cometadata/datacite-titles-descriptions-related-identifiers huggingface dataset:
DOI: 10.6084/m9.figshare.21975890
title: Supplementary Material for: Efficacy and Safety of a Persian Medicine Formula on Functional Dyspepsia Symptoms: A Randomized Double-blind Active-control Clinical Trial

description: Introduction: Functional Dyspepsia (FD) is one of the most frequent gastrointestinal disorders with a high burden. Although FD is a heterogeneous disorder without any standard therapy (treatment/treatment – repetition), there is a wide history of using herbal remedies for its treatment. Two of these herbal remedies quoted in various Persian medicine resources are Celery and Ajwain. Their effects have been investigated in recent clinical trials in FD patients. We aimed to compare the effect of Celery and Ajwain to that of Domperidone in patients with postprandial distress syndrome. Methods: This is a randomized double-blind active-control clinical trial, conducted at Kerman, Iran in 2020. Participants of this study were 100 patients suffering from FD (postprandial distress syndrome subtype). The intervention group received 1 g of the combination of Celery and Ajwain (Apium graveolens L. and Trachyspermum copticum (L.) Link) while the control group received 30 mg of Domperidone per day for 4 consecutive weeks. Primary outcomes were symptoms severity and frequency, and secondary outcomes were quality of life (QoL) and safety. All outcomes were assessed at the end of the trial, and also 4 weeks after the trial was finished. Results: No significant differences in symptoms frequency were found between groups. However, differences in symptoms severity were found at the end of the follow-up (week 8) (P= 0.001). The intra-group analysis showed a significant decrease in the symptoms severity and frequency at the end of week 4 in both groups (P&lt;0.001). QoL in the intervention group was higher compared to the control group at the end of the study (P&lt;0.001) and at the point of the follow-up (week 8; P&lt;0.001). No serious adverse events occurred in either group. Conclusion: The herbal mixture of Celery and Ajwain could be considered a safe and effective remedy, decreasing the severity of symptoms and increasing QoL in patients with postprandial distress syndrome. It is further suggested to confirm these findings in high-quality multi-center clinical trials.
DOI: 10.6084/m9.figshare.21981314
title: Supplementary Material for: Pulmonary Artery Intimal Sarcoma in a Patient with Lynch Syndrome: Response to an Immune Checkpoint Inhibitor

description: Intimal sarcoma is an extremely rare mesenchymal tumor arising in the great vessels. To date, intimal sarcoma has not been reported in patients with Lynch syndrome (LS), even though this syndrome lacks DNA mismatch repair ability genetically and is prone to various malignancies. This patient was diagnosed with LS by the Revised Amsterdam Criteria II, and she suffered from intimal sarcoma in the left pulmonary artery. She had a germline missense variant of <i>PMS2</i> (c.1399G&gt;A, pV467I) which is classified as a variant of unknown significance. In her intimal sarcoma, PMS2 expression was decreased. Additionally, it exhibited microsatellite instability and a high tumor mutational burden (69 mutations/Mb) which are features of mismatch repair deficiency, although <i>PMS2</i> (c.1399G&gt;A, pV467I) missense is a variant of unknown significance. The metastatic lesions of intimal sarcoma in this patient responded heterogeneously to pembrolizumab, an immune checkpoint inhibitor. Cytotoxic agents and radiation were also effective for some metastatic lesions, but some lesions, including her liver metastases, were resistant. The hypermutable nature of the LS genotype might acquire resistance to an immune checkpoint inhibitor and other cytotoxic agents such as occurred with her liver metastases.
DOI: 10.6084/m9.figshare.22006127
title: Supplementary Material for: Delayed diagnosis of indolent systemic mastocytosis as the cause of unexplained skin rash: a case report.

description: Mastocytosis is a heterogeneous group of disorders in which mast cells exhibit clonal proliferation that infiltrates one or more organs. In cutaneous mastocytosis, the mast cells infiltrate the skin only, whereas systemic mastocytosis is diagnosed when at least one extra-cutaneous site is involved with or without the skin being affected. Given the rarity of mastocytosis and the fact that skin rash can be a manifestation of different conditions and many clinicians aren’t familiar with this disorder, an accurate diagnosis may be delayed. We report a delayed diagnosis of indolent systemic mastocytosis in a 40-year-old gentleman who had been complaining of an unexplained skin rash for 6 years.
DOI: 10.6084/m9.figshare.22116200
title: Supplementary Material for: A recurrent Pleomorphic Xanthoastrocytoma in the cerebellum in a young adult: A case report and review of the literature.

