bluuebunny/crossref_metadata_2025_split huggingface dataset:
A136 MÉNÉTRIER’S DISEASE-LIKE PRESENTATION IN A PEDIATRIC PATIENT AS A FIRST PRESENTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS
Authors: Hussain M , Alsaffar Z , Ahmed N 
Published: February 2025

Abstract

Background
Ménétrier’s disease (MD) is an uncommon gastric disorder characterized by hypertrophic gastric mucosal folds, excess mucus secretion, and protein-losing gastropathy. It is mainly seen in adult patients, with around 100 pediatric cases reported to date, often associated with cytomegalovirus (CMV) infection. Systemic lupus erythematosus (SLE) is a autoimmune condition that can affect multiple systems. While MD and SLE are separately rare, there have been reports of MD preceding SLE in adult patients. However, no cases have documented MD as the initial presentation leading to a diagnosis of SLE in pediatric patients.

Aims
This case report aims to describe the first documented instance of a pediatric patient presenting with suspected Ménétrier’s disease, whose clinical course led to a diagnosis of systemic lupus erythematosus.

Methods: Case Summary
A 13-year-old boy with a history of autism spectrum disorder, presented with anasarca and multiple systemic symptoms, including musculoskeletal pain, fatigue, mouth ulcers, non-productive cough, and generalized edema. On admission, he was hypotensive with mild conjunctival injection, peri-orbital edema, ascites, and non-pitting edema in the lower limbs. Lab tests showed leukopenia, neutropenia, thrombocytopenia, elevated ferritin, and hypoalbuminemia. Imaging revealed bilateral pleural effusions and ascites, along with thickened gastric folds on CT scan raising suspicion for Ménétrier’s disease. An upper GI endoscopy showed atypical thick folds with scaliness in the stomach, but the biopsy was negative for H. pylori, CMV, showed no significant inflammation, no foveolar metaplasia, no viral cytopathic changes and had well-preserved morphology in esophageal, gastric and duodenal biopsies. A kidney biopsy was done in the context of proteinuria and confirmed lupus nephritis.

Results
He was diagnosed with systemic lupus erythematosus (SLE) and treated with steroids, Plaquenil, and Mycophenolate Mofetil. Post-treatment, his symptoms, including ascites and edema, resolved, and his lab values, such as blood counts, albumin, and C3/C4 levels, normalized. The patient showed significant clinical improvement following SLE-targeted therapy.

Conclusions
This case highlights the complexity and diagnostic challenges in differentiating Ménétrier’s disease from systemic lupus erythematosus, particularly when both disorders present with overlapping features. In addition, it suggests that in rare instances, MD may be a manifestation of a broader systemic inflammatory process such as SLE. Multidisciplinary approach and comprehensive evaluation are essential for accurate diagnosis and management in such complex cases.

Funding Agencies
None
A163 DIETARY PATTERNS OF BREASTFEEDING POST-PARTUM MOTHERS WITH INFLAMMATORY BOWEL DISEASE AND THEIR ASSOCIATIONS ON INFANT GROWTH
Authors: Dang R , Wu R Y , O’Connor K , Epstein-Shapiro A , Xiao K , Srikanth V , Maiocco F , Huang V 
Published: February 2025

Abstract

Background
Inflammatory bowel disease (IBD), which includes Crohn’s disease (CD) and ulcerative colitis (UC) is characterized by chronic intestinal inflammation. Since IBD is often diagnosed in young adulthood, many people will have IBD at the time of pregnancy and breastfeeding. Chronic inflammation can cause severe nutritional deficiencies; however, it is unclear whether nutrient deficiencies during postpartum affect patients with IBD.

Aims
To investigate the dietary patterns of breastfeeding mothers with IBD and correlate their dietary patterns with their infant growth within the first 6 months.

Methods
A total of 21 mothers with IBD (9 CD and 12 UC) were recruited from the Preconception and Pregnancy IBD clinic at Mount Sinai. Inclusion criteria include females between 18-45 years olds with a diagnosis of CD or UC, who are at least 24 weeks gestational age, and females with multi-fetal gestation, co-morbid intestinal disease, or autoimmune comorbidities were excluded. Mothers completed a 3-day food diary and clinical disease activity scores at 1-month (1M) and 3-months (3M) postpartum, and self-filled questionnaires related to infant growth over a 6-month follow-up. Food and dietary patterns were analyzed using a food analysis software called ESHA based on age, gender, weight and height. Infant growth was compared to the World Health Organization’s growth curve Z-scores.

