and extends to involve more regions according to distinct anatomical patterns, mainly in the cerebellum-neostriatum-cortical network. These findings advance our understanding on the natural history of structural damage in SCA3, while confirming known clinical features. This could provide unique insight into the ordered sequential process of regional brain atrophy that targets a particular network.
    Type of Medium: Online Resource
    ISSN: 0028-3878 , 1526-632X
    URL: Article
    DOI: 10.1212/WNL.0000000000010986
    RVK:
    XA 10000
    Language: English
    Publisher: Ovid Technologies (Wolters Kluwer Health)
    Publication Date: 2020
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  • 6
    Online Resource
    Online Resource
    Clinical Severity of Gitelman Syndrome Determined by Serum Magnesium
    S. Karger AG ; 2014
    In:  American Journal of Nephrology Vol. 39, No. 4 ( 2014), p. 357-366
    Details
    In: American Journal of Nephrology, S. Karger AG, Vol. 39, No. 4 ( 2014), p. 357-366
    Abstract: 〈 b 〉 〈 i 〉 Background/Aims: 〈 /i 〉 〈 /b 〉 Normomagnesemia is considered atypical in Gitelman syndrome (GS). Here, we describe clinical, pathological and genetic characteristics in Chinese GS patients with or without hypomagnesemia in order to determine whether serum magnesium concentration indicates the severity of the disease. 〈 b 〉 〈 i 〉 Methods: 〈 /i 〉 〈 /b 〉 7 normomagnesemic and 25 hypomagnesemic GS patients who were confirmed by direct sequencing of 〈 i 〉 SLC12A3 〈 /i 〉 gene were included. Clinical manifestation and laboratory tests were documented. Supine and upright plasma renin activity, angiotensin II and aldosterone were determined by radioimmunoassay. Transient receptor potential channel melastatin subtype 6 (TRPM6) was detected by immunohistochemistry in paraffin-embedded renal biopsy sections of 12 GS patients. 14 patients with glomerular minor lesion served as controls. The distribution of the mutations on the predicted NCC protein was analyzed and compared between two subgroups. 〈 b 〉 〈 i 〉 Results: 〈 /i 〉 〈 /b 〉 Clinical manifestations, electrolyte abnormalities, metabolic alkalosis and renin-angiotensin-aldosterone system activation were found to be milder in normomagnesemic compared with the hypomagnesemic group. Compared with glomerular minor lesion controls, the TRPM6-positive area was significantly decreased in hypomagnesemic patients (4.96 ± 1.88 vs. 8.63 ± 2.67%) while it was near normal (7.82 ± 5.23%) in 2 normomagnesemic GS patients. A higher percentage of intracellular mutations was observed in normomagnesemic patients than hypomagnesemic patients (92.31 vs. 56.52%, p = 0.02). 〈 b 〉 〈 i 〉 Conclusions: 〈 /i 〉 〈 /b 〉 Normomagnesemia is not rare in GS. Serum magnesium may indicate the severity of GS.
    Type of Medium: Online Resource
    ISSN: 0250-8095 , 1421-9670
    URL: Article
    DOI: 10.1159/000360773
    Language: English
    Publisher: S. Karger AG
    Publication Date: 2014
    detail.hit.zdb_id: 1468523-1
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  • 7
    Online Resource
    Online Resource
    Glucose tolerance and insulin responsiveness in Gitelman syndrome patients
    Bioscientifica ; 2017
    In:  Endocrine Connections Vol. 6, No. 4 ( 2017-05), p. 243-252
    Details
    In: Endocrine Connections, Bioscientifica, Vol. 6, No. 4 ( 2017-05), p. 243-252
    Abstract: Impaired glucose metabolism and insulin sensitivity have been reported in patients with Gitelman syndrome (GS), but insulin secretion and the related mechanisms are not well understood. Design and methods The serum glucose levels, insulin secretion and insulin sensitivity were evaluated in patients with GS ( n  = 28), patients with type 2 diabetes mellitus (DM) and healthy individuals ( n  = 20 in both groups) using an oral glucose tolerance test. Serum and urine sodium, potassium and creatinine levels were measured at 0, 30, 60, 120 and 180 min after an oral glucose load was administered. Results The areas under the serum glucose curves were higher in the GS patients than those in the healthy controls (17.4 ± 5.1 mmol·h/L vs 14.5 ± 2.8 mmol·h/L, P  = 0.02) but lower than those in the DM patients (24.8 ± 5.3 mmol·h/L, P   〈  0.001). The areas under the serum insulin curves and the insulin secretion indexes in GS patients were higher than those in DM patients and lower than those in healthy subjects. The insulin secretion-sensitivity index of GS patients was between that of healthy subjects and DM patients, but the insulin sensitivity indices were not different among the three groups. After one hour of glucose administration, the serum potassium level significantly decreased from baseline, and the urinary potassium-to-creatinine ratio increased gradually and peaked at 2 h. Conclusions Glucose metabolism and insulin secretion were impaired in GS patients, but insulin sensitivity was comparable between GS patients and patients with type 2 DM. After administration of an oral glucose load, the plasma potassium level decreased in GS patients due to the increased excretion of potassium in the urine.
