bluuebunny/crossref_metadata_2025_split huggingface dataset:
Ruxolitinib treatment permits lower cumulative glucocorticoid dosing in children with secondary hemophagocytic lymphohistiocytosis
Authors: Chi Ying , Liu Rong , Zhou Zhi-xuan , Shi Xiao-dong , Ding Yu-chuan , Li Jian-guo 
Published: April 2021

Abstract
Background
This study aimed to analyze the effects of ruxolitinib on children with secondary hemophagocytic lymphohistiocytosis (HLH).

Methods
Eleven pediatric patients diagnosed with HLH and treated with ruxolitinib (ruxolitinib group: group R) between November 2017 and August 2018 were retrospectively analyzed. Eleven age-matched pediatric patients with HLH undergoing conventional treatment (control group: group C) during the same period were also analyzed.

Results
In group R, three patients who did not respond to methylprednisolone (MP) pulse and intravenous immunoglobulin (IVIG) therapies were treated with Ruxolitinib and their temperature decreased to normal levels. Four patients had normal temperature after conventional treatment (dexamethasone and etoposide, with or without cyclosporine A), but they had severe organ involvement, including obvious yellowing of the skin, increased liver enzyme levels and neuropsychiatric symptoms, and they were all ameliorated with ruxolitinib treatment. Four patients were relieved with ruxolitinib therapy alone. In group C, the body temperatures of eleven patients decreased to normal levels after conventional treatment. The body temperature of group R patients decreased to normal levels more rapidly than that of group C patients. The glucocorticoid dosage in group R was significantly lower than that in group C. Both groups were followed-up for 2–2.5 years. No obvious adverse drug reactions to ruxolitinib were observed during treatment and follow-up.

Conclusion
Ruxolitinib might be an effective drug in controlling body temperature and reducing inflammation indicators. It might be a potential replacement for glucocorticoid therapy for HLH treatment in children, thereby reducing or avoiding glucocorticoid-related adverse reactions.
Associations between lifestyle behaviour changes and the optimal well-being of middle-aged Japanese individuals
Authors: Takao Toshihiro , Sumi Naoki , Yamanaka Yoshiyuki , Fujimoto Sohachi , Kamada Tomoari 
Published: April 2021

Abstract
Background
Psychological well-being has been associated with reduced mortality rates in both healthy and diseased populations. However, there is considerably less evidence on the effect of lifestyle behaviours on positive health outcomes such as well-being. This study examines the association between lifestyle behaviours and optimal well-being.

Methods
From a total of 4324 Japanese individuals who participated in an annual health check-up in 2017, this study recruited 2295 participants (mean age: 49.3 ± 8.4 years; female: 54.3%) without a history of cerebrovascular, cardiovascular, or chronic renal disease and not on medication for hypertension, diabetes, or dyslipidaemia. The World Health Organization-Five Well-Being Index (WHO-5) scores were compared to self-reported scores on each of the following items: dietary habits, physical activity, smoking, alcohol consumption, and sleep quality. Logistic regression analysis was used to examine the association between optimal well-being (the top quartile of WHO-5 scores) and individual lifestyle behaviours. The association between change in dietary habits and physical activity from 2016 to 2017 and optimal well-being was also investigated.

Results
Good dietary habits and regular physical activity were associated with higher raw WHO-5 scores and were positively associated with optimal well-being after adjusting for age, sex, body mass index (BMI), and sleep quality. Raw WHO-5 scores were significantly higher in those who maintained good dietary and physical activity behaviours than in those who did not. Furthermore, maintaining regular physical activity for two years was positively associated with optimal well-being, after adjusting for age, sex, BMI, and sleep quality.

Conclusion
These results demonstrate that not only currently practising good dietary and physical activity behaviours but also maintaining such behaviours over time is associated with optimal well-being. Maintaining good lifestyle behaviours, particularly regarding physical activity, could potentially improve people’s well-being.
Evaluation of Mosquito Magnet and other collection tools for Anopheles mosquito vectors of simian malaria
Authors: Jeyaprakasam Nantha Kumar , Pramasivan Sandthya , Liew Jonathan Wee Kent , Van Low Lun , Wan-Sulaiman Wan-Yusoff , Ngui Romano , Jelip Jenarun , Vythilingam Indra 
Published: April 2021

Abstract
Background
Vector surveillance is essential in determining the geographical distribution of mosquito vectors and understanding the dynamics of malaria transmission. With the elimination of human malaria cases, knowlesi malaria cases in humans are increasing in Malaysia. This necessitates intensive vector studies using safer trapping methods which are both field efficient and able to attract the local vector populations. Thus, this study evaluated the potential of Mosquito Magnet as a collection tool for Anopheles mosquito vectors of simian malaria along with other known collection methods.

