bluuebunny/crossref_metadata_2025_split huggingface dataset:
P32 Kawasaki disease: learning points from 6 years of audit data from a large university teaching hospital
Authors: Patel Fahim , Sridhar Arani 
Published: September 2019

Abstract

Background
Kawasaki disease is the most common cause of acquired childhood heart disease in developed countries. If left untreated, 15 to 25% of children develop coronary artery aneurysms and more worryingly 2 to 3% will die from coronary vasculitis. Prompt recognition and management including referral to a cardiologist can ameliorate this. The clinical challenge however is the lack of any diagnostic tests and that many cases do not fit the typical diagnostic criteria.
Here we present the results from an original audit assessing our practice against RCPCH guidelines. We show findings from audit cycles spanning six years from Leicester’s children’s hospital. We also take one step further to analyse this data looking for trends in presentation and lab findings.

Methods
Original audit data was collected from cases between 2008-2012 and 2015-2017 (audit standards are shown in figure 1). The data generated from these audits was re-analysed to assess for trends in presentation and laboratory findings.

Results
In six years we saw 41 cases: 23 were of atypical presentation and 18 were typical (figure 2); 27 patients were male and 14 were female. The age range was 1 month to 13 years. All patients received IVIG, aspirin and were referred to cardiology. No patients required biological therapies. Cardiological events: Data incomplete – patients disseminated across several tertiary units; acutely - 6 patients had a borderline/abnormal echocardiogram; long-term - 1 patient has persistent but resolving coronary artery aneurysms.

Mortalities
A 4-month old in 2008 died as a result of untreated atypical Kawasaki disease. This patient was diagnosed only at autopsy.

Conclusion
Over the 2 audit periods we noted a higher than expected caseload of atypical KD. At presentation: 88% had no desquamation, 30% had only mildly raised CRP, and 2% did not have fever for more than 5 days.

Conflicts of Interest
The authors declare no conflicts of interest.
P53 Paediatric non-infectious dacryoadenitis: a single-centre experience
Authors: Silva-Dinis Joana , Solebo Ameenat Lola , Abou-Rayyah Yassir , Compeyrot-Lacassagne Sandrine 
Published: September 2019

Abstract

Background
Dacryoadenitis is an inflammatory enlargement of the lacrimal gland and can be idiopathic or associated with infections, malignant or inflammatory diseases like Sjögren’s disease (SSj) or granulomatosis with polyangiitis (GPA). It remains the most common orbital inflammatory condition in children.
This study purposes to describe a paediatric cohort of non-infectious dacryoadenitis.

Methods
Identification of children who underwent lacrimal gland biopsy at our centre between 2000 and 2018 was done by searching the database using two key-words: “lacrimal” and “dacryoadenitis”. Patients with infectious or malignant dacryoadenitis were excluded. Medical notes were reviewed to collect data.

Results
Twenty-six patients underwent lacrimal gland biopsy at our centre between 2000 and 2018 and had inflammatory dacryoadenitis. 17 patients (65.4%) had non-specific chronic inflammation (NSCI) and 9 patients (34.6%) had an inflammatory systemic condition (Table 1). P53 Table 1Patients’ characteristics.NSCIGPASarcoidosisSSjogrenIgG4Number of patients17 (65.4%)4 (15.4%)3 (11.5%)1 (3.9%)1 (3.9%)Female, n (%)6 (35.3%)4 (100%)3 (100%)1 (100%)1 (100%)Median age (Y) (IQ)10.5 (4.9- 14.4)9.72 (8.7- 13.0)13.2 (11.45- 15.7)11.911.2Lacrimal gland involvementUnilateral13 (74.5%)3 (75.0%)2 (66.7%)00Bilateral4 (23.5%)1 (25.0%)1 (33.3%)1 (100%)1 (100%)Orbital symptomsPtosis4 (23.5%)1 (25.0%)000Erythema3 (17.5%)1 (25.0%)1 (33.3%)00Pain2 (11.8%)1 (25.0%)000Extra-orbital involvementExocrine glands001 (33.3%)00ENT03 (75.0%)000Renal1 (14.3%)2 (50.0%)01 (100%)1 (100%)Pulmonary1 (14.3%)1 (25.0%)1 (33.3%)01 (100%)Myositis1 (14.3%)0000Arthralgia1 (14.3%)01 (33.3%)00Thyroiditis1 (14.3%)0000CNS0001 (100%)0Lymphadenopaties00001 (100%)
4/13 children tested had raised ACE (2 sarcoidosis and 2 NSCI). 7/17 had raised amylase (2 sarcoidosis,1 SSj, 4 NSCI). 3/16 had raised IgG (IgG4 disease, GPA and NSCI). ANA was positive in 4/19 patients (NSCI, GPA, Sarcoidose, SSj) and ANCA was positive in 4/19 patients (GPA).
Thirteen patients received steroids (oral or IV) and elevan received DMARDS (MMF, methotrexate, azathioprine and hydroxychloroquine). Four patients received biologic (rituximab, adalimumab or infliximab). Six patients underwent orbitotomy. Patients still on follow-up had good response to treatment.
One patient with NSCI presented with dacryoadenitis and later developed auto-immune thyroiditis and polymyositis, with positive ANA and anti-SRP antibodies. He was treated with steroids, methotrexate, MMF and IVIG.