    Type of Medium: Online Resource
    ISSN: 2049-3614
    URL: Article
    DOI: 10.1530/EC-17-0014
    Language: Unknown
    Publisher: Bioscientifica
    Publication Date: 2017
    detail.hit.zdb_id: 2668428-7
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  • 8
    Online Resource
    Online Resource
    Table_3.docx
    Frontiers Media SA ; 2022
    In:  Frontiers in Aging Neuroscience Vol. 14 ( 2022-4-11)
    Details
    In: Frontiers in Aging Neuroscience, Frontiers Media SA, Vol. 14 ( 2022-4-11)
    Abstract: Spinocerebellar ataxia type 3 (SCA3) is a rare movement disorder characterized with ataxia. Previous studies on movement disorders show that the whole-brain functional network tends to be more regular, and these reconfigurations correlate with genetic and clinical variables. Methods To test whether the brain network in patients with SCA3 follows a similar reconfiguration course to other movement disorders, we recruited 41 patients with SCA3 (mean age = 40.51 ± 12.13 years; 23 male) and 41 age and sex-matched healthy individuals (age = 40.10 ± 11.56 years; 24 male). In both groups, the whole-brain network topology of resting-state functional magnetic resonance imaging (rs-fMRI) was conducted using graph theory, and the relationships among network topologies, cytosine-adenine-guanine (CAG) repeats, clinical symptoms, and functional connectivity were explored in SCA3 patients using partial correlation analysis, controlling for age and sex. Results The brain networks tended to be more regular with a higher clustering coefficient, local efficiency, and modularity in patients with SCA3. Hubs in SCA3 patients were reorganized as the number of hubs increased in motor-related areas and decreased in cognitive areas. At the global level, small-worldness and normalized clustering coefficients were significantly positively correlated with clinical motor symptoms. At the nodal level, the clustering coefficient and local efficiency increased significantly in the visual (bilateral cuneus) and sensorimotor (right cerebellar lobules IV, V, VI) networks and decreased in the cognitive areas (right middle frontal gyrus). The clustering coefficient and local efficiency in the bilateral cuneus gyrus were negatively correlated with clinical motor symptoms. The functional connectivity between right caudate nucleus and bilateral calcarine gyrus were negatively correlated with disease duration, while connectivity between right posterior cingulum gyrus and left cerebellar lobule III, left inferior occipital gyrus and right cerebellar lobule IX was positively correlated. Conclusion Our results demonstrate that a more regular brain network occurred in SCA3 patients, with motor and visual-related regions, such as, cerebellar lobules and cuneus gyrus, both forayed neighbor nodes as “resource predators” to compensate for normal function, with motor and visual function having the higher priority comparing with other high-order functions. This study provides new information about the neurological mechanisms underlying SCA3 network topology impairments in the resting state, which give a potential guideline for future clinical treatments. Clinical Trial Registration [ www.ClinicalTrials.gov ], identifier [ChiCTR1800019901] .
