bluuebunny/crossref_metadata_2025 huggingface dataset:
OCULAR MANIFESTATIONS OF PORETTI-BOLTSHAUSER SYNDROME: FINDINGS FROM MULTIMODAL IMAGING AND ELECTROPHYSIOLOGY
Authors: Cai Cindy X. , Go Michelle , Kelly Michael P. , Holgado Sandra , Toth Cynthia A.
Published: March 2020

Background/Purpose:

Poretti-Boltshauser syndrome is a rare, nonprogressive neurologic syndrome with characteristic cerebellar cysts on neuroimaging due to mutations in LAMA1. The ophthalmic findings in Poretti-Boltshauser syndrome are not well described. Here, we report the ophthalmic findings from multimodal imaging and electrophysiology of a patient with genetically confirmed Poretti-Boltshauser syndrome.

Methods:

A 3-year-old boy with confirmed mutations in LAMA1 underwent examination under anesthesia with electroretinography and multimodal imaging including fundus photography, fluorescein angiography, optical coherence tomography, and optical coherence tomography angiography.

Results:

Dilated fundus examination was notable for retinal vascular anomalies, including a large area of nonperfusion in the temporal macula with corresponding retinal thinning on optical coherence tomography. There was an absence of a distinct foveal avascular zone and decreased density of both the superficial and deep vascular plexuses in the macula on optical coherence tomography angiography. There was diffuse loss of choriocapillaris architecture and decreased choroidal thickness.

Conclusion:

Patients with Poretti-Boltshauser syndrome may possess chorioretinal thinning and retinal vascular abnormalities appreciable on examination and multimodal imaging. These findings suggest a role for LAMA1 in retinal and choroidal vascular development.
Are weather conditions associated with chronic musculoskeletal pain? Review of results and methodologies
Authors: Beukenhorst Anna L. , Schultz David M. , McBeth John , Sergeant Jamie C. , Dixon William G.
Published: March 2020

Many people believe that weather influences chronic musculoskeletal pain. Previous studies on this association are narratively reviewed, with particular focus on comparing methodologies and summarising study findings in light of study quality. We searched 5 databases (Medline, Embase, Web of Science, PsycINFO, and Scopus) for observational studies on the association between weather variables and self-reported musculoskeletal pain severity. Of 4707 located articles, 43 were eligible for inclusion. The majority (67%) found some association between pain and a weather variable. Temperature, atmospheric pressure, relative humidity, and precipitation were most often investigated. For each weather variable, some studies found an association with pain (in either direction), and others did not. Most studies (86%) had a longitudinal study design, usually collecting outcome data for less than a month, from fewer than 100 participants. Most studies blinded participants to study aims but were at a high risk of misclassification of exposure and did not meet reporting requirements. Pain severity was most often self-reported (84%) on a numeric rating scale or visual analog scale. Weather data were collected from local weather stations, usually on the assumption that participants stayed in their home city. Analysis methods, preparation of weather data, and adjustment for covariates varied widely between studies. The association between weather and pain has been difficult to characterise. To obtain more clarity, future studies should address 3 main limitations of the previous literature: small sample sizes and short study durations, misclassification of exposure, and approach to statistical analysis (specifically, multiple comparisons and adjusting for covariates).
Treatment of chronic neuropathic pain: purine receptor modulation
Authors: Jacobson Kenneth A. , Giancotti Luigino Antonio , Lauro Filomena , Mufti Fatma , Salvemini Daniela
Published: March 2020

Extracellular nucleosides and nucleotides have widespread functions in responding to physiological stress. The “purinome” encompasses 4 G-protein-coupled receptors (GPCRs) for adenosine, 8 GPCRs activated by nucleotides, 7 adenosine 5′-triphosphate-gated P2X ion channels, as well as the associated enzymes and transporters that regulate native agonist levels. Purinergic signaling modulators, such as receptor agonists and antagonists, have potential for treating chronic pain. Adenosine and its analogues potently suppress nociception in preclinical models by activating A1and/or A3adenosine receptors (ARs), but safely harnessing this pathway to clinically treat pain has not been achieved. Both A2AAR agonists and antagonists are efficacious in pain models. Highly selective A3AR agonists offer a novel approach to treat chronic pain. We have explored the structure activity relationship of nucleoside derivatives at this subtype using a computational structure-based approach. Novel A3AR agonists for pain control containing a bicyclic ring system (bicyclo [3.1.0] hexane) in place of ribose were designed and screened using an in vivo phenotypic model, which reflected both pharmacokinetic and pharmacodynamic parameters. High specificity (>10,000-fold selective for A3AR) was achieved with the aid of receptor homology models based on related GPCR structures. These A3AR agonists are well tolerated in vivo and highly efficacious in models of chronic neuropathic pain. Furthermore, signaling molecules acting at P2X3, P2X4, P2X7, and P2Y12Rs play critical roles in maladaptive pain neuroplasticity, and their antagonists reduce chronic or inflammatory pain, and, therefore, purine receptor modulation is a promising approach for future pain therapeutics. Structurally novel antagonists for these nucleotide receptors were discovered recently.
Influence of gut microbiota dysbiosis on brain function: a systematic review
Authors: Almeida Cátia , Oliveira Rita , Soares Raquel , Barata Pedro
Published: March 2020

Background:

For almost a century it has been recognized that human possess a varied and dens microbial ecosystem called the human microbiota, yet we are still beginning to understand many of the roles that these microorganisms play in human health and development. It is thought that under certain circumstances such as dysbiosis, the microbiota can cause diseases, where the central nervous system (CNS) has an important relevance and where the “gut-brain axis” will play a major role.