Conclusion
Paediatric rheumatic inflammatory disorders have a diverse presentation and dacryoadenitis may be the initial manifestation. Biopsy is mandatory to exclude infection and malignancy. A full diagnostic workup is essential, especially if patients have positive ANA or ANCA.

Conflicts of Interest
The authors declare no conflicts of interest.
Does the Duration of Cervical Radicular Symptoms Impact Outcomes After Anterior Cervical Discectomy and Fusion?
Authors: Basques Bryce A. , Ahn Junyoung , Markowitz Jonathan , Harada Garrett , Louie Philip K. , Mormol Jeremy , Varthi Arya , Goldberg Edward J. , An Howard S. 
Published: September 2019

Study Design:
Retrospective cohort study.

Objective:
To compare clinical outcomes after anterior cervical discectomy and fusion (ACDF) based on preoperative duration of radiculopathy symptoms.

Summary of Background Data:
There is no clear indication of when outcomes after ACDF become less effective in the setting of acute versus chronic symptoms from cervical nerve root compression.

Materials and Methods:
A retrospective cohort study of consecutive patients who underwent an ACDF between 2008 and 2015 for radiculopathy was performed. Patients were divided into 3 groups: radicular symptoms lasting for <6 months, symptoms lasting for 6–12 months, and those with symptoms for ≥12 months. Neck Disability Index (NDI) and Visual Analog Scale (VAS) scores for both the neck and arms were obtained at preoperative and final postoperative visits. Radiographs were assessed for adjacent segment degeneration, fusion, and subsidence. Bivariate and multivariate regressions were subsequently used to compare outcomes between groups.

Results:
A total of 379 patients were included. Patients with symptoms lasting for 6–12 months and those with symptoms lasting for ≥12 months had worse preoperative VAS neck pain compared with patients with symptoms lasting for < 6 months (P=0.000 and P=0.004, respectively). Patients with symptoms lasting for ≥12 months had worse baseline functional status compared with patients with symptoms lasting for <6 months (P=0.009). At final follow-up, there were no significant differences in VAS neck, VAS arm, or NDI between symptom duration groups. However, differences in outcomes were noted when considering the type of preoperative symptom. Patients with pain symptoms of ≥12 months had worse preoperative VAS neck (P=0.001), VAS arm (P=0.008), and NDI (P<0.001) and had significantly worse final VAS arm (P=0.019) and NDI (P=0.016) compared with patients with preoperative pain symptoms lasting for <6 months. The overall reoperation rate was 4.5%. Fusion was achieved in 97.6% of the patients, and subsidence was observed in 5.5%. There were no significant differences in the risk of adjacent segment degeneration, reoperations, fusion rates, or subsidence rates based on preoperative symptom duration.

Conclusions:
Despite worse preoperative neck pain and functional status in patients with preoperative duration of symptoms >6 months, there were no differences in final clinical outcomes across groups.
Pathologic Response to Primary Systemic Therapy With FOLFIRINOX in Patients With Resectable Pancreatic Cancer
Authors: Polish Ariel , Joseph Nora E. , Marsh Robert de W. 
Published: September 2019

Background:
Primary systemic therapy in resectable pancreatic cancer is currently under investigation. FOLFIRINOX has been shown to be effective in both the adjuvant and metastatic settings and is increasingly being used on and off study in the neoadjuvant setting. The objective pathologic response elicited by this regimen in truly resectable disease has not as yet been widely reported.

Methods:
This analysis focuses on 14 patients with resectable pancreatic cancer who were treated in a pilot study of primary systemic therapy, using 4 cycles of neoadjuvant FOLFIRINOX before surgery. A dedicated pancreatic pathologist reviewed all of the subsequent surgical specimens to assess the degree of tumor regression elicited by this approach, according to the scoring system proposed by Evans.

