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  • 1
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    Online Resource
    Abstracts of the 52nd Workshop for Pediatric Research : Frankfurt, Germany. 27-28 October 2016
    Springer Science and Business Media LLC ; 2017
    In:  Molecular and Cellular Pediatrics Vol. 4, No. S1 ( 2017-5)
    Details
    In: Molecular and Cellular Pediatrics, Springer Science and Business Media LLC, Vol. 4, No. S1 ( 2017-5)
    Type of Medium: Online Resource
    ISSN: 2194-7791
    URL: Article
    DOI: 10.1186/s40348-017-0071-0
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2017
    detail.hit.zdb_id: 2785551-X
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  • 2
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    Lipoprotein‐associated phospholipase A2 activity in obese adolescents with and without type 2 diabetes
    Wiley ; 2018
    In:  Journal of Inherited Metabolic Disease Vol. 41, No. 1 ( 2018-01), p. 73-79
    Details
    In: Journal of Inherited Metabolic Disease, Wiley, Vol. 41, No. 1 ( 2018-01), p. 73-79
    Abstract: Lipoprotein‐associated phospholipase A2 (Lp‐PLA2) was identified as a strong predictor for cardiovascular events. Furthermore, it is highly associated with obesity. The role of Lp‐PLA2 in diabetes mellitus is controversial and analyses, especially in adolescents with type 2 diabetes (T2D), are missing. Therefore, we compared Lp‐PLA2 activity between two obese age‐, sex‐, and BMI‐matched cohorts of adolescents with and without T2D. Relationships between Lp‐PLA2 activity and age, BMI, hemoglobin A1c, lipids, and adipokines were evaluated. Lp‐PLA2 activity was analyzed in serum of 72 obese adolescents without T2D (mean age 15.2 ± 1.6 years) and in 65 obese adolescents with T2D (mean age 15.5 ± 1.8 years). Clinical data were obtained from the Diabetes‐Patienten‐Verlaufsdokumentation (DPV) registry. Surprisingly, obese adolescents with T2D had lower levels of Lp‐PLA2 activity than obese children without T2D (160.2 ± 45.0 versus 180.9 ± 35.6 nmol/min/ml, p = 0.003), but this decrease could only be detected in male (158.8 ± 45.3 versus 190.8 ± 31.3 nmol/min/ml, p 〈 0.001) and not in female adolescents (162.1 ± 45.5 versus 167.7 ± 37.1 nmol/min/ml, p = 0.60). In multiple linear regression analysis, differences in Lp‐PLA2 activity between cohorts remained large and significant (ß‐coefficient: −31.60, 95% confidence interval [−49.27;−13.93], p 〈 0.001). Furthermore, Lp‐PLA2 activity was positively associated with BMI (ß‐coefficient: 2.04 [0.68;3.40], p = 0.004) and negatively associated with the adipokines leptin (ß‐coefficient: −0.53 [−0.89;−0.17], p = 0.004) and adiponectin (ß‐coefficient: −3.06, [−5.63;−0.48] , p = 0.02). Elevated mean glucose concentrations in adolescents with T2D were not associated with an increase but with a decrease of Lp‐PLA2 activity. Hence, in young patients with T2D the Lp‐PLA2 activity as a risk predictor for cardiovascular events needs further investigation.
