cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.30277297.v1
title: Supplementary Material for: Darier’s Disease Successfully Treated with Tofacitinib: A Case Report

description: Darier’s disease (DD) is a rare autosomal dominant genodermatosis characterized by keratotic papules, predominantly affecting seborrheic areas. We report a 42-year-old woman who developed pruritic brown papular lesions on her trunk, particularly under the breasts, groin, and neck. A biopsy confirmed Darier’s disease, and she was treated with adalimumab, which failed to provide clinical improvement. The patient was then started on tofacitinib 10 mg daily, leading to disease stabilization. Due to persistent itching and new lesions, the dose was increased to 15 mg daily, resulting in significant improvement. After a few months, with most lesions resolved, the dose was reduced to 10 mg daily, successfully maintaining disease control. This case highlights the potential role of JAK inhibitors as an effective treatment option for refractory Darier’s disease.
DOI: 10.6084/m9.figshare.30277300
title: Supplementary Material for: Sensory Profiles in Children with Speech Sound Disorders

description: Introduction: Intact sensory processing functions are essential in the development of speech and language, cognitive and motor milestones, and for supporting participation in daily activities. This study investigated in detail which specific modalities of sensory processing are affected in children with speech sound disorders (SSDs), including relevant clinical subgroups.
Methods: A consecutive sample of 143 children between 4 and 9 years (6:0 years) of age referred for differential diagnosis of oral and speech motor disorders to the Centre of Eating, Speech and Oral Motor Function (OMC) was included. Caregivers completed the Swedish version of the Sensory Profile 2 (SP2), which assesses sensory responses in everyday situations. Standardized norms were used for comparison.
Results: Children with motor speech disorders (MSD) (n = 82) and those with a language-oriented speech disorder (LD) with concomitant oral motor developmental delay (ODD) (n = 27) exhibited significantly more sensory processing difficulties than typically developing peers. Children in the MSD group also frequently presented with co-occurring LD and ODD diagnoses (MSD+).
Conclusion: The findings of this study indicate that children with SSD, particularly those in the MSD+ and LD+ODD subgroups, show a higher degree of sensory processing difficulties. These difficulties may significantly affect their participation in daily life activities. Gaining an understanding of how sensory processing dysfunctions shape a child’s actions and reactions is of vital importance for speech language pathologists (SLPs) working with these children. This knowledge can inform more tailored and effective intervention strategies within speech and language therapy.
DOI: 10.6084/m9.figshare.30277300.v1
title: Supplementary Material for: Sensory Profiles in Children with Speech Sound Disorders

description: Introduction: Intact sensory processing functions are essential in the development of speech and language, cognitive and motor milestones, and for supporting participation in daily activities. This study investigated in detail which specific modalities of sensory processing are affected in children with speech sound disorders (SSDs), including relevant clinical subgroups.
Methods: A consecutive sample of 143 children between 4 and 9 years (6:0 years) of age referred for differential diagnosis of oral and speech motor disorders to the Centre of Eating, Speech and Oral Motor Function (OMC) was included. Caregivers completed the Swedish version of the Sensory Profile 2 (SP2), which assesses sensory responses in everyday situations. Standardized norms were used for comparison.
Results: Children with motor speech disorders (MSD) (n = 82) and those with a language-oriented speech disorder (LD) with concomitant oral motor developmental delay (ODD) (n = 27) exhibited significantly more sensory processing difficulties than typically developing peers. Children in the MSD group also frequently presented with co-occurring LD and ODD diagnoses (MSD+).
Conclusion: The findings of this study indicate that children with SSD, particularly those in the MSD+ and LD+ODD subgroups, show a higher degree of sensory processing difficulties. These difficulties may significantly affect their participation in daily life activities. Gaining an understanding of how sensory processing dysfunctions shape a child’s actions and reactions is of vital importance for speech language pathologists (SLPs) working with these children. This knowledge can inform more tailored and effective intervention strategies within speech and language therapy.
DOI: 10.6084/m9.figshare.30277303
title: Supplementary Material for: Personalized treatment in rare genetic syndromes: a case-report in Witteveen-Kolk Syndrome (SIN3A)