Results:
Four patients (28.6%) had Evans grade I, 4 (28.6%) Evans grade IIa, 2 (14.2%) Evans grade IIb, and 4 (28.6%) Evans grade III response to the primary systemic therapy. There were no Evans grade IV responses.

Conclusions:
The results are intriguing with 28% of the specimens showing destruction of <10% of tumor cells, and only 28% achieving >90% destruction of tumor cells. The significant variation in response once again confirms the known heterogeneity in the biology of this cancer and clearly FOLFIRINOX is not equally effective in all patients. Future studies evaluating primary systemic therapy in pancreatic cancer should examine the optimal duration of therapy before surgery and should include a standardized pathologic grading scheme to better enable comparison of results.
Prognostic Value of Serum NPY Hypermethylation in Neoadjuvant Chemoradiotherapy for Rectal Cancer
Authors: Appelt Ane L. , Andersen Rikke F. , Lindebjerg Jan , Jakobsen Anders 
Published: September 2019

Objectives:
Long-term prevention of metastatic disease remains a challenge in locally advanced rectal cancer, and robust pretreatment prognostic factors for metastatic progression are lacking. We hypothesized that detecting circulating tumor-specific DNA (ctDNA) based on hypermethylation of the neuropeptide Y gene (meth-ctDNA) could be a prognostic marker in the neoadjuvant setting; we examined this in a secondary, explorative analysis of a prospective trial.

Materials and Methods:
Serum samples were prospectively collected in a phase III trial for locally advanced rectal cancer. Positivity for and fractional abundance of meth-ctDNA in baseline samples were estimated. Overall survival (OS) and the rate of distant metastases were compared between meth-ctDNA positive and negative patients; other prognostic factors were controlled for in multivariate Cox regression. Importance of quantitative load was examined by considering the fractional abundance of meth-ctDNA relative to total circulating DNA.

Results:
Baseline serum samples were available for 146 patients. In total, 30 patients had presence of meth-ctDNA, with no correlation with cT (P=0.8) or cN (P=0.6) stages. Median follow-up was 10.6 years for OS and 5.1 years for freedom from distant metastases. Patients with meth-ctDNA had significantly worse 5-year OS (47% vs. 69%), even when controlling for other prognostic factors (hazard ratio=2.08; 95% confidence interval, 1.23-1.51). This seemed mainly driven by disparity in the rate of distant metastases (55% vs. 72% at 5 y, P=0.01); hazard ratio=2.20 (95% confidence interval, 1.19-4.07, P=0.01) in multivariate analysis. Increased quantitative load was highly significant for worse outcomes.

Conclusions:
Meth-ctDNA could be a potential prognostic marker in the neoadjuvant setting and may, if validated, identify patients at increased risk of distant metastases.
Short- and Long-Term Results of Xenogeneic-Free Cultivated Autologous and Allogeneic Limbal Epithelial Stem Cell Transplantations
Authors: Behaegel Joséphine , Zakaria Nadia , Tassignon Marie-José , Leysen Inge , Bock Felix , Koppen Carina , Ní Dhubhghaill Sorcha 
Published: September 2019

Purpose:
To evaluate the short- and long-term success rates of xenogeneic-free cultivated limbal epithelial stem cell transplantation (CLET) for the treatment of limbal stem cell deficiency (LSCD).

Methods:
Thirteen patients with LSCD underwent an autologous (n = 9) or allogeneic (n = 4) CLET. The primary end point was to assess the long-term anatomical success rate of transplanted grafts at a follow-up of at least 3 years, in comparison with the short-term outcomes. Secondary end points involved reviewing functional improvement, patient-reported symptoms, and change in percentage area of corneal vascularization in both short-term and long-term.

Results:
The mean short- and long-term follow-up periods were 2.1 ± 0.38 years and 6.7 ± 1.81 years, respectively. The total anatomical success rate was 46.1% in the short-term, but it decreased to 23.1% in the long-term. A partial success rate of 30.8% was observed in both short- and long-term, and the failure rate increased from 23.1% to 46.1%. The mean percentage of vessel area decreased from 12.11% ± 5.29% preoperatively to 7.82% ± 6.70% in the short-term and increased to 8.70% ± 6.32% in the long-term. There was a significant improvement in best-corrected visual acuity (P = 0.044) in the short-term although not in the long-term (P = 0.865).

Conclusions:
This study shows that anatomical and functional success rates of CLET decrease over time. We believe that the decline of success is related to the extent of disease, cell origin, and lack of niche protection because subtotal LSCD and autologous donor cells confer a higher chance of success in the long-term.
Tear Film and Meibomian Gland Characteristics in Adolescents
Authors: Tichenor Anna A. , Ziemanski Jillian F. , Ngo William , Nichols Jason J. , Nichols Kelly K. 
Published: September 2019

Purpose:
To establish normative values of ocular surface parameters in adolescents and explore factors associated with meibomian gland (MG) dropout.

