bluuebunny/crossref_metadata_2025 huggingface dataset:
Artisanal Spearfishery in Temperate Nearshore Ecosystems of Chile: Exploring the Catch Composition, Revenue, and Management Needs
Authors: Godoy Natalio , Gelcich Stefan , Castilla Juan C. , Lima Mauricio , Smith Andres
Published: September 2016

We used extensive field data on catch and effort as well as fisher interviews to characterize the catch composition and revenue associated with the unregulated artisanal spearfishery in Chile (18–33°S). Sampling was performed on commercial spearfishing trips (snorkel and hookah diving gear) between spring 2010 and summer 2011. Two‐way crossed ANOVA showed significant effects of region (latitude) and dive gear on fishery variables such as biomass CPUE (CPUEb), numeric CPUE (CPUEn), catch species richness, fishing depth, cost, and income. Catches included 22 fish species from 15 families. Among the 23 species, 17 were associated with temperate rocky reef habitats: 14 carnivorous species, 2 omnivorous species, and 1 herbivorous species. Our results indicated that smaller, less‐valuable rocky reef fishes (e.g., Peruvian Morwong Cheilodactylus variegatus, Chilean Sandperch Pinguipes chilensis, and Peruvian Rock Seabass Paralabrax humeralis) supported higher CPUEb and CPUEn than large, high‐value, emblematic rocky reef species (e.g., Vieja Graus nigra, Galapagos Sheephead Wrasse Semicossyphus darwini, and Acha Medialuna ancietae). The CPUEb was significantly higher for hookah fishers than for snorkel fishers. Our results revealed that artisanal spearfishing activities provide important revenue for the fishers (2–3 times the minimum monthly wage in Chile), thereby incentivizing a rapid expansion of this unregulated fishery. Management options based on territorial user rights and catch and size restrictions are discussed in light of these findings.

Received December 19, 2013; accepted March 29, 2016
Upstream Movements of Atlantic Salmon in the Lower Penobscot River, Maine Following Two Dam Removals and Fish Passage Modifications
Authors: Izzo Lisa K. , Maynard George A. , Zydlewski Joseph
Published: September 2016

The Penobscot River Restoration Project (PRRP), to be completed in 2016, involved an extensive plan of dam removal, increases in hydroelectric capacity, and fish passage modifications to increase habitat access for diadromous species. As part of the PRRP, Great Works and Veazie dams were removed, making Milford Dam the first impediment to federally endangered Atlantic Salmon Salmo salar. Upstream habitat access for Atlantic Salmon is dependent upon successful and timely passage at Milford Dam because nearly all suitable spawning habitat is located upstream. In 2014 and 2015, a total of 73 adult salmon were radio-tagged to track their upstream movements through the Penobscot River to assess potential delays at (1) the dam remnants, (2) the confluence of the Stillwater Branch and the main stem of the Penobscot River below the impassable Orono Dam, and (3) the Milford Dam fish lift (installed in 2014). Movement rates through the dam remnants and the Stillwater confluence were comparable to open river reaches. Passage efficiency of the fish lift was high in both years (95% and 100%). However, fish experienced long delays at Milford Dam, with approximately one-third of fish taking more than a week to pass in each year, well below the Federal Energy Regulatory Commission passage standard of 95% within 48 h. Telemetry indicates most fish locate the fishway entrance within 5 h of arrival and were observed at the entrance at all hours of the day. These data indicate that overall transit times through the lower river were comparable to reported movement rates prior to changes to the Penobscot River due to the substantial delays seen at Milford Dam. The results of this study show that while adult Atlantic Salmon locate the new fish lift entrance quickly, passage of these fish was significantly delayed under 2014–2015 operations.

Received January 27, 2016; accepted April 14, 2016
Localization, metabelian groups, and the isomorphism problem
Authors: Baumslag Gilbert , Mikhailov Roman , Orr Kent
Published: September 2016

If

G
G

and

H
H

are finitely generated, residually nilpotent metabelian groups,

H
H

is termed para-

G
G

if there is a homomorphism of

G
G

into

H
H

which induces an isomorphism between the corresponding terms of their lower central quotient groups. We prove that this is an equivalence relation. It is a much coarser relation than isomorphism, our ultimate concern. It turns out that many of the groups in a given equivalence class share various properties, including finite presentability. There are examples, such as the lamplighter group, where an equivalence class consists of a single isomorphism class and others where this is not the case. We give several examples where we solve the Isomorphism Problem. We prove also that the sequence of torsion-free ranks of the lower central quotients of a finitely generated metabelian group is computable. In a future paper we plan on proving that there is an algorithm to compute the numerator and denominator of the rational Poincaré series of a finitely generated metabelian group and will carry out this computation in a number of examples, which may shed a tiny bit of light on the Isomorphism Problem. Our proofs use localization, class field theory and some constructive commutative algebra.
Isomorphisms of tensor algebras arising from weighted partial systems
Authors: Dor-On Adam
Published: September 2016

We continue the study of isomorphisms of tensor algebras associated to

C
∗

C^*

-correspondences in the sense of Muhly and Solel. Inspired by recent work of Davidson, Ramsey, and Shalit, we solve isomorphism problems for tensor algebras arising from weighted partial dynamical systems. We provide complete bounded / isometric classification results for tensor algebras arising from weighted partial systems, both in terms of the

