bluuebunny/crossref_metadata_2025 huggingface dataset:
A coadapted KNL1 and spindle assembly checkpoint axis orchestrates precise mitosis in Arabidopsis
Authors: Deng Xingguang , He Ying , Tang Xiaoya , Liu Xianghong , Lee Yuh-Ru Julie , Liu Bo , Lin Honghui
Published: January 2024

The kinetochore scaffold 1 (KNL1) protein recruits spindle assembly checkpoint (SAC) proteins to ensure accurate chromosome segregation during mitosis. Despite such a conserved function among eukaryotic organisms, its molecular architectures have rapidly evolved so that the functional mode of plant KNL1 is largely unknown. To understand how SAC signaling is regulated at kinetochores, we characterized the function of the
KNL1
gene in
Arabidopsis thaliana
. The KNL1 protein was detected at kinetochores throughout the mitotic cell cycle, and null
knl1
mutants were viable and fertile but exhibited severe vegetative and reproductive defects. The mutant cells showed serious impairments of chromosome congression and segregation, that resulted in the formation of micronuclei. In the absence of KNL1, core SAC proteins were no longer detected at the kinetochores, and the SAC was not activated by unattached or misaligned chromosomes. Arabidopsis KNL1 interacted with SAC essential proteins BUB3.3 and BMF3 through specific regions that were not found in known KNL1 proteins of other species, and recruited them independently to kinetochores. Furthermore, we demonstrated that upon ectopic expression, the KNL1 homolog from the dicot tomato was able to functionally substitute KNL1 in
A
.
thaliana
, while others from the monocot rice or moss associated with kinetochores but were not functional, as reflected by sequence variations of the kinetochore proteins in different plant lineages. Our results brought insights into understanding the rapid evolution and lineage-specific connection between KNL1 and the SAC signaling molecules.
‘Until’ constructions and expletive negation in Huasteca Nahuatl
Authors: Olguin Martinez Jesus
Published: January 2024

A number of works have explored expletive negation in clause-linkage constructions. Most of them have shown that
this type of negative marker can be omitted from the adverbial clause without affecting the interpretation holding between
clauses. The study shows, based on the analysis of natural discourse data, that expletive negation has developed an intriguing
discourse function in three types of ‘until’ constructions in Huasteca Nahuatl: ‘not…until’ constructions, scalar additive ‘until’
constructions, and beginning-to-end constructions. When the negative marker amo appears in the ‘until’ clause,
the proposition should be characterized as expressing surprise. When it is absent from the ‘until’ clause, the proposition does
not express surprise. This function of the expletive negative marker amo does not appear in elicited sentences,
but only in spontaneous speech. It is proposed that Huasteca Nahuatl developed expletive negation in ‘not…until’ constructions due
to contact with Spanish. However, the development of expletive negation in scalar additive ‘until’ constructions and
beginning-to-end constructions is an internally motivated development in Huasteca Nahuatl that cannot be attributed to
Spanish.
Electron and ion emission characteristics of metal irradiated by nanosecond laser
Authors: Sun Guoxiang , Nie Shaohao , Wang Xia , Ding Weidong , Yuan Qi , Yan Jiaqi
Published: January 2024

Clarifying the electron and ion emission characteristics of metals irradiated by nanosecond focused laser in low pressure environment is crucial for improving applications based on laser-produced plasmas. This paper investigates the emission characteristics through electrical and optical diagnosis. The emission process is investigated through joint analysis of electrons and ions behavior, and relevant influencing factors are studied. The emission process of electrons and ions is divided into four stages, characterized by the arrival of prompt electrons e1, ultrafast electrons e2/ions i1, fast ions i2 and thermal electrons e3/ions i3, respectively. e1 is mainly related to thermal emission and photoemission, which can be improved by high electric field, gas pressure (within a certain range), laser energy and melting boiling points of target. e2/e3 and i1/i2/i3 mainly originate from laser ablation, and their expansion process follows obvious bipolar diffusion characteristics, while the latter is related to the different mass and charge states of the ions. The amplitude of i2 and e3 can be improved by using low density and melting point metals, and they are easily blocked by background gas, almost independent of the weak electric field.
Macro-dipoles in soft/hard expanded-polytetrafluoroethylene + fluoroethylenepropylene (ePTFE + FEP) fluoropolymer-film systems for high-output piezoelectric ferroelectret-transducer applications
Authors: Wang Ningzhen , Baferani Mohamadreza Arab , Daniels Robert , Wu Chao , Huo Jindong , van Turnhout Jan , Sotzing Gregory A , Gerhard Reimund , Cao Yang
Published: January 2024

Multi-layer ferroelectrets consisting of fluoroethylenepropylene (FEP) copolymer and open-porous expanded polytetrafluoroethylene (ePTFE) films exhibit stable internal electret charges, high piezoelectric coefficients and heat resistance, making them promising candidates for wearable sensors or nanogenerators in body-area networks. Here, three- and five-layer (FEP/ePTFE/FEP and FEP/ePTFE/FEP/ePTFE/FEP) ferroelectret stacks were laminated and poled in a corona discharge. The resulting charge distributions were measured by use of the pulsed electro-acoustic (PEA) method and revealed that charges of opposite polarity were trapped at the interfaces between the FEP and ePTFE layers. Thus, the existence of one macro-dipole in the three-layer structure and of two macro-dipoles in the five-layer structure was directly shown for the first time. Moreover, electric-displacement-versus-electric-field (D-E) loops revealed that remnant polarization is given by the number of macro-dipoles in the respective stack. Due to the addition of the macro-dipoles, the piezoelectric d
33 coefficient of the FEP/ePTFE/FEP/ePTFE/FEP stack reaches 200 pC/N even under a potentially non-uniform compression of the soft ePTFE layers. The results should be useful for a better understanding and a performance optimization of ferroelectrets in self-powered intelligent devices.
Polarization image fusion method with image enhancement
Authors: Wang Chenguang , Ma Ruyue , Yan Deli , Cao Huiliang , Shen Chong
Published: January 2024

