Molecularly Confirmed Pseudomyogenic Hemangioendothelioma with Unusual EGFL7::FOSB Fusion in the Head and Neck Region of an Older Patient.

Pseudomyogenic hemangioendothelioma (PMHE), a rare vascular neoplasm, was first described in 1992 asa fibroma-like variant of epithelioid sarcoma, and would be termed as epithelioid sarcoma-like hemangioendothelioma a decade later due to its significant histologic overlap with epithelioid sarcoma and diffuse cytokeratin expression. PHME is currently defined as a distinct, potentially intermediate malignant, rarely metastasizing neoplasm with vascular/endothelial differentiation. It is characterized by young age (typically less than 40 years old), extremity location (approximately ~80%), and t(7:19) SERPINE1::FOSB fusion as the most common molecular alteration. Herein, we report a case of a 59-year-old male presenting with multifocal lesions, including in the right temporalis muscle, right frontoparietal calvarium, right pterygoid muscles, and right mandibular condyle. Histologic examination of the right temporal lesion revealed a multinodular biphasic lesion composed of sheets and fascicles of elongated spindle and epithelioid cells infiltrating into the adjacent skeletal muscle. Admixed abundant neutrophilic infiltration is noted; however, areas of necrosis, increased mitosis, nuclear atypia, or rhabdomyoblast-like cells are absent. Immunohistochemical (IHC) staining showed that the tumor cells were diffusely and strongly positive for FOSB, pan-cytokeratin (AE1/AE3), CD31, and ERG. Molecular testing demonstrated a t(9:19) EGFL7::FOSB fusion mRNA. This constellation of morphological, IHC and molecular findings was consistent with a diagnosis of PMHE. This is the first reported case of multifocal PMHE with EGFL7::FOSB fusion in the head and neck area of a patient aged more than 50 years old. Since the differential diagnoses for PMHE includes high-grade malignancies with aggressive clinical behavior, coupled with the rare reports of PMHE in the head and neck region, awareness of this tumor in the head and neck region will avoid the misdiagnosis and overtreatment of this entity.

A 50-Year-Old Man Presenting with Multiple Bone Lesions and a Diagnosis of Phosphaturic Mesenchymal Tumor of the Femur.

BACKGROUND Phosphaturic mesenchymal tumor (PMT) is an extremely rare mesenchymal neoplasm that is commonly seen in bone and soft tissue. It is associated with a paraneoplastic syndrome, oncogenic osteomalacia, due to tumor-induced urinary phosphate wasting. It is demonstrated to be predominantly mediated by fibroblast growth factor 23 (FGF23)/fibroblast growth factor receptor 1 (FGFR1) axis. Clinically, PMT usually presents as a solitary lesion in the bone. The diagnosis of PMT is challenging due to its non-specific clinical manifestation, radiologic findings, and morphological features. CASE REPORT We report the case of a 50-year-old man presenting with multiple lytic bone lesions and associated pathologic fracture of the right femur, clinically suspicious for multiple myeloma or other metastatic malignant process. Resection from the right femur showed a hypercellular lesion composed of oval-to-spindled cells infiltrating the native trabecular bone with admixed multinucleated giant cells. Immunohistochemical (IHC) staining and in situ hybridization (ISH) demonstrated the tumor cells were positive for SATB2, ERG, FGFR1, and FGF23 ISH. DNA and RNA next-generation sequencing showed marked increases in mRNA levels of FGF23 and FGFR1. The constellation of clinicoradiologic, histomorphologic, IHC, and molecular findings supported a diagnosis of primary benign PMT. CONCLUSIONS This case report discusses a patient with PMT presenting with multifocal lesions due to tumor-induced osteomalacia at initial presentation. We hope that this report will increase the awareness of clinician and pathologists of PMT as a differential diagnosis in patients presenting with multifocal lytic bone lesions. In turn, this will prevent misdiagnosis and overtreatment of a typically benign process.

10-Year observation of a rare presentation of pure fibromyxoid nephrogenic adenoma in the renal pelvis.

Nephrogenic adenoma (NA) is an unusual benign epithelial tumor in the genitourinary tract. Here we report a fibromyxoid nephrogenic adenoma in a 37-year-old female presenting with over 10-year slow-growing renal pelvic mass that was diagnosed with bland spindle cell lesion in multiple previous biopsies. This is the first reported case of pure fibromyxoid NA in renal pelvis with close comparison and correlation of biopsy and resection findings over a 10-year span. This will enhance awareness of pathologists to consider this unusual entity when examining spindle cell lesions in this setting, and prevent misdiagnosis and overtreatment of a typically benign process.