C
∗

C^*

-correspondences associated to them and in terms of the original dynamics. We use this to show that the isometric isomorphism and algebraic / bounded isomorphism problems are two distinct problems that require separate criteria to be solved. Our methods yield alternative proofs to classification results for Peters’ semi-crossed product due to Davidson and Katsoulis and for multiplicity-free graph tensor algebras due to Katsoulis, Kribs, and Solel.
Nuclear dimension and classification of 𝐶*-algebras associated to Smale spaces
Authors: Deeley Robin , Strung Karen
Published: September 2016

We show that the homoclinic

C

∗

\mathrm {C}^*

-algebras of mixing Smale spaces are classifiable by the Elliott invariant. To obtain this result, we prove that the stable, unstable, and homoclinic

C

∗

\mathrm {C}^*

-algebras associated to such Smale spaces have finite nuclear dimension. Our proof of finite nuclear dimension relies on Guentner, Willett, and Yu’s notion of dynamic asymptotic dimension.
Kleshchev’s decomposition numbers for diagrammatic Cherednik algebras
Authors: Bowman C. , Speyer L.
Published: September 2016

We construct a family of graded isomorphisms between certain subquotients of diagrammatic Cherednik algebras as the quantum characteristic, multicharge, level, degree, and weighting are allowed to vary; this provides new structural information even in the case of the classical

q
q

-Schur algebra. This also allows us to prove some of the first results concerning the (graded) decomposition numbers of these algebras over fields of arbitrary characteristic.
Arsenobetaine: the ongoing mystery
Authors: Popowich Aleksandra , Zhang Qi , Le X. Chris
Published: September 2016

Arsenobetaine (AsB) is a non-toxic organoarsenical identified as a major arsenic species in marine animals and a number of terrestrial mushrooms. Since its first identification nearly 40 years ago, numerous studies investigating the biosynthesis and function of AsB have been carried out, although molecular mechanisms have not been fully elucidated. Where and how is AsB formed? Why do marine animals acquire high concentrations of AsB? This review briefly summarizes the current progress of AsB research toward understanding its origin, function and the putative pathways for its biosynthesis. This paper also suggests potential future studies in the attempt to solve the AsB mystery.
Understanding sexual assault
Authors: Oldham Jennifer , Symonds Anita
Published: September 2016

Will you be ready when you encounter a patient who's been sexually assaulted?
Sex and the older adult
Authors: Cooper Karen , Kent Vicky P. , Crusse Elizabeth P.
Published: September 2016

Learn how to help your older patients down a safe PATH involving advocacy, communication, and education on topics related to sexuality and intimacy.
A Dexamethasone-regulated Gene Signature Is Prognostic for Poor Survival in Glioblastoma Patients
Authors: Luedi Markus M. , Singh Sanjay K. , Mosley Jennifer C. , Hatami Masumeh , Gumin Joy , Sulman Erik P. , Lang Frederick F. , Stueber Frank , Zinn Pascal O. , Colen Rivka R.
Published: September 2016

Background:

Dexamethasone is reported to induce both tumor-suppressive and tumor-promoting effects. The purpose of this study was to identify the genomic impact of dexamethasone in glioblastoma stem cell (GSC) lines and its prognostic value; furthermore, to identify drugs that can counter these side effects of dexamethasone exposure.

Methods:

We utilized 3 independent GSC lines with tumorigenic potential for this study. Whole-genome expression profiling and pathway analyses were done with dexamethasone-exposed and control cells. GSCs were also co-exposed to dexamethasone and temozolomide. Risk scores were calculated for most affected genes, and their associations with survival in The Cancer Genome Atlas and Repository of Molecular Brain Neoplasia Data databases. In silico Connectivity Map analysis identified camptothecin as antagonist to dexamethasone-induced negative effects.

Results:

Pathway analyses predicted an activation of dexamethasone network (z-score: 2.908). Top activated canonical pathways included “role of breast cancer 1 in DNA damage response” (P=1.07E–04). GSCs were protected against temozolomide-induced apoptosis when coincubated with dexamethasone. Altered cellular functions included cell movement, cell survival, and apoptosis with z-scores of 2.815, 5.137, and –3.122, respectively. CCAAT/enhancer binding protein beta (CEBPB) was activated in a dose dependent manner specifically in slow-dividing “stem-like” cells. CEBPB was activated in dexamethasone-treated orthotopic tumors. Patients with high risk scores had significantly shorter survival. Camptothecin was validated as potential partial neutralizer of dexamethasone-induced oncogenic effects.

Conclusions:

Dexamethasone exposure induces a genetic program and CEBPB expression in GSCs that adversely affects key cellular functions and response to therapeutics. High risk scores associated with these genes have negative prognostic value in patients. Our findings further suggest camptothecin as a potential neutralizer of adverse dexamethasone-mediated effects.
Systemic vasculitis and the eye
Authors: Tugal-Tutkun Ilknur
Published: September 2016

Purpose of review

The primary systemic vasculitides represent a spectrum of rare but life-threatening conditions that may also affect the eye in various forms. This article reviews recently published data on ocular manifestations of systemic vasculitis.

Recent findings

Early diagnosis and timely treatment has led to better visual outcomes in giant cell arteritis (GCA). Gene expression from orbital tissues could distinguish granulomatosis with polyangiitis (GPA) from sarcoidosis and Graves disease, but not from idiopathic orbital inflammation. Rituximab was an effective therapeutic option in ocular GPA. An ocular attack severity scoring system, flare levels, fluorescein angiography and optical coherence tomography parameters have been suggested as predictors of visual prognosis in Behçet disease. Efficacy of tumor necrosis factor-alpha inhibitors, interferon α and anti-interleukin-1 agents was shown in noncontrolled studies of ocular Behçet disease.