Aims:

This review investigated the influence of the gut microbiota on brain function, trying to demonstrate whether dysbiosis influences CNS diseases or whether it is the disease that causes dysbiosis, highlighting the existing literature within this field.

Methods:

We performed a systematic literature search in EMBASE, PubMed, and Cochrane combining the terms “gut microbiota,” “dysbiosis,” and “CNS diseases” to identify those whom reported some influence or relation between dysbiosis of gut microbiota and CNS diseases. For the present systematic review, we only included systematic reviews or meta-analysis.

Results:

The EMBASE, PubMed, and Cochrane were systematically searched, considering only systematic reviews or meta-analysis. Nine studies comprising 705 articles were included in this review. Those 9 systematic reviews consist in 2 about autism spectrum disorder, 1 in dementia, 1 in depression, 2 in autoimmune diseases, 1 in schizophrenia, and 2 in some altered brain function. Available data characterizing several neural diseases demonstrate a significant correlation between dysbiosis and CNS diseases, strengthen the evidence that dysbiosis of gut microbiota may correlate with abnormalities in CNS patients.

Conclusions:

Although there is a clear need for more investigations to better understand the role of the gut microbiota in CNS diseases, the modulation of the nervous system by the microbiota is clear, continuing to be the subject of continuous research. We need to fully understand the mechanisms by which the microbiota interacts with the human brain, and therefore what's the connection between dysbiosis and pathologies such depression, dementia, autism, or schizophrenia.
Progressing Toward the Application of Artificial Intelligence for Medical Equipment Replacement in Canadian Hospitals —An Idea From the Biomedical Engineering Department at Northern Health
Authors: Kanamala Nikhil
Published: March 2020

How do biomedical engineering departments in hospitals effectively utilize the Computerized Maintenance Management System (CMMS) databases to replace medical equipment? What are the current tools available that have enhanced the utilization of data in the CMMS database for better decision making? Applying artificial intelligence for better health technology management in areas such as maintenance scheduling and replacement is progressing. And adopting it for analyzing CMMS data and efficient medical equipment replacement is a good start.
Assessing mental health stigma: Nurse practitioners' attitudes regarding managing patients with mental health disorders
Authors: Young Cara C. , Calloway Susan J.
Published: March 2020

Background:

A contributing factor in the provision of suboptimal mental health care is the presence of stigmatizing attitudes among health care providers. Although numerous studies have investigated stigmatizing attitudes among physicians, nurses in psychiatric settings, and various populations of students, mental health stigma among nurse practitioners (NPs) has not yet been addressed.

Purpose:

The purpose of this study was to assess NPs' attitudes and beliefs regarding working with individuals with a mental health disorder.

Methods:

A cross-sectional descriptive design was used. Nurse practitioners attending a national practice conference completed a survey for demographic and practice information and a standardized measure for assessing stigmatizing attitudes regarding three mental health conditions: (a) anxiety/depression, (b) attention deficit hyperactivity disorder, and (c) substance use disorders (SUDs).

Results:

A total of 141 NPs participated in this study. The most favorable attitudes were reported for working with individuals with anxiety/depression, and the least favorable attitudes were reported for working with those with an SUD.

Implications for practice:

Stigmatizing attitudes are present among practicing NPs. Effective bias-reducing interventions are indicated to improve the patient-centered care NPs provide to individuals with mental health disorders.
Altered Expression of the Epithelial Mucin MUC1 Accompanies Endoscopic Recurrence of Postoperative Crohn’s Disease
Authors: Hashash Jana G. , Beatty Pamela L. , Critelli Kristen , Hartman Douglas J. , Regueiro Matthew , Tamim Hani , Regueiro Miguel D. , Binion David G. , Finn Olivera J.
Published: March 2020

Background:

MUC1-glycoprotein is expressed at low levels and in fully glycosylated form on epithelial cells. Inflammation causes MUC1 overexpression and hypoglycosylation. We hypothesized that overexpression of hypoglycosylated MUC1 would be found in postoperative Crohn’s disease (CD) recurrence and could be considered an additional biomarker of recurrence severity.

Methods:

We examined archived neo-terminal ileum biopsies from patients with prior ileocecal resection who had postoperative endoscopic assessment of CD recurrence and given a Rutgeerts ileal recurrence score. Consecutive tissue sections were stained using 2 different anti-MUC1 antibodies, HMPV that recognizes all forms of MUC1 and 4H5 that recognizes only inflammation-associated hypoglycosylated MUC1.

Results:

A total of 71 postoperative CD patients were evaluated. There was significant increase in MUC1 expression of both glycosylated/normal (P<0.0001) and hypoglycosylated/abnormal (P<0.0001) forms in patients with severe endoscopic CD recurrence (i3+i4), ileal score i2, compared with patients in endoscopic remission (i0+i1). Results were similar regardless of anti-TNF-α use. Although MUC1 expression and Rutgeerts scores were in agreement when characterizing the majority of cases, there were a few exceptions where MUC1 expression was characteristic of more severe recurrence than implied by Rutgeerts score.

