bluuebunny/crossref_metadata_2025_split huggingface dataset:
The functional effect of 3D-printing individualized orthosis for patients with peripheral nerve injuries
Authors: Chae Dong-Sik , Kim Da-Ham , Kang Kyung-Yil , Kim Doo-Young , Park Si-Woon , Park Sung-Jun , Kim Jae-Hyung 
Published: April 2020

Abstract

Rationale:
In the medical field, the use of 3-dimensional (3D) printing is increasing explosively and it is especially widespread in the clinical application of fabricating orthosis. Advantages of 3D-printed orthosis compared to conventional ones include its lower cost, easier modification, and faster fabrication. The 3D-printing technique makes it possible for physicians to easily create individual-tailored products. Recently, many kinds of orthosis through 3D printing have been studied and used. The knee orthosis, ankle-foot orthosis, wrist orthosis, hand orthosis, and foot orthotics are examples used in the rehabilitation fields of orthotics. We reported 3 cases of 3D-printed orthoses in patients with peripheral nerve injuries.

Patients concerns:
In spite of the rapid development of the clinical use of 3D printing, to our knowledge, its application to patients with peripheral nerve injuries has not yet been reported. Two patients suffered from upper limb problems and 1 patient had a foot drop associated with peripheral nerve injury.

Diagnosis:
Three patients diagnosed with median neuropathy, ulnar neuropathy, and right lower lumbar radiculopathy, respectively, by electromyography.

Interventions:
Herein we present 3 case reports of patients with peripheral nerve injuries whose orthotic needs were fulfilled with the application of 3D-printed wrist orthosis and ankle-foot orthosis.

Outcomes:
For hand function evaluation, we assessed the Jebsen–Taylor hand function test. Grasp and pinch powers were assessed by a hand dynamometer before and after orthosis application. For lower limb functional evaluation, we used a 6-minute walking test and modified Emory Functional Ambulation Profile for ambulatory function.

Lessons:
The 3D-printed orthosis could help functional improvement in patients with peripheral nerve injuries.
Impact of Epstein-Barr virus coinfection in Mycoplasma pneumoniae pneumonia
Authors: Xu Yingchun , Li Shuxian , Liu Jinling , Zhou Junfen , Jin Fang , Chen Xiaoyang , Wang Yingshuo , Jiang Yuan , Chen Zhimin 
Published: April 2020

Abstract

Mycoplasma pneumoniae (MP) is one of the most common pathogens of respiratory infection in children, while Epstein-Barr virus (EBV) infection is usually subclinical in immunocompetent children. Although single MP infection is common enough, MP and EBV coinfection have received little attention. Especially, the pathogenic role of EBV in lung when coinfection with MP, has not been clarified. The purpose of this study was to investigate the impact of EBV on MP pneumonia (MPP) in hospitalized children. We retrospectively reviewed the clinical data of MPP children who underwent screening for EBV by polymerase chain reaction in bronchoalveolar lavage fluid during hospitalization in 2014. Of total 147 patients, 68 patients were in the MP group and 79 were in the MP/EBV coinfection group. We found longer fever duration and higher CRP, IgA, IgG, interleukin-2 (IL-2), percentage of peripheral neutrophils levels, higher incidence of pulmonary consolidation and percentage of refractory MPP in coinfection group, when compared to those in MP group. In ROC curve analysis, IL-2 was useful for differentiating patients with coinfection from those with MP infection. Logistic regression analysis showed that the IL-2 ≥ 3.35 pg/ml (OR = 3.677) was a significant predictor regarding to MP/EBV coinfection. In conclusion, coinfection of EBV and MP poses a higher risk for prolonged symptoms. IL-2 could be used as a good predictor of coinfection.
Association of APC gene promoter methylation and the risk of gastric cancer
Authors: Zhou Xueliang , Jiao Dechao , Dou Mengmeng , Zhang Weijie , Hua Hao , Chen Jianjian , Li Zhaonan , Li Lifeng , Han Xinwei 
Published: April 2020

Abstract

Background:
The methylation status of the adenomatous polyposis coli (APC) promoter has been shown to be associated with the occurrence of gastric cancer, but this finding remains controversial. The aim of this study was to investigate the relationship between methylation of the APC gene promoter and gastric cancer.