description: Introduction: Neurodevelopmental disorders (NDDs) include a broad spectrum of disorders. NDDs caused by rare gene mutations are referred to as Rare Genetic Neurodevelopmental Syndromes (RGNS). Witteveen-Kolk syndrome (WITKOS) is such a RGNS, caused by heterozygous mutations in SIN3A and characterized by developmental problems, facial dysmorphisms, short stature, brain abnormalities, intellectual disability and behavioral problems. Care for individuals with WITKOS mainly focuses on somatic characteristics. To address this issue, we present a case-study of the first documented treatment of behavioral problems in WITKOS.
Case Presentation: The patient is a 27-year-old single female with known developmental problems during childhood and a history of severe psychiatric problems. She received the genetically confirmed diagnosis of WITKOS at the age of 25 and based upon extensive intellectual and neuropsychological examination, a personalized psychological treatment could be performed. This treatment consisted of twelve individual sessions aimed to reduce anxiety, fatigue and thought disorder by treating factors contributing to cognitive overload. Evaluation during treatment and at three-months follow-up showed a positive increase in functioning and acceptance as well as the acceptance of successive follow-up treatment. However, she was unable to maintain favorable changes in emotion-regulation strategies at follow up, emphasizing the need for prolonged professional guidance and support.
Conclusion: Neurocognitive evaluation enabled the adaptation of treatment strategies to the patients developmental and neurocognitive functioning, provided guidance in treatment choice, supported the rewriting of her personal narrative and resulted in successive care.
DOI: 10.6084/m9.figshare.30277303.v1
title: Supplementary Material for: Personalized treatment in rare genetic syndromes: a case-report in Witteveen-Kolk Syndrome (SIN3A)

description: Introduction: Neurodevelopmental disorders (NDDs) include a broad spectrum of disorders. NDDs caused by rare gene mutations are referred to as Rare Genetic Neurodevelopmental Syndromes (RGNS). Witteveen-Kolk syndrome (WITKOS) is such a RGNS, caused by heterozygous mutations in SIN3A and characterized by developmental problems, facial dysmorphisms, short stature, brain abnormalities, intellectual disability and behavioral problems. Care for individuals with WITKOS mainly focuses on somatic characteristics. To address this issue, we present a case-study of the first documented treatment of behavioral problems in WITKOS.
Case Presentation: The patient is a 27-year-old single female with known developmental problems during childhood and a history of severe psychiatric problems. She received the genetically confirmed diagnosis of WITKOS at the age of 25 and based upon extensive intellectual and neuropsychological examination, a personalized psychological treatment could be performed. This treatment consisted of twelve individual sessions aimed to reduce anxiety, fatigue and thought disorder by treating factors contributing to cognitive overload. Evaluation during treatment and at three-months follow-up showed a positive increase in functioning and acceptance as well as the acceptance of successive follow-up treatment. However, she was unable to maintain favorable changes in emotion-regulation strategies at follow up, emphasizing the need for prolonged professional guidance and support.
Conclusion: Neurocognitive evaluation enabled the adaptation of treatment strategies to the patients developmental and neurocognitive functioning, provided guidance in treatment choice, supported the rewriting of her personal narrative and resulted in successive care.
DOI: 10.15468/dl.xn23gm
title: Occurrence Download

description: A dataset containing 0 species occurrences available in GBIF matching the query:
{
 "and" : [
 "TaxonKey is one of ()",
 "HasCoordinate is true",
 "HasGeospatialIssue is false",
 "Year is greater than or equal to 1980",
 "OccurrenceStatus is Present",
 {
 "not" : {
 "BasisOfRecord" : [
 "is one of (Fossil, Living Specimen)"
 ]
 }
 },
 "Geometry POLYGON((-30 -60,0 -60,0 -30,-30 -30,-30 -60))"
 ]
}

 The dataset includes 0 records from 0 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0045251-250920141307145/datasets/export for details.
DOI: 10.6084/m9.figshare.30277306
title: Supplementary Material for: A quasi-experimental study on feeding behaviors and related outcomes by provision of molded puree in aged care facilities