Because of their complementary characteristics, intensity images and polarization images are often fused to produce information-rich images. However, the polarization characteristics are easily affected by the object’s environment, and the image fusion process may lose important information. In this paper, we propose an unsupervised end-to-end network framework based on a CNN for intensity images and degree of linear polarization images. First, we construct our own polarization dataset to solve the limitations of the training dataset; a hybrid loss function is designed to form an unsupervised learning process; and a Laplace operator enhancement layer is introduced into the network to further improve the quality of the fused images. Subjective and objective comparison experiments prove that the proposed fusion network is visually superior to several classical fusion methods.
Monolayer WS2 electro- and photo-luminescence enhancement by TFSI treatment
Authors: Cadore A R , Rosa B L T , Paradisanos I , Mignuzzi S , De Fazio D , Alexeev E M , Dagkli A , Muench J E , Kakavelakis G , Shinde S M , Yoon D , Tongay S , Watanabe K , Taniguchi T , Lidorikis E , Goykhman I , Soavi G , Ferrari A C
Published: January 2024

Layered material heterostructures (LMHs) can be used to fabricate electroluminescent devices operating in the visible spectral region. A major advantage of LMH-based light emitting diodes (LEDs) is that electroluminescence (EL) emission can be tuned across that of different exciton complexes (e.g. biexcitons, trions, quintons) by controlling the charge density. However, these devices have an EL quantum efficiency as low as ∼10−4%. Here, we show that the superacid bis-(triuoromethane)sulfonimide (TFSI) treatment of monolayer WS2-LEDs boosts EL quantum efficiency by over one order of magnitude at room temperature. Non-treated devices emit light mainly from negatively charged excitons, while the emission in treated ones predominantly involves radiative recombination of neutral excitons. This paves the way to tunable and efficient LMH-based LEDs.
Control of stripe, skyrmion and skyrmionium formation in the 2D magnet
Fe3−xGeTe2
by varying composition
Authors: Birch Max T , Powalla Lukas , Litzius Kai , Nehruji Vanessa , Hovorka Ondrej , Wintz Sebastian , Schulz Frank , Mayoh Daniel A , Balakrishnan Geetha , Weigand Markus , Burghard Marko , Schütz Gisela
Published: January 2024

Two-dimensional (2D) van der Waals (vdW) magnets have recently emerged as novel skyrmion hosts. This discovery has opened a new material platform for tuning the properties of topological spin textures, such as by exploiting proximity effects induced by stacking of 2D materials into heterostructures, or by directly manipulating the structural composition of the host material. Previous works have considered the effect of varied composition in the bulk crystals of the vdW magnet

F
e

3
−
x

G
e
T
e

2

, but so far the effects on the hosted spin textures have not been thoroughly investigated. In this work, real-space x-ray microscopy is utilized to image magnetic stripe domain, skyrmion and composite skyrmion states in exfoliated flakes of

F
e

3
−
x

G
e
T
e

2

with varying Fe deficiency x. In combination with supporting mean-field and micromagnetic simulations, the significant alterations in the magnetic phase diagrams of the flakes, and thus the stability of the observed spin textures, are revealed. These arise as a result of the varying temperature dependence of the fundamental magnetic properties, which are greater than can be explained by the removal of spins, and are consistent with previously reported changes in the electronic band structure via the Fe deficiency.
Exploring graphene-substrate interactions: plasmonic excitation in Sn-intercalated epitaxial graphene
Authors: Mamiyev Zamin , Tegenkamp Christoph
Published: January 2024

Graphene plasmons, including those in intercalated graphene, are an important research focus, with the promise of enabling light manipulation and providing a unique platform for gaining fundamental insights into many-body electronic interactions. In the present work, we discuss the results of low-energy plasmonic excitations in epitaxial quasi-free monolayer graphene formed by intercalation of Sn beneath the buffer layer (BL) on 4 H-SiC(0001). The quantitative analysis of the sheet plasmon dispersion revealed that the Sn-induced (

1
×
1

) interface is metallic and results in formation of charge-neutral graphene. A redshift of the 2D plasmon was found, but only after doping with potassium. The Sn-diluted interface, revealing a (

3

×

3

) reconstruction and resulting in intrinsically n-type doped graphene, behaves comparably to the BL for epitaxial monolayer graphene (MLG). Furthermore, it seems that a dipolar coupling of the longitudinal charge density fluctuations in graphene to the interface layer triggers the formation and the loss energy of a plasmonic multipole component, which therefore makes it suitable for studying proximity effects of excitations in electronically weakly coupled 2D heterosystems.
Optimization of parameters for the best shot peening effect based on surface response and neural network model
Authors: Wang Chengan , Kim Taehyung
Published: January 2024