Methods:
We searched the Web of Science, EMBASE, Medline, and Cochrane Central Register of Controlled Trials (CENTRAL) databases from the date of creation until August 1, 2019. According to the inclusion criteria, the relationship between the methylation status of the APC gene promoter and gastric cancer was investigated. The incidence of APC promoter methylation in the tissues or blood of patients with and without gastric cancer was compared. The results are expressed as the odds ratio (OR) and 95% confidence interval (CI). The pooled OR of each study was estimated using a fixed effects model or a random effects model to generate forest plots. We further validated the results using the MethHC database.

Results:
Eight studies (985 samples) were included. Our meta-analysis showed that the incidence of APC promoter methylation in patients with gastric cancer was higher than that of patients without gastric cancer (OR = 3.86, 95% CI 1.71–8.74, P = .001). Methylation of the APC promoter is associated with the incidence of gastric cancer, and it increases the risk of gastric cancer.

Conclusion:
This study provides a new strategic direction for research on gastric cancer. Methylation of the APC promoter may be a potential biomarker for the diagnosis of gastric cancer, but the results of this work require further confirmation.
Outcomes of patients with spinal metastases from renal cell carcinoma treated with conventionally-fractionated external beam radiation therapy
Authors: Lee Chia Ching , Tey Jeremy Chee Seong , Cheo Timothy , Lee Chau Hung , Wong Alvin , Kumar Naresh , Vellayappan Balamurugan 
Published: April 2020

Abstract
Renal cell carcinoma (RCC) has been traditionally thought to be radioresistant. This retrospective cohort study aims to assess the outcomes of patients with spinal metastases from RCC treated with conventionally-fractionated external beam radiation therapy (cEBRT) in our institution.
Patients diagnosed with histologically or radiologically-proven RCC who received palliative cEBRT to spinal metastases, using 3-dimensional conformal technique between 2009 and 2018 were reviewed. Local progression-free survival (PFS), overall survival (OS) and common terminology criteria for adverse events version 4.0-graded toxicity were assessed. Univariable and multivariable Cox proportional hazards regression analyses were performed to evaluate for predictors associated with survivals.
Thirty-five eligible patients with forty spinal segments were identified, with a median follow-up of 7 months (range, 0–47). The median equivalent dose in 2 Gy fractions (EQD2) was 32.5 Gy 10 (range, 12–39). Thirty-seven percent of patients underwent surgical intervention. At the time of last follow-up, all but 1 patient had died. Seven patients developed local progression, with the median time to local progression of 10.2 months. The median local PFS and OS were 3.3 and 4.8 months. There was no grade 3 or higher toxicity. A higher radiation dose (equivalent dose to 2 Gy fraction <32.5 Gy 10 vs ≥32.5Gy 10) (hazard ratio [HR], 0.47; 95% confidence interval [CI], 0.17–3.18; P-value (P) = .68) and spinal surgery (HR, 2.35; 95% CI, 0.53–10.29; P = .26) were not significantly associated with local PFS on univariable analysis. Multivariable analysis showed that higher Tokuhashi score (HR, 0.41; 95% CI, 0.19–0.88; P = .02), lower number of spinal segments irradiated (HR, 1.18; 95% CI, 1.01–1.37; P = .04) and use of targeted therapy (HR, 0.41; 95% CI, 0.18–0.96; P = .04) were independent predictors for improved OS.
For an unselected group of patients with RCC, there is no significant association between higher radiation dose and improved local control following cEBRT. This may be due to their short survivals. With the use of more effective systemic therapy, including targeted therapy and immunotherapy, survival will likely be prolonged. A tailored-approach is needed to identify patients with good prognosis who may still benefit from aggressive local treatments.
Surgical treatment of asymptomatic epithelioid hemangioendothelioma originating from the superior vena cava
Authors: Lee Seok Soo , Lee Jang Hoon 
Published: April 2020

Abstract

Rationale:
Epithelioid hemangioendothelioma is a rare endothelial tumor with a low-grade malignancy. This tumor can be treated with complete resection.

