bluuebunny/crossref_metadata_2025_split huggingface dataset:
Extremely Early Onset Transthyretin Familial Amyloid Polyneuropathy with a Leu55Pro Mutation: A Pediatric Case Report and Literature Review
Authors: Lee Yun Jeong , Oh Jeeyoung , Hwang Su-Kyeung , Lee Eun Joo , Yang Dong Heon , Kim Yong-Jin , Kwon Soonhak , Hyun Myung Chul 
Published: July 2019

AbstractTransthyretin familial amyloid polyneuropathy (TTR-FAP) is a life-threatening autosomal dominant disease caused by the deposition of amyloid fibrils composed of TTR proteins. Symptoms of this disease include progressive sensorimotor neuropathy, cardiomyopathy, and involvement of other organs. We described a pediatric case of extremely early onset TTR-FAP with a TTR Leu55Pro mutation. A 17-year-old boy began to suffer from lower limb weakness, gait disturbance, and decreased sensation from 14 years of age onward. He presented with hypertrophic cardiomyopathy, periorbital and scleral ecchymosis, anhidrosis, orthostatic intolerance, and gastrointestinal autonomic dysfunction including nausea, vomiting, and diarrhea alternating with constipation. The patient's older sister had developed similar gastrointestinal symptoms from 20 years of age onward and was diagnosed as having hypertrophic cardiomyopathy. The boy's biopsy results showed infiltrated amyloid deposition on subcutaneous fat tissue and endocardium. Genetic analysis of the TTR gene demonstrated that both the patient and his sister had a pathogenic mutation, c.224T > C (Leu55Pro). Both patients were prescribed tafamidis, a TTR stabilizing agent. Although a majority of TTR-FAPs occur during adulthood, it should be suspected, even in pediatric populations, when symmetric length dependent neuropathy occurs in conjunction with a family history of neuropathy, autonomic neuropathy, and/or cardiomyopathy.
The Rho-family GTPase
OsRac1
controls rice grain size and yield by regulating cell division
Authors: Zhang Ying , Xiong Yan , Liu Renyi , Xue Hong-Wei , Yang Zhenbiao 
Published: July 2019

Significance
Feeding the increasing world population continues to be a great challenge in agriculture. In this study, we demonstrate that OsRac1 Rho-like GTPase from plants (ROP GTPase), an important signaling protein, promotes rice grain yield at least in part through its promotion of grain size. OsRac1 increases grain size by regulating OsMAPK6 and enhancing cell division, another signaling molecule recently shown to promote cell division and grain size in rice. Hence, this study has established a ROP GTPase signaling pathway that regulates grain size in rice, and has potentially illuminated a strategy for enhancing agricultural productivity.
Courthouse Size and Its Impact on Judicial Performance
Authors: Smith Sven , Askew Robert , Lang Emily , Smith Justin 
Published: July 2019

AbstractSectors of the criminal justice system have bureaucratized to such an extent that their management has supplanted the values for which they were created. Weber predicted this phenomenon, arguing that substantive rationality would be replaced by formal rationality as organizations grew. We test the relationship between size and these two types of rationality with the use of judicial performance checks in arraignments created from conversations with administrative courthouse staff and pilot observations at courthouses. We measure judicial performance through arraignment checklists (n = 481). Findings from multilevel models suggest that there is a positive relationship between size and formal rationality and a negative relationship between size and substantive rationality, even when controlling for workload by research design. Results suggest that abundance of formal rationality or substantive rationality facilitates injustice. These results begin a discourse encouraging quantitatively measuring formal and substantive rationality and that size be considered regarding judicial administrative policy.
Crystal melting processes of propylene carbonate and 1,3-propanediol investigated by the reed-vibration mechanical spectroscopy for liquids*
Authors: Wang Li-Na , Zhao Xing-Yu , Zhou Heng-Wei , Zhang Li , Huang Yi-Neng 
Published: July 2019