Widely used fast-track pathway care as well as ocular imaging to detect subclinical involvement may enable earlier diagnosis of GCA and prevention of permanent visual loss. Orbital inflammation may not remain idiopathic with advances in gene expression profiling of orbital tissues. With an increased availability and the use of biologic agents, visual prognosis will improve in patients with severe ocular complications of systemic vasculitides.
Diagnostic Accuracy of a Host Gene Expression Signature That Discriminates Clinical Severe Sepsis Syndrome and Infection-Negative Systemic Inflammation Among Critically Ill Children
Authors: Zimmerman Jerry J. , Sullivan Erin , Yager Thomas D. , Cheng Catherine , Permut Lester , Cermelli Silvia , McHugh Leo , Sampson Dayle , Seldon Therese , Brandon Richard B. , Brandon Roslyn A.
Published: September 2016

Objectives:

SeptiCyte Lab (Immunexpress, Seattle, WA), a molecular signature measuring the relative expression levels of four host messenger RNAs, was developed to discriminate critically ill adults with infection-positive versus infection-negative systemic inflammation. The objective was to assess the performance of Septicyte Lab in critically ill pediatric patients.

Design:

Prospective observational study.

Setting:

Pediatric and Cardiac ICUs, Seattle Children’s Hospital, Seattle, WA.

Patients:

A cohort of 40 children with clinically overt severe sepsis syndrome and 30 children immediately postcardiopulmonary bypass surgery was recruited. The clinically overt severe sepsis syndrome children had confirmed or highly suspected infection (microbial culture orders, antimicrobial prescription), two or more systemic inflammatory response syndrome criteria (including temperature and leukocyte criteria), and at least cardiovascular ± pulmonary organ dysfunction.

Interventions:

None (observational study only).

Measurements and Main Results:

Next-generation RNA sequencing was conducted on PAXgene blood RNA samples, successfully for 35 of 40 (87.5%) of the clinically overt severe sepsis syndrome patients and 29 of 30 (96.7%) of the postcardiopulmonary bypass patients. Forty patient samples (~ 60% of cohort) were reanalyzed by reverse transcription-quantitative polymerase chain reaction, to check for concordance with next-generation sequencing results. Postcardiopulmonary bypass versus clinically overt severe sepsis syndrome descriptors included the following: age, 7.3 ± 5.5 versus 9.0 ± 6.6 years; gender, 41% versus 49% male; Pediatric Risk of Mortality, version III, 7.0 ± 4.6 versus 8.7 ± 6.4; Pediatric Logistic Organ Dysfunction, version II, 5.1 ± 2.2 versus 4.8 ± 2.8. SeptiCyte Lab strongly differentiated postcardiopulmonary bypass and clinically overt severe sepsis syndrome patients by receiver operating characteristic curve analysis, with an area-under-curve value of 0.99 (95% CI, 0.96–1.00). Equivalent performance was found using reverse transcription-quantitative polymerase chain reaction. There was no significant correlation between the score produced by the SeptiCyte Lab test and measures of illness severity, immune compromise, or microbial culture status.

Conclusions:

SeptiCyte Lab is able to discriminate clearly between clinically well-defined and homogeneous postcardiopulmonary bypass and clinically overt severe sepsis syndrome groups in children. A broader investigation among children with more heterogeneous inflammation-associated diagnoses and care settings is warranted.
Statewide Study to Assess Nurses' Experiences With Meaningful Use–Based Electronic Health Records
Authors: McBride Susan , Tietze Mari , Hanley Mary Anne , Thomas Laura
Published: September 2016

Nursing professionals are at the frontline of the health information technology revolution. The Texas Nurses Association and Texas Organization of Nurse Executives partnered to evaluate the changing health technology environment in Texas, in particular the nurses' satisfaction with the use of clinical information systems. A descriptive exploratory study using the Clinical Information System Implementation Evaluation Scale and a newly developed Demographic Survey and the Meaningful Use Maturity-Sensitive Index, with a narrative component, was conducted in 2014 and 2015. Nurses across Texas received an electronic invitation to participate in the survey, resulting in 1177 respondents. Exploratory factor analysis revealed that variables of the Meaningful Use Maturity-Sensitive Index and Clinical Information System Implementation Evaluation Scale show strong interrater reliability, with Cronbach's α scores of .889 and .881, respectively, and thereby inform the survey analysis, indicating and explaining variations in regional and institutional trends with respect to satisfaction. For example, the maturity of a clinical information system within an organization and age of the nurse significantly influence the probability of nurse satisfaction (P < .05). Qualitative analysis of nurses' narratives further explained the nurses' experiences. Recommendations for future research and educational were identified.
Pembrolizumab-triggered Uveitis: An Additional Surrogate Marker for Responders in Melanoma Immunotherapy?
Authors: Diem Stefan , Keller Fabienne , Rüesch Reinhard , Maillard Samia A. , Speiser Daniel E. , Dummer Reinhard , Siano Marco , Urner-Bloch Ursula , Goldinger Simone M. , Flatz Lukas
Published: September 2016