Patient concerns:
A 20-year-old Korean man visited our hospital due to an abnormal finding on standing chest PA X-ray. He did not have any past medical history.

Diagnosis:
Chest computed tomography shows a well-defined, oval-shaped tumor invading the brachiocephalic vein and superior vena cava. A malignant tumor of vascular origin was diagnosed by a percutaneous needle biopsy.

Interventions:
We performed en-bloc resection including the great vessels for complete resection of the tumor. Histologic evaluation confirmed the lesion to be a hemangioendothelioma and the surgical margins were free from tumor invasion.

Outcomes:
Fourteen days later, the patient was discharged without any complication. Thirty months after surgery, recurrences, or metastasis were not detected.

Lessons:
Epithelioid hemangioendothelioma is a rare malignant endothelial tumor in the central vein. Surgery is the treatment of choice and shows good results. We introduce and appropriate surgical method to ensure successful treatment for rare disease.
Postoperative Crohn's disease management
Authors: Barnes Edward L. 
Published: April 2020

Purpose of review
Despite multiple advances in the medical treatment of Crohn's disease, it is recognized that a significant proportion of patients will ultimately require surgical resection at some point during their disease course. This review focuses on the postoperative management of patients with Crohn's disease, with the goal of preventing endoscopic and clinical recurrence, and future surgery.

Recent findings
Surgery is an integral part of the multidisciplinary approach to the management of patients with Crohn's disease. Recent literature and guidelines have focused on a standardized and evidence-based approach to the management of patients in the postoperative setting. This approach should utilize risk stratification at the time of surgery, initiation of appropriate therapy among high-risk patients, and objective evaluation in the postoperative period, including ileocolonoscopy at 6–12 months after a surgical resection.

Summary
A standardized approach to the postoperative management of patients with Crohn's disease is critical. Identifying those patients at high-risk for endoscopic and clinical recurrence is essential, as these patients will likely require early medical therapy. While novel objective assessments are emerging, all patients should undergo ileocolonoscopy at 6–12 months and further decision making should be based on the presence of endoscopic recurrence.
Heart transplantation in cardiac storage diseases: data on Fabry disease and cardiac amyloidosis
Authors: Di Nora Concetta , Livi Ugolino 
Published: April 2020

Purpose of review
To deeply investigate one type of intracellular cardiac storage disease, the Fabry disease and one example of extracellular cardiac storage disease, the cardiac amyloidosis, with the aim to collect data about cardiac treatment at the end-stage level and the feasibility of heart transplantation (HTx) in this setting of patients.

Recent findings
Some registry describes that Fabry disease and cardiac amyloidosis showed similar characteristics as hypertrophic cardiomyopathies; thus, their correct diagnosis is often missing or time consuming. A multiorgan approach is mandatory to recognize the main systemic findings of these diseases, involving also the kidneys, the brain, the autonomous system and the skin.

Summary
The early diagnosis of these diseases is required to start as soon as possible the correct therapy for the patients affected. However, the end-stage heart failure is common and HTx could be offered to selected patients, especially if affected by light chain cardiac amyloidosis, to allow to perform the autologous stem cell transplantation after the cardiac transplant. Considering the Fabry disease patients, the enzyme recombinant therapy is also mandatory after HTx to avoid potential release of the systemic disease on the donor graft.
Current status of pediatric mechanical circulatory support
Authors: Spigel Zachary A. , Cho Junsang , Adachi Iki 
Published: April 2020

Purpose of review
The field of pediatric mechanical circulatory support has experienced exponential evolution in recent decades. With favorable complication profiles, implantable continuous-flow ventricular assist devices (VADs) have become a standard option in children, as has been seen in the adult counterpart. Nevertheless, there still exists room for further advances, not just for survival, but throughout the whole trajectory of treatment courses. With reviewing the current state of pediatric VAD support, including existing challenges, we aim to highlight the targets clinicians should focus on for further improvement of pediatric VAD support.