Results
About half (52% at 1M and 48% at 3M) of mothers with IBD met the protein recommendation. Over half (62% at 1M and 3M) met their total fat recommendation. However, most did not meet the carbohydrate (33% at 1M and 19% at 3M) and fiber (24% at 1M and 10% at 3M) recommendations. All infants included in the analysis were breastfed at 1M and 3M postpartum. Breastfed infants born to mothers who met their total fat recommendation had higher length measurements compared to those who did not at 6 months.

Conclusions
Our study findings show that breastfeeding mothers with IBD may not meet their recommended carbohydrate and fiber dietary intake, and that infants breastfed by mothers who do not meet their macronutrient dietary intake and lengths in the lower percentile range. Future research will delineate the specific nutrient composition in mothers’ breastmilk.

Funding Agencies
American College of Gastroenterology, University of Toronto, CAN-TAP-TALENT
A217 OUTCOMES OF BALLOON ASSISTED ENDOSCOPIC STRICTURE DILATION IN PATIENTS WITH SMALL BOWEL CROHN’S DISEASE
Authors: Bowron J C , Fazal M , Reeson M , Halloran B , Zepeda-Gomez S 
Published: February 2025

Abstract

Background
Crohn’s Disease (CD) is a subset of inflammatory bowel disease (IBD) that has unique pharmacologic, endoscopic and surgical considerations secondary to chronic, transmural inflammation that affects the entire gastrointestinal tract. The natural history of this disease is complicated by formation of fibro-stenotic strictures, adhesions and penetrating fistulae. Balloon-assisted endoscopy (BAE) has significantly improved the ability to assess and treat small bowel CD.

Aims
We present a Canadian cohort of patients that have undergone BAE for stricture dilation of fibrostenotic CD. We retrospectively analyzed demographic, clinical and endoscopic risk factors that could be associated with increased risk of requiring surgical management.

Methods
Retrospective analysis of 157 patients undergoing BAE for stricture dilation of known CD from Apr 2012 – Jan 2024 at the University of Alberta Hospital. A total of 282 procedures were performed and 629 strictures dilated. Patients were then subdivided into two groups: patients that required surgery at any point after their first BAE compared with patients managed with balloon-assisted endoscopic stricture dilation alone.

Results
Of 157 patients with confirmed stricturing CD, 65/157 (41.4%) required surgery between Apr 2012- Jan 2024 and 92/157 (58.6%) patients were managed with endoscopic intervention. The average age at the time of their first BAE was 55.9 years and mean disease duration was 20.76 years in the surgery group. Average patient age in the non-surgical group was 52 years and mean disease duration was 17.9 years. 43.08% (28/65) of patients were active smokers in the surgical group vs. 26.09% (24/92) in the non-surgical group. Concurrent or previous biologic use was comparable at 76.9% and 73.9% in the surgical and non-surgical groups, respectively. The average minimum diameter dilated was 15.2(+/- 0.25) mm in the surgical group vs. 16.2(+/- 0.16) mm in the non-surgical group. The incidence of non-traversable strictures post-dilation was higher in the surgical group (34/231, 14.72%) compared with the non-surgical group (16/398, 9.20%).
In total, 97 surgeries were performed and average time to surgery from first BAE was 32.22 months (excluding emergent/urgent surgeries). Average time from most recent BAE to surgery was 8.79 months. There were 3 emergent surgeries (&lt;24 hours from BAE to surgery, including one perforation) and 7 urgent surgeries (&lt;30 days from BAE to surgery).

Conclusions
BAE has revolutionized endoscopic management of small bowel stricturing CD. Based on our retrospective case cohort, most patients with symptomatic strictures in the small bowel secondary to CD can be successfully managed with balloon-assisted endoscopic dilation. BAE may also play an important role in preventing emergent/unplanned surgical interventions.

