bluuebunny/crossref_metadata_2025_split huggingface dataset:
Long term longitudinal follow-up of an AD-HIES cohort: the impact of early diagnosis and enrollment to IPINet centers on the natural history of Job’s syndrome
Authors: Carrabba Maria , Dellepiane Rosa Maria , Cortesi Manuela , Baselli Lucia Augusta , Soresina Annarosa , Cirillo Emilia , Giardino Giuliana , Conti Francesca , Dotta Laura , Finocchi Andrea , Cancrini Caterina , Milito Cinzia , Pacillo Lucia , Cinicola Bianca Laura , Cossu Fausto , Consolini Rita , Montin Davide , Quinti Isabella , Pession Andrea , Fabio Giovanna , Pignata Claudio , Pietrogrande Maria Cristina , Badolato Raffaele 
Published: April 2023

AbstractJob’s syndrome, or autosomal dominant hyperimmunoglobulin E syndrome (AD-HIES, STAT3-Dominant Negative), is a rare inborn error of immunity (IEI) with multi-organ involvement and long-life post-infective damage. Longitudinal registries are of primary importance in improving our knowledge of the natural history and management of these rare disorders. This study aimed to describe the natural history of 30 Italian patients with AD-HIES recorded in the Italian network for primary immunodeficiency (IPINet) registry. This study shows the incidence of manifestations present at the time of diagnosis versus those that arose during follow up at a referral center for IEI. The mean time of diagnostic delay was 13.7 years, while the age of disease onset was < 12 months in 66.7% of patients. Respiratory complications, namely bronchiectasis and pneumatoceles, were present at diagnosis in 46.7% and 43.3% of patients, respectively. Antimicrobial prophylaxis resulted in a decrease in the incidence of pneumonia from 76.7% to 46.7%. At the time of diagnosis, skin involvement was present in 93.3% of the patients, including eczema (80.8%) and abscesses (66.7%). At the time of follow-up, under therapy, the prevalence of complications decreased: eczema and skin abscesses reduced to 63.3% and 56.7%, respectively. Antifungal prophylaxis decreased the incidence of mucocutaneous candidiasis from 70% to 56.7%. During the SARS-CoV-2 pandemic, seven patients developed COVID-19. Survival analyses showed that 27 out of 30 patients survived, while three patients died at ages of 28, 39, and 46 years as a consequence of lung bleeding, lymphoma, and sepsis, respectively. Analysis of a cumulative follow-up period of 278.7 patient-years showed that early diagnosis, adequate management at expertise centers for IEI, prophylactic antibiotics, and antifungal therapy improve outcomes and can positively influence the life expectancy of patients.
DQN-based resource allocation for NOMA-MEC-aided multi-source data stream
Authors: Ling Jing , Xia Junjuan , Zhu Fusheng , Gao Chongzhi , Lai Shiwei , Balasubramanian Venki 
Published: April 2023

AbstractThis paper investigates a non-orthogonal multiple access (NOMA)-aided mobile edge computing (MEC) network with multiple sources and one computing access point (CAP), in which NOMA technology is applied to transmit multi-source data streams to CAP for computing. To measure the performance of the considered NOMA-aided MEC network, we first design the system cost as a linear weighting function of energy consumption and delay under the NOMA-aided MEC network. Moreover, we propose a deep Q network (DQN)-based offloading strategy to minimize the system cost by jointly optimizing the offloading ratio and transmission power allocation. Finally, we design experiments to demonstrate the effectiveness of the proposed strategy. Specifically, the designed strategy can decrease the system cost by about 15% compared with local computing when the number of sources is 5.
Study on Hermite-Hadamard-type inequalities using a new generalized fractional integral operator
Authors: Ni Jinbo , Chen Gang , Dong Hudie 
Published: April 2023