Recent findings
The field of pediatric VAD has been steadily growing, as evidenced by an increasing number of total VAD implants, particularly with continuous-flow VAD. Currently, HeartWare HVAD (Medtronic Inc., Mounds View, MN) is the most widely used continuous-flow VAD in children with excellent performance. However, only half of the children with HVAD are discharged home, which is drastically different from adult patients, suggesting that the pediatric field is still in the process of maturation. Additionally, outcomes of VAD support for complex congenital heart defect, particularly single ventricle physiology, remain suboptimal, despite an increasing number of such patients.

Summary
With the ongoing advancement, the field of pediatric VAD support is undergoing a rapid maturation process. This will eventually lead to further paradigm changes, including the use of VAD as permanent therapy.
Redefining marginality: donor lung criteria
Authors: Kukreja Jasleen , Chen Joy , Brzezinski Marek 
Published: April 2020

Purpose of review
Despite an increase in lung transplantation globally, the waitlist mortality persists due to organ shortage. In order to keep up with the demand, the marginal lung donor criteria need to be revisited. The goal of this review is to redefine the lung donor criteria based on the most recent data.

Recent findings
Recent evidence indicates that lungs from donor with traditional extended criteria such as, age more than 55, PaO
2/FiO2 threshold of less than 300, ischemia time more than 6 h, positive sputum microbiology, abnormal radiography no longer represent contraindications to lung transplantation by themselves. In addition, the introduction of new antiviral agents, organs from Hepatitis C positive donors, though not extended in the traditional sense, have shown positive outcomes.

Summary
The decision to use such organs, including those with smoking history, should be taken in totality weighing both donor and recipient characteristics. Hence, authors would advocate removing the term ‘marginal’ or ‘extended’ from lung donor criteria.
Celiac Disease in Patients With Cystic Fibrosis on Ivacaftor
Authors: Hjelm Michelle , Shaikhkhalil Ala K. 
Published: April 2020

ABSTRACTCystic fibrosis transmembrane conductance regulator (CFTR) protein modulators have revolutionized care for individuals with cystic fibrosis (CF) with positive effects on the gastrointestinal (GI) tract. There is emerging evidence linking CFTR dysfunction to celiac disease (CD). We present 3 cases of patients with CF, genotype F508del/G551D, treated with CFTR modulator, ivacaftor, and diagnosed with CD. These patients tested for CD because they had persistent GI symptoms that had partially improved with ivacaftor. This case series highlights the importance of a better understanding of how CFTR modulators impact the GI tract, their possible link to CD, and the importance of considering CD when evaluating GI symptoms in individuals with CF.
Mutations in Myosin 5B in Children With Early‐onset Cholestasis
Authors: Cockar Iram , Foskett Pierre , Strautnieks Sandra , Clinch Yasmin , Fustok Jana , Rahman Obydur , Sutton Harry , Mtegha Marumbo , Fessatou Smaragdi , Kontaki Elena , Papaevangelou Vassiliki , Deheragoda Maesha , Thompson Richard J. , Grammatikopoulos Tassos 
Published: April 2020

ABSTRACTObjectives:Mutations in Myosin 5B (MYO5B) are known to be associated with microvillous inclusion disease (MVID) a genetic cause of neonatal intractable diarrhoea. More recently, they have been reported in children with cholestasis but without typical gastrointestinal symptoms of MVID. We describe our series of children with cholestasis and mutations in MYO5B.Methods:Clinical, laboratory, and histological data were collected from patients with cholestasis and pathogenic mutations in MYO5B, found by next generation sequencing (NGS) but with minimal gastrointestinal disease.Results:Six patients (3 boys) were identified. Median age at presentation was 19 months (range, 3–92). Presenting features were jaundice, pale stools, pruritus, and failure to thrive. Patients 5 and 6 had intractable diarrhoea until the age of 3 and 7 years, respectively, but currently are on full enteral diet with no intestinal symptoms. Median values for serum total bilirubin were 55 μmol/L (2–500), alanine aminotransferase 73I IU/L (32–114), γ‐glutamyltransferase 7 IU/L (7–10), and serum bile acids 134 μmol/L (18–274). Three patients underwent 1 or more types of biliary diversion for symptom control. Median follow‐up was 5 years (2–22). At most recent follow‐up, they all reported pruritus while on antipruritics. Patient 1 had a liver transplant.Conclusions:We identified 6 patients, with mutations in MYO5B, early‐onset cholestasis and pruritus, with variable response to biliary diversion without typical MVID.
Children with Attention-deficit/Hyperactivity Disorder Show an Altered Eye Movement Pattern during Reading
Authors: Molina Rubén , Redondo Beatriz , Vera Jesús , García José Antonio , Muñoz-Hoyos Antonio , Jiménez Raimundo 
Published: April 2020

