cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.31077391
title: Supplementary Material for: Long-Term Mortality and 10-Year Outcomes after Subthalamic Nucleus Deep Brain Stimulation in Parkinson’s Disease

description: Background: Subthalamic nucleus deep brain stimulation (STN-DBS) effectively improves motor symptoms in Parkinson’s disease (PD), but long-term survival outcomes and mortality predictors remain unclear. This study aimed to evaluate survival and 10-year clinical outcomes after STN-DBS.
Methods: We retrospectively analyzed 608 patients with PD who underwent bilateral STN-DBS between 2006 and 2023. Kaplan–Meier and Cox regression analyses assessed survival and preoperative predictors. Motor (MDS-UPDRS Part III), cognitive (MoCA, MMSE), and medication (LEDD) data were compared between baseline and 10 years postoperatively.
Results: During a mean follow-up of 4.8 ± 3.5 years, 42 deaths (6.9%) occurred, yielding an age-adjusted mortality rate of 3.3 per 100,000 population. The estimated 5- and 10-year survival rates were 95% and 77%, respectively. The most frequent cause of death was aspiration pneumonia (11.9%). In multivariate Cox analysis, lower preoperative MoCA scores (HR = 0.83, 95% CI 0.73–0.95, p &lt; 0.01) and higher OFF-state MDS-UPDRS Part III scores (HR = 1.03, 95% CI 1.00–1.07, p &lt; 0.05) independently predicted mortality, whereas age at surgery was not significantly associated with survival. Among 118 patients with 10-year follow-up, OFF-state motor and cognitive scores worsened significantly (p &lt; 0.01), while ON-state motor scores (p = 0.21) and total LEDD (p = 0.06) remained stable, suggesting sustained motor control and medication-sparing effects.
Conclusions: Long-term survival after STN-DBS in PD was favorable. Preoperative cognitive and motor status, rather than age, determined long-term prognosis, emphasizing the enduring therapeutic value of STN-DBS.
DOI: 10.6084/m9.figshare.31077391.v1
title: Supplementary Material for: Long-Term Mortality and 10-Year Outcomes after Subthalamic Nucleus Deep Brain Stimulation in Parkinson’s Disease

description: Background: Subthalamic nucleus deep brain stimulation (STN-DBS) effectively improves motor symptoms in Parkinson’s disease (PD), but long-term survival outcomes and mortality predictors remain unclear. This study aimed to evaluate survival and 10-year clinical outcomes after STN-DBS.
Methods: We retrospectively analyzed 608 patients with PD who underwent bilateral STN-DBS between 2006 and 2023. Kaplan–Meier and Cox regression analyses assessed survival and preoperative predictors. Motor (MDS-UPDRS Part III), cognitive (MoCA, MMSE), and medication (LEDD) data were compared between baseline and 10 years postoperatively.
Results: During a mean follow-up of 4.8 ± 3.5 years, 42 deaths (6.9%) occurred, yielding an age-adjusted mortality rate of 3.3 per 100,000 population. The estimated 5- and 10-year survival rates were 95% and 77%, respectively. The most frequent cause of death was aspiration pneumonia (11.9%). In multivariate Cox analysis, lower preoperative MoCA scores (HR = 0.83, 95% CI 0.73–0.95, p &lt; 0.01) and higher OFF-state MDS-UPDRS Part III scores (HR = 1.03, 95% CI 1.00–1.07, p &lt; 0.05) independently predicted mortality, whereas age at surgery was not significantly associated with survival. Among 118 patients with 10-year follow-up, OFF-state motor and cognitive scores worsened significantly (p &lt; 0.01), while ON-state motor scores (p = 0.21) and total LEDD (p = 0.06) remained stable, suggesting sustained motor control and medication-sparing effects.
Conclusions: Long-term survival after STN-DBS in PD was favorable. Preoperative cognitive and motor status, rather than age, determined long-term prognosis, emphasizing the enduring therapeutic value of STN-DBS.
DOI: 10.6084/m9.figshare.31077394
title: Supplementary Material for: Sequential Multiple-Organ Immune-Related Adverse Events During and After Pembrolizumab Therapy in Triple-Negative Breast Cancer: A Case Report