Conclusions:

MUC1 is overexpressed and hypoglycosylated in neo-terminal ileum tissue of patients with postoperative CD recurrence. Increased levels are associated with more severe endoscopic recurrence scores, and this is not influenced by anti-TNF-α use. Discrepancies found between Rutgeerts scores and MUC1 expression suggest that addition of MUC1 as a biomarker of severity of postoperative CD recurrence may improve categorization of recurrence status and consequently treatment decisions.
Mild to moderate blepharoptosis correction
Authors: Pan Er , Chen Wen-li , Zhang Sheng-chang , Chen Yi , Yu Jian-gang
Published: March 2020

Conventional levator aponeurosis plication is a widely accepted technique for correction of mild to moderate ptosis. However, this method is associated with a high recurrence rate. The objective of this study was to investigate the clinical efficacy of levator aponeurosis posterior layer plication technique for correction of mild to moderate ptosis.

A convenience sampling approach was used to recruit 450 patients with mild to moderate blepharoptosis at the Guangzhou Eye-Nose-Face Aesthetic Plastic Surgery Hospital between August, 2015 and December, 2017. All participants were treated with levator aponeurosis posterior layer plication technique. The primary outcome was the postoperative change in marginal reflex distance 1 (MRD1). The paired t test was used to determine the clinical efficacy. Outcomes were assessed at 1 week, 1 month, 3 months, and 6 months after surgery.

The mean preoperative MRD1 was 1.7 ± 0.5 mm, and the mean postoperative MRD1 at 6-month follow-up was 3.7 ± 0.4 mm (P < .0001). According to the postoperative survey, 427 (94.9%) patients were satisfied with surgical outcomes.

This modified levator aponeurosis plication technique is a simple and effective procedure for correction of mild to moderate blepharoptosis. It results in good MRD1 and high patient satisfaction.
Fatal pediatric Stevens–Johnson syndrome/toxic epidermal necrolysis
Authors: Shi Tingting , Chen Huan , Huang Li , Fan Huifeng , Yang Diyuan , Zhang Dongwei , Lu Gen
Published: March 2020

Rationale:

Stevens–Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are extremely rare but potentially life-threatening disorders. We presented 3 fatal pediatric SJS/TEN cases.

Patient concerns:

Our patients had some severe complications such as septic shock, respiratory failure and obliterans bronchiolitis (BO) etc.

Diagnosis:

Three patients diagnosed SJS/TEN with clinical symptoms that were triggered by antibiotics, nonsteroidal anti-inflammatory drugs, previous infection, or neoplasms.

Interventions:

All of them accepted mechanical ventilation, intravenous immunoglobulin (IVIG), blood transfusion, glucocorticoid, and multi-anti-infectious therapy.

Outcomes:

They all died because of out-of-control severe infections. In Patient 1, he died 6 days after being admitted to the PICU on the 28th day from onset. In Patient 2, he died on the 211th day from the onset of illness during the third time of PICU admission. In Patient 3, she died 12 days after PICU admission on the 87th day from onset.

Lessons:

We should be aware that mucosal damage occurs on the skin and within the mucosa of visceral organs, leading to the occurrence of bronchiectasia, BO, enterocolitis, acute renal failure, and severe secondary infections. Establish a clinically predictive score that includes severe infection for pediatric patients to evaluate the risk of mortality in children in order to improve poor outcomes.
Pharmacokinetic evaluation of MFF in combinations with tacrolimus and cyclosporine. Findings of C0 and AUC
Authors: Radzevičienė Aurelija , Stankevičius Edgaras , Saint-Marcoux Franck , Marquet Pierre , Maslauskienë Rima , Kaduševičius Edmundas
Published: March 2020

We hypothesized that area under the concentration time curve (AUC(0-12)) is more accurate pharmacokinetic predictor vs trough level of mycophenolic acid (C0).

Study was performed at the University Hospital of Limoges (France) and included 238 renal recipients aged 22 to 82 years. Risk of nephropathy was evaluated by analyzing data of protocol biopsies according to the Banff 97 classification.

Assessment of immunosuppressants’ exposures was based on the calculation of the mean of AUC(0-12). The AUC(0-12) was estimated using a Bayesian estimator and a 3-point limited sampling strategy. Cyclosporine and tacrolimus analyses were performed using liquid chromatography–mass spectrometry method. The measurement of total mycophenolic acid was performed using a validated high-performance liquid chromatography method with ultraviolet detection. IBM SPSS 20.0 was used for statistical analysis.

The most accurate dosing of mycophenolate mofetil (MMF) was observed in patients receiving MMF with tacrolimus, 70.6% of patients’ AUC(0-12) exposures were within the therapeutic range. The highest rates of low dosing were observed in patients receiving MMF with cyclosporine, 30.9% of patients had AUC(0-12) exposures below the therapeutic range. The assessment of AUC(0-12) revealed 38% of chronic nephropathy cases, while C0 enables to identify only 20% of chronic nephropathy cases.

Probability test results showed that more likely AUC(0-12) and C0 will be maintained within the therapeutic width if patients receive MMF with tacrolimus vs MMF with cyclosporine: 0.6320 vs 0.6410 for AUC(0-12) determination and 0.8415 vs 0.4827 for C0 determination.

Combination of MMF with tacrolimus is dosed more precisely vs dosing of MMF with cyclosporine. 72 (70.6%) patients AUC(0-12) and 79 (77.5%) patients C0 out of 102 patients were within the therapeutic range. The AUC(0-12) monitoring of mycophenolic acid in patients receiving MMF with tacrolimus or in patients receiving MMF with cyclosporine enabled to identify more overdosing and possible risky cases.