To optimize the peening effect of different shot peening process parameters on metal surfaces, the mapping relationship between different shot peening process parameters and metal surface integrity was obtained. In this paper, ABAQUS software was used to establish a DE-FE (Discrete element-Finite element) random multi-shot analysis model to simulate shot peening, then optimize the shot peening process parameters based on the surface response method(RSM), and finally validate it through experiments and BP(back propagation) neural network model. The result shows that when the shot velocity is 70 m s−1, the impact angle of shot is 61.45°, and the shot diameter is 0.78 mm, the shot peening effect is the best, the surface roughness value is reduced by 101.84%, and the arc height value is increased by 54.66%; the error between the predicted results of BP neural network and the results of numerical analysis is less than 8%. Therefore, the optimized process parameters significantly improve the shot peening effect, but also shows that the BP neural network prediction model can more accurately predict the mapping relationship between the input parameters of shot velocity, shot diameter, and impact angle of shot and the output parameters of roughness value and arc height value.
Characteristics of polypropylene-based antistatic bio-nano composites reinforced with mono-diacylglycerols and cellulose nanocrystals
Authors: Setyaningsih Dwi , Sarfat Muhammad Syukur , Fahma Farah , Indrasti Nastiti Siswi
Published: January 2024

Polypropylene (PP) is known as a polymer without antistatic properties that is susceptible to the use of high temperatures. Therefore, to improve the thermal and antistatic properties of PP, it is necessary to modify PP to antistatic bio-nanocomposites with mono-diacylglycerols (M-DAG) as an antistatic agent and cellulose nanocrystals (CNC) as a reinforcement. This research aimed to characterize the electrical resistivity and thermal properties of PP-based antistatic bio-nanocomposites reinforced with M-DAG and CNC at different concentrations of CNC (0%–5%), and 2% of M- DAG, compared to pure PP. The results showed that the addition of 2% CNC (AS-BNC-2) gave the melting temperature of 125.0 °C, which was higher than pure PP of 118.3 °C. The thermal stability of the antistatic bio-nano composites with 3% CNC (AS-BNC-3) was 457.10 °C, which was higher than pure PP of 441.56 °C. The electrical resistivity of the antistatic bio-nano composites from all treatments was still in the range of the antistatic category of 1010–1012 Ω/sq. The melting temperature and thermal stability of bio-nano composites were higher than those of pure PP and they have antistatic properties. This indicates the potential application of these materials in the electronics devices and packaging industries.
The structure of the spin^{ℎ} bordism spectrum
Authors: Mills Keith
Published: January 2024

Spin

h

^h

manifolds are the quaternionic analogue to

spin
c

\text {spin}^c

manifolds. We compute the

spin
h

\text {spin}^h

bordism groups at the prime

2
2

by proving a structure theorem for the cohomology of the

spin
h

\text {spin}^h

bordism spectrum

M
S
p
i

n
h

\mathrm {MSpin^h}

as a module over the mod 2 Steenrod algebra. This provides a 2-local splitting of

M
S
p
i

n
h

\mathrm {MSpin^h}

as a wedge sum of familiar spectra. We also compute the decomposition of

H
∗

(

M
S
p
i

n
h

;

Z

/

2

Z

)

H^*(\mathrm {MSpin^h};\mathbb {Z}/2\mathbb {Z})

explicitly in degrees up through 30 via a counting process.
Improved inequalities between Dirichlet and Neumann eigenvalues of the biharmonic operator
Authors: Lotoreichik Vladimir
Published: January 2024

We prove that the

(
k
+
d
)

(k+d)

-th Neumann eigenvalue of the biharmonic operator on a bounded connected

d
d

-dimensional

(
d
≥
2
)

(d\ge 2)

Lipschitz domain is not larger than its

k
k

-th Dirichlet eigenvalue for all

k
∈

N

k\in \mathbb {N}

. For a special class of domains with symmetries we obtain a stronger inequality. Namely, for this class of domains, we prove that the

(
k
+
d
+
1
)

(k+d+1)

-th Neumann eigenvalue of the biharmonic operator does not exceed its

k
k

-th Dirichlet eigenvalue for all

k
∈

N

k\in \mathbb {N}

. In particular, in two dimensions, this special class consists of domains having an axis of symmetry.
A trace Trudinger-Moser inequality involving 𝐿^{𝑝}-norm on a compact Riemann surface with boundary
Authors: Zhang Mengjie
Published: January 2024

In this paper, using the method of blow-up analysis, we establish a trace Trudinger-Moser inequality involving

L
p

L^p

-norm and obtain the corresponding extremal on a compact Riemann surface with a smooth boundary. The result generalizes those of Li-Liu [Math. Z. 250 (2005), pp. 363–686] and Zhang [Commun. Pure Appl. Anal. 20 (2021), pp. 1721–1735].
Positive intermediate curvatures and Ricci flow
Authors: González-Álvaro David , Zarei Masoumeh
Published: January 2024

We show that, for any

n
≥
2

n\geq 2

, there exists a homogeneous space of dimension

d
=
8
n
−
4

d=8n-4

with metrics of

R
i

c

d
2

−
5

>
0

Ric_{\frac {d}{2}-5}>0

if

n
≠
3

n\neq 3

and

R
i

c
6

>
0

Ric_6>0

if

n
=
3

n=3

which evolve under the Ricci flow to metrics whose Ricci tensor is not

(
d
−
4
)

(d-4)

-positive. Consequently, Ricci flow does not preserve a range of curvature conditions that interpolate between positive sectional and positive scalar curvature. This extends a theorem of Böhm and Wilking [Geom. Funct. Anal. 17 (2007), pp. 665–681] in the case of

n
=
2

n=2

.
Fixed points of continuous pseudocontractive mappings on convex, closed unbounded domains
Authors: Garcia-Falset Jesús
Published: January 2024