    Type of Medium: Online Resource
    ISSN: 0141-8955 , 1573-2665
    URL: Article
    DOI: 10.1007/s10545-017-0100-0
    RVK:
    YC 6270
    Language: English
    Publisher: Wiley
    Publication Date: 2018
    detail.hit.zdb_id: 2006875-X
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  • 3
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    Online Resource
    Relationship of Serum Fetuin A with Metabolic and Clinical Parameters in German Children and Adolescents with Type 1 Diabetes
    S. Karger AG ; 2018
    In:  Hormone Research in Paediatrics Vol. 89, No. 2 ( 2018), p. 73-81
    Details
    In: Hormone Research in Paediatrics, S. Karger AG, Vol. 89, No. 2 ( 2018), p. 73-81
    Abstract: 〈 b 〉 〈 i 〉 Background and Aim: 〈 /i 〉 〈 /b 〉 The hepatokine fetuin A is upregulated in the metabolic syndrome and in type 2 diabetes (T2D), while its role in adolescent type 1 diabetes (T1D) is unclear. We assessed the relationship between circulating fetuin A levels and metabolic control, comorbidities, and complications in adolescent T1D patients. 〈 b 〉 〈 i 〉 Methods: 〈 /i 〉 〈 /b 〉 We studied the relationship between serum fetuin A and clinical diabetes-related data from the DPV registry (Diabetes-Pa­tienten-Verlaufsdokumentation) in 172 adolescent T1D patients with early-onset ( & #x3c;5 years) long-standing ( & #x3e;10 years) T1D. Fetuin A levels were further compared between adolescent T1D and T2D patients. 〈 b 〉 〈 i 〉 Results: 〈 /i 〉 〈 /b 〉 Serum fetuin A levels in T1D patients (mean 0.267 ± 0.043 g/L) did not correlate with age, diabetes duration, gender, body mass index (BMI), glycated hemoglobin, serum lipid levels, blood pressure, celiac or thyroid disease, nephropathy, or retinopathy. An association of fetuin A levels with insulin requirements was only evident within the subgroup of overweight T1D patients ( 〈 i 〉 r 〈 /i 〉 〈 sub 〉 s 〈 /sub 〉 = 0.439, 〈 i 〉 p 〈 /i 〉 = 0.028, 〈 i 〉 n 〈 /i 〉 = 25, BMI & #x3e;90th percentile), disappearing after adjustment for multiple testing. Adolescent T1D patients showed distinctly lower fetuin A levels than patients with T2D ( 〈 i 〉 p 〈 /i 〉 ≤ 0.001). 〈 b 〉 〈 i 〉 Conclusion: 〈 /i 〉 〈 /b 〉 Overall, we did not observe a clinically relevant association of fetuin A levels with surrogate parameters for insulin sensitivity in our juvenile T1D cohort. A correlation with insulin requirements was detectable in overweight patients only. We hypothesize that multiple factors, such as obesity, puberty, inadequate metabolic control, and hepatic steatosis, have to add up before a clinically relevant effect of fetuin A on insulin sensitivity becomes evident.
    Type of Medium: Online Resource
    ISSN: 1663-2818 , 1663-2826
    URL: Article
    DOI: 10.1159/000484896
    Language: English
    Publisher: S. Karger AG
    Publication Date: 2018
    detail.hit.zdb_id: 2540224-9
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  • 4
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    Online Resource
    Recommendation for standardized medical care for children and adolescents with long COVID : Statement of a multidisciplinary pediatric expert group of the DGKJ convent societies (status: February 2022)
    Springer Science and Business Media LLC ; 2022
    In:  Monatsschrift Kinderheilkunde Vol. 170, No. 6 ( 2022-06), p. 539-547
    Details
    In: Monatsschrift Kinderheilkunde, Springer Science and Business Media LLC, Vol. 170, No. 6 ( 2022-06), p. 539-547
    Abstract: This current consensus paper for long COVID complements the existing AWMF S1 guidelines for long COVID with a detailed overview on the various clinical aspects of long COVID in children and adolescents. Members of 19 different pediatric societies of the DGKJ convent and collaborating societies together provide expert-based recommendations for the clinical management of long COVID based on the currently available but limited academic evidence for long COVID in children and adolescents. It contains screening questions for long COVID and suggestions for a structured, standardized pediatric medical history and diagnostic evaluation for patients with suspected long COVID. A time and resource-saving questionnaire, which takes the clinical complexity of long COVID into account, is offered via the DGKJ and DGPI websites as well as additional questionnaires suggested for an advanced screening of specific neurocognitive and/or psychiatric symptoms including post-exertional malaise (PEM) and myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). According to the individual medical history as well as clinical signs and symptoms a step by step diagnostic procedure and a multidisciplinary therapeutic approach are recommended.