Methods
A randomized 4 × 4 Latin square designed experiment was conducted to compare the efficiency of the Mosquito Magnet against three other common trapping methods: human landing catch (HLC), CDC light trap and human baited trap (HBT). The experiment was conducted over six replicates where sampling within each replicate was carried out for 4 consecutive nights. An additional 4 nights of sampling was used to further evaluate the Mosquito Magnet against the “gold standard” HLC. The abundance of Anopheles sampled by different methods was compared and evaluated with focus on the Anopheles from the Leucosphyrus group, the vectors of knowlesi malaria.

Results
The Latin square designed experiment showed HLC caught the greatest number of Anopheles mosquitoes (n = 321) compared to the HBT (n = 87), Mosquito Magnet (n = 58) and CDC light trap (n = 13). The GLMM analysis showed that the HLC method caught significantly more Anopheles mosquitoes compared to Mosquito Magnet (P = 0.049). However, there was no significant difference in mean nightly catch of Anopheles mosquitoes between Mosquito Magnet and the other two trapping methods, HBT (P = 0.646) and CDC light traps (P = 0.197). The mean nightly catch for both An. introlatus (9.33 ± 4.341) and An. cracens (4.00 ± 2.273) caught using HLC was higher than that of Mosquito Magnet, though the differences were not statistically significant (P > 0.05). This is in contrast to the mean nightly catch of An. sinensis (15.75 ± 5.640) and An. maculatus (15.78 ± 3.479) where HLC showed significantly more mosquito catches compared to Mosquito Magnet (P < 0.05).

Conclusions
Mosquito Magnet has a promising ability to catch An. introlatus and An. cracens, the important vectors of knowlesi and other simian malarias in Peninsular Malaysia. The ability of Mosquito Magnet to catch some of the Anopheles mosquito species is comparable to HLC and makes it an ethical and safer alternative.

Graphical Abstract
H3K36 methylation reprograms gene expression to drive early gametocyte development in Plasmodium falciparum
Authors: Connacher Jessica , Josling Gabrielle A. , Orchard Lindsey M. , Reader Janette , Llinás Manuel , Birkholtz Lyn-Marié 
Published: April 2021

AbstractBackgroundThePlasmodiumsexual gametocyte stages are the only transmissible form of the malaria parasite and are thus responsible for the continued transmission of the disease. Gametocytes undergo extensive functional and morphological changes from commitment to maturity, directed by an equally extensive control program. However, the processes that drive the differentiation and development of the gametocyte post-commitment, remain largely unexplored. A previous study reported enrichment of H3K36 di- and tri-methylated (H3K36me2&3) histones in early-stage gametocytes. Using chromatin immunoprecipitation followed by high-throughput sequencing, we identify a stage-specific association between these repressive histone modifications and transcriptional reprogramming that define a stage II gametocyte transition point.ResultsHere, we show that H3K36me2 and H3K36me3 from stage II gametocytes are associated with repression of genes involved in asexual proliferation and sexual commitment, indicating that H3K36me2&3-mediated repression of such genes is essential to the transition from early gametocyte differentiation to intermediate development. Importantly, we show that the gene encoding the transcription factor AP2-G as commitment master regulator is enriched with H3K36me2&3 and actively repressed in stage II gametocytes, providing the first evidence ofap2-ggene repression in post-commitment gametocytes. Lastly, we associate the enhanced potency of the pan-selective Jumonji inhibitor JIB-04 in gametocytes with the inhibition of histone demethylation including H3K36me2&3 and a disruption of normal transcriptional programs.ConclusionsTaken together, our results provide the first description of an association between global gene expression reprogramming and histone post-translational modifications duringP. falciparumearly sexual development. The stage II gametocyte-specific abundance of H3K36me2&3 manifests predominantly as an independent regulatory mechanism targeted towards genes that are repressed post-commitment. H3K36me2&3-associated repression of genes is therefore involved in key transcriptional shifts that accompany the transition from early gametocyte differentiation to intermediate development.
Novel operative technique of advancement urethral meatoplasty utilizing buccal mucosa for Vulvar Paget’s disease with urethral invasion: two case reports
Authors: Nakamura Masaki , Hakozaki Yuji , Iwata Shohei , Sato Yusuke , Makino Katsuhiro , Kawai Taketo , Yamada Yuta , Yamada Daisuke , Suzuki Motofumi , Omatsu Jun , Abe Masanobu , Hoshi Kazuto , Kume Haruki , Igawa Yasuhiko 
Published: March 2021