Immunotherapy leads to significantly prolonged survival of patients with metastatic melanoma. Autoimmune side effects including colitis, dermatitis, and endocrine abnormalities are common in patients treated with ipilimumab [anti-CTLA4 (cytotoxic T-lymphocyte-associated protein 4)]. Antibodies such as pembrolizumab that interfere with the PD-1 (programmed cell death 1)/PD-L1 pathway show greater efficacy and less toxicity than ipilimumab. Here we report 2 cases of pembrolizumab-induced uveitis associated with complete or partial tumor response. We suggest that uveitis may serve as a surrogate marker for a tumor response to therapy with pembrolizumab.
Clear Cell Papulosis: Report of a Case With Unique Clinical and Histologic Findings
Authors: Bisi dos Santos Josie Eiras , Ribeiro de Miranda Mario Fernando
Published: September 2016

Clear cell papulosis is a rare, self-limited, benign disease of early childhood, characterized by white macules and flat papules over the milk line. Histopathologically, it is characterized by scattered clear cells throughout the basal and/or suprabasal epidermal layers, which—as clear cells of Toker of the nipple do—typically express cytokeratin 7. They also exhibit other markers expected for adenoid differentiation, such as low-molecular weight cytokeratins, carcinoembryonic antigen, epithelial membrane antigen, and mucin. The age of onset, distribution of lesions, histopathology, and its benign behavior nature help to exclude clinically similar conditions, either benign or malignant. The authors report a case of clear cell papulosis in a 7-year-old Brazilian girl in whom lesions were observed on the legs and histologically formed by solid and adenoid aggregates of clear cells, in a similar fashion than clear fetal cells of Toker.
Restrained Terminal Differentiation and Sustained Stemness in Neonatal Skin by Ha-Ras and Bcl-2
Authors: Lee Sangjun , Rodriguez-Villanueva Julio , McDonnell Timothy
Published: September 2016

Nonmelanoma skin cancer is the most frequently diagnosed cancer in the United States. Deregulation of bcl-2 and ras family members is commonly observed in nonmelanoma skin cancer. It has been previously demonstrated that simultaneous bcl-2 and Ha-ras gene expression in keratinocytes results in resistance to cell death induced by ultraviolet radiation and enhanced multistep skin carcinogenesis. In this study, we aimed to elucidate the central roles of Ha-Ras and Bcl-2 in maintaining epidermal homeostasis. To assess the effect of deregulated Ha-Ras and Bcl-2 on skin differentiation, we have generated skin-specific transgenic mouse model constitutively expressing both oncogenic Ha-Ras and Bcl-2. Ectopic expression of Ha-Ras and Bcl-2 in newborn double transgenic epidermal keratinocytes induced abnormal epidermal differentiation accompanied by increased cell proliferation and suppressed apoptotic cell death, which resulted in thickened and wrinkled skin morphology in neonate skins. Expression of epidermal differentiation marker cytokeratin 1 was decreased. Expression of other differentiation markers loricrin and filaggrin was also decreased and delayed to be detected only in the upper stratum granulosum, whereas the proliferative markers cytokeratin 14 and cytokeratin 6, which are expressed in constitutively proliferative basal layer and stem cell niches such as hair follicles or neoplastic lesions, respectively, were highly expressed. The abnormal expression of epidermal cytokeratins suggests that Ha-Ras and Bcl-2 suppress the terminal differentiation and sustain the stem cell–like features in epidermal keratinocytes.
Extraskeletal Myxoid Chondrosarcoma of the Foot Clinically Mimicking Plantar Fibromatosis
Authors: Saraggi Deborah , Salmaso Roberto , Montesco Maria Cristina , Zamuner Carolina , Tiengo Cesare , Rugge Massimo , Fassan Matteo
Published: September 2016

Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma usually presenting in proximal extremities of middle-aged men. The authors discuss a unique case of EMC, localized in the plantar foot of a 76-year-old woman, clinically suspected as plantar fibromatosis. It is important to avoid misdiagnosis of EMC because of their propensity for late recurrence and their metastatic potential.
An Epidemic Outbreak of Cutaneous Leishmaniasis Presenting as Suppurative Folliculitis: A Study of 6 Cases
Authors: Fernandez-Flores Angel , Valerio Lluís , Carrato Cristina , Hernández-Gallego Alba , Fernández-Figueras Maria Teresa
Published: September 2016

Folliculitis is defined as the inflammation of the follicles. The most common cause of folliculitis is infection. Here, we report an unusual cause of suppurative infundibulitis—which had not yet been described in the literature—due to Leishmania infection, and exemplified by 6 cases that occurred in the setting of an epidemic outbreak. The 6 individuals were immigrants from Morocco. Most of them were men (4 men and 2 women), and most of them were less than 30 years old (apart from one 40-year-old woman). In all cases, a cutaneous biopsy was performed. There was a granulomatous folliculitis with suppurative granulomas in all the cases. All cases showed prominence of plasma cells in the inflammatory infiltrate, and leishmanias were found in all cases. They were mainly seen in the abscessified central areas. The amount of organisms varied from a few to a moderate amount. They were stained by the anti-CD1a antibody (Novocastra) and by a polyclonal homemade anti–leishmania antibody. In addition, in 1 case, microbiological culture was performed, and Leishmania major was demonstrated as the causative agent of the infection.
Dexmedetomidine Depresses Sinoatrial and Atrioventricular Nodal Function Without Any Change in Atrial Fibrillation Inducibility
Authors: Sairaku Akinori , Nakano Yukiko , Suenari Kazuyoshi , Tokuyama Takehito , Kawazoe Hiroshi , Matsumura Hiroya , Tomomori Shunsuke , Amioka Michitaka , Kihara Yasuki
Published: September 2016