Methods:
Subjects between 8 and 17 years of age were enrolled in this cross-sectional study. All subjects were given dry eye and lifestyle questionnaires. Tear film assessments and meibography were performed. Statistical tests included a one-way analysis of variance to test differences in ocular surface parameters between age groups and linear correlations between clinical findings and lifestyle factors.

Results:
Two hundred twenty-five subjects completed the study. Thirty-four subjects (15%) reported ocular discomfort, primarily itching. Tear meniscus height increased with age and was greatest in the oldest subjects (mean = 0.25 mm, P < 0.01). Across all subjects, meibography showed that 39% of the upper and 39% of the lower eyelids had MG dropout. The average MG dropout score was 0.50 ± 0.57 for the upper eyelids and was 0.67 ± 0.93 for the lower eyelids. There was no correlation between phone/tablet usage and MG dropout for either the upper (P = 0.39) or lower (P = 0.56) eyelids.

Conclusions:
The frequency of ocular symptoms in these adolescents was 15%. Because MG dropout is thought to increase with age, it was unexpected to observe that most subjects in this study had mild MG dropout in 1 or both eyelids. Although electronic device usage did not correlate with MG dropout in this study sample, it is still unclear what the effects of long-term digital device usage may have as the subjects age.
Analysis of Compensatory Corneal Epithelial Thickness Changes in Keratoconus Using Corneal Tomography
Authors: Franco Jimena , White Craig A. , Kruh Jonathan N. 
Published: September 2019

Purpose:
To determine patterns of epithelial remodeling in keratoconus and to assess changes in these patterns as the disease progresses.

Methods:
This is a prospective case series. Patients with keratoconus undergoing corneal collagen crosslinking underwent Scheimpflug imaging before and after epithelial debridement. Analysis was performed to determine maps of epithelial thickness and change in keratometry. Maps were analyzed for patterns, and map SD was quantified. Measures were compared across the patients as grouped by the severity of disease.

Results:
The study comprised 38 eyes from 30 patients. Patients were stratified using the Amsler–Krumeich classification of keratoconus severity, with 17, 14, and 7 patients in the stage I, stage II, and stage III groups, respectively. A pattern of central epithelial thinning (to approximately 20 μm) with an annulus of epithelial thickening (to approximately 30–40 μm) was demonstrated. Changes were more pronounced in the later stages of the disease, with the average central thickness decreasing from 23 μm in stage I to 18 μm in stage III. Central corneal steepening of 1.5 to 1.9 diopters and peripheral flattening of 1.4 to 2.0 diopters after epithelial debridement were demonstrated. Analysis of map SD revealed a significant difference between stage III patients and patients at earlier stages of disease.

Conclusions:
The “doughnut pattern” of epithelial remodeling in keratoconus is supported by Scheimpflug imaging. This pattern is demonstrated to partially compensate for central corneal steepening seen in keratoconus.
MicroRNA-19b predicts widespread pain and posttraumatic stress symptom risk in a sex-dependent manner following trauma exposure
Authors: Linnstaedt Sarah D. , Rueckeis Cathleen A. , Riker Kyle D. , Pan Yue , Wu Alan , Yu Shan , Wanstrath Britannia , Gonzalez Michael , Harmon Evan , Green Paul , Chen Chieh V. , King Tony , Lewandowski Christopher , Hendry Phyllis L. , Pearson Claire , Kurz Michael C. , Datner Elizabeth , Velilla Marc-Anthony , Domeier Robert , Liberzon Israel , Mogil Jeffrey S. , Levine Jon , McLean Samuel A. 
Published: September 2019