AbstractIn this study, a new definition of the fractional integral operator is first proposed, which generalizes some well-known fractional integral operators. Then, by using this newly generalized fractional integral operator, we proved several new Hermite-Hadamard-type inequalities for convex functions. Finally, we provided some corollaries to show that the current results extend and enrich the previous results in the literature.
Positive continuous solutions for some semilinear elliptic problems in the half space
Authors: Alsaedi Ramzi , Ghanmi Abdeljabbar , Zeddini Noureddine 
Published: April 2023

AbstractThe aim of this article is twofold. The first goal is to give a new characterization of the Kato class of functions $K^{\infty}({\mathbb{R}}_{+}^{d})$

K
∞

(

R
+
d

)
that was defined in (Bachar et al. 2002:41, 2002) for $d=2$
d
=
2
and in (Bachar and Mâagli 9(2):153–192, 2005) for $d\geq 3$
d
≥
3
and adapted to study some nonlinear elliptic problems in the half space. The second goal is to prove the existence of positive continuous weak solutions, having the global behavior of the associated homogeneous problem, for sufficiently small values of the nonnegative constants λ and μ to the following system: $\Delta u=\lambda f(x,u,v)$
Δ
u
=
λ
f
(
x
,
u
,
v
)
, $\Delta v=\mu g(x,u,v)$
Δ
v
=
μ
g
(
x
,
u
,
v
)
in ${\mathbb{R}}_{+}^{d}$

R
+
d

, $\lim _{x\rightarrow (\xi ,0)}u(x)=a_{1}\phi _{1}(\xi )$

lim

x
→
(
ξ
,
0
)

u
(
x
)
=

a
1

ϕ
1

(
ξ
)
, $\lim _{x\rightarrow (\xi ,0)}v(x)=a_{2}\phi _{2}(\xi )$

lim

x
→
(
ξ
,
0
)

v
(
x
)
=

a
2

ϕ
2

(
ξ
)
for all $\xi \in {\mathbb{R}}^{d-1}$
ξ
∈

R

d
−
1

, $\lim _{x_{d} \rightarrow \infty}\frac{u(x)}{x_{d}}=b_{1}$

lim

x
d

→
∞

u
(
x
)

x
d

=

b
1

, $\lim _{x_{d} \rightarrow \infty}\frac{v(x)}{x_{d}}=b_{2}$

lim

x
d

→
∞

v
(
x
)

x
d

=

b
2

, where $\phi _{1}$

ϕ
1

and $\phi _{2}$

ϕ
2

are nontrivial nonnegative continuous functions on $\partial {\mathbb{R}}_{+}^{d}= {\mathbb{R}}^{d-1}\times \{0\}$
∂

R
+
d

=

R

d
−
1

×
{
0
}
, $a_{1}, a_{2}, b_{1}, b_{2}$

a
1

,

a
2

,

b
1

,

b
2

are nonnegative constants such that $(a_{1}+b_{1})(a_{2}+b_{2})>0$
(

a
1

+

b
1

)
(

a
2

+

b
2

)
>
0
. The functions f and g are nonnegative and belong to a class of functions containing in particular all functions of the type $f(x,u,v)=p(x) u^{\alpha}g_{1}(v)$
f
(
x
,
u
,
v
)
=
p
(
x
)

u
α

g
1

(
v
)
and $g(x,u,v)=q(x)g_{2}(u)v^{\beta}$
g
(
x
,
u
,
v
)
=
q
(
x
)

g
2

(
u
)

v
β

with $\alpha \geq 1$
α
≥
1
, $\beta \geq 1$
β
≥
1
, $g_{1}$

g
1

, $g_{2}$

g
2

are continuous on $[0,\infty )$
[
0
,
∞
)
, and $p,q$
p
,
q
are nonnegative functions in $K^{\infty}({\mathbb{R}}_{+}^{d})$

K
∞

(

R
+
d

)
.
Exploring the eating disorder curricula of accredited university dietetic programs in Australia and New Zealand
Authors: Parker Elizabeth Kumiko , Ashley Mellisa Anne , Harris Deanne Maree , Stefoska-Needham Anita 
Published: April 2023