Funding Agencies
None
A246 EXPLORING THE ROLE OF LUMINAL PROTEASES IN NOCICEPTION MODULATION FROM PATIENTS WITH ACTIVE AND REMISSIVE INFLAMMATORY BOWEL DISEASE
Authors: Wood H M , Blazkova K , Faucher F F , Sheth P M , Vanner S J , Reed D E , Bogyo M , Lomax A E 
Published: February 2025

Abstract

Background
Abdominal pain poses a significant challenge for individuals with inflammatory bowel disease (IBD). Despite current treatments that target inflammation, IBD-associated abdominal pain often persists even in the absence of inflammation, negatively impacting patients’ quality of life. This persistence suggests that factors other than inflammation may be at play. Our previous research suggests that bacterial proteases can directly influence the excitability of dorsal root ganglia neurons, many of which are pain-sensing. Building on this, we hypothesize that proteases, both of host and bacterial origin, play a role in pain modulation during the active and remission phases of IBD.

Aims
Determine whether luminal proteases in IBD fecal samples induce changes in pain signalling.

Methods
The effects of fecal supernatants (FS) from patients with active or remissive IBD and healthy volunteers (HV), on pain-sensing neurons were assessed using ex-vivo single-unit afferent nerve recordings from mouse colons. A protease inhibitor cocktail (PIC; 1:1000) and a protease-activated receptor (PAR)-2 antagonist (GB83; 10µM) were independently applied in the bioassay to determine whether these inhibited the excitatory effect of the FS. In addition, the participant FS were tested for proteolytic cleavage of the N-terminal domain of protease-activated receptor PAR2 using a novel enzymatic assay.

Results
FS from HV [N=5] had no effect on afferent nerve excitability (p&gt;0.05). FS from active IBD patients [N=15] increased action potential discharge from colonic afferent nerves by 85% (p&lt;0.0001) and selectively increased the activation of high-threshold units, which are putative nociceptors, by 44% (p&lt;0.01). A protease inhibitor cocktail and PAR2 antagonist both independently inhibited the excitatory effects of IBD FS (p&gt;0.05) on afferent nerve activity. In contrast, FS from IBD patients in remission [N=15] did not excite colonic afferent nerves (p&gt;0.05). Interestingly, these findings were found to be consistent when IBD was split into disease subtypes: Crohn’s disease and ulcerative colitis. Furthermore, when normalized to total protein content, active disease yielded significantly greater PAR2 cleavage activity (p&lt;0.05), while the remission samples were not significantly different than the HV (p&gt;0.05). This PAR2 cleavage activity was found to be correlated to neuronal excitation (R2=0.4721, p&lt;0.001).

Conclusions
Our findings suggest that active IBD leads to the generation of luminal mediators, including proteases acting on PAR2, that activate visceral nociceptive neurons. These luminal mediators are less abundant when inflammation is in remission. These data suggest that targeting proteases could offer a promising therapeutic approach for pain management in IBD.

Funding Agencies
CIHR
Hospital environment as reservoir of Pseudomonas aeruginosa in human cases: a molecular epidemiology investigation in a hospital setting in central Italy
Authors: Lombardi Adele , Tamburro Manuela , Sammarco Michela Lucia , Ripabelli Giancarlo 
Published: February 2025

Abstract
Pseudomonas aeruginosa is one of the main causes of hospital infections that are difficult to manage because of multidrug resistance (MDR). The aim of this study was to describe a molecular investigation on 19 clinical and 14 isolates from the environment in the Hub hospital of the Molise region, central Italy. Antimicrobial susceptibility was evaluated using BD Phoenix™ Automated Microbiology System. Pulsed-field gel electrophoresis (PFGE) with SpeI, enterobacterial repetitive intergenic consensus (ERIC-PCR), and random amplified polymorphic DNA-PCR were performed for genotyping. All 33 P. aeruginosa showed MDR phenotype. PFGE had 0.99 discriminatory power, underlining high heterogeneity among the strains. The genetic relatedness between two human isolates (H12 and H15) from neonatal intensive care (NICU) and one environmental strain (E1) collected from siphon of sink in the delivery room was noticeable, as well as between one strain from faucet in NICU (E2) and siphon sink (E5) from delivery room. The link between H12, H15, and E1 strains was corroborated by ERIC-PCR showing epidemiological concordance, although with a lower discriminatory power. The study findings strengthened the critical correlation between clinical P. aeruginosa and environment, according to previous molecular surveys on outbreaks occurred in Italy.
Genomic investigation of an antifungal-resistant Aspergillus fumigatus outbreak in a French hospital
Authors: Joste Valentin , Delouis Maëlys , Mouhajir Abdelmounaim , Gera Denis-Petit Stuti , Moënne-Locoz Pierre , Kernéis Solen , Houzé Sandrine , Bonnal Christine , Coppée Romain 
Published: February 2025

