bluuebunny/crossref_metadata_2025_split huggingface dataset:
Nager syndrome and Pierre Robin sequence
Authors: Rosa Rafael Fabiano Machado , Guimarães Victória Bernardes , Beltrão Luciana Amorim , Trombetta Júlia Santana , Puma Lliguin Karen Lizeth , de Mattos Vinicius Freitas , Zen Paulo Ricardo Gazzola 
Published: March 2015

AbstractNager syndrome is considered a rare genetic syndrome characterized by craniofacial and radial anomalies. Pierre Robin sequence is a triad that includes micrognathia, cleft palate and glossoptosis. The present patient had typical findings of Nager syndrome and Pierre Robin sequence. He progressed to severe respiratory distress, requiring mechanical ventilation and tracheostomy. At 1 year and 11 months, he had episodes of cardiorespiratory arrest and died. In the literature review, we identified the clinical description of 44 patients with Nager syndrome. Among them, 93.1% had micrognathia, 38.6% cleft palate and 11.3% glossoptosis. Only one (2.3%) had all three features, as observed in the present patient. Therefore, despite the fact that the features of Pierre Robin sequence are common, there are few patients who have the complete triad. It is noteworthy, however, that they may be associated with respiratory distress, which may put the patient's life at risk.
Combination of flecainide and propranolol for congenital junctional ectopic tachycardia
Authors: Imamura Tomohiko , Tanaka Yuji , Ninomiya Yumiko , Yoshinaga Masao 
Published: March 2015

AbstractCongenital junctional ectopic tachycardia is a rare tachyarrhythmia with high mortality. A pharmacological approach in early infancy is regarded as the first‐line therapeutic option. Pharmacologically, amiodarone alone or in combination with other drugs is the most commonly reported effective agent for congenital junctional ectopic tachycardia, but it has many adverse effects. Here we report the case of a 40‐day‐old infant. The clinical course suggests that combined oral flecainide and propranolol is an effective alternative therapy for early infants. Esophageal lead electrocardiography may give a clear diagnosis of junctional ectopic tachycardia.
Fibrodysplasia ossificans progressiva in a newborn with cardiac involvement
Authors: Marseglia Lucia , D'Angelo Gabriella , Manti Sara , Manganaro Alessandro , Calabrò Maria Pia , Salpietro Carmelo , Gitto Eloisa 
Published: March 2015

AbstractFibrodysplasia ossificans progressiva is a rare genetic disease that manifests in early life with malformed big toes and progressive heterotopic ossification that forms qualitatively normal bone in characteristic extraskeletal sites. Mutation c.617G>A in the activin A receptor type I gene is reported in all patients with fibrodysplasia ossificans progressiva. No cases of cardiac involvement have been described in children. We report the case of a child with halluces valgi at birth, along with two tender, firm, immovable masses located on the right and left parietal–occipital region, a transitory subluxation of the right hip and an unusual ventricular septal hypertrophy. We hypothesize that the ventricular septal hypertrophy could be the result of a thickening of the fibrous portion of the septum, and a possible new element of the phenotype, probably resulting from the mechanical stimuli secondary to the significant hemodynamic changes occurring at birth.
Suspected fetal onset of neonatal transient eosinophilic colitis and development of respiratory distress
Authors: Tanaka Katsunori , Koshida Shigeki , Yanagi Takahide , Tsutsui Hidemi , Nakahara Sayuri , Furukawa Ouki , Tsuji Shunichiro 
Published: March 2015

AbstractNeonatal transient eosinophilic colitis (NTEC) is a new disease concept within eosinophilic gastroenteritis, which was proposed by Ohtsuka et al. It causes hematochezia as a result of eosinophilia, in neonates who have not yet started to receive enteral nutrition, although the whole‐body status of the infant is in fact relatively good. To date, there have been no reports of this disease in which abnormalities were noted during gestation, and the clinical phenomena surrounding it, along with any complications, are not yet clear. We encountered a suspected case of NTEC causing respiratory distress with aspiration of hematochezia, in which dilated bowel was noted during gestation. This case indicates that NTEC may occur at the fetal stage and be complicated by respiratory distress.
Pulmonary arterial hypertension associated with chronic active Epstein–Barr virus infection
Authors: Fukuda Yutaka , Momoi Nobuo , Akaihata Mitsuko , Nagasawa Katsutoshi , Mitomo Masaki , Aoyagi Yoshimichi , Endoh Kisei , Hosoya Mitsuaki 
Published: March 2015