Study results show that standard MMF dosing without monitoring and with mycophenolic acid level within the therapeutic width is possible and demonstrates less risky cases in patients receiving MMF with tacrolimus, while patients receiving MMF with cyclosporine should be intensively monitored to achieve the highest safety. However, AUC(0-12) monitoring is advised showing better compliance vs C0 monitoring.
Primary testicular diffuse large B-cell lymphoma
Authors: Wang Qi , Zheng Dafang , Chai Damin , Wu Shiwu , Wang Xiaolin , Chen Shaonan , Wu Linhui , Cao Ruoxue , Tao Yisheng
Published: March 2020

Rationale:

Primary testicular lymphoma (PTL) is a rare type of extranodal non-Hodgkin's lymphoma (NHL). Although data of PTL in patients with diffuse large B-cell lymphoma (DLBCL) are accumulating, there are still patients respond poorly to prognosis.

Patient concerns:

All patients had disease of the DLBCL subtype and those patients had primary involvement of the testis. In our studies, eleven patients had stage I/II disease, and 3 patients had advanced disease with B symptoms. Four patients exhibited a MYC+, BCL2+, and BCL6- expression pattern, 4 patients had a MYC+, BCL6+, and BCL2- expression pattern, and 3 patients had a MYC+, BCL2+, and BCL6+ expression pattern. Additionally, 43% (7/16) of PT-DLBCL patients had a germinal center B-cell-like (GCB) phenotype, while the others had a non-GCB phonotype.

Diagnoses:

In our case, most patients presented with unilateral painless scrotal swelling and the enlargement of the testicles in the first examination. After hospitalization, all patients underwent preoperative imageological examination of the testis and epididymis and postoperative revealed that all patients were the diffuse infiltration of a large number of anomalous lymphocytes. In addition, no invasion of other sites was observed within 3 months after diagnosis.

Interventions and outcomes:

Underwent orchiectomy on the affected side was performed by urologists after all patients were diagnosed with PTL. Meanwhile, some patients received at least one course of chemotherapy, or received postoperative combined RT and chemotherapy. Because of it particularity, nineteen instances of lymph node region involvement were discovered in 12 patients since the operation.

Lessons:

PT-DLBCL has unique biological characteristics, and its treatment modalities are becoming increasingly standardized. In the future, systematic interventions need to be actively considered in the early stages of PTL.
The association between HHEX single-nucleotide polymorphism rs5015480 and gestational diabetes mellitus
Authors: Wang Xingjie , Ding Yuanlin , Zhang Xinshan , Rao Jiawei , Yu Haibin , Pan Haiyan
Published: March 2020

Objective:

To evaluate the association between the rs5015480 single-nucleotide polymorphism of hematopoietically expressed homeobox (HHEX) and gestational diabetes mellitus (GDM) via meta-analysis.

Methods:

A comprehensive electronic search was performed of the PubMed, Springer, Science Direct, China National Knowledge Infrastructure (CNKI), Wanfang, and VIP databases for studies worldwide on the relationship between HHEX rs5015480 and GDM published up to July 2019. Rigorous inclusion and exclusion criteria were developed, and the quality of studies was assessed using the Newcastle–Ottawa scale, followed by heterogeneity evaluation using the Q test and I
2 statistic and data pooling. A meta-analysis was then performed on the included studies using RevMan 5.3.

Results:

A total of 4 eligible case–control studies were included, involving a total of 1651 patients and 3513 controls. The meta-analysis showed the following odds ratios: C allele vs T allele, 1.24 (95% confidence interval [CI]: 1.12–1.38); CC genotype vs TT genotype, 1.65 (95% CI: 1.26–2.17); CC genotype vs CT genotype, 1.22 (95% CI: 1.00–1.50); and CC genotype vs CT + TT genotype, 1.32 (95% CI: 1.09–1.61).

Conclusions:

HHEX rs5015480 represents a risk factor for the development of GDM, and pregnant women carrying the CC genotype have an increased risk of GDM.
Evidence on factors influencing contraceptive use and sexual behavior among women in South Africa
Authors: Hlongwa Mbuzeleni , Mashamba-Thompson Tivani , Makhunga Sizwe , Hlongwana Khumbulani
Published: March 2020

Introduction:

Contraceptive use and sexual health behavior remain a prominent public health concern in South Africa (SA). Despite many government interventions, unintended pregnancies and termination of pregnancies remain relatively high. This review aimed to map evidence on factors influencing contraceptive use and sexual behavior in SA.

Methods:

We conducted a scoping review guided by Arksey and O’Malley's framework. We searched for articles from the following databases: PubMed/MEDLINE, American Doctoral Dissertations via EBSCO host, Union Catalogue of Theses and Dissertations (UCTD) and SA ePublications via SABINET Online and World Cat Dissertations, Theses via OCLC and Google Scholar. Studies published from January 1990 to March 2018 were included. We used the Population, Concept, and Context (PCC) framework and the PRISMA chart to report the screening of results. The Mixed Method Appraisal Tool (MMAT) version 11 and ACCODS tools were used to determine the quality of the included studies.