Abstract
Aspergillus fumigatus is associated with various invasive, chronic, and allergic fungal diseases. The emergence of environmental azole-resistant strains complicates the treatment of these infections. The use of whole-genome sequencing (WGS), which is widely used to study bacterial and viral outbreaks, could be beneficial for characterizing azole-resistant A. fumigatus outbreaks. Here, nine azole-resistant cyp51A TR34/L98H A. fumigatus strains isolated during an outbreak in a French hospital between November 2021 and October 2022 were studied. The genetic relatedness of these strains was assessed using microsatellites, high-quality single-nucleotide polymorphisms (SNPs) from WGS data, phylogenetic reconstruction, and principal component analysis. Multiple sequenced but independently cultured A. fumigatus strains were used as identical strain controls. Among the nine unrelated patients infected by TR34/L98H A. fumigatus, five had previously isolated A. fumigatus strains without the cyp51A TR34/L98H mutation. Both microsatellites and WGS confirmed that the initial cyp51A wild-type strains for these five patients were genetically different from the new mutant strains. Eight of the nine TR34/L98H strains were genetically close, with a number of SNPs similar to the controls. These resistant strains were not related to four environmental strains isolated in the hospital. Altogether, the results suggest that at least eight patients were exposed to a common source, although its exact origin could not be determined. The presence of antifungal-resistant strains in healthcare settings underscores the need for active research into resistant strains and that both microsatellites and WGS techniques have their place in the management of A. fumigatus epidemics.
Compact protoplanetary discs can be produced by dead zones
Authors: Tong Simin , Alexander Richard 
Published: February 2025

ABSTRACT
Radially compact protoplanetary discs ($\lesssim$ $50\, \mathrm{au}$) are ubiquitous in nearby star-forming regions. Multiple mechanisms have been invoked to interpret various compact discs. In this paper, we propose that fragmentation of fragile dust grains in moderate turbulence, as expected beyond the dead zone, provides an effective alternative mechanism to form compact discs which are consistent with current observations. We run 1D dust transport and collision models with dustpy and generate synthetic observations, and find that discs formed by this mechanism have sizes determined by the radial extent of their dead zones. Accounting for dust porosity and considering less fragile dust do not change disc sizes significantly. The smooth dust morphology can be altered only when pressure bumps are present in the dead zone. However, when present at small radii ($\lesssim$ 10 au), pressure bumps cannot effectively trap dust. Dust in these bumps fragments and replenishes the inner discs, effectively hiding dust traps in the optically thick inner disc from observations. We note a striking resemblance in the radial intensity profile between our synthetic observations and some recent high-resolution observations of compact discs. We discuss how such observations can inform our understanding of the underlying disc physics.
Obesity reduces the urate-lowering efficacy among patients with primary gout: a prospective cohort study
Authors: Cheng Zan , Xu Xinmiao , Qi Han , Li Xiaoyu , Li Yushuang , Jiang Chang , Miao Xinyuan , Ji Xiaopeng , Wang Yunyang , Dong Bingzi , Li Changgui , Lu Jie 
Published: February 2025

Abstract

Objectives
Obesity affects 53% of gout patients, while its effect on urate-lowering therapy (ULT) is unclear. This study aimed to compare the response to febuxostat among different BMI catalogs of male gout patients.

Methods
A prospective study recruited 633 men with gout, classified by BMI into normal-weight, overweight and obese groups. Baseline age, disease duration and serum urate (SU) levels were matched simultaneously. All participants received febuxostat for 12 weeks, increasing the dosage from 20 to 40 mg daily. We compared the efficacy of ULT and the incidence of gout flares in three groups. Cox regression analysis was used to identify risk factors for achieving target SU levels before and after matching. Restricted cubic spline (RCS) curves were used to illustrate the relationship between BMI and hazard ratio (HR) for SU achievement.

Results
The proportion of participants achieving SU &lt; 6.0 mg/dl in the obese group was 38.9%, significantly lower than the overweight (54.2%) and normal-weight groups (63.8%) (P &lt; 0.05), with no significant difference between the overweight and normal-weight groups at week 12. High BMI (HR = 0.92, 95% CI 0.89–0.96, P &lt; 0.001) independently prevented achieving the SU target, with a similar effect after matching. Obese and overweight individuals had a higher cumulative incidence of gout flares compared with the normal-weight group (P &lt; 0.05). The linear relationship between BMI and HR for achieving the SU target was suggested by RCS (nonlinear P &gt; 0.05).