It has been reported that dexmedetomidine (dex) has an impact on the cardiac conduction system and even has potential antiarrhythmic actions. We examined the influence of dex on the cardiac electrophysiological properties and atrial fibrillation (AF) inducibility. Adult paroxysmal AF patients were randomly assigned to receive (N = 107) or not receive (N = 108) dex during cardiac electrophysiological studies. The corrected sinus node recovery time (558 ± 331 vs. 459 ± 260 milliseconds; P = 0.02), Wenckebach cycle length (P < 0.001), atrioventricular nodal effective refractory period (317 ± 76 vs. 252 ± 54 milliseconds; P < 0.001), and atrio-His interval (P < 0.001) were longer in patients with dex than in those without. We tested the induction of repetitive atrial firing (RFA) defined as the occurrence of ≥2 successive atrial activities induced by single premature atrial stimuli to determine the AF inducibility. RFA was seen with a similar proportion (41.1% vs. 44.4%), yet it was evoked at a longer stimulus coupling interval in the dex patients, which was potentially attributed to the longer atrial effective refractory period (237 ± 36 vs. 213 ± 27 milliseconds; P < 0.001) and more prolonged atrial conduction delay seen in the dex group. In conclusion, dex may depress the sinus and atrioventricular nodal function, however, it may not reduce the AF inducibility.
Update on the Protective Role of Regulatory T Cells in Myocardial Infarction: A Promising Therapy to Repair the Heart
Authors: Kaplan Abdullah , Altara Raffaele , Eid Ali , Booz George W. , Zouein Fouad A.
Published: September 2016

Myocardial infarction (MI) remains one of the leading causes of heart failure development and death worldwide. To date, interventional and pharmacological therapies are effective in reducing the onset of heart failure and promoting survival. However, progressive maladaptive remodeling post-MI persists in a large fraction of patients resulting in poor prognosis. Immune cell responses and an inflammatory environment largely contribute to adverse cardiac remodeling post-MI. CD4+FOXP3+ regulatory T cells (Tregs) are known for their immunosuppressive capacity and have been successfully implemented in multiple preclinical studies of permanent and ischemia–reperfusion MI. In this review, we highlight the important cardioprotective role of Tregs at the cardiac tissue, cellular, and molecular level, as well as the most prominent pharmacological venues that could be used to exploit Tregs as a novel therapeutic intervention to lessen myocardial injury post-MI.
Maternal Endogenous Forces and Shoulder Dystocia
Authors: GRIMM MICHELE J.
Published: September 2016

Childbirth is a complicated biomechanical process that many take for granted. However, the delivery forces generated by a mother (uterine contractions and maternal pushing) are strong and have a significant effect on the body and tissues of the fetus, especially during the second stage of labor. Although most infants are born without negative, force-related outcomes, in some infants the normal forces of labor cause an injury that can have either temporary or permanent sequelae. The biomechanical situation is further complicated when an infant’s shoulder impacts the maternal pelvis, which provides increased resistance and creates added stresses within the neonatal body and tissues.
MULTIMODAL IMAGING AND ELECTRORETINOGRAPHY IN LONG-CHAIN 3-HYDROXYACYL COENZYME A DEHYDROGENASE DEFICIENCY
Authors: Lin James , Boudreault Katherine , Tsang Stephen
Published: September 2016

Purpose:

To report a case of pigmentary retinopathy in long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency using multimodal imaging techniques.

Methods:

Case report.

Results:

An 8-year-old boy with a history of failure to thrive and a diagnosis of long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency was referred for examination. Examination revealed a pigmentary retinopathy with macular atrophy; electroretinography results were consistent with a rod–cone dystrophy. Fundus autofluorescence and optical coherence tomography revealed retinal pigment epithelium atrophy. Follow-up examination findings showed increased severity of retinopathy on electroretinography, with optical coherence tomography angiography revealing enhanced visualization of choroidal vessels.

Conclusion:

This report reveals that long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency can be characterized as a progressive rod–cone dystrophy, with multi-modal imaging techniques used to describe this condition. In particular, optical coherence tomography angiography can be used to further characterize this condition.
Congenital Glaucoma and CHARGE Syndrome: A Case Report
Authors: Benson Matthew D. , Jivraj Imran , Damji Karim F. , Solarte Carlos E.
Published: September 2016

Purpose:

To report a rare case of congenital glaucoma in a patient with CHARGE syndrome, present gonioscopic photographs, and explore mechanisms of disease that may account for this association.

Patients and Methods:

We describe a 35-week-old girl with previously diagnosed CHARGE syndrome who presented with corneal edema, buphthalmos, and elevated intraocular pressure in the left eye. She was subsequently diagnosed with congenital glaucoma and started on topical and oral therapy.

Results:

Examination under anesthesia confirmed the above findings as well as bilateral abnormal angles with an anterior iris insertion at the level of the posterior trabecular meshwork, prominent iris vasculature and stromal strands, and nonvisible scleral spur and ciliary body bands. Trabeculotomy and trabeculectomy were performed in the left eye with a poor outcome. CHARGE syndrome is a complex neurocristopathy, and we propose that the abnormal angle findings and associated asymmetric glaucoma in our patient share a common mechanism of neural crest cell dysfunction.

Conclusions:

CHARGE syndrome can be associated with congenital glaucoma and we emphasize the importance of a thorough ophthalmic examination to detect glaucoma with surgical management as deemed appropriate.
The Effect of Labor on the Intraocular Pressure in Healthy Women
Authors: Meshi Amit , Armarnik Sharon , Mimouni Michael , Segev Fani , Segal Ori , Kaneti Hagai , Assia Ehud I. , Geffen Noa
Published: September 2016

Purpose:

To investigate the effect of modern vaginal labor using epidural anesthesia on the intraocular pressure (IOP) and on the mean ocular perfusion pressure (MOPP) in healthy women.