AbstractChronic active Epstein–Barr virus (EBV) infection (CAEBV), characterized by persistent infectious mononucleosis‐like symptoms, can lead to cardiovascular complications including coronary artery aneurysm or myocarditis. Here, we present the case of an 11‐year‐old boy with pulmonary arterial hypertension (PAH) and junctional ectopic tachycardia associated with CAEBV. The patient did not have any major symptoms attributed to CAEBV, such as fever, lymphadenopathy or splenomegaly when the PAH developed. Mild liver dysfunction was found at the first examination, and it persisted. Two years after the PAH symptoms appeared, CAEBV was evident, based on deteriorated liver function, hepatosplenomegaly, and coronary artery aneurysms. CAEBV should be considered as a cause of secondary PAH, particularly when liver dysfunction coexists.
Total anomalous pulmonary venous connection with ccTGA and VSD: Can pulmonary artery banding avert pulmonary venous obstruction?
Authors: Hayashi Taiyu , Hirata Yoichiro , Inuzuka Ryo , Hirata Yasutaka 
Published: March 2015

AbstractWe describe a rare case of infracardiac total anomalous pulmonary venous connection (TAPVC), associated with congenitally corrected transposition of the great arteries (ccTGA) and ventricular septal defect, in which the patient had undergone pulmonary artery banding (PAB) at 16 days of age. She began to have episodes of severe cyanosis while crying, 2 weeks after PAB. Cardiac catheterization at 34 days of age showed severe pulmonary hypertension and a transhepatic pressure gradient of 7 mmHg. The infant underwent TAPVC repair and conventional repair for ccTGA at 35 days of age. Although PAB might have the provisional effect of delaying the manifestation of pulmonary venous obstruction (PVO), it is unable to prevent the development of PVO due to the high resistance of the hepatic sinusoids. Signs of PVO should be closely monitored so that TAPVC can be repaired in a timely fashion.
The relationship power inventory: Development and validation
Authors: FARRELL ALLISON K. , SIMPSON JEFFRY A. , ROTHMAN ALEXANDER J. 
Published: March 2015

AbstractPower is a central concept in relationships, yet existing self‐report measures of relationship power are not well validated and do not assess all aspects of power. To address this, we developed the Relationship Power Inventory (RPI), a self‐report measure of power for romantic partners. In Study 1, we identified the most important decision‐making domains in romantic relationships. In Study 2, we generated an item pool assessing relationship power, selected the best performing items for inclusion, and tested the convergent and divergent validity properties of the RPI. Study 3 revealed RPI scores predict observer ratings of power during decision‐making discussions and showed the RPI has good test–retest reliability. We discuss how the RPI can advance research on power in close relationships
Friends with benefits? Gendered performances in women's casual sexual relationships
Authors: FAHS BREANNE , MUNGER ADRIELLE 
Published: March 2015

AbstractAlthough some research has examined “friends with benefits” relationships (FWBRs), women's subjective accounts of FWBRs remains notably understudied. Utilizing attachment theory, scripting theory, and social constructionist theories of gender, this study drew upon qualitative interviews with a community sample of 20 women (mean age = 34, SD = 13.35) from diverse ages, races, and sexual identity backgrounds to illuminate five themes in women's FWBR narratives: (a) regulation and suppression of emotions, (b) performance and idealization of detachment and emotionlessness, (c) lack of clear communication combined with “other‐defined” experiences, (d) replication of racist and sexist scripts, and (e) transitional qualities of the relationship. Implications for the power differentials present in FWBRs, and tensions between subverting and further entrenching relationship scripts, are explored.
The Administrative Presidency as Reactive Oversight: Implications for Positive and Normative Theory
Authors: West William F. 
Published: March 2015

AbstractThe fact that centralized executive oversight of agency policy making is primarily reactive reflects the motives and constraints that define the president's relationship with the domestic bureaucracy. Such a strategy allows for the allocation of limited resources to agency initiatives that are inconsistent with the president's agenda or that evoke conflict within the executive branch or the larger political system. This calls into question the descriptive model of presidential administration as proactive management by a unitary actor, as well as its prescriptive corollary that equates a strong executive with coordination across the bureaucracy. Reactive presidential oversight can produce a kind of ad hoc coordination that can be salutary, but its often pluralistic character can also undermine other important values in the administrative process.
Prostasomes of Canine Seminal Plasma – Zinc‐Binding Ability and Effects on Motility Characteristics and Plasma Membrane Integrity of Spermatozoa
Authors: Mogielnicka‐Brzozowska M , Strzeżek R , Wasilewska K , Kordan W 
Published: March 2015