Results:

A total of 2030 articles were identified by our search criteria for title screening. Only 21 studies met our inclusion criteria and were included in quality assessment stage. We found that knowledge of a contraceptive method, length of a relationship, sexual debut, age difference between partners availability of a contraceptive method, long waiting hours, and nurse's attitudes toward human immunodeficiency virus (HIV) positive or younger clients predict whether or not women use a contraceptive method or improve sexual behavior.

Conclusion:

There remains a necessity for improving educational programs aimed at transferring knowledge on contraceptives and sexual behavior to both women and their male counterparts, alongside the public health systems’ improvements.
Comparison of preoxygenation with a high-flow nasal cannula and a simple mask before intubation during induction of general anesthesia in patients undergoing head and neck surgery
Authors: Jo Jun-Young , Kim Wook-Jong , Ku Seungwoo , Choi Seong-Soo
Published: March 2020

Background:

To assess the arterial oxygen partial pressure (PaO2) at defined time points during preoxygenation and to compare high-flow heated humidified nasal oxygenation with standard preoxygenation using oxygen insufflation via a facemask for at least 5 minutes, before intubation during induction of general anesthesia.

Methods:

This randomized, single-blinded, prospective study will be conducted in patients undergoing head and neck surgery. After standard monitoring, the artery catheter at the radial artery or dorsalis pedis artery will be placed and arterial blood gas analysis (ABGA) for baseline values will be performed simultaneously. Each group will be subjected to 1 of 2 preoxygenation methods (high-flow nasal cannula or simple facemask) for 5 minutes, and ABGA will be performed twice. After confirming intubation, we will start mechanical ventilation and check the vital signs and perform the final ABGA.

Discussion:

This trial aims to examine the trajectory of PaO2 levels during the whole preoxygenation procedure and after intubation. We hypothesize that preoxygenation with the high-flow nasal cannula will be superior to that with the face mask.

Study registration:

This trial was registered with the Clinical Trial Registry (NCT03896906; ClinicalTrials.gov).
The roles of first phase, second phase insulin secretion, insulin resistance, and glucose effectiveness of having prediabetes in nonobese old Chinese women
Authors: Lu Chieh-Hua , Teng Sen-Wen , Wu Chung-Ze , Hsieh Chang-Hsun , Chang Jin-Biou , Chen Yen-Lin , Liang Yao-Jen , Hsieh Po-Shiuan , Pei Dee , Lin Jiunn-Diann
Published: March 2020

It has been established that prediabetes can causes significant comorbidities, particularly in the elderly. The deterioration of glucose metabolism are generally considered to be results of the impairment of the 4 factors: first, second insulin secretion (FPIS, SPIS, respectively), glucose effectiveness (GE), and insulin resistance. In this study, we enrolled older women to investigate their relationships with prediabetes.

Five thousand four hundred eighty-two nonobese, nondiabetic women were included. They were divided into normal glucose tolerance and prediabetes groups. Receiver operating characteristic curve was performed to investigate the effects on whether to have prediabetes for each factors. Two models were built: Model 1: FPIS + SPIS, and Model 2: model 1 + GE. The area under the receiver operating characteristic (aROC) curve was used to determine the predictive power of these models.

The aROC curve of GE was significantly higher than the diagonal line followed by SPIS and FPIS accordingly. The aROC curve of Model 1 (0.611) was not different from GE. However, Model 2 improved significantly up to 0.663. Based on this model, an equation was built (−0.003 × GE − 212.6 × SPIS − 17.9 × insulin resistance + 4.8). If the calculated value is equal or higher than 0 (≥0), then the subject has higher chance to have prediabetes (sensitivity = 0.607, specificity = 0.635).

Among the 4 factors, GE is the most important contributor for prediabetes in older women. By building a model composed of FPIS, SPIS, and GE, the aROC curve increased significantly. The equation built from this model could predict prediabetes precisely.
Over expression of CDK4 and MDM2 in a patient with recurrent ALK-negative mediastinal inflammatory myofibroblastic tumor
Authors: Hou Tien-Chi , Wu Pao-Shu , Huang Wen-Yu , Yang Yi-Ting , Tan Kien Thiam , Liu Shih-Hua , Chen Yu-Jen , Chen Shu-Jen , Su Ying-Wen
Published: March 2020

Rationale:

The diagnosis of anaplastic lymphoma kinase (ALK)-negative inflammatory myofibroblastic tumors (IMT) remains challenging because of their morphological resemblance with spindle cell sarcoma with myofibroblastic characteristics.

Patient concerns:

A 69-year-old female patient presented with loco-regional recurrent IMT several times within 8 years after primary treatment and neck lymph node metastasis 3.5 years after last recurrence.

Diagnosis:

The primary, recurrence, and lymph node metastasis lesions were diagnosed as ALK-negative IMTs based on the histopathological features.

Interventions:

Biopsy samples were obtained during repeated surgeries and evaluated for genomic alterations during first and recurrent presentations. The evaluation was done using pathway-driven massive parallel sequencing, and genomic alterations between primary and recurrent tumors were compared.

Outcomes:

Copy number gains and overexpression of mouse double minute 2 homolog (MDM2) and cyclin dependent kinase 4 (CDK4) were observed in the primary lesion, and additional gene amplification of Discoidin Domain Receptor Tyrosine Kinase 2 (DDR2), Succinate Dehydrogenase Complex II subunit C (SDHC), and thyroid stimulating hormone receptor (TSHR) Q720H were found in the recurrent tumors. Metastases to the neck lymph node were observed 3.5 years after recurrence.

