cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.5121628
title: Supplementary Material for: Disorder-Specific Cognitive-Behavioral Therapy for Separation Anxiety Disorder in Young Children: A Randomized Waiting-List-Controlled Trial

description: <i>Background:</i> Separation anxiety disorder (SAD) is one of the earliest and most common mental disorders in childhood, and a strong predictor of adult psychopathology. Despite significant progress in psychotherapy research on childhood anxiety disorders, no randomized controlled trial has been conducted with a disorder-specific treatment program for young children suffering from SAD. <i>Methods:</i> Forty-three children (ages 5–7) with SAD and their parents were assigned to either a 16-session disorder-specific SAD treatment program including parent training and classical cognitive-behavioral therapy (CBT) components, or to a 12-week waiting list group. Categorical and/or continuous data for anxiety, impairment/distress and quality of life were collected at baseline, after treatment/waiting list condition, and at a 4-week follow-up. <i>Results:</i> Intention-to-treat analyses indicate that 76.19% of children allocated to the treatment group definitively no longer fulfilled DSM-IV criteria for SAD at follow-up, compared to 13.64% in the waiting list group. Between 91 and 100% of children rated themselves or were rated by their father, mother or therapist as very much or much improved on the global success rating immediately after treatment. Results indicated large time by treatment condition interaction effect sizes (d = 0.98–1.41) across informants for reduction of distress/avoidance in separation situations after the test for the treatment condition. Further, parents reported significant improvements in impairment/distress in the child’s major life domains and the child’s quality of life. Treatment gains were maintained at the 4-week follow-up assessment. <i>Conclusions:</i> Results indicate the short-term efficacy of a disorder-specific treatment approach for SAD, and are among the first to indicate that CBT programs work with young children.
DOI: 10.6084/m9.figshare.5121631.v1
title: Supplementary Material for: The Progressive Ankylosis Protein Regulates Cementum Apposition and Extracellular Matrix Composition

description: <i>Background/Aims:</i> Tooth root cementum is sensitive to modulation of inorganic pyrophosphate (PP<sub>i</sub>), an inhibitor of hydroxyapatite precipitation. Factors increasing PP<sub>i</sub> include progressive ankylosis protein (ANK) and ectonucleotide pyrophosphatase/phosphodiesterase 1 (NPP1) while tissue nonspecific alkaline phosphatase hydrolyzes PP<sub>i</sub>. Studies here aimed to define the role of ANK in root and cementum by analyzing tooth development in <i>Ank</i> knock-out (KO) mice versus wild type. <i>Materials and Methods:</i> Periodontal development in KO versus control mice was analyzed by histology, histomorphometry, immunohistochemistry, in situ hybridization, electron microscopy, and nanoindentation. Cementoblast cultures were used in vitro to provide mechanistic underpinnings for PP<sub>i</sub> modulation of cell function. <i>Results:</i> Over the course of root development, <i>Ank</i> KO cervical cementum became 8- to 12-fold thicker than control cervical cementum. Periodontal ligament width was maintained and other dentoalveolar tissues, including apical cementum, were unaltered. Cervical cementum uncharacteristically included numerous cells, from rapid cementogenesis. <i>Ank </i>KO increased osteopontin and dentin matrix protein 1 gene and protein expression, and markedly increased NPP1 protein expression in cementoblasts but not in other cell types. Conditional ablation of <i>Ank</i> in joints and periodontia confirmed a local role for ANK in cementogenesis. In vitro studies employing cementoblasts indicated that <i>Ank</i> and <i>Enpp1</i> mRNA levels increased in step with mineral nodule formation, supporting a role for these factors in regulation of cementum matrix mineralization. <i>Conclusion: </i>ANK, by modulating local PP<sub>i</sub>, controls cervical cementum apposition and extracellular matrix. Loss of ANK created a local environment conducive to rapid cementogenesis; therefore, approaches modulating PP<sub>i</sub> in periodontal tissues have potential to promote cementum regeneration.
DOI: 10.6084/m9.figshare.5121616
title: Supplementary Material for: Effectiveness of Unilateral Pallidotomy for Meige Syndrome Confirmed by Motion Analysis