Patients and Methods:

In this prospective observational study, eligible candidates were healthy pregnant women for vaginal delivery with epidural anesthesia, with a singleton pregnancy, who were admitted to the delivery room in the first phase of the first stage of labor. Demographic data as well as medical and obstetric history were obtained at baseline, followed by performance of a biomicroscopic examination. IOP, maternal heart rate, systolic blood pressure, and diastolic blood pressure were measured in the first, second and third stages of labor, 24±2 and 48±2 hours postpartum. IOP was not measured during the contraction phase of stage 2. MOPP was expressed as the difference between the arterial blood pressure and the IOP. Measurements were performed throughout the different stages of labor and were compared with baseline values.

Results:

Thirty healthy white women were enrolled. Four women dropped out due to unexpected cesarean sections. The average age was 31.8±4.7 years. Nineteen participants received oxytocin during delivery. Twenty-three women were placed in a supine position, whereas 3 in a left decubitus position. No statistically significant changes were recorded in IOP (P=0.50) or MOPP (P=0.17) throughout the different stages of vaginal labor in this study.

Conclusions:

Vaginal delivery under epidural anesthesia is unlikely to cause significant damage to the optic nerve in healthy women.
XEN-augmented Baerveldt: A New Surgical Technique for Refractory Glaucoma
Authors: D’Alessandro Elisa , Guidotti Jacopo M. , Mansouri Kaweh , Mermoud André
Published: September 2016

Background:

Glaucoma drainage devices have traditionally been reserved for patients with refractory glaucoma. However, these devices are prone to various sight-threatening complications. To prevent hypotony after placement of the Baerveldt tube, surgeons traditionally tie the tube with an absorbable suture until encapsulation occurs around the plate. We hypothesized that combining the XEN tube, placing it in the anterior chamber and connecting it to the Baerveldt tube posteriorly, outside the anterior chamber, would minimize 2 main potentially blinding complications: hypotony and corneal disease.

Purpose:

To describe a new surgical technique for refractory glaucoma, combining both the Baerveldt and the XEN tubes in the same surgery.

Surgical Technique:

The Baerveldt implant was positioned in the superotemporal quadrant and sutured to the sclera. A scleral flap was executed extending from the anterior margin of the plate for the entire length of the tube to the limbus. The Baerveldt tube was correctly positioned by removing a deeper scleral flap. The XEN tube was then inserted ab externo and inserted into the Baerveldt tube’s lumen. The newly formed double tube was then sutured and covered by the first scleral flap and usual suturing of the conjunctiva was performed.

Conclusions:

This technique is simple and potentially increases the safety of refractory glaucoma surgeries.
Minimally Invasive Mitral Valve Surgery I: Patient Selection, Evaluation, and Planning
Authors: Ailawadi Gorav , Agnihotri Arvind K. , Mehall John R. , Wolfe J. Alan , Hummel Brian W. , Fayers Trevor M. , Farivar R. Saeid , Grossi Eugene A. , Guy T. Sloane , Hargrove W. Clark , Khan Junaid H. , Lehr Eric J. , Malaisrie S. Chris , Murphy Douglas A. , Rodriguez Evelio , Ryan William H. , Salemi Arash , Segurola Romualdo J. , Shemin Richard J. , Smith J. Michael , Smith Robert L. , Weldner Paul W. , Goldman Scott M. , Lewis Clifton T. P. , Barnhart Glenn R.
Published: September 2016

Widespread adoption of minimally invasive mitral valve repair and replacement may be fostered by practice consensus and standardization. This expert opinion, first of a 3-part series, outlines current best practices in patient evaluation and selection for minimally invasive mitral valve procedures, and discusses preoperative planning for cannulation and myocardial protection.
Breakthrough cancer pain: twenty-five years of study
Authors: Mercadante Sebastiano , Portenoy Russell K.
Published: September 2016

Breakthrough cancer pain (BTcP) is an episode of severe pain that “breaks through” a period of persistent pain at least partly controlled by a stable opioid regimen. Although mentioned in the literature decades ago, it has been only 25 years since the first effort to define and measure it. Controversy about the definition of BTcP continues despite an international effort to achieve consensus. Nevertheless, common approaches to measurement of BTcP have led to a robust literature, including many surveys that have described prevalence, characteristics, and association with adverse outcomes. Measurement also has been important for clinical trials of new drug formulations specifically designed for BTcP. Several approaches have been reported in the literature, although most of them have never been substantiated with appropriate studies. Administration of an opioid as needed is the most common treatment. Twenty-five years of research has produced a more refined understanding of the safety and efficacy of oral opioids in this context, and provided the clinical trials data necessary to attain regulatory approval of multiple new formulations specifically developed for BTcP. Transmucosal formulations of fentanyl may provide meaningful analgesia within 5 to 15 minutes. Given the difference in cost, transmucosal formulations should be considered in a subset of patients with BTcP, including those with pain that are not adequately controlled with an oral drug and those with distress associated with the rapid pain onset. The long-term use of opioids for BTcP remains to be clarified. Future studies should assess the potential of personalized treatment of BTcP.
Medication-induced acute kidney injury
Authors: Goldstein Stuart L.
Published: September 2016

Purpose of review

The present article will review the current state of our understanding of nephrotoxic medication-associated acute kidney injury (AKI) and provide strategies to reduce its impact.