description: Immune checkpoint inhibitors (ICIs) are an important component of triple-negative breast cancer (TNBC) treatment, and the KEYNOTE-522 regimen has established pembrolizumab combined with chemotherapy, as the standard of care. While ICIs improve outcomes, they can also induce immune-related adverse events (irAEs), affecting diverse organs. However, renal, pancreatic, and ocular toxicities are rare, and there have been few reports of sequential multi-organ irAEs in breast cancer. This case study describes a 63-year-old woman with stage IIA TNBC who was treated with the KEYNOTE-522 regimen. During neoadjuvant therapy, she developed fever, malaise, and mild laboratory abnormalities. Her symptoms progressed to acute kidney injury and elevated pancreatic enzymes. She was diagnosed with immune-related acute interstitial nephritis and pancreatitis and improved promptly with high-dose corticosteroids. After tapering immunosuppression, the patient developed Pneumocystis jirovecii pneumonia. Subsequently, she suffered bilateral anterior uveitis consistent with an immune-related ocular event. Surgical resection revealed a pathological complete response (pCR), and she remains under surveillance. This case included three distinct irAEs affecting the kidney, pancreas, and eye. Each occurred at a different time point, including after discontinuation of immunotherapy. The opportunistic infection that she developed during steroid therapy emphasizes the need for prophylaxis in patients requiring prolonged immunosuppression. This case underscores the importance of multidisciplinary management and long-term vigilance for delayed and multi-organ irAEs in TNBC patients treated with ICIs.
DOI: 10.6084/m9.figshare.31077394.v1
title: Supplementary Material for: Sequential Multiple-Organ Immune-Related Adverse Events During and After Pembrolizumab Therapy in Triple-Negative Breast Cancer: A Case Report

description: Immune checkpoint inhibitors (ICIs) are an important component of triple-negative breast cancer (TNBC) treatment, and the KEYNOTE-522 regimen has established pembrolizumab combined with chemotherapy, as the standard of care. While ICIs improve outcomes, they can also induce immune-related adverse events (irAEs), affecting diverse organs. However, renal, pancreatic, and ocular toxicities are rare, and there have been few reports of sequential multi-organ irAEs in breast cancer. This case study describes a 63-year-old woman with stage IIA TNBC who was treated with the KEYNOTE-522 regimen. During neoadjuvant therapy, she developed fever, malaise, and mild laboratory abnormalities. Her symptoms progressed to acute kidney injury and elevated pancreatic enzymes. She was diagnosed with immune-related acute interstitial nephritis and pancreatitis and improved promptly with high-dose corticosteroids. After tapering immunosuppression, the patient developed Pneumocystis jirovecii pneumonia. Subsequently, she suffered bilateral anterior uveitis consistent with an immune-related ocular event. Surgical resection revealed a pathological complete response (pCR), and she remains under surveillance. This case included three distinct irAEs affecting the kidney, pancreas, and eye. Each occurred at a different time point, including after discontinuation of immunotherapy. The opportunistic infection that she developed during steroid therapy emphasizes the need for prophylaxis in patients requiring prolonged immunosuppression. This case underscores the importance of multidisciplinary management and long-term vigilance for delayed and multi-organ irAEs in TNBC patients treated with ICIs.
DOI: 10.6084/m9.figshare.31077397
title: Supplementary Material for: Obstructive Jaundice Revealing Primary Diffuse Large B-Cell Lymphoma of the Ampulla of Vater in a Child

description: Introduction: Primary lymphoma of the ampulla of Vater is an exceptionally rare entity, with most reported cases being low-grade B-cell lymphomas in adults. Diffuse Large B-Cell Lymphoma (DLBCL) at this site is markedly aggressive and exceedingly uncommon in the pediatric population.
Case Presentation: An 11-year-old male presented with obstructive jaundice. Abdominal ultrasound revealed a dilated gallbladder. Endoscopic Retrograde Cholangiopancreatography (ERCP) identified a fungating mass at the ampulla of Vater, causing significant biliary dilation. Histopathological and immunohistochemical analysis of biopsy samples (positive for LCA and CD20, negative for CD3) confirmed the diagnosis of DLBCL. Staging CT scan showed localized disease with cervical lymphadenopathy. The patient was managed with biliary stenting and systemic immunochemotherapy (R-COP protocol). Following an episode of hepatotoxicity, doxorubicin was introduced. Post-treatment imaging showed an excellent therapeutic response, and the patient was scheduled for consolidative radiotherapy.
Conclusion: This case underscores that lymphoma, though rare, should be considered in the differential diagnosis of obstructive jaundice even in children. A multimodal approach involving ERCP with biopsy and imperative immunohistochemistry is crucial for an accurate diagnosis to avoid unnecessary surgery. Management centered on immunochemotherapy, with adjuncts like biliary decompression and radiotherapy, can lead to successful outcomes in pediatric ampullary DLBCL.
DOI: 10.6084/m9.figshare.31077397.v1
title: Supplementary Material for: Obstructive Jaundice Revealing Primary Diffuse Large B-Cell Lymphoma of the Ampulla of Vater in a Child