Abstract
The melting of crystals is one of the most common and general phase transition phenomena. However, the mechanism of crystal melting is not well understood, and more experimental measurements and explorations are still needed. The mechanical spectra of propylene carbonate and 1,3-propanediol during the crystal melting processes are measured by the reed vibration mechanical spectroscopy for liquids (RMS-L) for the first time. The experimental results show that as the temperature increases, the real part of the complex Young modulus first decreases slowly, and then quickly drops to zero; meanwhile, its imaginary part increases slowly at first, then goes up and drops quickly to zero, showing a peak of internal friction. Preliminary analyses indicate that both the real and imaginary parts can present some characteristics of the melting process, such as the transition from the disconnected liquid regions to the connected liquid regions, that from the connected crystal regions to the disconnected crystal regions, and so on. In addition, the results show that the melting rate per unit volume of crystalline phase versus temperature satisfies the Arrhenius relation at the initial stage of melting, and deviates from this relation as the temperature increases to a certain value. Therefore, the RMS-L will provide an effective supplement for the further study of melting.
Sperm chromosomal abnormalities and their contribution to human embryo aneuploidy
Authors: Rodrigo Lorena , Meseguer Marcos , Mateu Emilia , Mercader Amparo , Peinado Vanessa , Bori Lorena , Campos-Galindo Inmaculada , Milán Miguel , García-Herrero Sandra , Simón Carlos , Rubio Carmen 
Published: July 2019

AbstractIn this work we reviewed 18 years of experience using fluorescence in situ hybridization (FISH) for sperm aneuploidy testing. We evaluated parameters associated with increased numerical sperm chromosome abnormalities and determined the male contribution to embryo aneploidies in terms of reproductive outcome by increased sperm aneuploidy. This retrospective study analyzed data from 2008 sperm samples of infertile males undergoing FISH analysis because of clinical history of repetitive implantation failure, recurrent miscarriage, impaired sperm parameters, or mixed causes. Sperm concentration was the only sperm parameter associated with FISH results—we observed a gradual increase of abnormal sperm FISH results in males with decreasing sperm concentration. However, a great proportion of normozoospermic males also showed increased sperm aneuploidies, suggesting that sperm parameters alone do not enable identification of a substantial proportion of infertile males at risk of sperm aneuploidies. Regarding reproductive outcomes, couples with normal sperm FISH results for the male had similar outcomes regardless of conventional in vitro fertilization (IVF), intracytoplasmic sperm injection (ICSI), or preimplantation genetic testing for aneuploidies (PGT-A). However, couples with abnormal sperm FISH results for the male showed better clinical outcomes after PGT-A, suggesting a potential contribution of sperm to embryo aneuploidy. Moreover, PGT-A cycles showed better clinical outcomes when 24 chromosomes were analyzed by array comparative genome hybridization (aCGH) or next-generation sequencing (NGS) instead of only nine chromosomes analyzed by FISH. In conclusion, sperm FISH analysis offers clinical prognostic value to evaluate reproductive possibilities in infertile couples. Therefore, couples with abnormal sperm FISH results should be offered genetic counseling and presented with clinical options such as PGT-A.
Cholesterol-crystalized coronary atheroma as a potential precursor lesion causing acute coronary syndrome: a case report
Authors: Sugane Hiroki , Kataoka Yu , Otsuka Fumiyuki , Yasuda Satoshi 
Published: July 2019