    Type of Medium: Online Resource
    ISSN: 0026-9298 , 1433-0474
    URL: Article
    DOI: 10.1007/s00112-021-01408-1
    RVK:
    XA 10000
    Language: German
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2022
    detail.hit.zdb_id: 1462918-5
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  • 5
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    Dual-source computed tomography of the lung with spectral shaping and advanced iterative reconstruction: potential for maximum radiation dose reduction
    Springer Science and Business Media LLC ; 2020
    In:  Pediatric Radiology Vol. 50, No. 9 ( 2020-08), p. 1240-1248
    Details
    In: Pediatric Radiology, Springer Science and Business Media LLC, Vol. 50, No. 9 ( 2020-08), p. 1240-1248
    Abstract: Radiation dose at CT should be as low as possible without compromising diagnostic quality. Objective To assess the potential for maximum dose reduction of pediatric lung dual-source CT with spectral shaping and advanced iterative reconstruction (ADMIRE). Materials and methods We retrospectively analyzed dual-source CT acquisitions in a full-dose group (FD: 100 kV, 64 reference mAs) and in three groups with spectral shaping and differing reference mAs values (Sn: 100 kV, 96/64/32 reference mAs), each group consisting of 16 patients (age mean 11.5 years, standard deviation 4.8 years, median 12.8 years, range 1.3–18 years). Advanced iterative reconstruction of images was performed with different strengths (FD: ADMIRE Level 2; Sn: ADMIRE Levels 2, 3 and 4). We analyzed dose parameters and measured noise. Diagnostic confidence and detectability of lung lesions as well as anatomical structures were assessed using a Likert scale (from 1 [unacceptable] to 4 [fully acceptable] ). Results Compared to full dose, effective dose was reduced to 16.7% in the Sn 96 group, 11.1% in Sn64, and 5.5% in Sn32 ( P 〈 0.001). Noise values of Sn64 ADM4 did not statistically differ from those in FD ADM2 (45.7 vs. 38.9 Hounsfield units [HU]; P =0.132), whereas noise was significantly higher in Sn32 ADM4 compared to Sn64 ADM4 (61.5 HU; P 〈 0.001). A Likert score 〉 3 was reached in Sn64 ADM4 regarding diagnostic confidence (3.2) and detectability of lung lesions (3.3). For detectability of most anatomical structures, no significant differences were found between FD AM2 and Sn64 ADM4 ( P ≥0.05). Conclusion In pediatric lung dual-source CT, spectral shaping together with ADMIRE 4 enable radiation dose reduction to about 10% of a full-dose protocol while maintaining an acceptable diagnostic quality.
    Type of Medium: Online Resource
    ISSN: 0301-0449 , 1432-1998
    URL: Article
    DOI: 10.1007/s00247-020-04714-0
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2020
    detail.hit.zdb_id: 1463007-2
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  • 6
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    Sleep-Disordered Breathing in Children with Prader-Willi Syndrome in Relation to Growth Hormone Therapy Onset
    S. Karger AG ; 2020
    In:  Hormone Research in Paediatrics Vol. 93, No. 2 ( 2020), p. 85-93
    Details
    In: Hormone Research in Paediatrics, S. Karger AG, Vol. 93, No. 2 ( 2020), p. 85-93
    Abstract: 〈 b 〉 〈 i 〉 Objective: 〈 /i 〉 〈 /b 〉 The aim of this study was to consider sleep apnea in Prader-Willi syndrome (PWS) children depending on age at growth hormone (GH) therapy onset. 〈 b 〉 〈 i 〉 Study Design: 〈 /i 〉 〈 /b 〉 We analyzed longitudinally cardiorespiratory polygraphy of 62 PWS children (aged 0–2.5 years at baseline). Twenty-one children (Group A) started GH-therapy during and 41 children (Group B) after their first year of life. Data were acquired before, at 3 and 6 months, then 1.2, 2.2, and 3.2 years after GH onset. Outcomes were determined with the obstructive apnea hypopnea index (OAHI), central apnea index (CAI), oxygen desaturation index (ODI), and by measuring obstructive sleep apnea (OSA) and peripheral blood oxygen saturation (SpO 〈 sub 〉 2 〈 /sub 〉 ). 〈 b 〉 〈 i 〉 Results: 〈 /i 〉 〈 /b 〉 We observed no significant differences in OAHI, CAI, ODI, and SpO 〈 sub 〉 2 〈 /sub 〉 depending on treatment onset. At baseline, 5/21 patients (23.8%) in Group A versus 15/41 patients (36.6%) in Group B showed pathological sleep apnea (OAHI ≥1.5). Pathological OSA increased significantly in Group A during the first 3 months of therapy but dropped below baseline after 1 year in both groups. ODI changed during GH therapy in both groups (from 4.0 to 2.6 in Group A, and 3.6 to 1.6 in Group B; baseline to 3.2 years; 〈 i 〉 p 〈 /i 〉 & #x3c; 0.05). 〈 b 〉 〈 i 〉 Conclusions: 〈 /i 〉 〈 /b 〉 OSA in PWS children appears to develop independently of treatment onset. Treatment may therefore safely be initiated early but should be accompanied by regular sleep analysis.