description: Introduction
Texture-modified diets are commonly used in the management of dysphagia in older adults. Conventional pureed diets, although safe, are often visually unappealing, potentially leading to reduced intake and negative feeding behaviors. Molded purees are created using food molds and gelling agents and aim to improve mealtime experiences through enhanced presentation. This study evaluated the effects of molded puree on feeding behaviors and intake amount in residents of aged care facilities, and whether any factors were associated with more favorable outcomes.
Methods
A quasi-experimental repeated measures study design was used, involving 130 residents from four nursing homes. Participants received molded puree meals instead of their original prescribed diet once per week over 3 weeks. Feeding performance was assessed using the Edinburgh Feeding Evaluation in Dementia Scale (EdFED) and amount consumed were also recorded. Wilcoxon signed rank test was used to compare outcomes between molded puree and their original diets. Subgroup analyses were conducted based on medical diagnoses and swallowing status.
Results
Molded puree meals were associated with significantly lower EdFED scores compared to conventional purees, indicating improved feeding behaviors (p &lt; 0.001). Participants also consumed significantly more molded puree than their original diets (p &lt; 0.001). Subgroup analyses revealed participants with dementia showed greater benefit in feeding behaviors and amount consumed when they were provided with molded puree.
Discussion
Molded puree improved feeding behavior and food intake among residents in aged care, in particular patients with dementia, but independent of their swallowing function. These findings support the potential for molded purees to enhance mealtime experiences in this population, although further research is needed to evaluate long-term clinical outcomes.
DOI: 10.6084/m9.figshare.30277306.v1
title: Supplementary Material for: A quasi-experimental study on feeding behaviors and related outcomes by provision of molded puree in aged care facilities

description: Introduction
Texture-modified diets are commonly used in the management of dysphagia in older adults. Conventional pureed diets, although safe, are often visually unappealing, potentially leading to reduced intake and negative feeding behaviors. Molded purees are created using food molds and gelling agents and aim to improve mealtime experiences through enhanced presentation. This study evaluated the effects of molded puree on feeding behaviors and intake amount in residents of aged care facilities, and whether any factors were associated with more favorable outcomes.
Methods
A quasi-experimental repeated measures study design was used, involving 130 residents from four nursing homes. Participants received molded puree meals instead of their original prescribed diet once per week over 3 weeks. Feeding performance was assessed using the Edinburgh Feeding Evaluation in Dementia Scale (EdFED) and amount consumed were also recorded. Wilcoxon signed rank test was used to compare outcomes between molded puree and their original diets. Subgroup analyses were conducted based on medical diagnoses and swallowing status.
Results
Molded puree meals were associated with significantly lower EdFED scores compared to conventional purees, indicating improved feeding behaviors (p &lt; 0.001). Participants also consumed significantly more molded puree than their original diets (p &lt; 0.001). Subgroup analyses revealed participants with dementia showed greater benefit in feeding behaviors and amount consumed when they were provided with molded puree.
Discussion
Molded puree improved feeding behavior and food intake among residents in aged care, in particular patients with dementia, but independent of their swallowing function. These findings support the potential for molded purees to enhance mealtime experiences in this population, although further research is needed to evaluate long-term clinical outcomes.
DOI: 10.15468/dl.bwp3rv
title: Occurrence Download

description: A dataset containing 0 species occurrences available in GBIF matching the query:
{
 "and" : [
 "TaxonKey is one of ()",
 "HasCoordinate is true",
 "HasGeospatialIssue is false",
 "Year is greater than or equal to 1980",
 "OccurrenceStatus is Present",
 {
 "not" : {
 "BasisOfRecord" : [
 "is one of (Fossil, Living Specimen)"
 ]
 }
 },
 "Geometry POLYGON((0 -60,30 -60,30 -30,0 -30,0 -60))"
 ]
}

 The dataset includes 0 records from 0 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0045253-250920141307145/datasets/export for details.
DOI: 10.26023/g3a2-65ap-kf02
title: ICE-L: WRF 1KM Vertical Velocity Forecast Imagery. Version 1.0

description: This dataset contains Weather Research and Forecasting Model (WRF) 1 kilometer cross-sections of vertical velocity. The imagery was generated from Winter Park, CO to Platteville, CO during the ICE-L project. It is available in PNG format and covers the time span from 2007-11-19 00:00:00 to 2007-12-21 23:59:59.
DOI: 10.15468/dl.fqrv89
title: Occurrence Download

description: A dataset containing 599 species occurrences available in GBIF matching the query:
{
 "TaxonKey" : [
 "is Tulipa uniflora"
 ]
}

 The dataset includes 599 records from 39 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0045252-250920141307145/datasets/export for details.
 