SIGNIFICANCE
Attention-deficit/hyperactivity disorder (ADHD) is characterized by oculomotor abnormalities. However, the eye movement pattern of children with ADHD during reading has yet to be fully determined. This investigation provides novel insights into the altered eye movement pattern during oral reading of nonmedicated children with pure ADHD in comparison with age-matched controls.

PURPOSE
The purpose of this study was to objectively compare the eye movement pattern during oral reading in a group of nonmedicated children with pure ADHD and an age-matched control group.

METHODS
Forty-one children, 21 children with pure ADHD (9.3 ± 2.2 years, 15 boys) and 20 control children (9.3 ± 2.5 years, 10 boys), orally read a standardized text according to their age while the eye movement pattern was objectively recorded using the Visagraph Eye Movement recording system.

RESULTS
The Bayesian statistical analyses revealed that children with ADHD exhibited a significantly higher number of fixations (Bayes factor 10 [BF10] = 3.39), regressions (BF10 = 9.97), saccades in return sweeps (BF10 = 4.63), and anomalies of fixations and regressions (BF10 = 3.66) compared with controls. In addition, children with ADHD significantly showed longer reading times (BF10 = 31.29), as well as lower reading rate (BF10 = 156.74) and grade-level equivalent (BF10 = 168.24) in comparison with controls.

CONCLUSIONS
Our data showed that the nonmedicated children with pure ADHD have an altered eye movement pattern during oral reading when compared with controls, which cannot be attributable to any comorbid condition. The present outcomes may help to understand the link between ADHD and reading performance and design the most pertinent strategies to enhance the reading skills of this population.
Comparison of Two Lighting Assessment Methods when Reading with Low Vision
Authors: Henry Rebecca , Duquette Josée , Wittich Walter 
Published: April 2020

SIGNIFICANCE
Lighting assessments are an important component of low-vision rehabilitation but are rarely studied systematically. Our study indicates that preferred lighting levels support improvements in reading ease and enjoyment, independently of the evaluation technique. To determine preferred illumination level and color temperature, the LuxIQ is quicker to administer and covers broad options of settings.

PURPOSE
The purpose of this study was to determine if preferred lighting, as assessed by the LuxIQ versus a standard lighting assessment, leads to better reading outcomes in individuals with low vision.

METHODS
Preferred lighting was assessed at home with visually impaired persons (mean age, 75.3 years), using the LuxIQ (n = 18) or a standard technique based on trying out light bulbs of various intensity and color temperature (n = 16). Maximum reading speed and reading acuity were measured before the lighting intervention and then under the preferred lighting conditions. A 1-month telephone follow-up evaluated the (1) compliance with the lighting recommendations and, for those who modified their lighting, (2) their level of satisfaction with the length of reading time and eye strain felt during reading.

RESULTS
Compared with usual lighting conditions, most participants preferred a cooler temperature at a higher illuminance level. Neither lighting assessment type appeared to lead to substantially improved objectively measurable reading outcomes. At the 1-month follow-up, 56% of the participants had modified their lighting, having a significant effect on satisfaction (P < .01), independent of assessment method. Of 18 respondents, 16 (87.5%) mentioned that reading was more enjoyable or easier with the lighting modifications.

CONCLUSIONS
Both lighting assessment methods lead to comparable results, but the LuxIQ is easier and faster to use. More research is needed to determine whether the LuxIQ is suitable to be incorporated into clinical practice.