The purpose in this article is to discuss under what conditions a continuous pseudocontractive mapping, in particular a nonexpansive map, has a fixed point when its domain is a closed, convex and unbounded subset of a Banach space. In fact, we will give a characterization of this property. The Halpern iterative algorithm for nonexpansive mappings is also investigated.
Massive waves gravitationally bound to static bodies
Authors: Sussman Ethan
Published: January 2024

We show that, given any static spacetime whose spatial slices are asymptotically Euclidean (or, more generally, asymptotically conic) manifolds modeled on the large end of the Schwarzschild exterior, there exist stationary solutions to the Klein–Gordon equation having Schwartz initial data. In fact, there exist infinitely many independent such solutions. The proof is a variational argument based on the long range nature of the effective potential. We give two sets of test functions which serve to verify the hypothesis of the variational argument. One set consists of cutoff versions of the hydrogen bound states and is used to prove the existence of eigenvalues near the hydrogen spectrum.
The microvillar protocadherin CDHR5 associates with EBP50 to promote brush border assembly
Authors: Matoo Samaneh , Graves Maura J. , Choi Myoung Soo , Idris Rawnag A. El Sheikh , Acharya Prashun , Thapa Garima , Nguyen Tram , Atallah Sarah Y. , Tipirneni Ashna K. , Stevenson Phillip J. , Crawley Scott W.
Published: January 2024

Transporting epithelial cells of the gut and kidney interact with their luminal environment through a densely packed collection of apical microvilli known as a brush border (BB). Proper brush border assembly depends on the intermicrovillar adhesion complex (IMAC), a protocadherin-based adhesion complex found at the distal tips of microvilli that mediates adhesion between neighboring protrusions to promote their organized packing. Loss of the IMAC adhesion molecule Cadherin-related family member 5 (CDHR5) results in significant brush border defects, though the functional properties of this protocadherin have not been thoroughly explored. Here, we show that the cytoplasmic tail of CDHR5 contributes to its correct apical targeting and functional properties in an isoform-specific manner. Library screening identified the Ezrin-associated scaffolds EBP50 and E3KARP as cytoplasmic binding partners for CDHR5. Consistent with this, loss of EBP50 disrupted proper brush border assembly with cells exhibiting markedly reduced apical IMAC levels. Together, our results shed light on the apical targeting determinants of CDHR5 and further define the interactome of the IMAC involved in brush border assembly.
Lipid- and phospho-regulation of CTP:Phosphocholine Cytidylyltransferase α association with nuclear lipid droplets
Authors: Foster Jason , McPhee Michael , Yue Lambert , Dellaire Graham , Pelech Steven , Ridgway Neale D.
Published: January 2024

Fatty acids stored in triacylglycerol-rich lipid droplets are assembled with a surface monolayer composed primarily of phosphatidylcholine (PC). Fatty acids stimulate PC synthesis by translocating CTP:phosphocholine cytidylyltransferase (CCT) α to the inner nuclear membrane, nuclear lipid droplets (nLD) and lipid associated promyelocytic leukemia (PML) structures (LAPS). Huh7 cells were used to identify how CCTα translocation onto these nuclear structures are regulated by fatty acids and phosphorylation of its serine-rich P-domain. Oleate treatment of Huh7 cells increased nLDs and LAPS that became progressively enriched in CCTα. In cells expressing the phosphatidic acid phosphatase Lipin1α or 1β, the expanded pool of nLDs and LAPS had a proportional increase in associated CCTα. In contrast, palmitate induced few nLDs and LAPS and inhibited the oleate-dependent translocation of CCTα without affecting total nLDs. Phospho-memetic or phospho-null mutations in the P-domain revealed that a 70% phosphorylation threshold, rather than site-specific phosphorylation, regulated CCTα association with nLDs and LAPS. In vitro candidate kinase and inhibitor studies in Huh7 cells identified cyclin-dependent kinase (CDK) 1 and 2 as putative P-domain kinases. In conclusion, CCTα translocation onto nLDs and LAPS is dependent on available surface area and fatty acid composition, as well as threshold phosphorylation of the P-domain potentially involving CDKs.
Surveillance Cameras and Resistance: A Case Study of a Middle School in China
Authors: Shi Chen , Xu Jianhua
Published: January 2024

China has rapidly evolved into a surveillance society. While much attention has been paid to describing the leviathan represented by the presence of surveillance cameras in China, empirical evidence on the mechanisms of the creep of surveillance remains limited. Using data collected through fieldwork and in-depth interviews, this study explores the spread of surveillance cameras and the resistance encountered in a middle school in northern China. We find that surveillance cameras were first introduced for security purposes, but their application was quickly expanded to discipline students and avoid responsibilities in school management. We further explore the resistance to the creep made possible by the existence of exempted spaces, the difficulty of self-surveillance, and what might be called the boomerang effect. Through the case study of a middle school, this research sheds light on the formation of the Chinese surveillance society from a bottom-up approach and contributes to the global literature on surveillance creep.
O03 Sarcoidosis in a Boy with Severe Eczema on Dupilumab
Authors: Shahid Musarat , Davis Penny , Taibjee Saleem , Moss Ceilia , Ogboli Malobi
Published: January 2024

Sarcoidosis, a systemic granulomatous disease of unknown aetiology, is rare in children. Presentation varies according to the extent and severity of organ involvement and is typically multisystem in older children. Drug induced sarcoidosis-like reaction (DISR) has recently been reported in patients taking Dupilumab. We describe a patient who developed sarcoidosis while on Dupilumab and question whether this was caused by the drug.