AbstractBackgroundHistopathological studies have reported the presence of cholesterol crystals in the culprit lesion in patients with sudden cardiac death. Given that cholesterol crystals themselves promote pro-inflammatory cascades, they may destabilize atherosclerotic plaques, leading to the occurrence of acute coronary events.Case summaryA 60-year-old man presented with ST-segment elevation myocardial infarction. Emergent coronary angiography revealed a severely stenotic lesion (=culprit lesion) and another non-obstructive lesion in the proximal and middle segments of the left anterior descending artery (LAD), respectively. Optical coherence tomography (OCT) imaging showed that both lesions exhibited lipid-rich plaque with cholesterol crystals, and the non-obstructive lesion in the mid-LAD did not have a thin fibrous cap (its thickness = 230 μm). A drug-eluting stent was successfully implanted at the culprit lesion in the proximal LAD. On non-contrast T1-weighted magnetic resonance imaging performed 10 days after percutaneous coronary intervention (PCI), a high-intensity signal was identified at the non-obstructive mid-LAD lesion. This lesion was medically managed with aspirin, clopidogrel, and rosuvastatin due to the absence of myocardial ischaemia. However, 12 months after PCI, the patient was hospitalized again due to unstable angina pectoris. Coronary angiography revealed substantial progression of the mid-LAD lesion. Optical coherence tomography imaging prior to the second PCI showed a severely narrowed lesion containing cholesterol crystals and covered by organized thrombus. This lesion harbored an extensive amount of lipidic materials on near-infrared spectroscopy (maximum 4-mm lipid core burden index = 809).DiscussionIn our case, atherosclerotic plaques containing cholesterol crystals was associated with the occurrence of acute coronary syndrome. Our findings suggest that plaque with cholesterol crystals is a potential precursor to future acute coronary events.
Music Technology and Alternate Controllers for Clients with Complex Needs
Authors: Ward Asha , Davis Tom , Bevan Ann 
Published: July 2019

Abstract
Music technology can provide unique opportunities to allow access to music-making for clients with complex needs. While there is a growing trend of research in this area, technology has been shown to face a variety of issues leading to underuse in this context. This literature review is a collation of information from peer-reviewed publications, gray literature, and practice. Focusing on active music-making using new types of alternate controllers, this review aims to bring together information regarding the types of technology available, categorizes music technology and its use within the music therapy setting for clients with complex needs, catalogues work occurring within the field, and explores the issues and potentials surrounding music technology and its use in practice.
Primary intestinal lymphangiectasia in a 23-month- old girl
Authors: Mohammad Lava , Omran Razan , Ibrahim Alexandr , Hourieh Mohammad Adib , Ibrahim Ali 
Published: July 2019

Abstract
Primary intestinal lymphangiectasia (PIL) is a rare protein-losing gastroenteropathy which is defined as dilation of existing mucosal, submucosal, or subserosal lymphatics within the gastrointestinal tract. That causes loss of lymph fluid into the gastrointestinal tract, leading to the development of hypoproteinemia, edema, lymphocytopenia, hypogammaglobinemia, and immunologic anomalies. It is usually diagnosed in patients younger than 3 years old and is rarely first diagnosed in adulthood. Here we have a case report in a 23-month- old female presented with the complaint of peripheral edema and diarrhea. The diagnosis of PIL was made through upper gastrointestinal endoscopy and pathology histologic analysis. Patient placed on oral supplements of medium-chain triglycerides, a high protein diet, supplements of fat-soluble vitamins and responded well.
Follow the complex bread crumbs: A review of autoinflammation for the general paediatrician
Authors: Tsoukas Paul , Laxer Ronald M 
Published: July 2019

Abstract
Autoinflammatory diseases have emerged as a group of disorders that have significant morbidity, and even mortality. Since their onset predominately occurs during childhood, it is important that paediatricians are aware of what these diseases are, how they present, when to include them in differential diagnoses, and when to refer to a specialist. This review will focus on the clinical indicators suggestive of autoinflammatory disease, how the presence of an autoinflammatory disease may influence routine care, indications for immediate referral, and both their acute and chronic complications.
Faculty Ability to Care and Caring Characteristics in School of Nursing Climate: A National Survey
Authors: Hayne Arlene N. , Schlosser Sharron P. , McDaniel Gretchen S. 
Published: July 2019

Abstract

AIM
This study surveyed faculty ability to care and perception of caring characteristics within school of nursing climate.

BACKGROUND
Caring within schools of nursing is not well understood.

METHOD
A stratified, random, multistage cluster national sample of 2,043 faculty were surveyed using the Caring Ability Inventory and the Caring Characteristics Within the School of Nursing Climate instruments.

RESULTS
No significant differences on the Caring Ability Inventory were identified by school type. Significant differences were noted in faculty perceptions of a caring climate by school type. State universities and community colleges scored lower on the Trust/Teamwork, Support, and Respect subscales and total Caring Characteristics Within the School of Nursing Climate. Community college scores were lower than private, not-for-profit, and state university scores (p < .05) on the Valued subscale.