description: <i>Background:</i> We report the case of a 64-year-old woman with bilateral manifestation of Meige syndrome (MS) successfully treated with left-side unilateral ventroposterolateral pallidotomy. <i>Methods:</i> Symptoms were evaluated according to the Burke-Fahn-Marsden dystonia rating scale. Head tremor, blepharospasm and orofacial dyskinesia were measured with an infrared, video-based, computerized, real-time passive marker-based analyzer of motions (RTPAM). <i>Results:</i> The Burke-Fahn-Marsden score showed a 90.2% reduction (from 25.5 to 2.5) at 6 months, and an 88.2% long-lasting benefit (to 3.0) at the 3-year follow-up with good bilateral control of the blepharospasm and orofacial movements. The RTPAM showed a substantial regression of acceleration for all markers, and abolishment of the 4.8-Hz head tremor. The correlation between symmetrical markers, and between markers within the right and left sides, was significantly decreased. <i>Conclusions:</i> Pallidotomy with staged procedure is recommended for the treatment of MS in patients on whom deep brain stimulation could not be performed. In case of good bilateral benefits from the unilateral procedure, contralateral surgery is not needed. The RTPAM is a useful tool for the mapping of facial involuntary movements.
DOI: 10.6084/m9.figshare.5121619
title: Supplementary Material for: Interdisciplinary Evidence-Based Recommendations for the Follow-Up of Testicular Germ Cell Cancer Patients

description: Over the last years, clear treatment recommendations for patients with testicular cancer have been published. This has led to significant improvements in outcome and survival. Moreover, active surveillance has become a cornerstone in the management of clinical stage I seminomatous and non-seminomatous germ cell tumors. On the other hand, the existing recommendations for the follow-up of testis cancer patients are unclear and differ widely. Follow-up recommendations in this young patient population have to be as evidence based as possible, feasible in order to ensure adherence, and must not be harmful. Therefore, attention has to be paid to the negative impact of unnecessary radiation exposure. Recently, new evidence became available regarding the relapse pattern of different disease stages of testicular cancer, the use of imaging at follow-up, and the risks of excessive radiation due to imaging, and in particular that of computed tomography (CT) scans. This article summarizes the recommendations for follow-up of testicular cancer patients of an interdisciplinary multinational working group consisting of urologists, medical oncologists, and radiation oncologists.
DOI: 10.6084/m9.figshare.5121622
title: Supplementary Material for: Carboplatin Dosing in Children: Calculation by Different Formulae

description: Background: Carboplatin dosing in children is based on renal function and there exists a wealth of formulae available for calculating the body surface area (BSA), the glomerular filtration rate (GFR), and the carboplatin dose. Patients and Methods: A fictitious group of children with different ages and body builds was ‘constructed’. For comparison of formulae, bias and precision were assessed. Results: BSA calculations according to DuBois-DuBois, Gehan-George, Mosteller, and Boyd showed good agreement. GFR calculations according to the weight-based Cole formula and the Léger formula gave comparable results. Regarding GFR in young children, the weight-and creatinine-based Cole and the Schwartz formula showed clear differences. Again, carboplatin dose calculations according to Marina, Newell, and Chatelut are comparable. Moreover, the precision of the creatinine measurement has a clear influence on the result of the dose calculation. Conclusions: The choice of the GFR formula is more important for the carboplatin dose calculation compared to the BSA or dose equation. GFR calculations in children show marked, age-dependent variations. A sequence of multiple calculation steps (especially for the Schwartz and Marina formulae) may lead to considerable uncertainty and proneness to error in the clinical routine. In high-risk patients, GFR should be measured precisely and complemented by therapeutic drug monitoring.
DOI: 10.6084/m9.figshare.5121625
title: Supplementary Material for: Screening for New Biomarkers for Subcortical Vascular Dementia and Alzheimer’s Disease

description: <i>Background:</i> Novel biomarkers are important for identifying as well as differentiating subcortical vascular dementia (SVD) and Alzheimer’s disease (AD) at an early stage in the disease process. <i>Methods:</i> In two independent cohorts, a multiplex immunoassay was utilized to analyze 90 proteins in cerebrospinal fluid (CSF) samples from dementia patients and patients at risk of developing dementia (mild cognitive impairment). <i>Results:</i> The levels of several CSF proteins were increased in SVD and its incipient state, and in moderate-to-severe AD compared with the control group. In contrast, some CSF proteins were altered in AD, but not in SVD. The levels of heart-type fatty acid binding protein (H-FABP) were consistently increased in all groups with dementia but only in some of their incipient states. <i>Conclusions:</i> In summary, these results support the notion that SVD and AD are driven by different pathophysiological mechanisms reflected in the CSF protein profile and that H-FABP in CSF is a general marker of neurodegeneration.
DOI: 10.6084/m9.figshare.5121616.v1
title: Supplementary Material for: Effectiveness of Unilateral Pallidotomy for Meige Syndrome Confirmed by Motion Analysis