Data from some individual datasets included in this download may be licensed under less restrictive terms.
DOI: 10.15468/dl.pcxd2s
title: Occurrence Download

description: A dataset containing 0 species occurrences available in GBIF matching the query:
{
 "and" : [
 "TaxonKey is Periclimenes calmani",
 "Country is Egypt",
 "HasCoordinate is true"
 ]
}

 The dataset includes 0 records from 0 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0045254-250920141307145/datasets/export for details.
DOI: 10.15468/dl.uwxyav
title: Occurrence Download

description: A dataset containing 0 species occurrences available in GBIF matching the query:
{
 "and" : [
 "TaxonKey is one of ()",
 "HasCoordinate is true",
 "HasGeospatialIssue is false",
 "Year is greater than or equal to 1980",
 "OccurrenceStatus is Present",
 {
 "not" : {
 "BasisOfRecord" : [
 "is one of (Fossil, Living Specimen)"
 ]
 }
 },
 "Geometry POLYGON((30 -60,60 -60,60 -30,30 -30,30 -60))"
 ]
}

 The dataset includes 0 records from 0 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0045255-250920141307145/datasets/export for details.
DOI: 10.26023/dgvp-07k5-5d0c
title: ICE-L: WRF 1KM Radar Reflectivity Forecast Imagery. Version 1.0

description: This dataset contains Weather Research and Forecasting Model (WRF) 1 kilometer vertical cross-sections of radar reflectivity from Craig, CO to Denver, CO. The imagery was generated during the ICE-L project and is in PNG format. It covers the time span from 2007-11-19 00:00:00 to 2007-12-21 23:59:59.
DOI: 10.15468/dl.dz923r
title: Occurrence Download

description: A dataset containing 0 species occurrences available in GBIF matching the query:
{
 "and" : [
 "TaxonKey is one of ()",
 "HasCoordinate is true",
 "HasGeospatialIssue is false",
 "Year is greater than or equal to 1980",
 "OccurrenceStatus is Present",
 {
 "not" : {
 "BasisOfRecord" : [
 "is one of (Fossil, Living Specimen)"
 ]
 }
 },
 "Geometry POLYGON((60 -60,90 -60,90 -30,60 -30,60 -60))"
 ]
}

 The dataset includes 0 records from 0 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0045256-250920141307145/datasets/export for details.
DOI: 10.15468/dl.8zhhmf
title: Occurrence Download

description: A dataset containing 0 species occurrences available in GBIF matching the query:
{
 "and" : [
 "TaxonKey is one of ()",
 "HasCoordinate is true",
 "HasGeospatialIssue is false",
 "Year is greater than or equal to 1980",
 "OccurrenceStatus is Present",
 {
 "not" : {
 "BasisOfRecord" : [
 "is one of (Fossil, Living Specimen)"
 ]
 }
 },
 "Geometry POLYGON((90 -60,120 -60,120 -30,90 -30,90 -60))"
 ]
}

 The dataset includes 0 records from 0 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0045257-250920141307145/datasets/export for details.
DOI: 10.15468/dl.vxnvab
title: Occurrence Download

description: A dataset containing 5 species occurrences available in GBIF matching the query:
{
 "and" : [
 "TaxonKey is Leptochela aculeocaudata",
 "Country is Egypt",
 "HasCoordinate is true"
 ]
}

 The dataset includes 5 records from 1 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0045258-250920141307145/datasets/export for details.
 
Data from some individual datasets included in this download may be licensed under less restrictive terms.
DOI: 10.26023/hxry-3j7x-dw0r
title: ICE-L: WRF 1KM Mixing Ratio Forecast Imagery. Version 1.0

description: This dataset contains Weather Research and Forecasting Model (WRF) 1 kilometer vertical cross-sections of four different mixing ratios, ice number concentration, and temperature. The imagery contains cloud water, rain water, snow, and Graupel mixing ratios. The imagery was generated from Craig, CO to Denver, CO to during the ICE-L project. It is available in PNG format and covers the time span from 2007-11-19 00:00:00 to 2007-12-21 23:59:59.
DOI: 10.15468/dl.jcgxr2
title: Occurrence Download

description: A dataset containing 0 species occurrences available in GBIF matching the query:
{
 "and" : [
 "TaxonKey is one of ()",
 "HasCoordinate is true",
 "HasGeospatialIssue is false",
 "Year is greater than or equal to 1980",
 "OccurrenceStatus is Present",
 {
 "not" : {
 "BasisOfRecord" : [
 "is one of (Fossil, Living Specimen)"
 ]
 }
 },
 "Geometry POLYGON((120 -60,150 -60,150 -30,120 -30,120 -60))"
 ]
}

 The dataset includes 0 records from 0 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0045260-250920141307145/datasets/export for details.
DOI: 10.15468/dl.6nhqvk
title: Occurrence Download

description: A dataset containing 0 species occurrences available in GBIF matching the query:
{
 "and" : [
 "TaxonKey is Leptochela aculeocaudata",
 "Country is Syrian Arab Republic",
 "HasCoordinate is true"
 ]
}