CONCLUSION
Further research is needed to describe and evaluate the caring climate within schools of nursing and the effectiveness of specific strategies to facilitate caring in the school of nursing climate.
A Randomized Trial to Increase the Assessment Accuracy of Glaucoma and Optic Disc Characteristics by Orthoptists
Authors: Scheetz Jane , Koklanis Konstandina , McGuinness Myra , Long Maureen , Morris Meg E. 
Published: July 2019

Introduction:
The aim of this study was to determine the accuracy of orthoptists when examining the optic disc for signs of glaucoma, and to explore the impact of targeted clinical education on accuracy.

Methods:
In this randomized controlled trial, 42 monoscopic color optic disc images were presented to 46 orthoptists who assessed the likelihood of glaucoma as well as optic disc size, shape, tilting, vertical cup-to-disc ratio, cup shape, depth, presence of hemorrhage, peripapillary atrophy, and retinal nerve fiber layer. The level of agreement with specialist ophthalmologists was assessed. Participants were then randomly assigned to an experimental group (targeted postgraduate education on optic disc assessment) or to no intervention. The educational program was designed to increase knowledge of the characteristic features associated with glaucomatous optic neuropathy. All participants re-examined the included optic disc images after a period of 6 to 8 weeks. The primary outcome measure was a change in agreement between attempts.

Results:
The education group showed significant improvements between attempts for identifying hemorrhages (P = .013), retinal nerve fiber layer defects (0.035), disc size (P = .001), peripapillary atrophy (P = .030), and glaucoma likelihood (P = .023). The control group did not show any statistically significant improvement. The intervention group showed significantly more improvement when identifying hemorrhages (P = .013), disc size (P = .001), disc shape (P = .033), and cup shape (P = .020) compared with the control group.

Discussion:
Orthoptists who received additional postgraduate online education based on principles of adult learning were more accurate at assessing the optic disc for glaucoma. These results highlight the value of continuing education to optimize clinical practice in allied health professionals.
A Novel Laparoscopic Technique With a Bladder Peritoneum Flap Closure for Pelvic Cavity for Patients With Rigid Pelvic Peritoneum After Neoadjuvant Radiotherapy in Laparoscopic Extralevator Abdominoperineal Excision
Authors: Yang Tinghan , Wei Mingtian , Deng Xiangbing , Meng Wenjian , Wang Ziqiang 
Published: July 2019

BACKGROUND:
In laparoscopic extralevator abdominoperineal excision, reconstruction of the pelvic peritoneum helps to prevent the small intestine from adhering to pelvic wall tissues, thus avoiding small-bowel obstruction and perineal complications. However, there are difficulties in pelvic peritoneum closure if the patient has received preoperative radiotherapy and has a rigid pelvis. We report a novel laparoscopic method for pelvic peritoneum reconstruction using the bladder peritoneum flap in laparoscopic extralevator abdominoperineal excision after neoadjuvant radiotherapy.

TECHNIQUE:
After transection of the rectum, if the patient had a rigid pelvis, we chose to perform the novel technique for the pelvic peritoneum closure in a 3-step approach. The flap has an arch shape with the bottom at the anterior wall of the pelvic cavity entrance. The height of the arched flap is equal to the distance from the bladder to the sacral promontory. The peritoneum was incised with electrocautery at the planned level and peeled off the bladder. The bladder peritoneum flap was then rotated to cover the entrance of the pelvic cavity and sutured to the brim of the pelvis.

RESULTS:
Acceptable postoperative short-term and long-term outcomes (5- to 22-month follow-ups) were achieved in 3 patients who underwent bladder peritoneum flap closure.