description: <i>Background:</i> We report the case of a 64-year-old woman with bilateral manifestation of Meige syndrome (MS) successfully treated with left-side unilateral ventroposterolateral pallidotomy. <i>Methods:</i> Symptoms were evaluated according to the Burke-Fahn-Marsden dystonia rating scale. Head tremor, blepharospasm and orofacial dyskinesia were measured with an infrared, video-based, computerized, real-time passive marker-based analyzer of motions (RTPAM). <i>Results:</i> The Burke-Fahn-Marsden score showed a 90.2% reduction (from 25.5 to 2.5) at 6 months, and an 88.2% long-lasting benefit (to 3.0) at the 3-year follow-up with good bilateral control of the blepharospasm and orofacial movements. The RTPAM showed a substantial regression of acceleration for all markers, and abolishment of the 4.8-Hz head tremor. The correlation between symmetrical markers, and between markers within the right and left sides, was significantly decreased. <i>Conclusions:</i> Pallidotomy with staged procedure is recommended for the treatment of MS in patients on whom deep brain stimulation could not be performed. In case of good bilateral benefits from the unilateral procedure, contralateral surgery is not needed. The RTPAM is a useful tool for the mapping of facial involuntary movements.
DOI: 10.6084/m9.figshare.5121619.v1
title: Supplementary Material for: Interdisciplinary Evidence-Based Recommendations for the Follow-Up of Testicular Germ Cell Cancer Patients

description: Over the last years, clear treatment recommendations for patients with testicular cancer have been published. This has led to significant improvements in outcome and survival. Moreover, active surveillance has become a cornerstone in the management of clinical stage I seminomatous and non-seminomatous germ cell tumors. On the other hand, the existing recommendations for the follow-up of testis cancer patients are unclear and differ widely. Follow-up recommendations in this young patient population have to be as evidence based as possible, feasible in order to ensure adherence, and must not be harmful. Therefore, attention has to be paid to the negative impact of unnecessary radiation exposure. Recently, new evidence became available regarding the relapse pattern of different disease stages of testicular cancer, the use of imaging at follow-up, and the risks of excessive radiation due to imaging, and in particular that of computed tomography (CT) scans. This article summarizes the recommendations for follow-up of testicular cancer patients of an interdisciplinary multinational working group consisting of urologists, medical oncologists, and radiation oncologists.
DOI: 10.6084/m9.figshare.5121625.v1
title: Supplementary Material for: Screening for New Biomarkers for Subcortical Vascular Dementia and Alzheimer’s Disease

description: <i>Background:</i> Novel biomarkers are important for identifying as well as differentiating subcortical vascular dementia (SVD) and Alzheimer’s disease (AD) at an early stage in the disease process. <i>Methods:</i> In two independent cohorts, a multiplex immunoassay was utilized to analyze 90 proteins in cerebrospinal fluid (CSF) samples from dementia patients and patients at risk of developing dementia (mild cognitive impairment). <i>Results:</i> The levels of several CSF proteins were increased in SVD and its incipient state, and in moderate-to-severe AD compared with the control group. In contrast, some CSF proteins were altered in AD, but not in SVD. The levels of heart-type fatty acid binding protein (H-FABP) were consistently increased in all groups with dementia but only in some of their incipient states. <i>Conclusions:</i> In summary, these results support the notion that SVD and AD are driven by different pathophysiological mechanisms reflected in the CSF protein profile and that H-FABP in CSF is a general marker of neurodegeneration.
DOI: 10.6084/m9.figshare.5121610
title: Supplementary Material for: An Exploratory Study on the <i>CHRNA3-CHRNA5-CHRNB4 </i>Cluster, Smoking, and Parkinson’s Disease