Experimental and theoretical probe on mechano- and chemosensory integration in the insect antennal lobe.

In nature, olfactory signals are delivered to detectors-for example, insect antennae-by means of turbulent air, which exerts concurrent chemical and mechanical stimulation on the detectors. The antennal lobe, which is traditionally viewed as a chemosensory module, sits downstream of antennal inputs. We review experimental evidence showing that, in addition to being a chemosensory structure, antennal lobe neurons also respond to mechanosensory input in the form of wind speed. Benchmarked with empirical data, we constructed a dynamical model to simulate bimodal integration in the antennal lobe, with model dynamics yielding insights such as a positive correlation between the strength of mechanical input and the capacity to follow high frequency odor pulses, an important task in tracking odor sources. Furthermore, we combine experimental and theoretical results to develop a conceptual framework for viewing the functional significance of sensory integration within the antennal lobe. We formulate the testable hypothesis that the antennal lobe alternates between two distinct dynamical regimes, one which benefits odor plume tracking and one which promotes odor discrimination. We postulate that the strength of mechanical input, which correlates with behavioral contexts such being mid-flight versus hovering near a flower, triggers the transition from one regime to the other.

First-in-human immunoPET imaging of HIV-1 infection using 89Zr-labeled VRC01 broadly neutralizing antibody.

A major obstacle to achieving long-term antiretroviral (ART) free remission or functional cure of HIV infection is the presence of persistently infected cells that establish a long-lived viral reservoir. HIV largely resides in anatomical regions that are inaccessible to routine sampling, however, and non-invasive methods to understand the longitudinal tissue-wide burden of HIV persistence are urgently needed. Positron emission tomography (PET) imaging is a promising strategy to identify and characterize the tissue-wide burden of HIV. Here, we assess the efficacy of using immunoPET imaging to characterize HIV reservoirs and identify anatomical foci of persistent viral transcriptional activity using a radiolabeled HIV Env-specific broadly neutralizing antibody, 89Zr-VRC01, in HIV-infected individuals with detectable viremia and on suppressive ART compared to uninfected controls (NCT03729752). We also assess the relationship between PET tracer uptake in tissues and timing of ART initiation and direct HIV protein expression in CD4 T cells obtained from lymph node biopsies. We observe significant increases in 89Zr-VRC01 uptake in various tissues (including lymph nodes and gut) in HIV-infected individuals with detectable viremia (N = 5) and on suppressive ART (N = 5) compared to uninfected controls (N = 5). Importantly, PET tracer uptake in inguinal lymph nodes in viremic and ART-suppressed participants significantly and positively correlates with HIV protein expression measured directly in tissue. Our strategy may allow non-invasive longitudinal characterization of residual HIV infection and lays the framework for the development of immunoPET imaging in a variety of other infectious diseases.

Verapamil Use Is Associated With Reduction of Newly Diagnosed Diabetes Mellitus.

Objective: The mechanism of the beneficial effect of calcium-channel blockers (CCBs), especially verapamil, on the development of type 2 diabetes mellitus (T2DM) has been described. This study compared the incidence of T2DM in adults prescribed oral verapamil and propensity score-matched adults prescribed other oral CCBs. Methods: This retrospective population-based cohort study used Taiwan's National Health Insurance Research Database from 2000 to 2011. T2DM was defined according to the International Classification of Diseases, Ninth Revision, Clinical Modification. Results: During follow-up periods of 41,958 and 42,118 person-years, 269 of 4930 patients in the verapamil cohort and 340 of 4930 patients in the matched cohort, respectively, developed T2DM. The incidence rates were 6.41 and 8.07 per 1000 population per year among verapamil and other CCB users, respectively. The adjusted hazard ratio (HR) for T2DM associated with use of verapamil (vs. other CCBs) was 0.80 [95% confidence interval (CI), 0.68 to 0.94; P = 0.006]. After exclusion of patients followed for <180 days or <365 days (to avoid bias derived from delayed diagnosis), adjusted HRs remained significant [0.79 (95% CI, 0.67 to 0.93; P = 0.005) and 0.77 (95% CI, 0.65 to 0.91; P = 0.002), respectively]. Only the interaction term for age was significant (P = 0.009). Verapamil had a more prominent effect on patients aged older than 65 years (P < 0.001). Conclusions: In patients with no known history of diabetes mellitus, oral verapamil use was associated with a decreased incidence of T2DM compared with other CCBs.

Semi-Prone Position Can Influence Variability in Respiratory Rate of Premature Infants Using Nasal CPAP.