Abstract
Background
Dietitians are viewed as integral members of the multidisciplinary treatment team for people with eating disorders (EDs). However, low levels of perceived confidence, competence, and willingness to practice in this clinical area, have been reported by dietitians and student dietitians. As the extent of ED-specific knowledge and skills-based training within tertiary accredited dietetic programs is currently unknown, this research aimed to: (1) obtain insights into the current ED-specific knowledge base and training content of dietetic curricula in both Australian and New Zealand universities; (2) understand the perspectives of course convenors regarding the role of dietitians in ED treatment and their employment opportunities; and (3) identify gaps and opportunities for improving university programs and the dietetic workforce.

Methods
Course convenors (or their nominated representative) of Australian and New Zealand accredited dietetic programs were invited to participate in a semi-structured virtual interview. A purpose-built question guide was developed to explore the inclusion and/or integration of ED-specific content into the curricula, and the perspectives of course convenors toward the role of dietitians in the treatment of EDs, and their employment opportunities. The interviews were audio recorded, transcribed verbatim, and analysed qualitatively using inductive thematic analysis.

Results
Thirteen participants who represented 14 universities and 19 individual accredited dietetic programs, were interviewed, with some participants representing more than one university. Three dominant themes emerged: (1) varying ED-specific content and training in dietetic programs; (2) unclear dietitian's role in the treatment of EDs, and (3) contrasting views regarding ED clinical practice and employment.

Conclusions
ED-specific content was embedded within all the dietetic programs investigated in this study. However, this content was generally limited to an introductory level, with notable variations found between the depth of content and the type of training provided. Risk-mitigation skill development, such as screening for EDs, and early identification of symptoms, also varied between programs. Therefore, it is recommended that ED-specific skill development and knowledge is enhanced within Australian and New Zealand university programs, to support effective, safe, and timely care for people with EDs. This research has implications for current and future university dietetic program development and the broader dietetic workforce.
Remifentanil but not sufentanil induces cardioprotection in human ischemic heart muscle in vitro
Authors: Kunecki Marcin , Oleksy Tomasz , Martynów Jan , Zygmunt Michalina , Deja Marek , Kargul Tomasz , Biernat Jolanta , Podolec Piotr , Gołba Krzysztof S. , Płazak Wojciech 
Published: April 2023

Abstract
Background
Previous studies on animal models have suggested that δ-opioid receptor (OR) signaling is the primary pathway responsible for opioids' cardioprotective effect. We hypothesize that the μ-OR's activation protects the human heart muscle.

Methods
We performed the experiments on muscular trabeculae obtained from the right atrial appendages of 104 consecutive patients subjected to coronary artery bypass surgery. Two trabeculae from each patient were studied simultaneously and exposed to 60 min of hypoxia with subsequent 60 min of reoxygenation. Remifentanil (5 μM or 50 μM) or sufentanil (40 μM or 400 μM) was used from the time of reoxygenation. Trabeculae contractility was assessed as the maximal amplitude of the contraction at baseline, after 60 min of hypoxia, during reoxygenation, and after norepinephrine application.

Results
During reperfusion, the application of remifentanil improved cardiomyocytes' function as compared to the control group (time from reperfusion: 15 min: 39.8% vs. 21.7%, p = 0.01; 30 min: 41.4% vs. 21.8%, p = 0.01; 60 min: 42.7% vs. 26.9%, p = 0.04; after norepinephrine: 64.7% vs. 43.2%, p = 0.03). The application of sufentanil did not influence cardiomyocyte function as can be seen when comparing the results of the experimental and control group.

