bluuebunny/crossref_metadata_2025_split huggingface dataset:
PDOC-08 THE CLINICAL CHARACTERISTICS OF CHILDREN WITH CRANIOPHARYNGIOMA AND THEIR MANAGEMENT IN UGANDA: A RETROSPECTIVE REVIEW
Authors: Katasi Victoria Mwebe , Chintagumpala Murali , Munube Deogratius , Naitala Ronald , Kasirye Phillip , Muwonge Peter , Lubega Joseph , Zungu Jennifer , Akullo Anne , Namazzi Ruth , Nakirulu Annet , Nabaggala Cathy , Ssenyonga Peter , Onen Justine , Ssekabunga Julie , Kibudde Solomon , Wegoye Emmanuel 
Published: October 2024

Abstract

BACKGROUND
Craniopharyngiomas are benign brain tumors that mostly arise in the sellar/suprasellar region. Histologically though benign, these tumors can locally infiltrate with involvement of surrounding structures. They have a 5-year survival rate of above 80% in High Income Countries (HIC). Craniopharyngioma management includes surgery, radiotherapy and intracystic treatments, which requires a multi-disciplinary team. The morbidity associated with treatment must be weighed against tumor recurrence and quality of life after treatment. Endocrine deficiencies, especially growth hormone deficiency and visual deficits are common presenting features.

OBJECTIVE
To describe the clinical characteristics and management of children with craniopharyngioma managed at Mulago National Referral Hospital from March 2019 to December 2023. METHODOLOGY: A retrospective descriptive study was conducted among patients&lt; 18 years of age, admitted to the Mulago National Referral Hospital (MNRH) from March 2019 to December 2023 with a radiological diagnosis of a craniopharyngioma.

RESULTS
A total of 151 children with a diagnosis of a brain tumor during this period were identified and of these 40 (26%) were diagnosed with craniopharyngioma. The median age was 8.4 years and 22 (55%) were males. Only 2 children had maximal safe resection (sub-total resection), one of whom later received adjuvant external beam radiotherapy. Thirty-eight patients had insertion of Ommaya reservoirs as their tumors were predominantly cystic. Two patients received intracystic bleomycin and 11 received radical external beam radiotherapy. Twenty-seven patients had no additional therapy after Ommaya reservoir placement. Fifteen (39%) of the patients had endocrine deficits with hypothyroidism, hypocortisolism and growth hormone deficiency being the most common. The median follow-up time was 1.45 years. The overall survival was 67.5%, 95%CI (42.5-83.5).

CONCLUSION
Management of patients with craniopharyngioma in Uganda is mostly conservative with the aim of intervention to preserve quality of life. Limited access to radiation therapy contributed to the poorer survival compared to HIC.
PDOC-11 A STUDY TO DETERMINE THE TREATMENT OUTCOMES OF GANGLIONEUROBLASTOMA IN PEDIATRIC CANCER PATIENTS AT AIC KIJABE HOSPITAL IN KENYA
Authors: Girisa Peres , Michelle Obat , Sarah Muma , Musau Helena , Christine Mwakio 
Published: October 2024

Abstract

BACKGROUND
Ganglioneuroblastoma is one of neurogenic tumors characterized by an interval spectrum of differentiation with elements of both malignant neuroblastoma and benign ganglioneuroma. They are mostly diagnosed by the first decade of life with median age at diagnosis of 22-48 months.

OBJECTIVES
The main objective of this study is to determine treatment outcome of ganglioneuroblastoma in pediatric cancer patients at AIC Kijabe Hospital.

METHODOLOGY
This is a retrospective study where children diagnosed with ganglioneuroblastoma at AICKH over a period of three years have been assessed. Inclusion criteria included children from ages 0-14 years diagnosed with ganglioneuroblastoma. Data was entered in a questionnaire and was analyzed using descriptive statistical methods. Children were evaluated based on histologic tissue diagnosis, staging scans, risk stratification and monitored along the course of treatment that included during surgery, chemotherapy and radiotherapy and followed up in one year post treatment for recurrence.