UNLABELLED: To determine the effect of positions (supine, lateral, semiprone) on the physiological parameters (HR, RR, SpO2) of premature infants receiving NCPAP who were non-oxygen-dependent and non-BPD, and to identify significant clinical changes associated with these variables. METHODS: A crossover study design with three different positions in the assigned sequence (supine-supine, supine-lateral and supine-semiprone) was used, and each position was maintained for 1h. The subjects' vital signs were recorded 30 min after initiation of each position and measured for 30 min. RESULTS: Forty-seven infants with a median GA of 28.6 weeks (range 26-35) were studied, and their median BW was 1210g (range 776-2920). Overall, position-related effects showed significant difference in the variability in RR (OR=0.68; CI 0.51-0.89), with the variability in RR being significantly lower in the semiprone position. The lateral position was associated with increased RR (B=2.9; p=0.02). Previous use of ventilator (PUV) was associated with increased HR, whereas BW and GA were negatively associated with higher HR. Cesarean birth, use of surfactant and PUV were associated with lower SpO2, whereas BW and GA were correlated with higher SpO2. GA was identified as a protective factor, while PUV was a risk factor for the variability in both HR and SpO2. CONCLUSIONS: Premature infants receiving NCPAP sleeping in the semiprone position may have more stable RR, while the lateral position did not improve RR. Thus, the semiprone and supine positions may be considered preferable when positioning the monitored premature infants with NCPAP.

A correlational study of illness knowledge, self-care behaviors, and quality of life in elderly patients with heart failure.

BACKGROUND: Patients with heart failure experience adverse physical symptoms that affect quality of life. The number of patients with heart failure in Taiwan has been growing in recent years. PURPOSE: This article examines correlations among illness knowledge, self-care behaviors, and quality of life in elderly patients with heart failure. METHODS: A cross-sectional research design using three questionnaires was adopted. The study was undertaken in an outpatient department of a teaching hospital in Taiwan from January to June 2008. Potential participants aged 65 years or older were selected by a physician based on several diagnostic findings of heart failure that included an International Classification of Diseases' code 4280 or 4289. Patients who were bedridden or had a prognosis of less than 6 months were excluded from consideration. RESULTS: One hundred forty-one patients with heart failure were recruited. Most participants were men (51.8%), older adults (49.6% older than 71 years old), and either educated to an elementary school level or illiterate (69.5%) and have New York Heart Association class II (61.0%). Participants had an average left ventricular ejection fraction of 41.1%. The illness knowledge of participants was poor (accuracy rate: 29.3%), and most were unaware of the significance of self-care. Illness knowledge correlated with both self-care behaviors (r = -.42, p < .01) and quality of life (r = -.22, p < .01). Illness knowledge and age were identified as significant correlated factors of self-care behaviors (R = .22); and functional class, living independently, and age were identified as significant correlated factors of quality of life (R = .41). CONCLUSIONS/IMPLICATIONS FOR PRACTICE: Participants in this study with higher self-reported self-care behaviors and quality of life were younger in age and had better illness knowledge. Furthermore, physical function and independence in daily living significantly affected quality of life. Care for patients with heart failure, particularly older adults, should focus on teaching these patients about heart failure illness and symptom management. Assisting elderly patients with heart failure to promote and maintain physical functions to handle activities of daily living independently is critical to improving patient quality of life.

The experiences of adult ventilator-dependent patients: a meta-synthesis review.

Studies exploring the perceptions of patients whose lives are maintained by mechanical ventilation highlight the stressful nature of this type of experience. The objective of this meta-synthesis study was to describe the nature of the experience of adult ventilator-dependent patients. A systematic literature search of English and Chinese databases was undertaken, covering the period between 1970 and 2012. Qualitative research findings were extracted and pooled using the Joanna Briggs Institute Qualitative Assessment and Review Instrument. A total of 1004 papers were identified from various database and hand searches. Nineteen papers were critically appraised and 16 met inclusion criteria. Five meta-synthesis themes emerged from the analysis: (1) the feelings of fear due to being dependent on a ventilator and the loss of control of life; (2) disconnection with reality; (3) impaired embodiment; (4) construction of coping patterns; (5) trust and caring relationship. Suggested implications for practice include enhancing the trust relationship with health professionals, as well as nursing actions throughout the suction procedure relating to release of patient's psychological distress and empowering their resilience factors.

Mothers' experience with 1st-3rd-grade children with asthma assisting their child's adaptation of school life in Taiwan.