Conclusions
Remifentanil, but not sufentanil, induces a cardioprotective effect on human right atria muscle in in vitro conditions, manifested as the increased amplitude of their contraction during reperfusion after 60 min of ischemia.
Experimental study on convective heat transfer of an open-loop borehole heat exchanger
Authors: Bu Xianbiao , Jiang Kunqing , Li Huashan , Ma Feng , Wang Lingbao 
Published: April 2023

AbstractOpen-loop borehole heat exchanger (OBHE) is a single well geothermal heat exchanger with an open-loop structure that can realize the geothermal energy extraction without mining the geothermal water. In this paper, a sandbox experiment is designed to simulate the convective heat transfer process in the reservoir area of OBHE. The mechanism of convective heat transfer in the reservoir area is studied, and the key factors that affect the convection heat transfer intensity are analyzed. The results show that the convection heat transfer of OBHE in the reservoir area is affected by both the driving effect of fluid flow inside the screen tube and the buoyancy effect. In the forward flow mode, the two effects have the opposite direction. While in the backward mode, the two effects have the same direction. The backward flow mode is more conducive to convective heat transfer. In addition, many factors influencing significantly the convective heat transfer of OBHE include inlet temperature, inlet flow rate, reservoir temperature, fluid flow direction and inner tube diameter.
Identification of qBK2.1, a novel QTL controlling rice resistance against Fusarium fujikuroi
Authors: Chen Szu-Yu , Lai Ming-Hsin , Chu Yi-Ling , Wu Dong-Hong , Tung Chih-Wei , Chen Yue-Jie , Chung Chia-Lin 
Published: April 2023

Abstract
Background
Bakanae disease caused by Fusarium fujikuroi is an increasing threat to rice production. The infected plants show symptoms such as elongation, slenderness, chlorosis, a large leaf angle, and even death. Bakanae disease is traditionally managed by seed treatment. However, fungicide-resistant F. fujikuroi isolates have emerged in several Asian areas, including Taiwan. This study aimed to identify new bakanae resistance quantitative trait loci (QTLs) and provide molecular markers to assist future breeding.

Results
A population of F2:9 recombinant inbred lines (RILs) was derived from the cross between an elite japonica Taiwanese cultivar ‘Taikeng 16 (TK16)’ and an indica variety ‘Budda’. ‘Budda’ was found highly resistant to all 24 representative isolates of the F. fujikuroi population in Taiwan. For the RIL population, 6,492 polymorphic single nucleotide polymorphisms (SNPs) spanning the rice genome were obtained by genotyping-by-sequencing (GBS) technique, and the disease severity index (DSI) was evaluated by inoculation with a highly virulent F. fujikuroi isolate Ff266. Trait-marker association analysis of 166 RILs identified two QTLs in ‘Budda’. qBK2.1 (21.97–30.15 Mb) is a novel and first bakanae resistance QTL identified on chromosome 2. qBK1.8 (5.24–8.66 Mb) partially overlaps with the previously reported qBK1.3 (4.65–8.41 Mb) on chromosome 1. The log of odds (LOD) scores of qBK1.8 and qBK2.1 were 4.75 and 6.13, accounting for 4.9% and 8.1% of the total phenotypic variation, respectively. 64 RILs carrying both qBK1.8 and qBK2.1 showed lower DSI (7%) than the lines carrying only qBK1.8 (15%), only qBK2.1 (13%), or none of the two QTLs (21%). For the future application of identified QTLs, 11 KBioscience competitive allele-specific PCR (KASP) markers and 3 insertion-deletion (InDel) markers were developed.