Lessons:

Our results indicated genetic evolution in a microscopically benign condition and highlighted the importance of molecular characterization of fibro-inflammatory lesions of uncertain malignant potential.
Mid-lumbar traumatic spondyloptosis without neurological deficit
Authors: Xu Feng , Tian Zhisen , Fu Changfeng , Yao Liyu , Yan Mengjie , Zou Congcong , Liu Yi , Wang Yuanyi
Published: March 2020

Introduction:

Spondyloptosis is a form of vertebral dislocation and the most advanced form of spondylolisthesis. Traumatic spondyloptosis is usually caused by high-energy impact and results in unstable spine deformity and spinal canal deformation, which lead to severe spinal cord injury. Traumatic spondyloptosis is mostly reported in the lumbo-sacral junction, while it is rarely documented in mid-lumbar segments. To the best of the authors’ knowledge, only 16 cases of mid-lumbar spondyloptosis have been described previously. Herein, we present a L3 to L4 spondyloptosis case that did not involve neurological deficit.

Patient concerns:

A 42-year-old man presented to the emergency department after an accident involving a fall. The patient developed severe back pain and spinal deformity, while his neurologic function remained intact. Radiological examinations indicated complete posterior vertebral dislocation at L3 to L4 and a fracture at the bilateral pelvic ischial tuberosity without major vessel injury or severe dura sac compression.

Diagnoses:

L3 to L4 complete vertebral dislocation, pelvic ischial tuberosity fracture.

Interventions:

For treatment, the patient underwent fracture reduction, L3 to L4 intervertebral fusion, and internal fixation 7 days post-injury.

Outcomes:

Postoperative digital radiography showed the correction of the spinal deformity. The patient was pain-free and fully rehabilitated 3 months after the surgery. At the 1-year follow-up, the patient was completely asymptomatic and had achieved normal alignment.

Conclusions:

We reported an L3 to L4 traumatic spondyloptosis case that involved intact neurology, which is the first-ever reported mid-lumbar spondyloptosis case that involved complete posterior column and neural sparing. For the treatment of traumatic spondyloptosis without neurological deficit, restoring stability and preventing secondary cord injury should be taken into consideration.
Impact of HCV cure with drug-acting antivirals in the use of concomitant medication and lipid profile: follow-up data 2 years after the sustained virological response
Authors: González-Colominas Elena , Batlle Marc , Monge-Escartín Inés , Duran Xavier , Viu Anna , de Antonio-Cuscó Marta , Grau Santiago , Bessa Xavier , Carrión Jose Antonio
Published: March 2020

Background and aim:

Patients with chronic hepatitis C (CHC) frequently associated comorbidities and concomitant medication. Sustained virological response (SVR12) has been related to an increase in cholesterol serum levels and in peripheral vascular resistance. Our aim was to evaluate the impact of SVR12 on the use of concomitant medication and serum lipid profile.

Methods:

Prospective study including patients treated with direct-acting antivirals who had achieved the SVR12. Clinical data and concomitant drugs were analysed at baseline and at least 1 year after SVR12. Differences from baseline to follow-up in the concomitant medication were evaluated by Stuart–Maxwell test and lipid profile by Wilcoxon signed-rank test. Patients were categorized according to the increase/decrease in the number of drugs included in each class (Anatomical Therapeutic Chemical classification system).

Results:

Two hundred twenty-six patients with SVR12 were included, 73.5% were receiving concomitant drugs (49.6% with antihypertensive effect, 30.5% antacids, 16.4% anti-diabetic drugs, and 7.1% lipid-lowering agents). One year after SVR12, total cholesterol serum levels increased from 161 to 179 mg/dl (P < 0.001) and, after a median time of 25.7 months, the use of lipid-lowering drugs increased from 7.8 to 11.5% (P = 0.009). In addition, we observed a trend to use more antihypertensive drugs in older patients (P = 0.06), especially in those with cirrhosis. Anxiolytics decreased after SVR12 from 13.7 to 10.6% (P = 0.035).

Conclusion:

CHC cure is associated with a significant increase in cholesterol serum levels and the use of lipid-lowering agents, as well as the use of drugs with antihypertensive effect in older patients.
Why You Need a Nursing Strategic Plan
Authors: Lal M. Maureen
Published: March 2020

A successful nursing strategic plan establishes a roadmap for the future. It gives nurses a direction to follow and can refresh and reenergize an organization. A robust strategic plan is a critical component to ensure excellent patient care and the best possible outcomes. In this month's Magnet® Perspectives, we unpack the essential elements that make a strategic plan effective, how to create and implement a plan that will work for you, and how to engage nurses at all levels in the process. We explore how strategic planning is used to guide the work of Magnet-recognized organizations and support nurses with the tools and resources they need to contribute fully to the care team.
The Distinguished Nurse Clinician Academy
Authors: Burke Kathleen G. , Jablonski Juliane , Cunningham Regina
Published: March 2020

The complexity and growth of healthcare systems provide nurse leaders an opportunity to recognize and facilitate professional development for experienced frontline clinicians and to create formal structures that give expert direct care nurses a stronger voice. The purpose of this article is to describe how one health system developed and implemented an innovative Distinguished Nurse Clinician Academy. This elite academy demonstrates the longitudinal impact of a health systems' Magnet® culture.
Cell-Cycle Arrest Biomarkers: Usefulness for Cardiac Surgery-Related Acute Kidney Injury in Neonates and Infants*
Authors: Bojan Mirela , Pieroni Laurence , Semeraro Michaela , Froissart Marc
Published: March 2020

Objectives:

Cell cycle arrest urine biomarkers have recently been shown to be early indicators of acute kidney injury in various clinical settings in critically ill adults and children. The product of tissue inhibitor metalloproteinase -1 and insulin-like growth factor binding protein-7 concentrations/1,000 (TIMP-1) × (IGFBP-7) provides stratification of acute kidney injury-risk in adults with critical illness. The present study explores the predictive accuracy of (TIMP-1) × (IGFBP-7) measured early after cardiopulmonary bypass for cardiac surgery-related acute kidney injury in neonates and infants, a population in whom such data are not yet available.