description: Pleomorphic xanthoastrocytoma (PXA) is a rare glioma. It accounts for less than 1% of all astrocytomas. About 98% of PXAs originate supratentorially with the temporal lobe being the most common location. Cases of infratentorial PXAs are rarely reported in the medical literature. The tumor presents with a wide variation of symptoms based on the neuroanatomy involved with the location and size of the tumor, with seizures being the most reported symptom. The diagnosis depends on histological and clinical features along with radiologic features. We searched the keywords “Pleomorphic xanthastrocytoma, PXA, cerebellum, infratentorium, astrocytoma, gliomas” in the pubmed database; from 1979 to the current date, 28 cases were found in the medical literature featuring PXA in the infratentorium. We present the 29th case in the literature and the first in Syria. Our patient had a lesion in the right cerebellum and presented with a history of intermittent headache for 5 months followed by progressive gait disturbances and blurry vision and was misdiagnosed at the time of presentation with a high-grade glioma which is a common confusion because of the histological and clinical similarities. The patient underwent a suboccipital craniotomy and adjuvant therapy with a combination of radiotherapy and chemotherapy with Temozolomide (TMZ) was initiated at first. Then the patient presented with a relapse of symptoms and went through another surgery where frozen sections suggested the diagnosis of pleomorphic xanthoastrocytoma (PXA), further histopathological and immunohistochemical studies confirmed the diagnosis. Alongside highlighting the diagnostic challenge of this rare tumor, we did a brief review of the literature.
DOI: 10.6084/m9.figshare.22141409
title: Supplementary Material for: Olfactory Dysfunction, an Often Neglected Symptom of Hydrocephalus: Experience from a Case of Late-Onset Idiopathic Aqueductal Stenosis

description: Disturbance of smell is often accompanied with common neurodegenerative diseases such as Parkinson's and Alzheimer's disease. In addition, patients with head trauma, intracranial tumors, and hydrocephalus can also develop olfactory dysfunction, and some of which can improve with treatment of the underlying disease. In clinical practice, few patients complain of smell disturbances, thus olfactory dysfunction is often overshadowed by visible motor symptoms. Herein, we report a case of late-onset idiopathic aqueductal stenosis (LIAS), a rare form of adult-onset hydrocephalus in which olfactory dysfunction and gait disturbance was markedly improved after endoscopic ventriculostomy. This case report is expected to make more physicians aware that hydrocephalus can cause olfactory dysfunction and that it can be corrected postoperatively. Furthermore, in addition to motor and neuropsychological function, olfactory function test might be useful for functional assessment before and after surgical treatment of hydrocephalus.
DOI: 10.6084/m9.figshare.22141466
title: Supplementary Material for: Long-term safety, clinical and immunological outcomes in Primary Membranous Nephropathy with Severe Renal Impairment treated with cyclophosphamide and steroid-based regimen