Recent findings

Nephrotoxic medications contribute to a substantial proportion of AKI in hospitalized patients. The previous perspective of nephrotoxic medication-associated AKI as a nonmodifiable necessary evil of providing appropriate therapy to ill patients had led to an incomplete understanding of its epidemiology and provided little impetus to reduce its occurrence.

Recent work on understanding specific combinations, thresholds for nephrotoxic burden and systematic kidney function assessment had mitigated, and even in some cases reduced, nephrotoxic AKI rates and severity. Current initiatives are underway to further refine specific nephrotoxic medication AKI risk via novel urinary biomarkers and genetic susceptibility.
Palliative care in the trauma ICU
Authors: O’Connell Kathleen , Maier Ronald
Published: September 2016

Purpose of review

The benefits of palliative care for critically ill patients are well recognized, yet acceptance into surgical culture is lagging. With the increasing proportion of geriatric trauma patients, integration of palliative medicine within daily intensive care services to facilitate goal-concordant care is imperative.

Recent findings

Misconceptions of palliative medicine as it applies to trauma patients linger among trauma surgeons and many continue to practice without routine consultation of a palliative care service. Aggressive end-of-life care does not correlate with an improved family perception of medical care received near death. Additionally, elderly patients near the end of life often prefer palliative treatments over life-extending therapy, and their treatment preferences are often not achieved. A new geriatric-specific prognosis calculator estimates the risk of mortality after trauma, which is useful in starting goals of care discussions with older patients and their families.

Shifting our quality focus from 30-day mortality rates to measurements of symptom control and achievement of patient treatment preferences will prioritize patient beneficence and autonomy. Ownership of surgical palliative care as a service provided by acute care surgeons will ensure that our patients with incurable injury and illness will receive optimal patient-centered care.
Mortality, Disenrollment, and Spending Persistence in Medicaid and CHIP
Authors: DeLia Derek
Published: September 2016

Background:

Research on spending persistence has not focused on Medicaid and the Children’s Health Insurance Program (Medicaid/CHIP), which includes a complex and growing population.

Objective of the Study:

The objective of the study was to describe patterns of expenditure persistence, mortality, and disenrollment among nondually eligible Medicaid/CHIP enrollees and identify factors predicting these outcomes.

Research Design:

The study is based on New Jersey Medicaid/CHIP claims data from 2011 to 2014. Descriptive and multinomial regression methods were used to characterize persistently extreme spenders, defined as those appearing in the top 1% of statewide spending every year, according to demographics, Medicaid/CHIP eligibility, nursing facility residence, patient risk scores, and clinical diagnostic categories measured in 2011. Similar analyses were done for persistently high spenders (ie, always in the top 10% but not always top 1%) as well as decedents, disenrollees, and moderate spenders (ie, at least 1 year outside of the top 10%).

Subjects:

Nondually eligible NJ Medicaid/CHIP enrollees in 2011.

Results:

One fourth of extreme spenders in 2011 remained in that category throughout 2011–2014. Almost all (89.3%) of the persistently extreme spenders were aged, blind, or disabled. Within the aged, blind, or disabled population, the strongest predictors of persistently extreme spending were diagnoses involving developmental disability, HIV/AIDS, central nervous system conditions, psychiatric disorders, type 1 diabetes, and renal conditions. Individuals in nursing facilities and those with very high risk scores were more likely to die or have persistently high spending than to have persistently extreme spending.

Conclusions:

The study highlights unique features of spending persistence within Medicaid/CHIP and provides methodological contributions to the broader persistence literature.
Tracheoesophageal fistula: open versus endoscopic repair
Authors: Daniel Sam J. , Smith Mariana M.
Published: September 2016

Purpose of review

The management of primary or recurrent tracheoesophageal fistula (TEF) remains an important challenge for airway surgeons.

Recent findings

The accuracy of prenatal detection can be significantly improved in specialized centers. Routine preoperative and postoperative airway endoscopy is recommended to detect a proximal fistula and evaluate vocal cord motility. Minimally invasive thoracoscopic approaches have equal success and improved cosmesis and visualization as compared with thoracostomy. Novel open approaches for complex TEF include a transcervical, transtracheal approach, and slide tracheoplasty.

Endoscopic closure of TEF carries less morbidity. Options include de-epithelialization of the tract, interposed material, and combinations. The mean operative time is 30 min; however multiple treatments are required (average 2.1). Use of continuous positive airway pressure in the immediate postoperative period was not associated with increased leak or recurrence. Children post-TEF repair continue to have frequent gastrointestinal and respiratory symptoms.

Prenatal diagnosis is beneficial both for prenatal counseling and for planning care. The ideal endoscopic approach is undecided but remains an interesting alternative to open surgery provided failures are anticipated and prompt repeated treatments initiated to preclude ongoing respiratory complications. Transtracheal approaches and slide tracheoplasty are well tolerated and effective in complex/recurrent cases. Long-term follow-up of patients with TEF is important.
Indoor allergen exposure and asthma outcomes
Authors: Sheehan William J. , Phipatanakul Wanda
Published: September 2016

Purpose of review

The aim of the present review is to discuss updates on research regarding the relationship between indoor allergen exposure and childhood asthma with a focus on clinical effects, locations of exposure, and novel treatments.