Conclusions
Compared to other important rice diseases, knowledge of bakanae resistance has been insufficient, which limited the development and deployment of resistant cultivars. The discovery of qBK2.1 has provided a new source of bakanae resistance. The resistant RILs inheriting good plant type, good taste, and high yield characteristics from ‘TK16’ can be used as good resistance donors. Our newly developed markers targeting qBK2.1 and qBK1.8 can also serve as an important basis for future fine-mapping and resistance breeding.
First non-invasive magnetic phrenic nerve and diaphragm stimulation in anaesthetized patients: a proof-of-concept study
Authors: Panelli Alessandro , Bartels Hermann Georges , Krause Sven , Verfuß Michael André , Grimm Aline Michèle , Carbon Niklas Martin , Grunow Julius J. , Stutzer Diego , Niederhauser Thomas , Brochard Laurent , Weber-Carstens Steffen , Schaller Stefan J. 
Published: April 2023

Abstract
Background
Mechanical ventilation has side effects such as ventilator-induced diaphragm dysfunction, resulting in prolonged intensive care unit length of stays. Artificially evoked diaphragmatic muscle contraction may potentially maintain diaphragmatic muscle function and thereby ameliorate or counteract ventilator-induced diaphragm dysfunction. We hypothesized that bilateral non-invasive electromagnetic phrenic nerve stimulation (NEPNS) results in adequate diaphragm contractions and consecutively in effective tidal volumes.

Results
This single-centre proof-of-concept study was performed in five patients who were 30 [IQR 21–33] years old, 60% (n = 3) females and undergoing elective surgery with general anaesthesia. Following anaesthesia and reversal of muscle relaxation, patients received bilateral NEPNS with different magnetic field intensities (10%, 20%, 30%, 40%); the stimulation was performed bilaterally with dual coils (connected to one standard clinical magnetic stimulator), specifically designed for bilateral non-invasive electromagnetic nerve stimulation. The stimulator with a maximal output of 2400 Volt, 160 Joule, pulse length 160 µs at 100% intensity was limited to 50% intensity, i.e. each single coil had a maximal output of 0.55 Tesla and 1200 Volt. There was a linear relationship between dosage (magnetic field intensity) and effect (tidal volume, primary endpoint, p < 0.001). Mean tidal volume was 0.00, 1.81 ± 0.99, 4.55 ± 2.23 and 7.43 ± 3.06 ml/kg ideal body weight applying 10%, 20%, 30% and 40% stimulation intensity, respectively. Mean time to find an initial adequate stimulation point was 89 (range 15–441) seconds.

Conclusions
Bilateral non-invasive electromagnetic phrenic nerve stimulation generated a tidal volume of 3–6 ml/kg ideal body weight due to diaphragmatic contraction in lung-healthy anaesthetized patients. Further perspectives in critically ill patients should include assessment of clinical outcomes to confirm whether diaphragm contraction through non-invasive electromagnetic phrenic nerve stimulation potentially ameliorates or prevents diaphragm atrophy.
The MATE trial: a multicentre, mixed-methodology, pilot, randomised controlled trial in neovascular age-related macular degeneration
Authors: Airody Archana , Baseler Heidi A. , Seymour Julie , Allgar Victoria , Mukherjee Rajarshi , Downey Louise , Dhar-Munshi Sushma , Mahmood Sajjad , Balaskas Konstantinos , Empeslidis Theo , Hanson Rachel L. W. , Dorey Tracey , Szczerbicki Tom , Sivaprasad Sobha , Gale Richard P. 
Published: April 2023

Abstract
Background/objectives
In healthcare research investigating complex interventions, gaps in understanding of processes can be filled by using qualitative methods alongside a quantitative approach. The aim of this mixed-methods pilot trial was to provide feasibility evidence comparing two treatment regimens for neovascular age-related macular degeneration (nAMD) to inform a future large-scale randomised controlled trial (RCT).

Subjects/methods
Forty-four treatment-naïve nAMD patients were followed over 24 months and randomised to one of two treatment regimens: standard care (SC) or treat and extend (T&E). The primary objective evaluated feasibility of the MATE trial via evaluations of screening logs for recruitment rates, nonparticipation and screen fails, whilst qualitative in-depth interviews with key study staff evaluated the recruitment phase and running of the trial. The secondary objective assessed changes in visual acuity and central retinal thickness (CRT) between the two treatment arms.