 The dataset includes 0 records from 0 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0045259-250920141307145/datasets/export for details.
DOI: 10.26023/qfsn-efbz-we06
title: ICE-L: WRF 1KM Relative Humidity Forecast Imagery. Version 1.0

description: This dataset contains Weather Research and Forecasting Model (WRF) 1 kilometer vertical cross-sections of relative humidity. The imagery was generated from Craig, CO to Denver, CO to during the ICE-L project. It is available in PNG format and covers the time span from 2007-11-19 00:00:00 to 2007-12-21 23:59:59.
DOI: 10.15468/dl.jukezd
title: Occurrence Download

description: A dataset containing 0 species occurrences available in GBIF matching the query:
{
 "and" : [
 "ScientificName is Echeveria tencho",
 "Country is Mexico",
 "BasisOfRecord is Specimen"
 ]
}

 The dataset includes 0 records from 0 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0045235-250920141307145/datasets/export for details.
DOI: 10.15468/dl.g4rgzw
title: Occurrence Download

description: A dataset containing 0 species occurrences available in GBIF matching the query:
{
 "and" : [
 "TaxonKey is one of ()",
 "HasCoordinate is true",
 "HasGeospatialIssue is false",
 "Year is greater than or equal to 1980",
 "OccurrenceStatus is Present",
 {
 "not" : {
 "BasisOfRecord" : [
 "is one of (Fossil, Living Specimen)"
 ]
 }
 },
 "Geometry POLYGON((150 -60,180 -60,180 -30,150 -30,150 -60))"
 ]
}

 The dataset includes 0 records from 0 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0045261-250920141307145/datasets/export for details.
DOI: 10.15151/esrf-es-2210534260
title: Swiss consortium in macromolecular X-ray crystallography structure determination for academic researchers: EPFL, UNIL, UNIGE, Zurich and SLS

description: This proposal builds upon the foundation established in BAG MX 2521 and MX 2644 and represents a collaborative effort among prominent Swiss academic institutions, including EPFL, UNIL, UNIGE, university of Zurich and SLS. Under the leadership of Dr. Florence Pojer, the head of the Protein Production and Structure core facility at EPFL, this initiative aims to streamline and enhance the process of determination of macromolecules structures by X-ray crystallography. Notably, the automated data collection established at ESRF has revolutionized our current pipeline, enabling the analysis of over 1000 crystals and the finalization at high-resolution of around 50 different structures in the past year alone. These achievements span diverse projects ranging from drug discovery to the design of novel protein folds.
DOI: 10.15151/esrf-es-2250310051
title: Structural Biology at EMBL Grenoble, IBS-CIBB, IAB and ILL

description: This proposal clusters macromolecular crystallography (MX) and biological small angle X-ray scattering (bioSAXS) projects at the EMBL Grenoble, IBS-CIBB, IAB and ILL. It combines groups working on different topics with a major focus on cell signaling proteins, ubiquitination pathways, nucleic acid-protein complexes, sarcomere development, host-pathogen interactions and drug discovery. The BAG group has been quite productive this last year/reporting period, as summarized in the report, with many results still being further analysed and follow-up experiments planned. Most notably, the BAG has produced 7 PDB depositions, 1 SASDB deposition and 10 publications in peer reviewed journals. The proposers have long-standing experience in the use, as well as the development of beam lines for MX and bioSAXS, and have participated in successive successful BAG applications over the years, mostly at the ESRF. The BAG responsible is again Andrew McCarthy (EMBL Grenoble) for this application round.
DOI: 10.15151/esrf-es-2191614248
title: IBS BAG

description: The IBS BAG includes teams from IBS (Grenoble) and external groups from CEA-Cadarache (St Paul lez Durance) and IRBA (Brétigny-sur-Orge). We study the structure-function relationships of proteins with a focus on human health, biotechnology and methodology. Most of the projects are a continuation of the MX-2586 proposal and can be grouped under the following themes: host-pathogen interaction, new therapeutic targets, fundamental cellular mechanisms, membrane proteins, biotechnological applications and methodology. Some of these projects use integrative approaches (X-ray crystallography, cryo-EM, SAXS and NMR) together with other biophysical and biochemical studies and, more recently, the use of AI (AlphaFold) to fully understand the complexity of the biological macromolecular systems under study.