description: Introduction and Aims Therapy of Primary Membranous nephropathy (PMN) with progressive advanced kidney dysfunction is challenging with limited literature and no clear therapeutic strategies. This is due to the scant evidence of effectiveness and uncertainty around the risk-benefit profile of immunosuppression (ImS) when eGFR is less than 30 ml/min. We aimed to determine long term clinical outcomes in patients with PMN and severe renal impairment treated with combined cyclophosphamide and steroids. Methods The study is a single-centre retrospective longitudinal cohort study. All patients (between 2004-2019) with biopsy confirmed PMN who initiated combination therapy with steroids and cyclophosphamide and had an eGFR of ≤30ml/min/1.73m2 at the time of initiation of therapy were included for analysis. Clinical and laboratory parameters including Anti-PLA2R-Ab were monitored as per standard clinical guidance. Primary outcome was achievement of partial remission. Secondary outcomes included immunological remission, need for renal replacement therapy, and adverse effects. Results Eighteen patients with median age of 68 (IQR 58-73) years and 5:1 M:F ratio received the combination therapy when eGFR was ≤30ml/min/1.73m2 (CKD-EPI). At time of immunosuppression, median eGFR and uPCR were 23 (IQR 18-27) ml/min/1.73m2 and 1000 (IQR 838-1285) mg/mmol respectively. Median follow-up was for 67 (IQR 27-80) months. 16 patients (89%) achieved partial Remission and 7 (39%) achieved complete remission. eGFR increased by 7ml/min/1.73m2 (27%) after one year of starting immunosuppression treatment and 12ml/min/1.73m2 at end of follow-up. Two patients (11%) developed end stage renal disease needing renal replacement therapy. 67% achieved both immunological and clinical remission. Two (11%) patients required hospitalization secondary to infections, 4 (22%) patients developed cancer and 4 patients died (22%). Conclusion Combination therapy with cyclophosphamide and steroids is effective in achieving partial remission and improving renal function in PMN with advanced renal dysfunction. Prospective controlled studies are required to provide further evidence and improve outcomes in such patients.
DOI: 10.6084/m9.figshare.22147211
title: Supplementary Material for: Relationship between cognitive decline and daily life gait among elderly people living in the community: A preliminary report

description: Introduction: Early detection and intervention are important to prevent dementia. Gait parameters have been recognized as a potentially easy screening tool for mild cognitive impairment (MCI); however, differences in gait parameters between cognitive healthy individuals (CHI) and MCI are small. Daily life gait change may be used to detect cognitive decline earlier. In the present study, we aimed to clarify the relationship between cognitive decline and daily life gait. Methods: We performed 5-cog cognitive function tests and daily life and laboratory-based gait assessments on 155 community-dwelling elderly people (75.5 ± 5.4 years old). Daily life gait was measured for 6 days using an iPod-touch with an accelerometer. Laboratory-based 10 m gait (fast pace) was measured using an electronic portable walkway. Results: The subjects consisted of 98 CHI (63.2%) and 57 cognitive decline individuals (CDI 36.8%). Daily life maximum gait velocity in the CDI group (113.7 [97.0-128.5] cm/sec) was significantly slower than that in the CHI group (121.2 [105.8-134.3] cm/sec) (p = 0.032). In the laboratory-based gait, the stride length variability in the CDI group (2.6 [1.8-4.1] was significantly higher than that in the CHI group (1.8 [1.2-2.7]) (p &lt; 0.001). The maximum gait velocity in daily life gait was weakly but significantly correlated with stride length variability in laboratory-based gait (ρ = -0.260, p = 0.001). Conclusion: We found an association between cognitive decline and slower daily life gait velocity among community-dwelling elderly people.
DOI: 10.6084/m9.figshare.22189009
title: Supplementary Material for: Treatment of unresectable cutaneous squamous cell carcinoma with cemiplimab in a patient on dialysis

description: Program death-1 inhibitors, a class of immune-checkpoint inhibitors, are now the standard of care in a variety of cancer settings, including cutaneous malignancies, such as melanomas, merkel cell, and squamous cell carcinomas (cSCC). The clinical trials that led to the approval of the programmed death-1 inhibitor, cemiplimab-rwlc (Libtayo®) for use in advanced cutaneous squamous cell carcinoma excluded patients with autoimmune disease, and those that required systemic immunosuppressive treatments, or had undergone solid-organ transplantation. Also, to be eligible, patients had to have adequate organ function. Here we present the first report of a patient that has been successfully treated with cemiplimab for locally advanced cSCC while simultaneously on dialysis for treatment of renal failure following renal transplant.
DOI: 10.6084/m9.figshare.22270024
title: Supplementary Material for: Patterns of Renal Dysfunction and Profile of Kidney Biopsies in Hematopoietic Stem Cell Transplant Recipients