ContentsProstasomes are small lipid membrane‐confined vesicles that are involved in various fertilization‐related processes. The aim of this study was to demonstrate canine seminal plasma prostasomes' ability to bind zinc ions, as well as examining their effects on sperm motility characteristics and plasma membrane integrity during cold storage. Ejaculates, collected from five cross‐bred dogs (n = 50), were subjected to ultracentrifugation followed by gel filtration (GF) on a Superose 6 column. Prostasomes appeared as a single fraction in the elution profile. Transmission electron microscopy (TEM) analysis of canine prostasomes revealed the presence of membrane vesicles with diameters ranging from 20.3 to 301 nm. The zinc‐affinity chromatography on a Chelating Sepharose Fast Flow – Zn2 + showed that from 93 to 100% of the prostasome proteins bind zinc ions (P+Zn). SDS‐PAGE revealed that canine P+Zn comprised four protein bands, with low molecular weights (10.2–12 kDa). We have also shown a positive effect of prostasomes (p < 0.05), especially variant B (2% of total seminal plasma protein) on canine sperm motility parameters after 2 h storage at 5°C (TMOT%, 44.75 ± 5.18) and PMOT%, 12.42 ± 1.59) and VAP, VSL, VCL, when compared with Control (TMOT%, 7.30 ± 1.41 and PMOT%, 1.70 ± 0.42). Higher percentage of spermatozoa with intact plasma membrane (SYBR/PI dual staining) and intact acrosome (Giemsa stained), after 2 h storage at 5°C, was showed, in variant A (1.5% of total seminal plasma protein) and B, when compared with Control and variant C (2.5% of total seminal plasma protein). The prostasomes' effect on motility and plasma membrane integrity of canine cold‐stored spermatozoa may be related to their ability to bind zinc ions and regulate their availability to the sperm.
Efforts to reduce the disparity between permanent residents and temporary migrants: Stop TB experiences in Shanghai, China
Authors: Lu Hui , Chen Jing , Wang Wei , Wu Laiwa , Shen Xin , Yuan Zhengan , Yan Fei 
Published: March 2015

AbstractObjectiveEight of 17 districts of Shanghai have offered transportation and living allowances subsidies to patients with tuberculosis (TB) among the migrant population. The study aimed to assess the impact of the subsidising initiative on the treatment success rate (TSR) and identify the social determinants of treatment outcomes.MethodsThe participants included 7072 residents and 5703 migrants who were registered in the TB Information Management System with smear‐positive pulmonary TB from January 2006 to December 2010. The Cochran–Armitage test was employed to test the trends of TSR and logistic regressions to identify the factors associated with treatment outcome.ResultsWithout subsidies, migrant TB cases had lower odds of successful treatment [OR = 0.20 (95% CI 0.18–0.23)] than resident cases. Subsidisation was associated with a 65% increased odds ratio of success [1.65 (1.40–1.95)] among migrant cases. The TSR has stabilised at 87% for both permanent residents and temporary migrants since 2009. Living in districts with a population density ≥20 000/km2 was associated with a low odds ratio [0.42 (0.26–0.68)] among resident cases, whereas among migrant cases those living in districts out of central downtown had a higher odds ratio of treatment success [peripheral downtown: 1.73 (1.36–2.20), suburban: 1.69 (1.16–2.46)]. The TB cases in districts with 2.0–2.9 TB specialists/100 cases had a higher odds ratio [2.99 (1.91–4.69)] of successful treatment than cases from districts with fewer specialists.ConclusionsBesides free medical services, transport and living allowance subsidies to migrant patients with TB improved the treatment outcome significantly.
Middle East Respiratory Syndrome Coronavirus: Another Zoonotic Betacoronavirus Causing SARS-Like Disease
Authors: Chan Jasper F. W. , Lau Susanna K. P. , To Kelvin K. W. , Cheng Vincent C. C. , Woo Patrick C. Y. , Yuen Kwok-Yung 
Published: March 2015