RESULTS
Ganglioneuroblastoma constituted 1.3 % of all childhood malignancies diagnosed compared to the more common variant neuroblastoma with an incidence of 8-10%, mainly predominated in the males. More than 75% had remission after one-year follow up, those who died was as a result of other factors not related to the disease. Surgery and combination chemotherapy regimen COJEC administered yielded benefits in the treatment outcome. Radiotherapy was considered in high-risk tumors.

CONCLUSIONS
Ganglioneuroblastoma is commoner in older children with a median age of 3-4 years with incidence noted more in male with thoracic type tends to be a common presentation. The disease was noted to be rare after 10 years of age and generally has a good overall survival and prognosis compared to neuroblastoma.
PDOC-12 PEDIATRIC BRAIN TUMOR INCIDENCE, AND TREATMENT MODALITY AT KILIMANJARO CHRISTIAN MEDICAL CENTER
Authors: Kingazi Shammah , Majaliwa Esther , Kambainei Lossim , Gwanika Yotham 
Published: October 2024

Abstract

INTRODUCTION
Pediatric brain tumors (PBT) a significant health concern, they are the primary cause of cancer-related deaths among children. PBTs in Low- and Middle-Income Countries are diagnosed late due to insufficient resources. This abstract describes Pediatric Brain tumor incidence and treatment modalities at Kilimanjaro Christian Medical Center (KCMC) Cancer Care Center (CCC), Moshi, Tanzania from December 2016 to March 2024.

METHOD
Data on pediatric patients treated at the CCC between Dec. 2016 and March 2024, were analyzed. Inclusion criteria were a primary brain tumor and patients aged 0-19 at the time of diagnosis. All patients were previously diagnosed by computer tomography/ magnetic resonance imaging. Variables retrospectively collected from patient’s medical records were: demographic characteristics (age, gender, Place of residence, and insurance status), previous history of malignant diseases, incidence date, and treatment modality.

RESULTS
Total of 748 cases of pedeatric cancer were diagnosed at CCC. Of all the pediatric cancers diagnosed, brain cancer accounted for 22(2.9%). Out of these cases of brain cancer, 63.6% were males and 36.4% were females.Majority of them came within the Kilimanjaro region. 41% were palliated while 59% had a curative intent at the time of diagnosis, 22.7% died between December 2016 to March 2024.Embryonal tumors were (40.9%) with a breakdown that includes 6 Medulloblastoma and 3 cases of Craniopharyngioma (teratoid/rhabdoid), gliomas (36.4%) with sub-types such as Ependymoma, Anaplastic Astrocytoma, Pilocytic Astrocytoma, Pineocytoma, Stem glioma, and Tectal glioma, unspecified cases (13.6%), and Pineoblastoma were (9.1%). Surgery was used in 40.9% of cases, radiotherapy in 31.8%, and chemotherapy in 72.7%. Chemoradiotherapy was used in 31.8% of cases. 13.6% of cases utilized a combined approach, including chemotherapy, radiotherapy, and surgery.

CONCLUSION
PBT incidence at KCMC is 2.9%, improving access to screening, accurate diagnosis and treatment modalities are essential in identifying and managing PBT in our setting.
PDOC-16 A RETROSPECTIVE AUDIT ON PRIMARY PEDIATRIC BRAIN TUMOURS TREATED AT DR GEORGE MUKHARI ACADEMIC HOSPITAL, GA-RANKUWA, SOUTH AFRICA
Authors: Zimani Arthur 
Published: October 2024

Abstract

OBJECTIVE
Pediatric brain tumours are the most common solid tumours affecting 30 000-40 000 children per year worldwide. These tumours are a leading cause of morbidity and mortality in children and adolescents worldwide. A single-center retrospective audit was performed to establish the characteristics of primary pediatric brain tumours at a tertiary referral hospital in Pretoria, South Africa. The study will help with setting up efficient screening and treatment protocols for pediatric neuro-oncology guarded by the study’s findings.

METHODS
The results from the analysis of the data recorded for the 29 patients in the study are presented in this chapter. The statistical analysis was descriptive, and all the procedures were performed on SAS (SAS Institute Inc, Cary, NC, USA), Release 9.4.