AIMS AND OBJECTIVES: This study used purposive sampling through semi-structured interviews to obtain the experiences of mothers of 7-11-year-old children with asthma who were assisting their child's adaptation to school life. BACKGROUND: Children with asthma often have problems with social adaptation, including school absenteeism, limits to their activity and bullying by peers. From kindergarten to elementary school, it is a transitional process where the child experiences multiple changes in the body, mind and social situations. It can be difficult for mothers of children with asthma to assist their children with their adaptation to school life. DESIGN: A qualitative enquiry design was used. METHODS: A total of 15 mothers having elementary school children with asthma in grades 1-3 were interviewed. Participants were contacted at the outpatient department of a medical centre in Taiwan. Verbatim transcriptions of the interviews were examined by the content analysis method. After analysis of the interview data of the 15 informants, no new themes had emerged. Lincoln and Guba's trustworthiness criteria were employed to evaluate methodological rigour. RESULTS: There were four themes that formed part of the mothers' experiences. Those were: (1) being concerned about the child's adaptation to school life, (2) improving attitudes and relieving symptoms, (3) establishing the child's self-management abilities and (4) bearing role strain and normalising the life of the child. CONCLUSIONS: The findings of this study should help health professionals and schoolteachers to understand the needs of families and mothers who have a child with asthma. The findings provide appropriate information and resources to assist the children's adjustment to school life. RELEVANCE TO CLINICAL PRACTICE: The findings indicate that nursing professionals should provide child-focused and family-centred care that will assist parents of children with asthma to adjust to school life.

A systematic review of the experiences of adult ventilator-dependent patients.

OBJECTIVES: The objective of this meta-synthesis study was to describe the nature of the experience of adult ventilator-dependent patients. INCLUSION CRITERIA: This review considered studies utilized qualitative methods to examine the experience of adult with ventilator. The phenomena of interest were experiences of patients who were treated with ventilator for both short term and long term in ICU settings or home settings. The research was limited to studies published in Chinese or in English language. SEARCH STRATEGY: The searching strategy sought to find both published and unpublished studies. The CINAHL, PubMed, MEDLINE, Cochrane Library, Chinese Periodicals Index and JIB website were used to search the articles. The preliminary keywords were drawn from the natural language terms of the topic, in the title, abstract and subject descriptors. METHODOLOGICAL QUALITY: Each paper was assessed independently by two reviewers for methodological quality. The Joanna Briggs Institute's Qualitative Assessment and Review Instrument were used to appraisal the methodological quality of the articles. Any disagreements that arose between the reviewers were resolved through discussion with a third reviewer. DATA SYNTHESIS: Qualitative research findings were pooled and the data analysis process involved synthesizing findings to generate a set of statements that represent the nature of the experiences of ventilator-dependent adult patients. The categories and themes/meta-syntheses were emerged from the analysis process. RESULTS: A total of 997 papers were identified from various database and hand searches. Nineteen papers were critically appraised and 15 met inclusion criteria. Four papers were excluded because they did not meet the inclusion criteria. Five themes/meta-syntheses emerged from the analysis: 1). The feelings of fear due to being dependent on ventilator and the loss of control of life, 2). Disconnection with reality, 3). Impaired embodiment (body image and body boundary), 4). Construction of coping patterns, 5). Trust and caring relationship. CONCLUSION: The five themes/meta-syntheses derived from the review represent the patients' experiences in regarding the threatening of the integrity of self, self-other and self-environment relationships, the coping patterns and resilient resources to maintain their self-identify and the meaning of life. These findings also illustrate the resiliency factors for those patients to cope with this stressful situation.The implications to practice include enhancing the trust relationship with health professionals, as well as the nursing actions prior to suction, during the suction procedure and post suction in related to release their psychological distress and empower their resilience factors was suggested.Furthermore, the further research could focus on the development and implementation of support programs for the patients, families, and health professionals, as well as the research regarding the reduction of psychological distress and empower the coping patterns.

Removal of oral secretion prior to position change can reduce the incidence of ventilator-associated pneumonia for adult ICU patients: a clinical controlled trial study.