SUMMARY

The source of the severe acute respiratory syndrome (SARS) epidemic was traced to wildlife market civets and ultimately to bats. Subsequent hunting for novel coronaviruses (CoVs) led to the discovery of two additional human and over 40 animal CoVs, including the prototype lineage C betacoronaviruses,
Tylonycteris
bat CoV HKU4 and
Pipistrellus
bat CoV HKU5; these are phylogenetically closely related to the Middle East respiratory syndrome (MERS) CoV, which has affected more than 1,000 patients with over 35% fatality since its emergence in 2012. All primary cases of MERS are epidemiologically linked to the Middle East. Some of these patients had contacted camels which shed virus and/or had positive serology. Most secondary cases are related to health care-associated clusters. The disease is especially severe in elderly men with comorbidities. Clinical severity may be related to MERS-CoV's ability to infect a broad range of cells with DPP4 expression, evade the host innate immune response, and induce cytokine dysregulation. Reverse transcription-PCR on respiratory and/or extrapulmonary specimens rapidly establishes diagnosis. Supportive treatment with extracorporeal membrane oxygenation and dialysis is often required in patients with organ failure. Antivirals with potent
in vitro
activities include neutralizing monoclonal antibodies, antiviral peptides, interferons, mycophenolic acid, and lopinavir. They should be evaluated in suitable animal models before clinical trials. Developing an effective camel MERS-CoV vaccine and implementing appropriate infection control measures may control the continuing epidemic.
A Microbiological Revolution Meets an Ancient Disease: Improving the Management of Tuberculosis with Genomics
Authors: Wlodarska Marta , Johnston James C. , Gardy Jennifer L. , Tang Patrick 
Published: March 2015

SUMMARY

Tuberculosis (TB) is an ancient disease with an enormous global impact. Despite declining global incidence, the diagnosis, phenotyping, and epidemiological investigation of TB require significant clinical microbiology laboratory resources. Current methods for the detection and characterization of
Mycobacterium tuberculosis
consist of a series of laboratory tests varying in speed and performance, each of which yields incremental information about the disease. Since the sequencing of the first
M. tuberculosis
genome in 1998, genomic tools have aided in the diagnosis, treatment, and control of TB. Here we summarize genomics-based methods that are positioned to be introduced in the modern clinical TB laboratory, and we highlight how recent advances in genomics will improve the detection of antibiotic resistance-conferring mutations and the understanding of
M. tuberculosis
transmission dynamics and epidemiology. We imagine the future TB clinic as one that relies heavily on genomic interrogation of the
M. tuberculosis
isolate, allowing for more rapid diagnosis of TB and real-time monitoring of outbreak emergence.
Needle-Free Dermal Delivery of a Diphtheria Toxin CRM197Mutant on Potassium-Doped Hydroxyapatite Microparticles
Authors: Weissmueller Nikolas T. , Schiffter Heiko A. , Carlisle Robert C. , Rollier Christine S. , Pollard Andrew J. 
Published: March 2015

ABSTRACTInjections with a hypodermic needle and syringe (HNS) are the current standard of care globally, but the use of needles is not without limitation. While a plethora of needle-free injection devices exist, vaccine reformulation is costly and presents a barrier to their widespread clinical application. To provide a simple, needle-free, and broad-spectrum protein antigen delivery platform, we developed novel potassium-doped hydroxyapatite (K-Hap) microparticles with improved protein loading capabilities that can provide sustained local antigen presentation and release. K-Hap showed increased protein adsorption over regular hydroxyapatite (P< 0.001), good structural retention of the model antigen (CRM197) with 1% decrease in α-helix content and no change in β-sheet content upon adsorption, and sustained releasein vitro. Needle-free intradermal powder inoculation with K-Hap–CRM197induced significantly higher IgG1 geometric mean titers (GMTs) than IgG2a GMTs in a BALB/c mouse model (P< 0.001) and induced IgG titer levels that were not different from the current clinical standard (P> 0.05), namely, alum-adsorbed CRM197by intramuscular (i.m.) delivery. The presented results suggest that K-Hap microparticles may be used as a novel needle-free delivery vehicle for some protein antigens.
Gammaretrovirus-Specific Antibodies in Free-Ranging and Captive Namibian Cheetahs
Authors: Krengel Annika , Cattori Valentino , Meli Marina L. , Wachter Bettina , Böni Jürg , Bisset Leslie R. , Thalwitzer Susanne , Melzheimer Jörg , Jago Mark , Hofmann-Lehmann Regina , Hofer Heribert , Lutz Hans 
Published: March 2015