description: Introduction: Post hematopoietic stem cell transplant (HSCT), kidney can be subjected to injury by various causes. Of these, Graft versus Host Disease (GvHD) affecting the kidney is an under-recognized entity with no clear guidelines on its diagnosis, clinicopathological manifestations and outcomes. Material and Methods: Out of 2930 patients who underwent HSCT at our centre between 2005 and 2020, kidney biopsy was performed in 19 allogenic and 5 autologous recipients. Results: The mean age of the cohort at transplant was 33.2 ± 7 years and 15 (62%) were males. Median time to kidney biopsy from HSCT was 14 (IQR, 9-30) months. Aplastic anemia was the most common underlying hematological disease (54.2%). All 19 allogenic recipients were classified based on clinicopathological manifestations into either thrombotic microangiopathy [TMA, 12/19 (63%)] or nephrotic syndrome [NS, 7/19 (37%)] pattern. Glomerular tuft ‘mesangiolysis’ was the dominant pattern of injury noted in 9/12 cases of TMA pattern. There was a predominance of acute microangiopathic changes restricted primarily to the glomerular compartment. Of the seven patients with NS pattern, membranous nephropathy (MN) was seen in 4 (57%) and minimal change disease (MCD) in 3 (43%) patients. Thirty nine percent (7/18) stained positive for C4d which was predominantly glomerular. Allogenic recipients who did not receive immunosuppression (IS) for renal disease had a lower eGFR at biopsy, a longer latency between withdrawal of GvHD prophylaxis and biopsy and were significantly at higher risk of kidney failure (IS: 2/11, 18.1% vs. No IS: 2/6, 33.3%, p=0.04). ‘Associated extra-renal GvHD’ occurred in 11/19 (57.9%) allogenic recipients. Patients with ‘associated extra-renal GvHD’ had significantly more death (6/11, 60% vs. 0, p=0.02) but comparable renal outcomes. Conclusion: Renal GvHD can present with or without ‘associated extra-renal GvHD’ after a prolonged period of withdrawal of GvHD prophylaxis, requiring careful diagnostic vigilance and consideration of immunosuppression.
DOI: 10.6084/m9.figshare.22339462
title: Supplementary Material for: Durable Response to Pazopanib in a Patient with Fumarate Hydrogenase-Mutant Tubulocystic Renal Cell Carcinoma

description: Tubulocystic carcinoma of the kidney (TC-RCC) is a rare renal tumor and has been recently included as a distinct entity in WHO classification of renal neoplasms. We report the case of a patient with metastatic tubulocystic RCC whose disease progressed through standard of care treatment for non-clear RCC. However, genomic profiling revealed mutant fumarate hydrogenase (FH) gene, and the patient had a sustained and durable response to pazopanib.
DOI: 10.6084/m9.figshare.22609072
title: Supplementary Material for: Exceptional response to pembrolizumab for treatment of metastatic chemorefractory endometrial carcinoma in a patient with Lynch Syndrome: A case report

description: Advanced endometrial cancer is associated with poor outcomes and few treatment options exist. Recently, the US Federal Drug Administration (FDA) approved pembrolizumab for the treatment of endometrial cancers that are deficient in mismatch repair and have high microsatellite instability (MSI). Lynch syndrome is an autosomal dominant disease that causes MSI-high endometrial cancer. We report a case of a 46-year-old woman with Lynch syndrome and advanced endometrial cancer who experienced progressive disease after treatment with chemotherapy with carboplatin and paclitaxel. She was then treated with single-agent pembrolizumab and had an exceptional response. She was noted to have a significant decrease in the size of a large uterine mass extending into the vagina and vulva, as well as decrease in the size of lymphadenopathy. Data are limited at this time for patients with Lynch syndrome treated with single-agent pembrolizumab. Our case report seeks to add to the body of literature that suggests that this patient population may particularly benefit from this novel therapy.
DOI: 10.6084/m9.figshare.22656721
title: Supplementary Material for: A 23-year-old male testis cancer with spontaneously ruptured teratocarcinoma and no history of trauma: A Case Reports

description: Teratocarcinoma is a type of testis cancer that can be represented in the youth population. It usually shows itself with swelling of the testis and edema and a rise of BHCG and alpha-fetoprotein, but spontaneous rupture is a rare manifestation. A case 23-year-old was referred to The Sina hospital with testis pain and swelling complaints. Laboratory finding was alpha f.p was more than 2000, BHCG titer 255.21, and LDH 504. sonography findings show the right testis had been detected with a heterogeneous mass with vascularity and cystic area with microcalcification and 76*69 mm. in during surgery, we faced with rupture tumor that was unusual and rare. The radical orchidectomy was done successfully without any complications. After the surgery, pathology showed teratocarcinoma of the right testis, and a six-month observation and follow-up were done without difficulty.
DOI: 10.6084/m9.figshare.22657444
title: Supplementary Material for: Patient Perspectives on Psoriatic Disease Burden: Results from the Global Psoriasis and Beyond Survey