    Type of Medium: Online Resource
    ISSN: 1663-4365
    URL: Article
    URL: Article
    URL: Article
    URL: Article
    DOI: 10.3389/fnagi.2022.773119
    DOI: 10.3389/fnagi.2022.773119.s001
    DOI: 10.3389/fnagi.2022.773119.s002
    DOI: 10.3389/fnagi.2022.773119.s003
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2022
    detail.hit.zdb_id: 2558898-9
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  • 9
    Online Resource
    Online Resource
    MO029CLINICAL  CHARACTERISTICS AND PATHOGENIC GENES OF CONGENITAL SOLITARY KIDNEY WITH REPRODUCTIVE SYSTEM MALFORMATION*
    Oxford University Press (OUP) ; 2021
    In:  Nephrology Dialysis Transplantation Vol. 36, No. Supplement_1 ( 2021-05-29)
    Details
    In: Nephrology Dialysis Transplantation, Oxford University Press (OUP), Vol. 36, No. Supplement_1 ( 2021-05-29)
    Abstract: Congenital solitary kidney – one category of congenital anomalies of the kidney and urinary tract (CAKUT) may combine with other system malformations such as reproductive, cardiac, skeletal system, and so on. Our study analysed the clinical characteristics among congenital solitary kidney patients and their reproductive system malformations. And further work about probable pathogenic genes was explored. Method The information of CAKUT patients who were indicated by Doppler ultrasound was collected. The clinical and imaging features including reproductive system abnormalities were retrospectively reviewed in patients with congenital solitary kidney. In patients with Mayer-Rokitansky- Küster-Hauser (MRKH) syndrome, a disorder of congenital agenesis of uterus and vagina, whole exome sequencing was performed. Rare variants in CAKUT-related genes were analysed. Trio analysis was conducted to identify de novo mutations. Results We identified 209 patients with congenital solitary kidney from July 20, 2017 to July 19, 2020 among 1160 CAKUT patients in Peking Union Medical College Hospital. There were 152 females. The average age of congenital solitary kidney patients was 35.26±18.42 years when they were diagnosed. 53.2% showed different degrees of proteinuria and hematuria. Serum creatinine elevating was proved 13.1% and 40% in women and men separately. Among 81 females who also had a gynecological ultrasound report, 88.9% combined with genital malformation, oblique vaginal septum syndrome 48.7%, the Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome 22.2%, malformed uterus 23.5%, vaginal atresia and other genital malformation 7%. Congenital heart disease, complete transposition of viscera, and scoliosis were also found in some patients with congenital solitary kidney. Furthermore, based on the whole exome data of 443 patients with MRKH syndrome, seven function-lost mutations were confirmed. And also two De nove mutations (NOTCH2 (NM_024408.3: c.703A & gt;T(p.Thr235Ser), ESRRG (NM_001243512.1:c.-169-8delT)), one homozygous patients with parents heterozygous (NM_133433.3:c.8084C & gt;T(p.Thr2695Me) were identified as possible pathogenic genes caused CAKUT. Conclusion We should be aware of reproductive system malformations in CAKUT patients. Whole exome sequencing may suggest common pathogenic genes between the two kinds of diseases.
    Type of Medium: Online Resource
    ISSN: 0931-0509 , 1460-2385
    URL: Article
    DOI: 10.1093/ndt/gfab080.001
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2021
    detail.hit.zdb_id: 1465709-0
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  • 10
    Online Resource
    Online Resource
    MP035FREQUENT MUTATIONS OF CHINESE GITELMAN SYNDROME PATIENTS: FROM GENOTYPE TO CLINICAL AND PATHOLOGICAL FEATURES
    Oxford University Press (OUP) ; 2016
    In:  Nephrology Dialysis Transplantation Vol. 31, No. suppl_1 ( 2016-05), p. i354-i354
    Details
    In: Nephrology Dialysis Transplantation, Oxford University Press (OUP), Vol. 31, No. suppl_1 ( 2016-05), p. i354-i354
    Type of Medium: Online Resource
    ISSN: 1460-2385 , 0931-0509
    URL: Article
    DOI: 10.1093/ndt/gfw182.05
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2016
    detail.hit.zdb_id: 1465709-0
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