description: <i>Background:</i> Smokers have a lower risk of Parkinson’s disease (PD). Recent genome-wide association studies (GWAS) have consistently linked several single nucleotide polymorphisms (SNPs) in the <i>CHRNA3-CHRNA5-CHRNB4</i> cluster on chromosome 15.q25 to smoking behaviors and nicotine dependence. Investigations into these SNPs may help explain the nature and mechanisms of the smoking-PD relationship. <i>Objective:</i> To examine whether the genetic variations that were consistently associated with smoking or nicotine dependence in recent GWAS also predict the risk of PD. <i>Methods:</i> This is a population-based case-control study of 788 physician-diagnosed PD patients and 911 controls, all non-Hispanic Whites. Seven SNPs were selected based on findings from recent GWAS on smoking and nicotine dependence, all from the nicotinic acetylcholine receptor subunits <i>(CHRN)</i> A3-A5-B4. Odds ratios (ORs) and 95% confidence intervals were derived from logistic regression models under the assumption of logit-additive allelic effects. <i>Results:</i> Four SNPs in linkage disequilibrium from the <i>CHRNA3-CHRNA5-CHRNB4</i> cluster were associated with smoking duration (OR &gt;1.3, p &lt; 0.05). However, none of the SNPs from this cluster was associated with PD risk in the overall analysis or after stratifying on smoking status. <i>Conclusion:</i> This preliminary analysis does not support a relationship between these smoking-related GWAS SNPs and PD.
DOI: 10.6084/m9.figshare.5121613
title: Supplementary Material for: Different Molecular and Structural Adaptations with Eccentric and Conventional Strength Training in Elderly Men and Women

description: Reprogramming of gene expression contributes to structural and functional adaptation of muscle tissue in response to altered use. The aim of this study was to investigate mechanisms for observed improvements in leg extension strength, gain in relative thigh muscle mass and loss of body and thigh fat content in response to eccentric and conventional strength training in elderly men (n = 14) and women (n = 14; average age of the men and women: 80.1 ± 3.7 years) by means of structural and molecular analyses. Biopsies were collected from m. vastus lateralis in the resting state before and after 12 weeks of training with two weekly resistance exercise sessions (RET) or eccentric ergometer sessions (EET). Gene expression was analyzed using custom-designed low-density PCR arrays. Muscle ultrastructure was evaluated using EM morphometry. Gain in thigh muscle mass was paralleled by an increase in muscle fiber cross-sectional area (hypertrophy) with RET but not with EET, where muscle growth is likely occurring by the addition of sarcomeres in series or by hyperplasia. The expression of transcripts encoding factors involved in muscle growth, repair and remodeling (e.g. IGF-1, HGF, MYOG, MYH3) was increased to a larger extent after EET than RET. MicroRNA 1 expression was decreased independent of the training modality, and was paralleled by an increased expression of IGF-1 representing a potential target. IGF-1 is a potent promoter of muscle growth, and its regulation by microRNA 1 may have contributed to the gain of muscle mass observed in our subjects. EET depressed genes encoding mitochondrial and metabolic transcripts. The changes of several metabolic and mitochondrial transcripts correlated significantly with changes in mitochondrial volume density. Intramyocellular lipid content was decreased after EET concomitantly with total body fat. Changes in intramyocellular lipid content correlated with changes in body fat content with both RET and EET. In the elderly, RET and EET lead to distinct molecular and structural adaptations which might contribute to the observed small quantitative differences in functional tests and body composition parameters. EET seems to be particularly convenient for the elderly with regard to improvements in body composition and strength but at the expense of reducing muscular oxidative capacity.
DOI: 10.6084/m9.figshare.5121622.v1
title: Supplementary Material for: Carboplatin Dosing in Children: Calculation by Different Formulae

description: Background: Carboplatin dosing in children is based on renal function and there exists a wealth of formulae available for calculating the body surface area (BSA), the glomerular filtration rate (GFR), and the carboplatin dose. Patients and Methods: A fictitious group of children with different ages and body builds was ‘constructed’. For comparison of formulae, bias and precision were assessed. Results: BSA calculations according to DuBois-DuBois, Gehan-George, Mosteller, and Boyd showed good agreement. GFR calculations according to the weight-based Cole formula and the Léger formula gave comparable results. Regarding GFR in young children, the weight-and creatinine-based Cole and the Schwartz formula showed clear differences. Again, carboplatin dose calculations according to Marina, Newell, and Chatelut are comparable. Moreover, the precision of the creatinine measurement has a clear influence on the result of the dose calculation. Conclusions: The choice of the GFR formula is more important for the carboplatin dose calculation compared to the BSA or dose equation. GFR calculations in children show marked, age-dependent variations. A sequence of multiple calculation steps (especially for the Schwartz and Marina formulae) may lead to considerable uncertainty and proneness to error in the clinical routine. In high-risk patients, GFR should be measured precisely and complemented by therapeutic drug monitoring.
DOI: 10.6084/m9.figshare.5121601
title: Supplementary Material for: Treatment of Growth Hormone-Deficient Infants with Recombinant Human Growth Hormone to Near-Adult Height: Patterns of Growth