Conclusion
Obesity significantly reduces the efficacy of febuxostat-based ULT in male gout patients rather than being overweight.

Trial registration
China Clinical Trial Registry, #ChiCTR2300078804.
Cast Wedging in Pediatric Forearm and Tibia Fractures—A Safe Way to Avoid Surgery
Authors: Pathangey Sachin , Canizares Maria F. , Greenberg Michael , Miller Patricia E. , Watkins Colyn , Hedequist Daniel , Shore Benjamin J. 
Published: February 2025

Background:
Cast wedging is an established technique used to improve the alignment of pediatric fractures. The purpose of this study was to review the effectiveness of cast wedging to treat malaligned pediatric fractures and report the incidence of complications and failure associated with cast wedging.

Methods:
A retrospective review of a level one pediatric trauma center was performed. Children aged 3 to 18 who underwent cast wedging following a forearm shaft, distal radius, or tibia shaft fracture (January 2005 to January 2021) were reviewed. A total of 294 patients were identified for analysis. Coronal and sagittal angles were measured throughout the course of fracture management. Improved radiologic correction was defined as at least a 50% correction of malalignment in the plane of maximum deformity. Complications associated with cast wedging were recorded, and wedging failure was defined as patients who underwent a secondary procedure. Multivariable logistic regression analysis was used to identify variables affecting outcomes and odds ratios (ORs) with 95% CIs were estimated for final model effects.

Results:
Cast wedging generated a median 56% correction in angulation, with 74% of the cohort (217/294) achieving at least 50% correction. Twenty-two patients (7%) failed wedging treatment, with half undergoing a repeat closed reduction (12/22, 55%). Eighteen patients (6%) experienced at least 1 complication. Nine patients (3%) sustained a refracture after wedging an average of 8.9 months from the initial injury. Multivariable analysis identified older patients and those who did not achieve 50% radiologic correction after wedging to have increased odds of repeat surgery (OR=1.17; P=0.04 and OR=11.1; P=0.001, respectively).

Conclusions:
Cast wedging is a safe and effective method to correct malaligned pediatric fractures with a low incidence of complications and refracture. Cast wedging remains an important skill for orthopaedic surgeons managing pediatric fractures.

Level of Evidence:
Level IV.
Pediatric Chiari Malformation Management
Authors: Gepp Ricardo , Lee June Ho , Parthiban Jutty , Costa Francesco , Dantas Fernando , Sampaio Francisco , Chen Zan , Oertel Joachim , Sharif Salman , Klekamp Jörg , Botelho Ricardo , Zileli Mehmet , Alves Óscar L. 
Published: February 2025

Study Design.
A systematic literature review and consensus using Delphi method.

Objective.
This review aims to provide an overview on Chiari malformation in pediatric patients, highlighting the specific clinical manifestations and surgical treatment options.

Summary of Background.
Chiari malformation in children presents a real difficulty to the general neurosurgeon because children are not smaller adults. In the absence of pediatric neurosurgeons, as in many countries of the world, a need for education of general neurosurgeons on the management of Chiari malformation in children was identified.

Material and Methods.
The authors carried out an extensive review of the literature in PubMed database of the last 10 years addressing the topic of Chiari malformation in children. A total of 64 studies were selected for analysis and five statements were drawn to be voted by a panel of expert spine surgeons in two consensus meetings organized by the World Federation of Neurosurgical Societies (WFNS) Spine Committee. A consensus was reached using the Delphi method.

Results.
In children with CM1, a decompressive surgery with duraplasty before puberty may avoid scoliosis progression. In Chiari type 2, the recommendation is to perform urgently extensive decompression of the craniovertebral junction and cervical canal if there is no decompensation of hydrocephalus. Cranial vault expansion may be recommended in pediatric Chiari malformation associated with craniosynostosis. Children with Chiari type I can play sports because of the low risk of worsening.

Conclusion.
Pediatric age is a modifier for the management of Chiari Malformation (CM). Prompt diagnosis and appropriate decompressive surgery with duraplasty before puberty are essential to mitigate the impact of the condition on the child’s well-being. Increased awareness among healthcare professionals, timely access to specialized expertise in neurosurgical interventions are crucial, especially for type 2 CM patients that require urgent decompression of CVJ and cervical spine.