RESULTS
The most common age range was 0-4 years, with males (65.5%) affected more than females (34.5%). Headaches were reported in 69% of the patients, and vomiting was observed in 93.1%. Seizures were observed in 27.6% of patients and were associated with supratentorial tumours more than infratentorial tumours. On radiological examination, 45.5% of tumours were supratentorial, and the remainder, 54.5%, were infratentorial. Hydrocephalus was observed in 93.1% of patients and is associated mostly with infratentorial tumours. Pilocytic astrocytomas were the most common histological diagnosis at 20.7%, followed in second place by medulloblastomas and pineoblastomas, both at 10.3%.

CONCLUSION
The audit results are largely consistent with published literature in terms of gender profile, mean age at diagnosis, common presenting symptoms and signs and common histopathological types of Pediatric Brain Tumours. The small sample size and lack of sufficient clinical data make it difficult to make meaningful conclusions on some of the results.
TRLR-02 TARGETING GLIOBLASTOMA MICROENVIRONMENT TO UNLEASH IMMUNE FORCES
Authors: Janowski Miroslaw 
Published: October 2024

Abstract
Brain cancer has persistently eluded therapeutic advances over the past three decades. Here, we aim to raise awareness of the potential differences between the cancer microenvironment within the brain vs. peripheral organ cancers, which may contribute to the therapeutic setbacks observed in brain cancer treatment. While the blood-brain barrier has long been implicated in drug impermeability, its impact on brain cancer may extend far beyond mere drug exclusion. Studies have shown that soluble factors such as VEGF are more concentrated in brain cancer than in any other malignancies. Moreover, recent studies revealed that VEGF, TWEAK, and others can also press on the immune brakes. Therefore, we hypothesize that normalizing the tumor microenvironment could pave the way for effective therapeutic interventions, especially immunotherapy. Our in vitro studies confirmed abundant production of VEGF by glioblastoma 1 (GBM1) cell line, which is exceptionally well scavenged by the anti-VEGF antibody – bevacizumab. Moreover, in vitro, co-culture of T cells and GBM1 cell line without vascular component demonstrated no therapeutic activity of bevacizumab or typical immunotherapeutic drug – anti-PD1 checkpoint inhibitor – nivolumab given in monotherapies. However, a combination of both antibodies revealed therapeutic efficacy in a dose-dependent manner in our experimental system. Currently, intravenous delivery of bevacizumab is the standard of care in patients with glioblastoma recurrence, but its effectiveness has not been proven in this group of patients. Intravenous nivolumab failed in clinical trials. Based on our observations and the negligible penetration of antibodies to the brain, we hypothesize that transiently opening the blood-brain barrier may be necessary to enhance antibody delivery to the tumor microenvironment, alleviate immune suppression, and unlock immune cell activity. Therefore, achieving high antibody concentrations in brain cancer tissue, coupled with a combination of classic checkpoint inhibitors with scavengers of soluble immunosuppressive molecules, could herald a new era of brain cancer therapy.
High lipoprotein(a) is a risk factor for peripheral artery disease, abdominal aortic aneurysms, and major adverse limb events
Authors: Thomas Peter E. , Vedel-Krogh Signe , Kamstrup Pia R. 
Published: October 2024

Purpose of review
To summarize evidence from recent studies of high lipoprotein(a) as a risk factor for peripheral artery disease (PAD), abdominal aortic aneurysms (AAA), and major adverse limb events (MALE). Additionally, provide clinicians with 10-year absolute risk charts enabling risk prediction of PAD and AAA by lipoprotein(a) levels and conventional risk factors.

Recent findings
Numerous studies support high lipoprotein(a) as an independent risk factor for PAD, AAA, and MALE. The strongest evidence is from the Copenhagen General Population Study (CGPS) and the UK Biobank, two large general population-based cohorts. In the CGPS, a 50 mg/dl higher genetically determined lipoprotein(a) associated with hazard ratios of 1.39 (1.24–1.56) for PAD and 1.21 (1.01–1.44) for AAA. Corresponding hazard ratio in the UK Biobank were 1.38 (1.30–1.46) and 1.42 (1.28–1.59). In CGPS participants with levels at least 99th (≥143 mg/dl) vs, less than 50th percentile (≤9 mg/dl), hazard ratios were 2.99 (2.09–4.30) for PAD and 2.22 (1.21–4.07) for AAA, with a corresponding incidence rate ratio for MALE of 3.04 (1.55–5.98) in participants with PAD.