CONCLUSIONS:
The bladder peritoneum flap appears to be safe and feasible for intracorporeal closure of the pelvic cavity in laparoscopic extralevator abdominoperineal excision after neoadjuvant radiotherapy. The procedure provides a novel option for patients with severe fibrosis of the pelvis when another peritoneum reconstruction method is not feasible.
Increased Arbekacin Clearance in Patients With Febrile Neutropenia
Authors: Nakayama Takahiro , Chuma Masayuki , Tochikura Naohiro , Iwabuchi So , Suzuki Shinichiro , Matsumoto Chiaki , Imai Toru , Hamada Takashi , Nakagawa Masaru , Takahashi Hiromichi , Uchino Yoshihito , Miura Katsuhiro , Iriyama Noriyoshi , Hatta Yoshihiro , Takei Masami , Kimura Takahisa 
Published: July 2019

Background:
Arbekacin (ABK) is used to treat infections caused by methicillin-resistant Staphylococcus aureus and is used widely for the treatment of febrile neutropenia (FN). As ABK has a narrow therapeutic concentration window, the dosage must be adjusted via therapeutic drug monitoring. However, the influence of the physiology of patients with FN on the pharmacokinetic (PK) parameters of ABK remains unclear. Therefore, we examined this influence on ABK PK parameters.

Method:
We performed a retrospective cohort study using data from patients with a hematologic malignancy who were ≥18 years and had been administered ABK. We excluded patients who did not receive therapeutic drug monitoring and had an estimated glomerular filtration rate (eGFR) of <30 mL/min, because clinically sufficient data would not be available.

Result:
Of the 99 enrolled patients, 25 did not have FN and 74 had FN. Arbekacin clearance (CLabk) was shown to correlate with eGFR in patients with FN (r = 0.32, P = 0.0062) and without FN (r = 0.50, P = 0.01). CLabk was higher in patients with FN than in those without FN. In addition, in the eGFR of <100 mL/min group (normal renal function), CLabk and CLabk/eGFR were also higher in patients with FN than in those without FN.

Conclusions:
CLabk was increased in patients with FN and normal renal function; therefore, we propose an increased ABK dose for patients with FN and normal renal function.
Teledermatology Viewpoint
Authors: Martin Kari L. 
Published: July 2019

ABSTRACT
Teledermatology is a term to describe the provision of dermatologic medical services through telecommunication technology. In store-and-forward teledermatology, patient medical information (including history and visual data) obtained in one location is transferred electronically to a provider who is in another location (Roman & Jacob, 2014). The construct of this column is such that cases are presented in a standardized teledermatology reader format. This is a case of erythematous plaques with scattered erosions in the perineum.
Evaluation of the Effect of Diclofenac Sodium and 5-Fluourasil in a 3D Cholesteatoma Cell Culture Model
Authors: Kara Ahmet , Duman Busra Oncel , Yazır Yusufhan , SinanYilmaz Mahmut , Halbutogulları Zehra Seda Unal , Demir Deniz , Kara Rabia Oztas , Bayraktar Hakan , Guven Mehmet 
Published: July 2019

Introduction:
Middle ear cholesteatoma is a benign disease with invasive and destructive clinical behaviors. It increases the rate of both chronic otitis media complications and revision surgeries. The most effective treatment of middle ear cholesteatoma is surgical excision, and there is no medical treatment for this disease. Exploring new medical treatment options may help to create treatment alternatives instead of surgery.

Materials and Methods:
Required cholesteatoma tissues for cell culture were excised from 4 different participants who underwent surgery in our clinic and agreed to give tissue for the study. Cholesteatoma-derived keratinocytes and fibroblasts were cocultured in temperature-sensitive culture dishes to make a three-dimensional (3D) cholesteatoma model. Then, the effects of 1% and 2% diclofenac sodium on viability and cell proliferation rates were examined using WST-1 and annexin-V tests.

Results:
Cell viability and proliferation rates were found to be lower and apoptosis rates were higher in the diclofenac sodium group versus the negative and positive control groups.

Conclusion:
In this present study, we described a new 3D cholesteatoma cell culture model developed using cell sheet technology and demonstrated the efficacy of diclofenac sodium on cholesteatoma for the first time in the literature. It may be used in patients with chronic otitis media with cholesteatoma, but further studies investigating ototoxic and neurotoxic effects of this molecule are needed.