ABSTRACTThe cheetah population in Namibia is the largest free-ranging population in the world and a key population for research regarding the health status of this species. We used serological methods and quantitative real-time PCR to test free-ranging and captive Namibian cheetahs for the presence of feline leukemia virus (FeLV), a gammaretrovirus that can be highly aggressive in populations with low genetic diversity, such as cheetahs. We also assessed the presence of antibodies to other gammaretroviruses and the responses to a FeLV vaccine developed for domestic cats. Up to 19% of the free-ranging cheetahs, 27% of the captive nonvaccinated cheetahs, and 86% of the captive vaccinated cheetahs tested positive for FeLV antibodies. FeLV-antibody-positive free-ranging cheetahs also tested positive for Rauscher murine leukemia virus antibodies. Nevertheless, FeLV was not detectable by quantitative real-time PCR and no reverse transcriptase activity was detectable by product-enhanced reverse transcriptase assay in the plasma of cheetahs or the supernatants from cultures of peripheral blood mononuclear cells. The presence of antibodies to gammaretroviruses in clinically healthy specimens may be caused either by infection with a low-pathogenic retrovirus or by the expression of endogenous retroviral sequences. The strong humoral immune responses to FeLV vaccination demonstrate that cheetahs can respond to the vaccine and that vaccination against FeLV infection may be beneficial should FeLV infection ever become a threat, as was seen in Iberian lynx and Florida panthers.
First Probable Case of Subcutaneous Infection Due to Truncatella angustata: a New Fungal Pathogen of Humans?
Authors: Jagielski Tomasz , Żak Iwona , Tyrak Jerzy , Bryk Agata 
Published: March 2015

ABSTRACTTruncatella angustatais a coelomycetous fungus, typically associated with vascular plants as either an endophyte or a pathogen. This organism has not previously been implicated in human disease. This report describes a case ofT. angustatasubcutaneous infection in an immunocompetent patient. A conclusive diagnosis was achieved through partial sequencing of ribosomal DNA (rDNA) cluster. The patient was successfully treated with voriconazole followed by itraconazole.
Evaluation of the Qiagen
artus
C. difficile QS-RGQ Kit for Detection of Clostridium difficile Toxins A and B in Clinical Stool Specimens
Authors: Jazmati Nathalie , Wiegel Pia , Ličanin Božica , Plum Georg 
Published: March 2015

ABSTRACT

We compared the Qiagen
artus
C. difficile QS-RGQ kit, a new nucleic acid amplification test for the detection of
Clostridium difficile
toxins in stool specimens, with the Cepheid Xpert
C. difficile
test. The sensitivity, specificity, positive predictive value, and negative predictive value for the Qiagen
artus
C. difficile QS-RGQ test were 100%, 89.5%, 60.9%, and 100%, and those for the Cepheid Xpert
C. difficile
test were 100%, 90%, 62.2%, and 100%, respectively.
What Is the Origin of Livestock-Associated Methicillin-Resistant Staphylococcus aureus Clonal Complex 398 Isolates from Humans without Livestock Contact? An Epidemiological and Genetic Analysis
Authors: Lekkerkerk W. S. N. , van Wamel W. J. B. , Snijders S. V. , Willems R. J. , van Duijkeren E. , Broens E. M. , Wagenaar J. A. , Lindsay J. A. , Vos M. C. 
Published: March 2015

ABSTRACT

Fifteen percent of all methicillin-resistant
Staphylococcus aureus
(MRSA) clonal complex 398 (CC398) human carriers detected in The Netherlands had not been in direct contact with pigs or veal calves. To ensure low MRSA prevalence, it is important to investigate the likely origin of this MRSA of unknown origin (MUO). Recently, it was shown that CC398 strains originating from humans and animals differ in the presence of specific mobile genetic elements (MGEs). We hypothesized that determining these specific MGEs in MUO isolates and comparing them with a set of CC398 isolates of various known origin might provide clues to their origin. MUO CC398 isolates were compared to MRSA CC398 isolates obtained from humans with known risk factors, a MRSA CC398 outbreak isolate, livestock associated (LA) MRSA CC398 isolates from pigs, horses, chickens, and veal calves, and five methicillin-susceptible
Staphylococcus aureus
(MSSA) CC398 isolates of known human origin. All strains were
spa
typed, and the presence or absence of,
scn
,
chp
, φ3
int
, φ6
int
, φ7
int
,
rep7
,
rep27
, and
cadDX
was determined by PCRs. The MRSA CC398 in humans, MUO, or MRSA of known origin (MKO) resembled MRSA CC398 as found in pigs and not MSSA CC398 as found in humans. The distinct human MSSA CC398
spa
type, t571, was not present among our MRSA CC398 strains; MRSA CC398 was tetracycline resistant and carried no φ3 bacteriophage with
scn
and
chp
. We showed by simple PCR means that human MUO CC398 carriers carried MRSA from livestock origin, suggestive of indirect transmission. Although the exact transmission route remains unknown, direct human-to-human transmission remains a possibility as well.