description: Introduction: Primary lymphoma of the ampulla of Vater is an exceptionally rare entity, with most reported cases being low-grade B-cell lymphomas in adults. Diffuse Large B-Cell Lymphoma (DLBCL) at this site is markedly aggressive and exceedingly uncommon in the pediatric population.
Case Presentation: An 11-year-old male presented with obstructive jaundice. Abdominal ultrasound revealed a dilated gallbladder. Endoscopic Retrograde Cholangiopancreatography (ERCP) identified a fungating mass at the ampulla of Vater, causing significant biliary dilation. Histopathological and immunohistochemical analysis of biopsy samples (positive for LCA and CD20, negative for CD3) confirmed the diagnosis of DLBCL. Staging CT scan showed localized disease with cervical lymphadenopathy. The patient was managed with biliary stenting and systemic immunochemotherapy (R-COP protocol). Following an episode of hepatotoxicity, doxorubicin was introduced. Post-treatment imaging showed an excellent therapeutic response, and the patient was scheduled for consolidative radiotherapy.
Conclusion: This case underscores that lymphoma, though rare, should be considered in the differential diagnosis of obstructive jaundice even in children. A multimodal approach involving ERCP with biopsy and imperative immunohistochemistry is crucial for an accurate diagnosis to avoid unnecessary surgery. Management centered on immunochemotherapy, with adjuncts like biliary decompression and radiotherapy, can lead to successful outcomes in pediatric ampullary DLBCL.
DOI: 10.6084/m9.figshare.31077400
title: Supplementary Material for: Effects of N-Acetyl-L-Cysteine on the progression of kidney dysfunction in Acadian variant Fanconi syndrome: a case series

description: Introduction
Acadian variant Fanconi syndrome (AVFS) is an autosomal recessive disease caused by a mutation in the NDUFAF6 gene which results in mitochondrial dysfunction and oxidative damage to the kidneys. It presents as a slowly progressive chronic kidney disease and proximal tubular dysfunction that, in contrast to typical Fanconi Syndrome, leads to end-stage renal disease. It is diagnosed clinically and is present only in Acadian people. The objective of this study is to evaluate the effect of N-acetyl-L-cysteine (NAC), an antioxidant, on the progression of loss of renal function in patients with AVFS.
Case Presentation
Chart reviews were conducted on four active cases of AVFS not on kidney replacement therapy. Data on age, estimated glomerular filtration rate (eGFR), creatinine, medications and blood pressure were collected. Progression of eGFR was evaluated in each patient before and after NAC prescription, and the projected time to kidney replacement therapy (eGFR = 10 mL/min/1.73m2) with and without the use of NAC was calculated for each case. NAC had a positive effect on slowing the rate of eGFR loss in each patient. The projected need for kidney replacement therapy was delayed by an average of 9 years in the treated patients who otherwise would have required it by an estimated mean age of 28 years.
Conclusion
This study suggests that NAC has a beneficial effect on slowing the progression of kidney disease in patients with AVFS. This may be due to the modulation of oxidative stress by NAC. Further studies are needed to evaluate the potential benefits of NAC in other chronic kidney conditions.
DOI: 10.6084/m9.figshare.31077400.v1
title: Supplementary Material for: Effects of N-Acetyl-L-Cysteine on the progression of kidney dysfunction in Acadian variant Fanconi syndrome: a case series

description: Introduction
Acadian variant Fanconi syndrome (AVFS) is an autosomal recessive disease caused by a mutation in the NDUFAF6 gene which results in mitochondrial dysfunction and oxidative damage to the kidneys. It presents as a slowly progressive chronic kidney disease and proximal tubular dysfunction that, in contrast to typical Fanconi Syndrome, leads to end-stage renal disease. It is diagnosed clinically and is present only in Acadian people. The objective of this study is to evaluate the effect of N-acetyl-L-cysteine (NAC), an antioxidant, on the progression of loss of renal function in patients with AVFS.
Case Presentation
Chart reviews were conducted on four active cases of AVFS not on kidney replacement therapy. Data on age, estimated glomerular filtration rate (eGFR), creatinine, medications and blood pressure were collected. Progression of eGFR was evaluated in each patient before and after NAC prescription, and the projected time to kidney replacement therapy (eGFR = 10 mL/min/1.73m2) with and without the use of NAC was calculated for each case. NAC had a positive effect on slowing the rate of eGFR loss in each patient. The projected need for kidney replacement therapy was delayed by an average of 9 years in the treated patients who otherwise would have required it by an estimated mean age of 28 years.
Conclusion
This study suggests that NAC has a beneficial effect on slowing the progression of kidney disease in patients with AVFS. This may be due to the modulation of oxidative stress by NAC. Further studies are needed to evaluate the potential benefits of NAC in other chronic kidney conditions.
DOI: 10.6084/m9.figshare.31077403
title: Supplementary Material for: Successful Plasma Exchange and Steroid Maintenance Therapy for Severe Pembrolizumab-Induced Polyradiculoneuritis in Lung Cancer: A Case Report