Results
The overall recruitment rate was 3.07 participants per month with a 40.8% non-participation rate, 18.51% screen-failure rate and 15% withdrawal/non-completion rate. Key themes in the recruitment phase included human factors, protocol-related issues, recruitment processes and challenges. Both treatment regimens showed a trend towards a visual acuity gain at month 12 which was not maintained at month 24, whilst CRT reduced similarly in both regimens over the same time period. These were achieved with one less treatment following a T&E regimen.

Conclusion
This mixed-methodology, pilot RCT achieved its pre-defined recruitment, nonparticipation and screen failure rates, thus deeming it a success. With some minor protocol amendments, progression to a large-scale RCT will be achievable.
Updates in the perioperative management of postpartum hemorrhage
Authors: Samir Ghada M. 
Published: April 2023

Abstract
Background
Postpartum hemorrhage (PPH) is the leading cause of maternal death all over the world. It could be primary or secondary with uterine atony being the cause in 80% of cases.

Main body
For anticipated PPH, special antenatal preparation for abnormal placentation, inherited coagulation disorders, and Jehovah’s Witnesses must be done. Optimal surgical management of placenta accreta is done by scheduling delivery in an appropriate surgical facility, by insertion of prophylactic embolization catheters in the uterine or internal iliac arteries, and by rapid diagnosis of PPH. The obstetric shock index (SI) is highly specific for PPH. Optimal anesthetic management is done by oxygen supply, using warming devices, ensuring wide bore intravenous (IV) access with adequate volume replacement, and blood product preparation. The non-pneumatic anti-shock garment (NASG) could be used as first-aid compression device. Permissive resuscitation, uterotonic administration, tranexamic acid, recombinant active factor seven (VIIa), and lyophilized fibrinogen concentrate are beneficial. Hemostatic reanimation to correct coagulopathy and cell saver auto-transfusion are applied. For unanticipated PPH, guidelines and regular skill training reduce the incidence of severe PPH.

Conclusions
Anticipated PPH requires antenatal preparation, optimal anesthetic management with the implementation of permissive resuscitation, hemostatic reanimation, and optimal surgical management.
Pelvic venous congestion syndrome: female venous congestive syndromes and endovascular treatment options
Authors: Kashef Elika , Evans Elizabeth , Patel Neeral , Agrawal Deepsha , Hemingway Anne P 
Published: April 2023

AbstractPelvic venous congestion syndrome (PVCS) is a common, but underdiagnosed, cause of chronic pelvic pain (CPP) in women.PVCS occurs usually, but not exclusively, in multiparous women. It is characterized by chronic pelvic pain of more than six months duration with no evidence of inflammatory disease.The patients present to general practitioners, gynaecologists, vascular specialists, pain specialists, gastroenterologists and psychiatrists. Pain of variable intensity occurs at any time but is worse in the pre-menstrual period, and is exacerbated by walking, standing, and fatigue. Post coital ache, dysmenorrhea, dyspareunia, bladder irritability and rectal discomfort are also common. Under-diagnosis of this condition can lead to anxiety and depression.A multidisciplinary approach in the investigation and management of these women is vital.Non-invasive imaging (US, CT, MRI) are essential in the diagnosis and exclusion of other conditions that cause CPP as well in the definitive diagnosis of PVCS. Trans-catheter venography remains the gold standard modality for the definitive diagnosis and is undertaken as an immediate precursor to ovarian vein embolization (OVE). Conservative, medical and surgical management strategies have been reported but have been superseded by OVE, which has a reported technical success rates of 96–100%, low complication rates and long-term symptomatic relief in between 70–90% of cases.The condition, described in this paper as PVCS, is referred to by a wide variety of other terms in the literature, a cause of confusion.There is a significant body of literature describing the syndrome and the excellent outcomes following OVE however the lack of prospective, multicentre randomized controlled trials for both investigation and management of PVCS is a significant barrier to the complete acceptance of both the existence, investigation and management of the condition.