Abstract
Background
Vulvar Paget’s disease (VPD) is a rare malignant disorder originating in the external genitalia. It occasionally invades into urethral or vaginal mucosa of female, making surgical treatment more complicating. In case of urethral invasion of Paget’s cells, systematic mapping biopsy of urethral mucosa is the standard of care to determine the range of surgical resection. Resection of urethral mucosa and simple skin grafting often result in urethral stricture after surgery, which severely deteriorates patient’s quality of life.

Case presentation
We applied a new technique of advancement urethral meatoplasty using buccal mucosa, in two Japanese cases of VPD with urethral invasion. After broad resection of vulvar skin together with the urethral mucosa, buccal mucosa was implanted between advanced urethral mucosa and skin graft. In both cases, we could prevent urethral stricture one year and two years after surgery, respectively.

Conclusion
This technique prevented urethral stricture after surgery and could be a useful technique as part of urethroplasty for VPD.
Septic shock due to Escherichia coli meningoencephalitis treated with immunoglobulin-M-enriched immunoglobulin preparation as adjuvant therapy: a case report
Authors: Pota V. , Passavanti M. B. , Coppolino F. , Di Zazzo F. , De Nardis L. , Esposito R. , Fiore M. , Mangoni di Santostefano G. S. R. C. , Aurilio C. , Sansone P. , Pace M. C. 
Published: March 2021

Abstract
Background
Gram-negative bacteria are an uncommon etiology of spontaneous community-acquired adult meningitis and meningoencephalitis. Escherichia coli is a Gram-negative bacterium that is normally present in the intestinal microbial pool. Some Escherichia coli strains can cause diseases in humans and animals, with both intestinal and extraintestinal manifestations (extraintestinal pathogenic Escherichia coli) such as urinary tract infections, bacteremia with sepsis, and, more rarely, meningitis. Meningitis continues to be an important cause of mortality throughout the world, despite progress in antimicrobial chemotherapy and supportive therapy. The mortality rate fluctuates between 15% and 40%, and about 50% of the survivors report neurological sequelae. The majority of Escherichia coli meningitis cases develop as a result of hematogenous spread, with higher degrees of bacteremia also being related to worse prognosis. Cases presenting with impaired consciousness (that is, coma) are also reported to have poorer outcomes.

Case presentation
We describe the case of a 48-year-old caucasian woman with meningoencephalitis, with a marked alteration of consciousness on admission, and septic shock secondary to pyelonephritis caused by Escherichia coli, treated with targeted antimicrobial therapy and immunoglobulin-M-enriched immunoglobulin (Pentaglobin) preparation as adjuvant therapy.

Conclusion
Despite the dramatic presentation of the patient on admission, the conflicting data on the use of immunoglobulins in septic shock, and the lack of evidence regarding their use in adult Escherichia coli meningoencephalitis, we obtained a remarkable improvement of her clinical condition, accompanied by partial resolution of her neurological deficits.
The use of immunotherapy treatment in malignant pheochromocytomas/paragangliomas: a case report
Authors: Rodriguez Robin Raquel , Rizwan Saleha , Alhamad Khaled , Finley Gene Grant 
Published: March 2021

Abstract
Background
Pheochromocytomas are a subset of paragangliomas, which are a rare group of neural crest cell-derived tumors. Malignant cases of both pheochromocytomas and paragangliomas are even rarer, and currently there is no standard of care. This case report details the use of off-label immunotherapy and its efficacy in the management of the aforementioned tumor.