A 14-year-old boy with severe eczema developed widespread cutaneous nodules and papules after 30 months on Dupilumab. Histology showed non-caseating granulomas. A raised serum angiotensin converting enzyme level, asymptomatic coarse interstitial markings on chest x-ray and small lung nodules on CT scan were also consistent with sarcoidosis. Dupilumab was stopped and hydroxychloroquine and methotrexate commenced. Joint and liver involvement developed 4 months later so systemic glucocorticoids were and the methotrexate was changed to mycophenolate mofetil.

To our knowledge there have been 3 previous reports of DISR on Dupilumab. The clinical picture is almost indistinguishable from sarcoidosis. In this case continuing evolution of sarcoidosis 4 months after stopping Dupilumab argues against DISR. However, as Dupilumab becomes more widely used, it is important to be alert to the possibility of new adverse reactions.
O04 Vulval Psoriasis in Children: A Clinical Audit and Review of Literature
Authors: Wu Michelle , Fischer Gayle
Published: January 2024

Objective

Vulval psoriasis is frequently under-recognised, under-­diagnosed and under-treated in children due to its nonspecific presentation. This retrospective chart review is aimed to describe the symptoms, signs, and management of vulval psoriasis in children in a private vulval disease referral practice.

Materials and methods

A retrospective, single-centre cohort study of children was undertaken at a large, combined dermatology and gynaecology practice from January 2016 to January 2023. Clinical photographs and treatment data were systematically collected and recorded in an online patient database. Treatment regimens were individualised and titrated to clinical response.

Results

This seven-year retrospective cohort study included a total of 100 children with vulval psoriasis. The most common presentation was nonspecific erythema (85%) that is well-demarcated in one-third of children and appears as a raised plaque in one-fifth of children. In 78% of the patients, evidence of psoriasis was found on other parts of the skin. Initial induction treatment with potent topical corticosteroid followed by maintenance treatment with less potent topical steroids and topical tar creams. 5% of children required systemic treatment.

Conclusions and Relevance

Vulval psoriasis often presents as a nonspecific chronic erythematous vulvitis without vaginitis. It is a chronic relapsing skin condition that requires long-term management. Systematic treatment is required in a small proportion of children.
O05 A Qualitative Study of the Psychological Morbidity in Paediatric Survivors of Stevens Johnson Syndrome and Toxic Epidermal Necrolysis
Authors: Phillips Christopher , Mcniven Abigail , Yeung Michelle , McPherson Tess
Published: January 2024

Background

Steven Johnsons Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are rare but devastating skin conditions. In adults, the long-term psychological sequelae are known(1), however, there is no such research in the paediatric population(2).

Objective

Our study’s aim was to explore the experiences of children and young people (CYP) and their parents during and after their illness and the subsequent physical and psychological sequelae.

Method

A retrospective qualitative study with a grounded theory approach was undertaken using detailed semi-structured interviews. 13 CYP and families who had been admitted to a tertiary referral centre were interviewed. Interview transcripts were analysed by three different researchers and themes emerging were identified.

Results

Age range of those interviewed was 10-24 years. Following discharge, patients frequently had ongoing physical issues and psychological morbidity. Common themes included inadequate information on discharge and a desire for more psychological aftercare for both CYP and parents. SJS/TEN and its causes did not appear to have been clearly explained. While there was marked heterogeneity in the experiences and impact on CYP, all parents interviewed cited symptoms of health anxiety, and some believed they had developed subsequent mental health issues.

Conclusion

Subsequent physical disability, health anxiety, psychological morbidity and little understanding of SJS/TEN were themes that were prevalent from our interviews.

Families desire a more co-ordinated and holistic aftercare plan which may contribute to a better understanding of SJS/TEN as well as give opportunity to address the long-term physical and psychological morbidity of SJS/TEN.
O06 Clinical phenotype of the Klippel-Trenaunay syndrome with mosaic PIK3R1 mutations
Authors: Vabres Pierre , Engel Camille , Bessis Didier , Morice-Picard4 Fanny , Aubert Hélène , Isidor Bertrand , Phan Alice , Boccara Olivia , Mazereeuw-Hautier Juliette , Maruani-Raphaël Annabel , Puzenat Eve , Kuentz Paul
Published: January 2024

Segmental overgrowth syndromes with cutaneous vascular malformations (Klippel-Trenaunay syndrome (KTS), CLOVES syndrome) are mainly due to mosaic PIK3CA mutations, but the GNAQ, GNA11, HRAS, KRAS, MAP2K1, AKT1, AKT3, and PIK3R1 genes may also be involved. We sought to determine the frequency and phenotype of patients with PIK3R1-related segmental overgrowth.

Patients were ascertained nationwide in the French MUSTARD cohort of skin mosaic phenotypes. We searched for PIK3R1 mutations by next-generation sequencing on affected tissue in 297 patients with a clinical presentation suggestive of PIK3CA related overgrowth (PROS), but without PIK3CA variants. We retrospectively studied the clinical phenotype of PIK3R1-positive patients.

We identified three missense variants and six in-phase indels of PIK3R1 in 15 patients (5.0%), exclusively in affected tissue. Variant allele fraction was 2.0% to 12.0%. We found extensive pale or dark capillary malformations in 14 patients, segmental overgrowth in 11, venous truncal anomalies (varicose veins or persistent lateral marginal vein) in 10, lymphatic malformation in six and a café-­au-lait macule or naevus spilus in three, thus defining phacomatosis pigmento-vascularis (PPV). One patient had limb hypoplasia. None had MCAP (Megalencephaly-Capillary malformation).