Summary
Evidence from both observational and genetic studies support high lipoprotein(a) as a causal risk factor for PAD, AAA, and MALE, and highlight the potential of future lipoprotein(a)-lowering therapy to reduce the substantial morbidity and mortality associated with these diseases.
Corneal Epithelial Remodeling After Hyperopic SMILE: A 4-Year Study
Authors: Fu Dan , Zhang Luoli , Shang Jianmin , Lin Feng , Han Tian , Zhou Xingtao 
Published: October 2024

Purpose:
The purpose of this study was to investigate dynamic changes in corneal epithelial thickness (ET) after hyperopic small incision lenticule extraction (SMILE).

Methods:
This observational study included 18 eyes of 16 patients who were scheduled for hyperopic SMILE. High-resolution spectral-domain optical coherence tomography and a custom image segmentation algorithm were applied to measure corneal dynamic ET over a central 6 mm area. ET was analyzed among different corneal sectors (central, pericentral, and peripheral) at baseline and multiple postoperative time points (1 day, 1 month, 6 months, 2 years, and 4 years). Correlation analysis was performed between ET changes and preoperative and intraoperative parameters.

Results:
The mean spherical equivalent of hyperopia correction was +2.64 ± 1.00 D, and the average age of enrolled 16 patients was 32.4 ± 10.8 years. At the last follow-up, 72% of eyes kept or improved corrected distance visual acuity, with 2 eyes that lost 2 lines. 72% of eyes were within ±1.0 D of target refraction, and 56% were within ±0.5 D. Average ET decreased by 11.0% (5.9 ± 2.3 μm) from 53.2 ± 3.5 μm to 47.4 ± 4.2 μm 4 years postoperatively (P<0.001). The ET at center 2 mm decreased most significantly by 14.5% (7.9 ± 2.9 μm), followed by the pericentral area by 10.5% (5.6 ± 2.3 μm). ET and front corneal curvature stabilized 1 month after surgery. No correlation was found between refraction regression and ET changes.

Conclusions:
Hyperopic SMILE induced significant central ET thinning. ET remodeling in the 6 mm area stabilized after 1 month and did not underlie refractive changes.
Determination of Mandibular Morphology in a Turkish Population With Bipolar I Disorder Using Panoramic Radiography
Authors: Bahadir Hakan , Yetimoğlu Nihal , Oflezer Özlem , Erkiran Murat 
Published: September 2024

Abstract
The presence of specific craniofacial deviations may be evidence of abnormal early brain development in bipolar disorder (BD). This study aimed to compare mandibular morphology in patients with BD versus nonpsychiatric controls using panoramic radiography. A total of 215 recorded images, 70 patients with BD and 145 controls, were evaluated. Seven mandibular parameters of the ramus height (RH), condylar height (CH), gonial angle (GA), antegonial angle (AGA), antegonial notch depth (AND), ramal notch depth (RND), and bigonial width (BGW) were measured in a blinded manner. Group analysis was conducted separately for males and females. The univariate comparisons showed increased values in RH, CH, AND, RND, and BGW and decreased values in GA in males with BD compared with male controls. In addition, females with BD had higher RH, CH, RND, and BGW values in comparison to female controls. The differences were statistically significant. Data from multivariate analyses revealed that increases in RH and BGW values in both sexes were significant predictive variables that distinguished patients with BD from controls. Moreover, deeper AND was the most prominent predictor that distinguished male patients with BD from male controls. The identification of subtle changes in mandibular measurements is consistent with the notion of impaired neurodevelopment in BD.
Psychological Assessment of Health Care Workers in the Aftermath of the February 2023 Earthquakes in Turkey
Authors: Sehlikoğlu Şeyma , Yastibaş Kaçar Cennet , Yilmaz-Karaman Imran Gokcen 
Published: October 2024

Objective:
The goal of this study was to examine the psychological and physical effects experienced by health care workers (HCWs) participating in the response to the February 2023 earthquakes in Turkey and to identify any associated factors.

Methods:
An online survey was used to collect data from HCWs on duty in earthquake-stricken areas. The following assessment tools were utilized: Posttraumatic Stress Disorder (PTSD) Checklist for DSM-5, Patient Health Questionnaire-9, Generalized Anxiety Disorder-7, Maslach Burnout Inventory, Posttraumatic Growth Inventory, and Short Form-12.