Case presentation
Herein is presented a case of a 60-year-old Caucasian female with a rare malignant pheochromocytoma. The tumor was determined to be unresectable because of involvement of surrounding organs. Radiation therapy was also not a viable option because of concerns over appreciable toxicity in relation to mass size. As there is no standard of care for malignant cases, the patient was started on chemotherapeutic agents but was soon shown to be intolerant to this treatment. As she was ineligible for several clinical trials, the patient was started on the off-label immunotherapeutic agents nivolumab and ipilimumab. Immunotherapy use resulted in decreased tumor size, improved quality of life, and reconsideration for radiation therapy.

Conclusions
The use of immunotherapy in pheochromocytoma in this patient clearly demonstrated substantial benefit, as she was able to be reconsidered for radiation therapy. Not only has the patient been tolerant of this treatment, but she has exhibited progression-free survival of over 20 months. As there is no current standard treatment for malignant pheochromocytomas, the success of its use in this patient lends support to the ongoing clinical trials regarding the use of immunotherapy in rare tumors, including pheochromocytomas.
Epstein–Barr virus-positive lymphoproliferative disorder manifesting as pulmonary disease in a patient with acute myeloid leukemia: a case report
Authors: Dutta Ritika , Miao Susanna Y. , Phan Paul , Fernandez-Pol Sebastian , Shiraz Parveen , Ho Dora , Mannis Gabriel N. , Zhang Tian Y. 
Published: March 2021

Abstract
Background
Patients with lymphoproliferative disorders following hematopoietic stem cell transplant (HSCT) most commonly present with fever and lymphadenopathy within the first 5 months of transplant. Pulmonary post-transplant lymphoproliferative disorder (PTLD) is a particularly aggressive and rapidly progressive disease, with high morbidity and mortality. There are a very limited number of reported pulmonary PTLD cases following HSCT in patients with acute myeloid leukemia (AML). Early diagnosis and detection of pulmonary PTLD is critical given its high lethality. However, variable clinical presentations and nonspecific radiographic findings make pulmonary PTLD difficult to distinguish from other more common causes of pulmonary disease in AML patients.

Case presentation
Here, we describe a 68-year-old Caucasian man who presented for salvage induction therapy following relapse of his AML after a haploidentical allogeneic HSCT 10 months earlier. He developed recurrent fevers, dry cough, and hypoxemia, with chest computed tomography (CT) showing bibasilar consolidations and increased nodularity without increased lymphadenopathy. His symptoms initially improved with antibiotic and antifungal therapy, but his follow-up chest CT showed progression of disease despite symptomatic improvement. Epstein–Barr virus (EBV) was detected in his blood by polymerase chain reaction (PCR), and a lung biopsy revealed monomorphic PTLD with B cells positive for EBV. Unfortunately, the patient’s condition rapidly deteriorated, and he passed away prior to treatment initiation.

Conclusions
To our knowledge, this is the first reported case of an AML patient developing pulmonary PTLD relatively late in his post-transplant course in the setting of relapsed disease and salvage therapy. Pulmonary PTLD, a rare but highly lethal disorder, can imitate the symptoms and radiographic findings of pneumonia, a common diagnosis in immunocompromised AML patients. This case illustrates the importance of considering pulmonary PTLD in the differential diagnosis for pulmonary disease in AML patients with a history of HSCT, especially in the setting of progressive radiographic findings despite broad antibacterial and antifungal therapy. Further, our case demonstrates the importance of biopsy and uninterrupted EBV DNA monitoring in the definitive diagnosis of PTLD, given nonspecific symptomatology and radiographic findings.
Ophthalmological symptoms in a patient with reversible cerebral vasoconstriction syndrome: a case report
Authors: Hayashi Rijo , Hayashi Shimmin , Machida Shigeki 
Published: March 2021