PIKR1 mutations account for a minority of patients with KTS/CLOVES (5%). They are about 20 times less common than PIK3CA mutations. Truncal venous malformations, limb hypoplasia and PPV (spilorosea or melanorosea type) may be more common in PIK3R1-related KTS. No patient had severe or life-threatening involvement. Whether Sirolimus or Alpelisib are treatment options remains to be determined.
P01 Pigmented and purpuric patches in sun protected areas of a 9 year old child
Authors: Grechin Cristina , Law Ruth , Collins Sinead
Published: January 2024

Pigmented purpuric dermatosis-like mycosis fungoides (PPD-like MF) is a rare variant of paediatric mycosis fungoides (MF). Clinically, it can be challenging to diagnose and differentiate from pigmented purpuric dermatosis (PPD). Usually, has an indolent course if diagnosed in the early stage. The lesions often localize to sun-protected areas with three stages: patch, plaque, and tumour. Clinical features alone are insufficient for diagnosis and a skin biopsy is mandatory.1

We report a nine year old boy who presented with a three year history of a persistent, slowly progressing, mildly pruritic and scaling rash on the right buttock and medial thighs bilaterally. He was otherwise well with no other relevant medical or family history of note. The physical examination revealed erythematous to purpuric, mild scaly patches with confluent involvement on the right buttock extending to less defined involvement of the both inner thighs. The body surface area involved was less than <10%. No other clinical findings on examination. A punch biopsy for haematoxylin and eosin (H&E) staining was consistent with mycosis fungoides. T cell receptor gene rearrangement testing detected a monoclonal T-cell population. A diagnosis of pigmented purpuric dermatosis-like mycosis fungoides was made.

The patient responded well to a potent topical steroid (mometasone furoate) daily which was gradually tapered to twice weekly/intermittent usage to maintain response.
P02 Erythrokeratodermia variabilis et progressiva due to a pathogenic variant in GJB3 gene
Authors: Bageta Maria , Balboa Pablo Lopez , Lomas Debra , Petrof Gabriela , Martinez Anna E
Published: January 2024

Erythrokeratodermia variabilis et progressiva (EKVP) is a clinically heterogeneous group of inherited disorders characterized by the coexistence of localized or generalized hyperkeratotic plaques and transient, stationary or migratory erythematous patches. EKPV is most often transmitted in an autosomal dominant manner. Causal pathogenic variants have been detected in the GJB3, GJB4, GJA1 KDSR and KRT83 genes encoding connexins 31, 30.3, 43, 3-ketodihydrosphingosine reductase and keratin 83, respectively. Connexins are expressed in almost all tissues and pathogenic variants in the connexin genes can cause skin diseases, cardiovascular disorders, myelin-related diseases, craniofacial disorders and hearing loss.

We present a 2-year-old girl who was referred to our centre with well-demarcated hyperkeratotic plaques symmetrically distributed with a predilection for the distal extremities and buttocks as well as erythematous plaques on her cheeks. The hyperkeratotic plaques were hyperpigmented, exhibited a geographic morphology and had prominent hypertrichosis. Her trunk and upper extremities were spared and her hair, teeth and nails were normal. There was family history of mild psoriasis in her mother and mild eczema in her father. Next generation sequencing confirmed she was heterozygous for the GJB3 c.625C>T p.(Leu209Phe) pathogenic variant which is associated with erythrokeratodermia variabilis et progressiva-1 (MIM 133200) and autosomal dominant deafness-2B (MIM 612644).

Erythrokeratodermia variabilis is largely a skin-limited condition however GJB3 pathogenic variants can cause haring loss and neuropathy due to its expression in peripheral nerves and the cochlea. Our patient’s hearing and development have been normal to date and will be monitored.
P04 A Complex Case of Urticaria
Authors: Madhwapathi Vidya , Diba Vicky , Ogboli Malobi
Published: January 2024

An 11 year old female, with Type 1 diabetes and no previous atopy or allergy , presented with a 6 week history of facial swelling and a generalised urticaria on the trunk and limbs.

She had been seen privately by “Allergy” clinic and diagnosed with chronic spontaneous urticaria with angioedema. Blood tests for common allergens were negative, with a total IgE <20 kU/L. Treatment with Loratadine, Cetirizine 10mg, Fexofenadine 120mg QDS, Acrivastine 8mg BD, Montelukast 10mg OD and prednisolone 20mg OD had only a limited effect.

Skin biopsy confirmed urticaria with no evidence of vasculitis. Immunology bloods were negative.

Despite reporting compliance, she continued to experience debilitating symptoms with raised UAS scores. Desloratadine 2.5mg BD and multiple intermittent short courses of prednisolone were needed.

Omalizumab was started at 300mg monthly for 6 months with partial improvement. However symptoms flared on cessation and following tertiary advice, a 2nd course of Omalizumab was commenced at 300 mg s/c monthly for 3 months & 300mg s/c fortnightly for 3 months with the addition of 100mg BD Ciclosporin and Famotidine 10mg BD. Ciclosporin was discontinued after 1 month due to side effects and substituted with Methotrexate 15mg OW.

If control is not obtained, management plans include Mycophenolate Mofetil, or trialing omalizumab 450mg S/C monthly.