description: Introduction: Neurological immune-related adverse events (n-irAEs) caused by immune checkpoint inhibitors (ICIs) are rare but can be life-threatening.
Case Presentation: A 43-year-old man with stage IVB squamous cell carcinoma of the lung developed progressive polyradiculoneuropathy 180 days after the first pembrolizumab administration. Initial treatments with high-dose corticosteroids and intravenous immunoglobulin (IVIg) were ineffective. Cerebrospinal fluid (CSF) analysis revealed albuminocytologic dissociation, and magnetic resonance imaging (MRI) revealed cervical nerve root hypertrophy. Plasma exchange (PE) was initiated along with steroid maintenance, resulting in gradual neurological recovery, as evidenced by improvements in the MRC score (from 32 to 56, on a scale from 0 to 60, with higher scores indicating greater muscle strength) and resolution of MRI abnormalities. Tumor control was sustained throughout the treatment.
Conclusion: This case highlights the potential efficacy of combining plasma exchange and steroid maintenance therapy in managing severe, refractory n-irAEs without compromising oncologic outcomes.
DOI: 10.6084/m9.figshare.31077403.v1
title: Supplementary Material for: Successful Plasma Exchange and Steroid Maintenance Therapy for Severe Pembrolizumab-Induced Polyradiculoneuritis in Lung Cancer: A Case Report

description: Introduction: Neurological immune-related adverse events (n-irAEs) caused by immune checkpoint inhibitors (ICIs) are rare but can be life-threatening.
Case Presentation: A 43-year-old man with stage IVB squamous cell carcinoma of the lung developed progressive polyradiculoneuropathy 180 days after the first pembrolizumab administration. Initial treatments with high-dose corticosteroids and intravenous immunoglobulin (IVIg) were ineffective. Cerebrospinal fluid (CSF) analysis revealed albuminocytologic dissociation, and magnetic resonance imaging (MRI) revealed cervical nerve root hypertrophy. Plasma exchange (PE) was initiated along with steroid maintenance, resulting in gradual neurological recovery, as evidenced by improvements in the MRC score (from 32 to 56, on a scale from 0 to 60, with higher scores indicating greater muscle strength) and resolution of MRI abnormalities. Tumor control was sustained throughout the treatment.
Conclusion: This case highlights the potential efficacy of combining plasma exchange and steroid maintenance therapy in managing severe, refractory n-irAEs without compromising oncologic outcomes.
DOI: 10.15468/dl.8fydrz
title: Occurrence Download

description: A dataset containing 154 species occurrences available in GBIF matching the query:
{
 "and" : [
 "TaxonKey is Procapra gutturosa",
 "HasCoordinate is TRUE",
 "HasGeospatialIssue is FALSE",
 "Year is 2010,2025"
 ]
}

 The dataset includes 154 records from 5 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0013465-260108223611665/datasets/export for details.
 
Data from some individual datasets included in this download may be licensed under less restrictive terms.
DOI: 10.15468/dl.z4rcc5
title: Occurrence Download

description: A dataset containing 6 species occurrences available in GBIF matching the query:
{
 "and" : [
 "TaxonKey is Nanger dama",
 "HasCoordinate is TRUE",
 "HasGeospatialIssue is FALSE",
 "Year is 2010,2025"
 ]
}

 The dataset includes 6 records from 2 constituent datasets; see https://api.gbif.org/v1/occurrence/download/0013464-260108223611665/datasets/export for details.
 