Design:

Prospective, observational.

Setting:

A tertiary referral pediatric cardiac ICU.

Patients:

Fifty-seven neonates and 110 infants undergoing surgery with cardiopulmonary bypass.

Interventions:

None.

Measurements and Main Results:

(TIMP-1) × (IGFBP-7) was measured on the NephroCheck (Astute Medical, San Diego, CA) platform preoperatively, less than 1 hour of cardiopulmonary bypass and 1–3 hours of cardiopulmonary bypass. The incidence of postoperative acute kidney injury, dialysis, and/or death were compared among quintiles of postoperative (TIMP-1) × (IGFBP-7). Multivariable regression was used to assess the added predictive value for renal events of (TIMP-1) × (IGFBP-7) over clinical models. Basal (TIMP-1) × (IGFBP-7) increased with age at surgery (regression coefficient = 0.004 ± 0.001; p = 0.005). (TIMP-1) × (IGFBP-7) increased after cardiopulmonary bypass. Neonates had lower postoperative (TIMP-1) × (IGFBP-7) compared with older infants, despite undergoing longer surgeries and experiencing a higher incidence of postoperative renal events. (TIMP-1) × (IGFBP-7) was not associated with acute kidney injury, dialysis, and/or death and was not a predictor of the aforementioned events when added to a clinical acute kidney injury model including age, duration of cardiopulmonary bypass, and mechanical ventilation prior to surgery.

Conclusions:

These findings question the usefulness of (TIMP-1) × (IGFBP-7) for the prediction of cardiac surgery-related acute kidney injury in neonates and infants when measured within 3 hours of cardiopulmonary bypass.
Dose-Response Study on Surfactant Nebulization Therapy During Nasal Continuous Positive Airway Pressure Ventilation in Spontaneously Breathing Surfactant-Deficient Newborn Piglets*
Authors: Rey-Santano Carmen , Mielgo Victoria , Gomez-Solaetxe Miguel Angel , Ricci Francesca , Bianco Federico , Salomone Fabrizio , Loureiro Begoña
Published: March 2020

Objectives:

The current clinical treatment of neonates with respiratory distress syndrome includes endotracheal intubation and intratracheal instillation of exogenous surfactant. Nebulization of surfactant offers an attractive alternative. The aims of this study were to test nebulization as a noninvasive method of administering surfactant and determine the optimal dose for the treatment of respiratory distress syndrome–associated pathophysiology of the neonatal lungs.

Design:

Prospective, randomized, animal model study.

Setting:

An experimental laboratory.

Subjects:

Thirty-six newborn piglets.

Interventions:

Different doses (100, 200, 400, and 600 mg/kg) of poractant alfa were administered via a vibrating membrane nebulizer (eFlow-Neos; Pari Pharma GmbH, Starnberg, Germany) or a bolus administration using the intubation–surfactant–extubation (Insure) technique (200 mg/kg) to spontaneously breathing newborn piglets (n = 6/group) with bronchoalveolar lavage–induced respiratory distress syndrome during nasal continuous positive airway pressure (180 min).

Measurements and Main Results:

Pulmonary, hemodynamic, and cerebral effects were assessed. Histologic analysis of lung and brain tissue was also performed. After repeated bronchoalveolar lavage, newborn piglets developed severe respiratory distress syndrome. Rapid improvement in pulmonary status was observed in the Insure group, whereas a dose-response effect was observed in nebulized surfactant groups. Nebulized poractant alfa was more effective at doses higher than 100 mg/kg and was associated with similar pulmonary, hemodynamic, and cerebral behavior to that in the Insure group, but improved lung injury scores.

Conclusions:

In newborn piglets with severe bronchoalveolar lavage–induced respiratory distress syndrome, our results demonstrate that the administration of nebulized poractant alfa using an investigational customized eFlow-Neos nebulizer is an effective and safe noninvasive surfactant administration technique.
Decreased Brain Volumes and Infants With Congenital Heart Disease Undergoing Venoarterial Extracorporeal Membrane Oxygenation
Authors: Chao Brandon K. , Claessens Nathalie H. P. , Lim Jessie Mei , Gorodetsky Carolina , Au-Young Stephanie H. , Guerguerian Anne-Marie , Marini Davide , Blaser Susan , Shroff Manohar , Saini Amandeep K. , Seed Mike , Chau Vann , Miller Steven P.
Published: March 2020

Objectives:

The aims of this study were to: i) determine the spectrum of brain injury and ii) compare brain volumes between pre- and postoperative brain MRI in the infants receiving extracorporeal membrane oxygenation compared with those who did not require extracorporeal membrane oxygenation.