This is a case of extremely difficult to control urticaria in a child highlighting available systemic treatment options1.
P06 Outcomes from a New Combined Allergy and Dermatology Clinic- A Retrospective Review
Authors: Liew Su Yi , Ahmed Samid , Abdul-Wahab Alya , Wells Rosy , Ferguson Leila
Published: January 2024

Early introduction to known allergens such as peanuts can reduce subsequent development of allergies [1]. In 2019, an audit in our department of 35 babies under age 1 referred with eczema showed that 4 were concomitantly referred to allergy and 20 were referred to allergy after dermatology consultation. The majority had moderate/severe eczema and 31% had implemented dietary restrictions before the dermatology consultation. In 2021, a combined allergy and dermatology clinic was introduced to provide a holistic approach, particularly for young children. Patients are triaged from GP referrals or as follow-up from either team. We conducted a retrospective notes review to explore the benefits/outcomes. 57 patients were included with an average age of 4.75 years and 58% were below age 2. 58% had their dermatology plans changed. Before the clinic, 81% had dietary exclusions but only 46% had new allergies confirmed. 29% of the patients who underwent testing tested negative to all allergens, which allowed parents to reintroduce foods at an early age. This review shows that the combined clinic allowed young patients early access to allergy testing due to streamlined services, preventing unnecessary dietary exclusions. Additionally, patients with severe eczema without allergies benefit from early dermatology intervention. In fact, 44% of patients were suitable for discharge from dermatology directly from the combined clinic or at first follow-up appointment.
P08 Shingles in two children with no history of chicken pox
Authors: Eisner Mark , Darné Sharmela
Published: January 2024

We present two cases of dermatomal, vesicular eruptions in young children. Both were otherwise well with no history of immunocompromise or prior chickenpox (varicella).

Child A was 3 years old and presented with painful erythema and pustules to the left side of his nose and cheek with a sharp midline demarcation. He was admitted by the paediatricians, unwell and treated with antibiotics and acyclovir for presumed impetigo or herpes simplex virus infection. However, the swab was positive for varicella zoster virus (VZV), which in this dermatomal distribution is in keeping with shingles.

Child B was an 18-month-old girl who was referred with a dermatomal vesico-pustular eruption on the left forehead, scalp and nose unresponsive to antibiotics. Despite the lack of varicella history, shingles was suspected, and the child was started on aciclovir. Swabs were positive for VZV.

Shingles is rare in children and usually associated with immunocompromise. It is especially rare in those with no prior history of chicken pox and of the cases in the literature, almost all have had previous vaccination to chicken pox resulting in latent infection and subsequent shingles. We surmise that our otherwise well patients, with no prior vaccination, had previous asymptomatic chicken pox. There was a delay in the diagnosis of patient A who went on to develop trigeminal trophic syndrome with recurrent paranasal ulceration over 18 months. We highlight these cases to make clinicians aware of the possibility of shingles even in very young, immunocompetent children with no prior history of chickenpox.
P09 A prospective study of Narrowband UVB (nb-UVB) Phototherapy for atopic dermatitis (AD) in children and adolescents.
Authors: Sambi Pavan , Warburton Katharine , Montgomery Rachel , Majeethia Parisha , Thompson Mia , Clark Sheila
Published: January 2024

We evaluated response to thrice weekly nb-UVB for AD (maximum 40 exposures), to assess outcomes immediately post-treatment and 12 months later. Post-treatment follow up was 3 monthly for 12 months. We sub-analysed response in different ethnicities.

58 patients were recruited: 37 (64%) male, 21 (36%) female, age-range 4.1-16.9 (mean 10.6) years. Ethnicities were: White Caucasian 22 (38%), Southern Asian (Pakistani/Indian/Bangladeshi) 17 (29%), Black African/Caribbean 9 (16%), Other 10 (17%).

Immediately post-treatment, EASI 75 was achieved in: Overall 49%, Caucasian 48%, Southern Asian 50%, African-Caribbean 44%, other 56%. Mean EASI reduction was: Whole group 10.0, Caucasian 12.4, Southern Asian 8.6, African-Caribbean 2.5, Other 7.5.

12 months post-treatment (compared to pre-treatment), EASI 75 was achieved in: Overall 28%, Caucasian 33%, Southern Asian 40%, African-Caribbean14%, other 13%. Mean EASI reduction was: Whole group 7.6, Caucasian 9.0, Southern Asian 8.6, African-Caribbean -2.1 (worsening), Other 8.0.

Erythema was the most common side effect noted in 33% of patients, the incidence in Caucasians 50% (n=11), other 30% (n=3), African-Caribbean 22% (n=2) and Southern Asian 12% (n=2).

A similar proportion achieved EASI 75 across the different skin types, however, more variation in EASI score reduction was noted in skin of colour. A lower incidence of post-treatment erythema noted in darker skin types may be due to erythema underscoring or treatment underdosing.

Our study showed sustained AD improvement up to a year after stopping treatment in 28% overall, highest in Caucasian and Southern Asian patients, with greater risk of relapse noted in African-Caribbean patients.
P10 New rash in a child of skin of colour
Authors: Aslam Muhammad Fawad , Hoey Susannah
Published: January 2024

A 7 year old Nigerian girl was referred by Haematology to the Paediatric Dermatology team with “suspected eczema”.

She had known sickle cell disease, for which she was on hydroxycarbamide, Mum reported an 8 month history of a new rash with no systemic features. She had marked indurated plaques on face, forehead and forearms which were associated with scarring and hyperpigmentation. She had a number of investigation including negative urine dipstick. ANA was positive with elevated RO and RNP antibodies. Inflammatory markers were normal. Skin biopsy results were consistent with discoid lupus erythematosus. Stringent photo protection and sun blocking creams were advised. She was commenced on potent topical steroids and hydroxychloroquine which is first line systemic treatment. Within 4 weeks of commencing hydroxychloroquine there was significant flattening of the plaques. She was referred to Paediatric Rheumatology for assessment, given her high risk features.