AIM: The purpose of this study was to explore the effect of oral secretion on aspiration and reducing ventilator-associated pneumonia. BACKGROUND: Ventilator-associated pneumonia is a serious hospital-acquired infection with reported incidence rate of 12.2% and mortality rate of 29.3%. Oral secretion is purported as a media which brings the oropharyngeal pathogens down to the respiratory track. METHODS: Two-group comparison study design was adopted. Subjects were recruited from an adult general intensive care unit of a medical centre in Taipei city. Patients in the study group received suction of oral secretion before each positional care, in contrast with patients in the control group who received routine care. RESULTS: Ventilator-associated pneumonia was found in 24 of 159 (15.1%) patients in the control group and in five of 102 (4.9%) patients in the study group with a reduction of risk ratio of 0.32 (95% CI 0.11-0.92). Eight of the 24 ventilator-associated pneumonia patients died in the control group; however, none of those ventilator-associated pneumonia patients died in the study group. The increased chance of survival was 1.50 (95% CI 1.13-1.99). The length of stay in ICU and duration of mechanical ventilation were reduced in the study group. In consideration of cost, the cost of tubes used to remove oral secretion is much less than the one used to do continuous subglottal suction. CONCLUSION: Removal of oral secretion is effective in reducing the incidence of ventilator-associated pneumonia with minimum cost intervention. RELEVANCE TO CLINICAL PRACTICE: This study provides evidence that removal of oral secretion prior to position change is cost effective to reduce the incidence of ventilator-associated pneumonia. As such intervention is an easy task, routine removal of oral secretion is recommended as the standard of daily nursing care of patients on ventilator.

Influence of physical restraint on unplanned extubation of adult intensive care patients: a case-control study.

BACKGROUND: Unplanned extubation commonly occurs in intensive care units. Various physical restraints have been used to prevent patients from removing their endotracheal tubes. However, physical restraint not only does not consistently prevent injury but also may be a safety hazard to patients. OBJECTIVES: To evaluate the effect of physical restraint on unplanned extubation in adult intensive care patients. METHODS: A total of 100 patients with unplanned extubations and 200 age-, sex-, and diagnosis-matched controls with no record of unplanned extubation were included in this case-control study. The 300 participants were selected from a population of 1455 patients receiving mechanical ventilation during a 21-month period in an adult intensive care unit at a medical center in Taiwan. Data were collected by reviewing medical records and incident reports of unplanned extubation. RESULTS: The incidence rate of unplanned extubation was 8.7%. Factors associated with increased risk for unplanned extubation included use of physical restraints (increased risk, 3.11 times), nosocomial infection (increased risk, 2.02 times), and a score of 9 or greater on the Glasgow Coma Scale on admission to the unit (increased risk, 1.98 times). Episodes of unplanned extubation also were associated with longer stays in the unit. CONCLUSIONS: An impaired level of consciousness on admission to the intensive care unit and the presence of nosocomial infection intensify the risk for unplanned extubation, even when physical restraints are used. To minimize the risk of unplanned extubation, nurses must establish better standards for using restraints.

Caregivers' experiences at home with a ventilator-dependent child.

With advancing technology and transformation in health care delivery, more chronically ventilator-dependent children are being discharged to home. Pediatric home health care aims to increase the quality of life of patients and families, decrease cost and duration of hospital stay, and promote community participation in health care delivery. The authors aimed to describe and identify the qualitatively different experiences of primary caregivers who support ventilator-dependent children at home. Through phenomenographic inquiry 17 primary caregivers described their experiences through interviews that were later analyzed using accepted qualitative methods. Seven categories of description and an outcome space were identified, with findings highlighting the unique experience of this group and providing insight into their personal, social, and collective experiences. Outcomes reveal the significant and distinctive nature of understanding and have implications for clinical practice development, health education, policy formulation, social support, and future research in pediatric home health care.

Technology-dependent children and their families: a review.

BACKGROUND: Advances in medical technology and nursing care have enabled children who rely on long-term medical and technical support to reunite with their families and community. The impact of discharging these children into the community involves a number of unprecedented social implications that warrant policy consideration. To begin with, an effort must be made to understand the phenomenon of caring for technology-dependent children living at home. AIM: The aim of this paper is to provide a comprehensive literature review on caring for technology-dependent children living at home. METHODS: The review was conducted via keyword searches using various electronic databases. These included CINAHL, MEDLINE, Social Science Index, Sociological Abstracts, Australian Family and Society Abstracts, and the Australian Bureau of Statistics. The articles and books found were examined for commonality and difference, significant themes were extracted, and the strength of the research methods and subsequent evidence were critiqued. FINDINGS: In this paper, themes relating to home care for technology-dependent children and their families are elucidated and summarized. These are: chronic illness and children; the impact of paediatric home care on children; the uniqueness of technology-dependent children and their families; and parents' experience of paediatric home care. DISCUSSION: Contentious issues, relevant to the social life of these children and their families, are raised and are discussed with the intention of extending awareness and provoking further debate among key stakeholders. These issues include: the changed meaning of home; family dynamics; social isolation; saving costs for whom?; shifts in responsibility; and parent-professional relationships. CONCLUSION: More research is needed in the arena of paediatric home care, to facilitate relevant policy formation and implementation.