Design:

Cohort study of infants with d-transposition of the great arteries or single ventricle physiology. Brain volume (cm3) was measured using a segmentation of a volumetric T1-weighted gradient echo sequence. Brain imaging findings (intraventricular hemorrhage, white matter injuries, and stroke) were analyzed with respect to known clinical risk factors for brain injury and adverse neurodevelopmental outcomes. Clinical factors were collected by retrospective chart review. The association between brain volume and extracorporeal membrane oxygenation was evaluated using generalized estimating equations to account for repeated measures.

Setting:

Prospective and single-centered study.

Patients:

One hundred nine infants (median gestational age, 39.1 wk) with d-transposition of the great arteries (n = 77) or single ventricle physiology (n = 32) were studied pre- and postoperatively with MRI as per clinical protocol.

Interventions:

None.

Measurements and Main Results:

Of the 28 infants (26%) receiving extracorporeal membrane oxygenation, 19 (68%) were supported with extracorporeal membrane oxygenation once, and nine (32%) were supported 2–4 times. On postoperative MRI, new white matter injury was found in only five (17%) of the extracorporeal membrane oxygenation infants versus 40 (49%) in the non–extracorporeal membrane oxygenation group (p = 0.073). The rate of stroke (9% vs 10%), intraventricular hemorrhage (24% vs 29%), and hypoxic ischemia (3% vs 14%) did not differ between the non–extracorporeal membrane oxygenation and extracorporeal membrane oxygenation groups (all p > 0.5). Accounting for d-transposition of the great arteries or single ventricle physiology diagnosis, infants requiring extracorporeal membrane oxygenation had slower brain volume with single (β = –1.67) or multiple extracorporeal membrane oxygenation runs ([β = –6.54]; overall interaction p = 0.012).

Conclusions:

Patients with d-transposition of the great arteries or single ventricle physiology undergoing extracorporeal membrane oxygenation at our center have a similar incidence of brain injury but more significant impairment of perioperative brain volumes than those not requiring extracorporeal membrane oxygenation.
Isolation in Parents and Providers of Children With Chronic Critical Illness*
Authors: Wright-Sexton Laura A. , Compretta Caroline E. , Blackshear Chad , Henderson Carrie M.
Published: March 2020

Objectives:

An increasing number of children with medical complexity spend months or more in PICUs, lending to isolation for their parents and providers. We sought to better describe the experiences of parents and providers of children with chronic critical illness specifically around isolation during PICU admission.

Design:

In-person interviews and surveys of pediatric critical care providers and parents of children with chronic critical illness. Interview transcripts were analyzed for themes.

Setting:

Academic institution; PICU.

Subjects:

Seven PICU physicians, eight nurse practitioners, and 12 parents of children with chronic critical illness.

Interventions:

Surveys and semi-structured interviews.

Measurements and Main Results:

PICU providers acknowledge feeling medically isolated from children with chronic critical illness, fueled by a lack of chronic critical illness training and burnout. Providers also perceive medical isolation in parents of children with chronic critical illness manifesting as a declining level of parental engagement. Parents did not feel medically isolated in our study. Providers also perceive social isolation in families of children with chronic critical illness, identifying the child’s protracted disease and lack of tangible support systems as contributing factors. Parents self-reported adequate social supports but scored high on depression scales suggesting a disconnect between perceived and actual support. Both parents and providers acknowledge that the child’s chronic critical illness could be a source of support.

Conclusions:

PICU providers perceived social and medical isolation in parents of children with chronic critical illness; however, parents did not endorse either directly. A majority of parents showed signs of depression despite reporting good social support. Providers reported feeling medically isolated from children with chronic critical illness and their families related to burnout and insufficient training. Novel methods to address these issues are needed.
A Neurological Appearance of Celiac Disease
Authors: Cavusoglu Dilek , Olgac Dundar Nihal , Oztekin Ozgur , Arican Pinar , Gencpinar Pinar , Baran Masallah
Published: March 2020

Objective

Celiac disease may present with one or more neurological signs and/or symptoms. We aimed to define the incidence of accompanying neurological manifestations in children diagnosed as having celiac disease.

Methods

The prospective study included 146 children diagnosed as having celiac disease. The medical records (presentation symptoms, clinical findings, serological test, duodenal biopsy results, lack/deficiency of vitamin, tissue type, accompanying autoimmune disorders) and demographic data of all patients were also reviewed.

Results

Thirty-five (23.9%) of the 146 celiac patients exhibited one or more neurological findings. Headache (11.6%) and dizziness (6.1%) were the most common symptoms among neurological manifestations. There was a significant difference between the patients with and without neurological manifestations in terms of sex, biopsy result, and tissue type (P < 0.05). Moreover, there was a statistically significant difference between tissue types of the patients with and without headache (P < 0.05). We found that grade 3a by Marsh classification was the most common type among the patients with and without neurological findings in celiac disease. On neuroimaging evaluation of patients, 1 patient with chronic focal ischemic lesion, 1 patient with Chiari type 1 malformation, and 1 patient with subcortical white matter changes were identified.

Conclusions

Pathophysiology of neurological involvement in celiac disease is liable for various neurological findings. This study contributes to data suggesting that female sex, mild histopathological form, and human leukocyte antigen DQ2 heterozygosity are related to neurological manifestations, and also human leukocyte antigen DQ2 heterozygosity is associated with headache in celiac disease.