Abstract
Posttraumatic widespread pain (PTWP) and posttraumatic stress symptoms (PTSS) are frequent comorbid sequelae of trauma that occur at different rates in women and men. We sought to identify microRNA (miRNA) that may contribute to sex-dependent differences in vulnerability to these outcomes. Monte Carlo simulations (x10,000) identified miRNA in which predicted targeting of PTWP or PTSS genes was most enriched. Expression of the leading candidate miRNA to target PTWP/PTSS-related genes, miR-19b, has been shown to be influenced by estrogen and stress exposure. We evaluated whether peritraumatic miR-19b blood expression levels predicted PTWP and PTSS development in women and men experiencing trauma of motor vehicle collision (n = 179) and in women experiencing sexual assault trauma (n = 74). A sex-dependent relationship was observed between miR-19b expression levels and both PTWP (β = −2.41, P = 0.034) and PTSS (β = −3.01, P = 0.008) development 6 months after motor vehicle collision. The relationship between miR-19b and PTSS (but not PTWP) was validated in sexual assault survivors (β = −0.91, P = 0.013). Sex-dependent expression of miR-19b was also observed in blood and nervous tissue from 2 relevant animal models. Furthermore, in support of increasing evidence indicating a role for the circadian rhythm (CR) in PTWP and PTSS pathogenesis, miR-19b targets were enriched in CR gene transcripts. Human cohort and in vitro analyses assessing miR-19b regulation of key CR transcripts, CLOCK and RORA, supported the potential importance of miR-19b to regulating the CR pathway. Together, these results highlight the potential role that sex-dependent expression of miR-19b might play in PTWP and PTSS development after trauma/stress exposure.
Breast Cancer and Hair Loss
Authors: Trusson Diane , Quincey Kerry 
Published: September 2019

Background
There are relatively few studies comparing men’s and women’s breast cancer experiences. Furthermore, men’s experiences of cancer treatment–induced alopecia have received scant academic attention compared with those of women.

Objective
To explore experiences of treatment-induced alopecia in both sexes and highlight ways in which they might be supported when undergoing breast cancer treatment.

Methods
Qualitative interviews and photographic data taken from 2 separate experiential inquiries were analyzed together, focusing on references made to treatment-induced alopecia in women’s and men’s breast cancer accounts.

Results
Hair loss was described as distressing by both sexes, affecting gendered identities and relationships. Men typically discussed loss of body hair, whereas women rarely referred to body hair explicitly, underlining gendered aspects of their experiences. Differences were also noted in coping strategies, with men using humor and asserting their masculinity. Women were better able to disguise hair loss, whereas men were forced to reveal their hairlessness.

Conclusions
The findings contribute a nuanced understanding of the experience of treatment-induced alopecia for both men and women, which will help to improve their care during cancer treatment.

Implications for Practice
Healthcare professionals should provide information about the possible implications of cancer-related alopecia for identities and social relationships for both sexes. Highlighting marked gender differences in cancer-related hair loss, advice and support specific to men’s needs would be particularly beneficial, enabling greater gender equality in clinical practice. Understanding the coping strategies employed by both sexes in relation to hair loss will help healthcare professionals to identify and address any underlying patient distress.
Intracranial Electroencephalography in Pediatric Severe Traumatic Brain Injury
Authors: Appavu Brian , Foldes Stephen , Temkit M’hamed , Jacobson Austin , Burrows Brian T. , Brown Danni , Boerwinkle Varina , Marku Iris , Adelson P. David 
Published: September 2019

Objectives:Electroencephalography is used in neurocritical care for detection of seizures and assessment of cortical function. Due to limited resolution from scalp electroencephalography, important abnormalities may not be readily detectable. We aimed to identify whether intracranial electroencephalography allows for improved methods of monitoring cortical function in children with severe traumatic brain injury.Design:This is a retrospective cohort study from a prospectively collected clinical database. We investigated the occurrence rate of epileptiform abnormalities detected on intracranial electroencephalography when compared with scalp electroencephalography. We also investigated the strength of association of quantitative electroencephalographic parameters and cerebral perfusion pressure between both intracranial and scalp electroencephalography.Setting:This is a single-institution study performed in the Phoenix Children’s Hospital PICU.Patients:Eleven children with severe traumatic brain injury requiring invasive neuromonitoring underwent implantation of a six-contact intracranial electrode as well as continuous surface electroencephalography.Interventions:None.Measurements and Main Results:Visual detection of epileptiform abnormalities was performed by pediatric epileptologists. Association of intracranial and scalp electroencephalography total power, alpha percentage, and alpha-delta power ratio to cerebral perfusion pressure was performed using univariate dynamic structural equations modeling. Demographic data were assessed by retrospective analysis. Intracranial and scalp electroencephalography was performed in 11 children. Three of 11 children had observed epileptiform abnormalities on intracranial electroencephalography. Two patients had epileptiform abnormalities identified exclusively on intracranial electroencephalography, and one patient had seizures initiating on intracranial electroencephalography before arising on scalp electroencephalography. Identification of epileptiform abnormalities was associated with subsequent identification of stroke or malignant cerebral edema. We observed statistically significant positive associations between intracranial alpha-delta power ratio to cerebral perfusion pressure in nine of 11 patients with increased strength of association on intracranial compared with scalp recordings.Conclusions:These findings suggest that intracranial electroencephalography may be useful for detection of secondary insult development in children with traumatic brain injury.