description: <i>Background/Aims:</i> To determine adult statures and linear growth patterns of children with growth hormone deficiency (GHD) who began treatment with recombinant human growth hormone (rhGH) in infancy. <i>Methods:</i> Forty-seven patients with GHD in whom administration of rhGH was initiated at or before 2 years of age and who had achieved near-adult heights (NAH) were identified in the database of the Genentech National Cooperative Growth Study. <i>Results:</i> After beginning treatment at a mean age of 0.9 years and height of –2.3 SD, these subjects achieved mean statures of –0.6, –0.3, and –0.4 SD at 5 and 10 years of age and at NAH, respectively. In 23 patients whose parental heights were known, mean NAH was comparable to the target height. Patients with uncomplicated courses whose heights were normal or tall when spontaneous puberty occurred or was induced realized the tallest NAHs. Patients with severe prenatal or perinatal, congenital and acquired neurologic insults, sexual precocity, or associated illnesses achieved less optimal NAHs. <i>Conclusion:</i> A normal pattern of linear growth during childhood and adolescence and satisfactory NAHs can be achieved in the majority of patients when treatment of the GHD subject is begun during infancy.
DOI: 10.6084/m9.figshare.5121607
title: Supplementary Material for: Brain-Derived Neurotrophic Factor Promotes Central Nervous System Myelination via a Direct Effect upon Oligodendrocytes

description: The extracellular factors that are responsible for inducing myelination in the central nervous system (CNS) remain elusive. We investigated whether brain-derived neurotrophic factor (BDNF) is implicated, by first confirming that BDNF heterozygous mice exhibit delayed CNS myelination during early postnatal development. We next established that the influence of BDNF upon myelination was direct, by acting on oligodendrocytes, using co-cultures of dorsal root ganglia neurons and oligodendrocyte precursor cells. Importantly, we found that BDNF retains its capacity to enhance myelination of neurons or by oligodendrocytes derived from p75NTR knockout mice, indicating the expression of p75NTR is not necessary for BDNF-induced myelination. Conversely, we observed that phosphorylation of TrkB correlated with myelination, and that inhibiting TrkB signalling also inhibited the promyelinating effect of BDNF, suggesting that BDNF enhances CNS myelination via activating oligodendroglial TrkB-FL receptors. Together, our data reveal a previously unknown role for BDNF in potentiating the normal development of CNS myelination, via signalling within oligodendrocytes.
DOI: 10.6084/m9.figshare.5121604
title: Supplementary Material for: Large Umbilical Artery Aneurysm with a Live Healthy Baby Delivered at 31 Weeks

description: Umbilical artery aneurysm is a rare structural anomaly. We report a case of a large cord mass monitored closely with MCA PSV aiding the decision for delivery in an appropriately grown fetus.
DOI: 10.6084/m9.figshare.5121607.v1
title: Supplementary Material for: Brain-Derived Neurotrophic Factor Promotes Central Nervous System Myelination via a Direct Effect upon Oligodendrocytes

description: The extracellular factors that are responsible for inducing myelination in the central nervous system (CNS) remain elusive. We investigated whether brain-derived neurotrophic factor (BDNF) is implicated, by first confirming that BDNF heterozygous mice exhibit delayed CNS myelination during early postnatal development. We next established that the influence of BDNF upon myelination was direct, by acting on oligodendrocytes, using co-cultures of dorsal root ganglia neurons and oligodendrocyte precursor cells. Importantly, we found that BDNF retains its capacity to enhance myelination of neurons or by oligodendrocytes derived from p75NTR knockout mice, indicating the expression of p75NTR is not necessary for BDNF-induced myelination. Conversely, we observed that phosphorylation of TrkB correlated with myelination, and that inhibiting TrkB signalling also inhibited the promyelinating effect of BDNF, suggesting that BDNF enhances CNS myelination via activating oligodendroglial TrkB-FL receptors. Together, our data reveal a previously unknown role for BDNF in potentiating the normal development of CNS myelination, via signalling within oligodendrocytes.