    Type of Medium: Online Resource
    ISSN: 1663-2818 , 1663-2826
    URL: Article
    DOI: 10.1159/000506943
    Language: English
    Publisher: S. Karger AG
    Publication Date: 2020
    detail.hit.zdb_id: 2540224-9
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  • 7
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    〈b〉〈i〉MKRN3〈/i〉〈/b〉 Mutations in Familial Central Precocious Puberty
    S. Karger AG ; 2014
    In:  Hormone Research in Paediatrics Vol. 82, No. 2 ( 2014), p. 122-126
    Details
    In: Hormone Research in Paediatrics, S. Karger AG, Vol. 82, No. 2 ( 2014), p. 122-126
    Abstract: Loss-of-function mutations in the gene encoding the makorin RING finger protein 3 (MKRN3) have recently been reported to underlie familial cases of central precocious puberty (CPP). The imprinted 〈 i 〉 MKRN3 〈 /i 〉 gene is expressed only from the paternal allele, and mutations inherited from the father affect boys and girls equally, which is in contrast to the known female preponderance in idiopathic CPP. By screening a series of 6 families and 1 male patient with idiopathic CPP, we identified 2 further families carrying loss-of-function mutations in 〈 i 〉 MKRN3 〈 /i 〉 , the previously reported variant c.475_476insC (p.Ala162Glyfs*14) and a novel one, c.331G 〉 T (p.Glu111*). We conclude that 〈 i 〉 MKRN3 〈 /i 〉 mutations appear to be a frequent cause of familial CPP and, considering the imprinted mode of inheritance, may also account for a certain proportion of isolated CPP cases. Remarkably, four out of six 〈 i 〉 MKRN3 〈 /i 〉 mutations described so far encode either a stop codon or a frameshift followed by a premature stop codon. Consequently, there may be less severe mutations that possibly associate with more subtle phenotypes, which could even explain variation within the physiological range. Mutation screening in larger cohorts is necessary in order to estimate the real prevalence of 〈 i 〉 MKRN3 〈 /i 〉 mutations in idiopathic CPP.
    Type of Medium: Online Resource
    ISSN: 1663-2818 , 1663-2826
    URL: Article
    DOI: 10.1159/000362815
    Language: English
    Publisher: S. Karger AG
    Publication Date: 2014
    detail.hit.zdb_id: 2540224-9
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  • 8
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    Increased Steroid Excretion in Children with Extremely Low Birth Weight at a Median Age of 9.8 years
    S. Karger AG ; 2015
    In:  Hormone Research in Paediatrics Vol. 84, No. 5 ( 2015), p. 331-337
    Details
    In: Hormone Research in Paediatrics, S. Karger AG, Vol. 84, No. 5 ( 2015), p. 331-337
    Abstract: 〈 b 〉 〈 i 〉 Background: 〈 /i 〉 〈 /b 〉 Events during foetal or early extrauterine life may affect bodily structure and/or functions and even pave the way for adult diseases. 〈 b 〉 〈 i 〉 Aims: 〈 /i 〉 〈 /b 〉 To find whether extremely low birth weight (ELBW) infants differ from healthy controls regarding the excretion of steroid metabolites. 〈 b 〉 〈 i 〉 Methods: 〈 /i 〉 〈 /b 〉 The study compared 17 female and 10 male ELBW infants, all prepubertal, aged 8-11 years, birth weight 〈 1,000 g, with 27 age- and sex-matched controls. All were healthy at the time of the study. Height, weight and BMI did not differ between the groups. Results were adjusted according to body surface area. 36 urinary steroid metabolites were quantified by gas chromatography-mass spectrometry. 〈 b 〉 〈 i 〉 Results: 〈 /i 〉 〈 /b 〉 In the ELBW girls 33/36 steroid metabolites were higher (19 significantly) than in the controls. All 36 steroid metabolites were higher in the ELBW boys (9 significantly) than in the controls. Sums of mineralocorticoid precursors, metabolites descriptive for cortisol and parameters of adrenal androgen production were significantly higher in ELBW infants (both sexes). Only the sum of the metabolites known to be illustrative for adrenal 11β-hydroxysteroid dehydrogenase activity was not different. 〈 b 〉 〈 i 〉 Conclusion: 〈 /i 〉 〈 /b 〉 Prepubertal ELBW children have an augmented urinary excretion of adrenal androgens, cortisol and mineralocorticoid precursors. These findings corroborate and help to explain the link between early-life adversity and subsequent adrenocortical function.