Recent findings

Recent data continue to demonstrate that early life sensitization to indoor allergens is a predictor of asthma development later in life. Furthermore, avoidance of exposure to these allergens continues to be important especially given that the vast majority of children with asthma are sensitized to at least one indoor allergen. New research suggests that mouse allergen, more so than cockroach allergen, may be the most relevant urban allergen. Recent evidence reminds us that children are exposed to clinically important levels of indoor allergens in locations away from their home, such as schools and daycare centers. Exposure to increased levels of indoor mold in childhood has been associated with asthma development and exacerbation of current asthma; however, emerging evidence suggests that early exposure to higher fungal diversity may actually be protective for asthma development. Novel treatments have been developed that target TH2 pathways thus decreasing asthmatic responses to allergens. These therapies show promise for the treatment of severe allergic asthma refractory to avoidance strategies and standard therapies.

Understanding the relationship between indoor allergens and asthma outcomes is a constantly evolving study of timing, location, and amount of exposure.
Lack of Knowledge and Low Readiness for Health Care Transition in Eosinophilic Esophagitis and Eosinophilic Gastroenteritis
Authors: Eluri Swathi , Book Wendy M. , Kodroff Ellyn , Strobel Mary Jo , Gebhart Jessica H. , Jones Patricia D. , Menard‐Katcher Paul , Ferris Maria E. , Dellon Evan S.
Published: September 2016

Objectives:

A growing population of adolescents/young adults with eosinophilic esophagitis (EoE) and eosinophilic gastroenteritis (EGE) will need to transition from pediatric to adult health providers. Measuring health care transition (HCT) readiness is critical, but no studies have evaluated this process in EoE/EGE. We determined the scope and predictors of HCT knowledge in patients and parents with EoE/EGE and measured HCT readiness in adolescents/young adults.

Methods:

We conducted an online survey of patients 13 years or older and parents of patients with EoE/EGE who were diagnosed when 25 years or younger. Parents answered questions regarding their children and their own knowledge of HCT. HCT readiness was assessed in adolescents/young adults aged 13 to 25 years with the Self‐Management and Transition to Adulthood with Rx Questionnaire (a 6‐domain self‐report tool) with a score range of 0 to 90.

Results:

Four hundred fifty participants completed the survey: 205 patients and 245 parents. Included in the analysis (those diagnosed with EoE/EGE at age 25 years or younger) were 75 of 205 patients and children of 245 parent respondents. Overall, 78% (n = 52) of the patients and 76% (n = 187) of parents had no HCT knowledge. Mean HCT readiness score in adolescents/young adults (n = 50) was 30.4 ± 11.3 with higher scores in domains of provider communication and engagement during appointments. Mean parent‐reported (n = 123) score was 35.6 ± 9.7 with higher scores in medication management and disease knowledge.

Conclusions:

There was a significant deficit in HCT knowledge, and HCT readiness scores were lower than other chronic health conditions. HCT preparation and readiness assessments should become a priority for adolescents/young adults with EoE/EGE and their parents.
Twelve Novel Mutations in the SLC26A3 Gene in 17 Sporadic Cases of Congenital Chloride Diarrhea
Authors: Amato Felice , Cardillo Giuseppe , Liguori Renato , Scorza Manuela , Comegna Marika , Elce Ausilia , Giordano Sonia , Lucaccioni Laura , Lugli Licia , Cardile Sabrina , Romano Claudio , Pezzella Vincenza , Castaldo Giuseppe , Berni Canani Roberto
Published: September 2016

Objectives:

We aimed to improve the knowledge of pathogenic mutations in sporadic cases of congenital chloride diarrhea (CCD) and emphasize the importance of functional studies to define the effect of novel mutations.

Methods:

All member 3 of solute carrier family 26 (SLC26A3) coding regions were sequenced in 17 sporadic patients with CCD. Moreover, the minigene system was used to analyze the effect of 2 novel splicing mutations.

Results:

We defined the SLC26A3 genotype of all 17 patients with CCD and identified 12 novel mutations. Using the minigene system, we confirmed the in silico prediction of a complete disruption of splicing pattern caused by 2 of these novel mutations: the c.971+3_971+4delAA and c.735+4_c.735+7delAGTA. Moreover, several prediction tools and a structure‐function prediction defined the pathogenic role of 6 novel missense mutations.

Conclusions:

We confirm the molecular heterogeneity of sporadic CCD adding 12 novel mutations to the list of known pathogenic mutations. Moreover, we underline the importance, for laboratories that offer molecular diagnosis and genetic counseling, to perform fast functional analysis of novel mutations.
Histopatholological Findings in a Fatal Case of Rinkhals Envenomation
Authors: Moar Jacob Joseph , Hill Lawrence , Stewart Michael
Published: September 2016

Although cobra envenomation continues to pose a threat to life and therefore constitutes a major cause of death in Africa, India, and Sri Lanka, the forensic pathology reports are not always histologically well documented or illustrated. The reports in the literature, although providing graphic evidence of the macroscopic appearances of the bite and its ensuing tissue necrosis, should the victim survive, often do not illustrate the histopathological findings to a sufficient degree. We present an unusual case of fatal envenomation by a rinkhals (Hemachatus haemachatus) in which the victim had been bitten a second time after a previous rinkhals' bite. Two issues therefore arose: (a) whether the histopathological findings in the spleen were an acute reaction to the snake bite or were due to immunostimulation as a consequence of the previously inflicted bite, and (b) the previously undocumented finding of extravasation of erythrocytes into the surrounding Virchow-Robin spaces in the brain, a finding usually associated with blunt head trauma and therefore interpreted as a cortical contusion hemorrhage by forensic pathologists.