description: Background: Patients’ understanding of the systemic nature of psoriatic disease (PsD) remains insufficiently explored. Objectives: To assess patients’ understanding of PsD, associated comorbidities, disease burden, and relationships with healthcare professionals (HCPs). Methods: Psoriasis and Beyond was a cross-sectional, quantitative online survey conducted in patients with a self-reported, physician-given diagnosis of moderate to severe psoriasis (body surface area [BSA] &gt;5% to &lt;10%, affecting sensitive and/or prominent body parts or BSA ≥10%) at its worst, with/without psoriatic arthritis (PsA). Patients were recruited through online panels by the Institut de Publique Sondage d'Opinion Secteur (Ipsos SA) and patient advocacy groups. Results: Overall, 4978 respondents with psoriasis completed the online survey from 20 countries across Australia, Asia, Europe, and the Americas; 30% of patients also reported having concomitant PsA. Overall, 69% of patients with psoriasis had heard that their disease was part of a systemic disease, and 60% had heard of the term “psoriatic disease”. Despite this, recognition of common manifestations and comorbidities associated with PsD was low. Among psoriasis-only patients (n=3490), 38% screened positive using the Psoriasis Epidemiology Screening Tool (PEST), indicative of potential PsA. Overall, 48% of patients reported that their disease had a very large to extremely large effect on quality of life (QoL; Dermatology Life Quality Index [DLQI] score, 11–30); only 13% of patients reported no impact of the disease on QoL (DLQI, 0–1). Most patients had experienced stigma and discrimination (82%) and a negative impact on relationships (81%) in their lives. Overall, 59% of patients were not involved in deciding their treatment goals; 58% of all treated patients (n=4757) and 64% of treated patients with concomitant PsA (n=1409) were satisfied with their current treatment. Conclusions: These results highlight that patients may not fully understand the systemic nature of their disease, were frequently uninvolved in deciding treatment goals, and were often not satisfied with their current treatment. Increasing patients’ participation in their care can facilitate shared decision-making between patients and HCPs, which may result in better treatment adherence and patient outcomes. Furthermore, these data indicate that policies should be implemented to protect against stigma and discrimination, which are commonly experienced by patients with psoriasis.
DOI: 10.6084/m9.figshare.22657624
title: Supplementary Material for: Temporary Severe Neutropenia during Administration of Atezolizumab: A Novel Case Report

description: Here we describe a case of temporary severe neutropenia after atezolizumab monotherapy and its treatment course. Atezolizumab monotherapy was introduced as a 6th line treatment for a man in his late 60s who was diagnosed with stage Ⅳ lung adenocarcinoma. The first treatment cycle was administered under hospitalization, and the patients presented with a fever of 37.8°C on the first day. The fever resolved after administration of acetaminophen and naproxen, and the white blood cell count, neutrophil count, and other white blood cell fractions were normal. However, grade 3 leukopenia and grade 4 neutropenia appeared at the beginning of the third cycle, and the treatment was suspended. After treatment, monocytes in the leukocyte fraction increased from approximately 10% to 25.6%. Lenograstim 100 μg subcutaneous injection and oral levofloxacin 500 mg once daily were started of onset of neutropenia, and he was hospitalized on the next day. Laboratory findings upon admission showed significant improvement to 5300/µl for leukocytes and 3376/µl for neutrophils. Lenograstim was discontinued, with no further decrease in the neutrophil counts. Atezolizumab therapy was resumed and there was no further reduction in leukocytes, neutrophils, or leukocyte fractions over about 2-year period. Concomitant drugs were maintained during atezolizumab treatment, suggesting that they did not induce neutropenia. In conclusion, we observed temporary severe neutropenia during atezolizumab monotherapy. Neutrophil recovery with cautious monitoring has enabled longer efficacy. We should consider temporary symptom occurrence in cases of haematological immune-related adverse events.