AbstractBackgroundReversible cerebral vasoconstriction syndrome is characterized by severe headache with reversible segmental constriction of the cerebral arteries. We present details on a patient with reversible cerebral vasoconstriction syndrome who initially visited an ophthalmologist because of visual symptoms.Case presentationA 34-year-old Japanese woman complained of sustained headache and insomnia starting 2 days after her first childbirth. In addition to the severe headache, a visual field defect was also observed 10 days later. Best corrected visual acuity at the initial visit was 20/20 and 20/25 for the right and left eye, respectively. Exudative retinal detachment was noted surrounding both optic heads. Visual field testing revealed left homonymous hemianopsia, while magnetic resonance imaging demonstrated the presence of edema and infarction of the bilateral basal nuclei and right occipital lobe. The homonymous hemianopsia and exudative retinal detachment recovered immediately after treatment with a free-radical scavenger and anticoagulant. There has been no recurrence of symptoms during the 4 years of follow-up.ConclusionsWe report a case of reversible cerebral vasoconstriction syndrome with ophthalmological symptoms that were reversible, including serous retinal detachment and homonymous hemianopsia.
Chance diagnosis of mixed gonadal dysgenesis in an adult case of malignant gonadal germ cell tumor: a case report
Authors: Kurose Yoshiko , Nagai Tomonori , Shigematsu Kousuke , Uotani Takahiro , Akahori Taichi , Takai Yasushi , Seki Hiroyuki 
Published: March 2021

Abstract
Background
Mixed gonadal dysgenesis (MGD) is a subtype of the disorders of sex development (DSD) associated with sex chromosome abnormalities characterized by abnormal external genitalia, short stature, and primary amenorrhea. This disease is generally diagnosed from the neonatal stage to early childhood, and by puberty at the latest. Cases that are phenotypically female or those with ambiguous genitalia experience a high risk of gonadal tumor formation. As tumor risk is known to increase with age, prophylactic bilateral gonadectomy is recommended following early diagnosis.

Case presentation
Here we report a case of an adult Japanese woman diagnosed with MGD during treatment for a giant pelvic tumor. The patient initially visited a gynecology clinic during puberty for primary amenorrhea, at which time an abnormality was found with the external genitalia. However, a diagnosis of MGD was not made at this time, resulting in the development of a malignant gonadal germ cell tumor in adulthood.

Conclusions
For early diagnosis of MGD and the prevention of gonadal tumor formation, it is essential that gynecologists fully understand MGD and other DSD.
Perampanel overdose in low body mass index patients with epilepsy: a case report and review of the literature
Authors: Phabphal Kanitpong , Koonalintip Prut 
Published: March 2021

Abstract
Background
Perampanel (PER) is a novel antiepileptic drug (AED) which employs a completely different mechanism of action compared to existing medications. Overall, PER is considered to be safe up to a dose of 12 mg per day. When used to treat refractory and super-refractory status epilepticus, PER seems to be extremely well tolerated; this is true even when used at doses of up to 32 mg. There are currently only three case reports on the effects of acute PER overdose in epilepsy patients.

Case presentation
We report a 16-year-old Thai woman with a low body weight, who took PER at a dose of 40 times that of the prescribed daily dose. She experienced only an alteration of consciousness, without any systemic medical effects, and made a full recovery within 3 days without gastric lavage or specific treatment.

Conclusion
Our report demonstrates that an acute PER overdose may not produce serious adverse systemic effects. Individuals with adverse central nervous system (CNS) effects, such as altered consciousness, can experience a rapid recovery.
A hybrid access method for broadcasting of safety messages in IEEE 802.11p VANETs
Authors: Lei Xiaoying , Chen Xiangjin , Rhee Seung Hyong 
Published: April 2021

AbstractVehicular Ad-hoc Networks (VANETs) can improve the road safety by transmitting safety-critical messages such as beacons and emergency messages. IEEE 802.11p VANETs have adopted the carrier sense multiple access with collision avoidance (CSMA/CA) mechanism for the multiple access control. The 802.11p media access control (MAC) protocol, however, can not guarantee the reliability of broadcasting data, since the reception of transmitted messages are not acknowledged. Moreover, the backoff scheme of the 802.11p MAC utilizes a fixed-size contention window for safety message broadcasting, which causes high collision probabilities especially in dense environments. In order to improve such drawbacks, we propose a hybrid access method as follows: Nodes are equipped to reserve time slots for the next round of broadcasting, while unoccupied time slots are preserved for those which have emergency needs. In addition, implicit feedbacks are enabled for detecting collisions incurred during random channel accesses in preserved time slots. We devise a mathematical model which optimally controls the parameters of our scheme while minimizes the cost caused by idle channels and collisions. Extensive simulations show that our mechanism can remarkably improve the performance of VANETs in broadcasting of the safety messages.