Clinical features of DLE in children are similar to adult DLE in presentation and chronic course. However it is rare in children with less than 2% of patients developing DLE before 10 years of age. DLE tends to be more common and severe in skin of colour. There are complications of scarring and pigmentation. It is important to be aware of the psychological impact on both parent and child’s quality of life. There is high risk of transition to SLE in children with DLE and hence screening bloods and early referral to Rheumatology should be considered.
P12 Teleconsultations for Eczema in CHildren (TECH) feasibility study: a UK online survey of young people and parents
Authors: Stokes Natalie King , Daly Aoife , McCusker Sarah , Hunjan Manrup , Vincent Ellen , Lowe Ashima , Charman Carolyn , George Susannah , Solman Lea , Burden-Teh Esther
Published: January 2024

Providing teleconsultations for appropriate patients is a priority for outpatient service transformation, yet little evidence exists on their effectiveness and acceptability in paediatric eczema. We conducted a UK-wide online patient and parent survey to explore experiences of eczema teleconsultations. Further objectives included determining patient preferences and costs incurred with different appointments.

Young people (13-16 years) and parents of children (0-16 years) with eczema, who had had a follow-up teleconsultation under secondary/tertiary care dermatology were eligible. Ethical approval was sought and a Jisc Online survey was advertised on social media for 12 weeks. Data was analysed descriptively in Microsoft Excel.

There were 51 responses (31 parents, 20 adolescents) from across the UK. Most participants were receiving topical treatment, with 8 on systemic therapy. Of those who had experience of face-to-face appointments, 31/42 (73.8%) were ‘satisfied or very satisfied’, compared with 19/46 (41.3%) for telephone, 10/14 (71.4%) for video and 4/10 (40%) for online/mobile messaging. 49 (96.1%) participants reported they would feel ‘happy or very happy’ if their next appointment was face-to-face, compared with 14 (27.5%) for telephone, 14 (27.5%) for video and 6 (11.8%) for online/mobile messaging. 34 (66.7%) participants’ first-choice consultation was face-to-face, followed by 8 (15.7%) preferring a combination of face-to-face and telephone. Many commented that face-to-face was better for assessing the skin.

These results, along with a qualitative interview study currently underway, provide an insight into the experiences and preferences of young people and parents regarding eczema follow-up and will help inform a future trial.
P16 Hypopigmented Mycosis Fungoides in a Caucasian child
Authors: Bhadresha Sheena , Moledina Zahra , Tewari Angela
Published: January 2024

We report a case of hypopigmented MF in an 8-year-old Caucasian boy whose skin had been managed as pityriasis alba for a number of years.

On initial presentation to the emergency department, multiple non-itchy scattered hypopigmented macules were incidentally noted on the lower abdomen, groin, lateral flanks and axillae. Physical examination revealed widespread hypopigmented patches. Differential diagnoses included pityriasis alba or post inflammatory hypopigmentation secondary to atopic dermatitis.

He was initially managed with mild to moderate corticosteroids and emollients, but continued to develop multiple atypical asymmetrical macules, particularly on the lower abdomen and groin. Although the skin was responsive to anti-inflammatory treatment, given the progression, a skin biopsy was requested.

A lesional punch biopsy from the abdomen revealed focal lymphocyte exocytosis and interface change at the epidermis. Given the subtle findings the case was discussed at our histopathology MDT meeting. Further specialist review of the histopathology showed clinicopathological correlation in keeping with CD8+ mycosis fungoides (hypopigmented variant). He is currently managed with regular potent topical steroids, 0.1% topical tacrolimus. TL01 phototherapy has been suggested.

Juvenile onset MF constitutes hypopigmented, poikilodermatosis, pityriasis lichenoides-like, folliculotropic, and pigmented purpura- like presentations1. Generally there is no disease progression beyond stage 1A or 1B disease2. Hypopigmented MF is well described in black and Asian skin types but it is also important to appreciate that multiple reoccurring atypical hypopigmented patches in Caucasian skin may also be a variant of hypopigmented MF.
P17 A Multisite Service Evaluation of Paediatric Teleconsultations
Authors: McCusker Sarah , Daly Aoife , King Natalie , Hunjan Manrup , Solman Lea , Burden-Teh Esther , George Susannah , Hale Gordon
Published: January 2024

Paediatric dermatology teleconsultations have become familiar territory since the Covid-19 pandemic. There is a continued drive to provide teleconsultations, with efficiency and cost-saving cited as benefits. Yet, this is not supported by a recent UK-wide survey of health professionals.1 We evaluated practice across two paediatric dermatology centres, to assess time required for teleconsultations.

Data were collected prospectively from paediatric dermatology outpatient consultations for patients <16 years over 2 weeks in March and 4 weeks in September 2022. Consultation type (face-to-face/telephone/video), patient demographics, diagnosis and consultation length were reviewed. Caldicott approval was obtained.

A total of 173 consultations were included. There was a range of diagnoses including eczema (n=60), skin lesions (n=22), vascular lesions (n=28) and other inflammatory skin conditions (n=63). Most consultations were face-to-face (n=108, 62%), while 38% (n=65) were teleconsultations. Median consultation duration was 13 minutes, 45 seconds (range: 2.5min–1hour 5min 2s). Comparison of consultation duration across 3 modalities (face-to-face vs telephone vs video) demonstrated no statistical significance between groups. (H(2)=5.76, P=0.056).

There was a trend toward significance, likely due to 3 face-to-face consultations with duration of >50 minutes. Limitations include relatively small sample size and consultations carried out by few clinicians. Consultation modality is often influenced by case complexity, i.e., more complex consultations are likely to be held face-to-face.

Future work includes a health-economic analysis of teleconsultations to determine if these are cost-effective.