    Type of Medium: Online Resource
    ISSN: 1663-2818 , 1663-2826
    URL: Article
    DOI: 10.1159/000441031
    Language: English
    Publisher: S. Karger AG
    Publication Date: 2015
    detail.hit.zdb_id: 2540224-9
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  • 9
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    Pubertal Timing and Growth Dynamics in Children With Severe Primary IGF-1 Deficiency: Results From the European Increlex® Growth Forum Database Registry
    Frontiers Media SA ; 2022
    In:  Frontiers in Endocrinology Vol. 13 ( 2022-2-18)
    Details
    In: Frontiers in Endocrinology, Frontiers Media SA, Vol. 13 ( 2022-2-18)
    Abstract: Puberty is delayed in untreated children and adolescents with severe primary IGF-1 deficiency (SPIGFD); to date, it has not been reported whether recombinant human insulin-like growth factor-1 mecasermin (rhIGF-1) treatment affects this. Pubertal growth outcomes were extracted from the European Increlex ® Growth Forum Database (Eu-IGFD) Registry (NCT00903110). Methods The Eu-IGFD Registry includes children and adolescents aged 2 to 18 years with growth failure associated with SPIGFD who are treated with rhIGF-1. Reported outcomes include: age at last registration of Tanner stage 1 and first registration of Tanner stage 2-5 (T2-T5; based on breast development for girls and genital development for boys, respectively); maximum height velocity during each Tanner stage; and pubertal peak height velocity (PPHV). Data cut-off was 13 May 2019. Results This analysis included 213 patients (132 boys and 81 girls). Mean (SD) age at last registration of T1 and first registration of T5 was 13.0 (2.0) and 16.3 (1.6) years, respectively, in boys and 11.6 (1.8) and 14.7 (1.5) years, respectively, in girls. Among patients reaching the end of puberty (25 boys and 11 girls), mean (SD) height SDS increased from -3.7 (1.4) at baseline in the Eu-IGFD Registry to -2.6 (1.4) at T5 in boys and from -3.1 (1.1) to -2.3 (1.5) in girls. Maximum height velocity was observed during T2 in girls and T3 in boys. Median (range) PPHV was 8.0 (0.3–13.0) cm/year in boys and 6.8 (1.3–9.6) cm/year in girls and occurred most frequently during T2. Overall, the adverse events seen in this analysis were in line with the known safety profile of rhIGF-1. Conclusion Children and adolescents treated with rhIGF-1 for SPIGFD with growth failure experienced an increase in height SDS in prepubertal years compared with baseline. Despite 1.5 years delay in pubertal start and a delayed and slightly lower PPHV, height SDS gain during puberty was maintained.
    Type of Medium: Online Resource
    ISSN: 1664-2392
    URL: Article
    DOI: 10.3389/fendo.2022.812568
    Language: Unknown
    Publisher: Frontiers Media SA
    Publication Date: 2022
    detail.hit.zdb_id: 2592084-4
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  • 10
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    Potential for Radiation Dose Reduction in Dual-Source Computed Tomography of the Lung in the Pediatric and Adolescent Population Compared to Digital Radiography
    MDPI AG ; 2021
    In:  Diagnostics Vol. 11, No. 2 ( 2021-02-10), p. 270-
    Details
    In: Diagnostics, MDPI AG, Vol. 11, No. 2 ( 2021-02-10), p. 270-
    Abstract: Low-dose dual-source computed tomography (DSCT) protocols for the evaluation of lung diseases in children and adolescents are of importance since this age group is particularly prone to radiation damage. The aim of this study was to evaluate image quality of low-dose DSCT of the lung and to assess the potential of radiation dose reduction compared to digital radiographs (DR). Three groups, each consisting of 19 patients, were examined with different DSCT protocols using tin prefiltration (Sn96/64/32 ref. mAs at 100 kV). Different strengths of iterative reconstruction were applied (ADMIRE 2/3/4). DSCT groups were compared to 19 matched patients examined with posterior–anterior DR. Diagnostic confidence, detectability of anatomical structures and small lung lesions were evaluated on a 4-point Likert scale (LS 1 = unacceptable, 4 = fully acceptable; a value ≥ 3 was considered acceptable). Effective dose (ED) was 31-/21-/9-fold higher in Sn96/Sn64/Sn32 compared to DR. Diagnostic confidence was sufficient in Sn96/Sn64 (LS 3.4/3.2), reduced in Sn32 (LS 2.7) and the worst in DR (LS 2.4). In DSCT, detectability of small anatomical structures was always superior to DR (p 〈 0.05). Mean lesion size ranged from 5.1–7 mm; detectability was acceptable in all DSCT groups (LS 3.0–3.4) and superior to DR (LS 1.9; p 〈 0.05). Substantial dose lowering in DSCT of the pediatric lung enables acceptable detectability of small lung lesions with a radiation dose being about 10-fold higher compared to DR.
    Type of Medium: Online Resource
    ISSN: 2075-4418
    URL: Article
    DOI: 10.3390/diagnostics11020270
    Language: English
    Publisher: MDPI AG
    Publication Date: 2021
    detail.hit.zdb_id: 2662336-5
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