Data from some individual datasets included in this download may be licensed under less restrictive terms.
DOI: 10.6084/m9.figshare.31077406
title: Supplementary Material for: Gastrointestinal Bleeding after Particle Radiotherapy for Pancreatic Cancer

description: Introduction: Particle radiotherapy (PRT) is a new option for the treatment of unresectable pancreatic cancer (PC). While gastrointestinal bleeding (GIB) is a feared adverse event, real-world evidence in this setting is limited.
Methods: We conducted a single-center retrospective study to evaluate the frequency and outcomes of GIB and to elucidate the risk factors for GIB after PRT for PC.
Results: Thirty-four patients were included. Twenty-nine received PRT to the pancreatic primary, while five received PRT for metastases. Concurrent chemotherapy was given to 26 patients (76%). Eleven patients (32%) experienced acute GIB symptoms after PRT. Median time from PRT to GIB was 13.2 months. Endoscopic signs of hemorrhage were observed in eight patients (24%), and endoscopic hemostasis was performed in six (18%). Three cases presented with ruptured pseudoaneurysms, of which two were treated with transarterial embolization. Hemostasis was ultimately achieved in all cases, and no deaths occurred directly as a result of GIB. However, overall survival after GIB was short (median: 1.9 months). Median overall survival after PRT tended to be longer in bleeders than in non-bleeders (26.8 vs. 22.7 months, P = 0.03). Concurrent chemotherapy was associated with a lower risk of GIB in univariate analysis (P = 0.05).
Conclusion: Gastrointestinal bleeding after particle radiotherapy may not be as rare as previously believed, particularly in the terminal stages of pancreatic cancer.
DOI: 10.6084/m9.figshare.31077406.v1
title: Supplementary Material for: Gastrointestinal Bleeding after Particle Radiotherapy for Pancreatic Cancer

description: Introduction: Particle radiotherapy (PRT) is a new option for the treatment of unresectable pancreatic cancer (PC). While gastrointestinal bleeding (GIB) is a feared adverse event, real-world evidence in this setting is limited.
Methods: We conducted a single-center retrospective study to evaluate the frequency and outcomes of GIB and to elucidate the risk factors for GIB after PRT for PC.
Results: Thirty-four patients were included. Twenty-nine received PRT to the pancreatic primary, while five received PRT for metastases. Concurrent chemotherapy was given to 26 patients (76%). Eleven patients (32%) experienced acute GIB symptoms after PRT. Median time from PRT to GIB was 13.2 months. Endoscopic signs of hemorrhage were observed in eight patients (24%), and endoscopic hemostasis was performed in six (18%). Three cases presented with ruptured pseudoaneurysms, of which two were treated with transarterial embolization. Hemostasis was ultimately achieved in all cases, and no deaths occurred directly as a result of GIB. However, overall survival after GIB was short (median: 1.9 months). Median overall survival after PRT tended to be longer in bleeders than in non-bleeders (26.8 vs. 22.7 months, P = 0.03). Concurrent chemotherapy was associated with a lower risk of GIB in univariate analysis (P = 0.05).
Conclusion: Gastrointestinal bleeding after particle radiotherapy may not be as rare as previously believed, particularly in the terminal stages of pancreatic cancer.
DOI: 10.6084/m9.figshare.31077409
title: Supplementary Material for: Adapting and Validating the Communicative Activities Checklist (COMACT) to Portuguese: A Preliminary Investigation with People with Aphasia

description: Aims: To translate and adapt the Communicative Activities Checklist (COMACT), initially developed in Australian English, into European Portuguese (EP) and to analyse its validity and reliability, with a sample of Portuguese people with aphasia (PWA) and neurologically healthy people (NHP). Methods: The following phases were completed: forward translation, back-translation, development of a set of instructions, evaluation by a specialist committee of the different versions, cognitive debriefing, and a discussion group about the final COMACT-EP and its use with a sample of Portuguese PWA and NHP. Cognitive debriefing data were analysed both qualitatively (Thematic analysis) and quantitatively (Content Validity Index – CVI). Concurrent validity with the Communication Disability Profile - European Portuguese (CDP-EP) Activity subscale (Spearman’s correlation), internal consistency (Cronbach’s α) and test-retest (Wilcoxon test) were also analysed. Results: The CVI obtained was excellent. The instructions were considered clear, and COMACT-EP was found to be easy to complete and of adequate length. Most of its items were regarded as clear, easy to understand, and relevant. However, the need for improvement was also highlighted. It was suggested that certain items be added and others revised to enhance clarity. Test-retest results revealed stability in the majority of its items. The IC of the COMACT-EP categories “Talking” and “Listening” was found to be low, and the concurrent validity with the CDP-EP Activities subscale was somewhat limited. Conclusions: It is necessary to continue revising and rewording certain items of the COMACT-EP and to include a larger sample of PWA, representing a broader range of ethnic, geographic, and cultural backgrounds, as well as different types of aphasia and levels of severity.