Results:
A total of 175 HCWs with a mean age of 37.27 years participated in the study. Of these, 39.4% suffered from PTSD, 30.3% experienced depression, and 31.4% experienced anxiety. Female gender, loss of significant others, and previous psychiatric treatment were found to be associated with worse mental health. Nurses tended to have higher levels of PTSD than the medical doctors; the medical doctors had significantly lower scores on the Posttraumatic Growth Inventory compared with the nurses and the other HCWs and lower mental component summary scores on the Short Form-12 compared with the other HCWs. Meeting basic needs and subjective evaluation of teamwork were also linked to mental health. The study also found that marital status, age, and length of time spent in earthquake-stricken areas were associated with scores on the Maslach Burnout Inventory.

Conclusions:
After the earthquake in Turkey, HCWs experienced a significant amount of various adverse mental health outcomes related to certain demographic variables such as gender, profession, previous psychiatric treatment, loss of relatives, and evaluation of living conditions and teamwork. Since HCWs play an essential role in reducing the harmful effects of disasters, recognizing groups at risk and planning tailored interventions may help prevent mental health issues.
Patient-Reported Quality of Life for Osteoradionecrosis Reconstruction in the Head and Neck: A Longitudinal Framework and Risk Factors
Authors: Wu Robin T. , Lin Jennifer An‐Jou , Su Charlie Chun-Lin , Wei Fu-Chan 
Published: October 2024

Background:
Osteoradionecrosis (ORN) is an aggressive sequela of head and neck cancer, treatment of which focuses on functional restoration and quality-of-life (QoL). This study aims to identify risks for poor QoL in ORN reconstruction and build a chronologic, longitudinal framework for QoL.

Methods:
A prospective database of reconstructions performed by the senior author was reviewed from 2015-2023. QoL metrics (University of Washington [UWQoL]v4) were prospectively administered prior to surgery, one year post-operatively, and each yearly follow-up.

Results:
56 ORN patients were included (average 58.2years, mean 6,412Gy radiation). Reconstruction commonly was achieved with the fibula(55.4%) and anterolateral-thigh flaps(37.5%). The total complication rate was 23.2%, median 10.7mo post-operatively.
Both “health-related QoL in comparison with prior to cancer diagnosis” (62.5 vs 43.5;p=0.030) and “Overall QoL during the past 7-days” (50.5 vs 41.7;p=0.029) were higher post-ORN reconstruction than before. Physical-QoL was higher pre-cancer reconstruction (79.0) than prior to ORN reconstruction (50.6;p<0.001) and following reconstruction (52.5;p=0.001). Social-emotional function was higher following ORN reconstruction compared to pre-reconstruction (68.7 vs 59.6;p=0.010).
On multi-variate analysis, both post-operative social-emotional and physical function were impacted by betelnut use (p=0.038;p=0.025). Poor improvement in QoL from pre to post-ORN reconstruction were affected by maxilla involvement (p=0.048), fistula (p=0.004), and hardware issues (p=0.001).

Conclusions:
Our longitudinal experience trended decline in QoL at ORN diagnosis with gradual improvement following reconstruction, with eventual social-emotional, pain, anxiety, chewing, and global-QoL significantly improved following surgery. Betelnut was a risk factor for poor post-operative QoL. Maxillary involvement, post-op fistula and hardware issues were risks for non-improvement in QoL.
Diagnosis and Screening of Velocardiofacial Syndrome by Evaluating Facial Photographs Using a Deep Learning-Based Algorithm
Authors: Baek Rong-Min , Cho Anna , Chung Yoon Gi , Jeon Yonghoon , Kim Hunmin , Hwang Hee , Kang Jiwon , Myung Yujin 
Published: October 2024

Background:
Early detection of rare genetic diseases, including velocardiofacial syndrome (VCFS), is essential for patient well-being. However, their rarity and limited clinical experience of physicians make diagnosis challenging. Deep learning algorithms have emerged as promising tools for efficient and accurate diagnosis. This study investigates the use of a deep learning algorithm to develop a face recognition model for diagnosing VCFS.

Methods:
The study employed publicly available labeled face datasets to train the multitask cascaded convolutional neural networks (MTCNN) model. Subsequently, we examined the binary classification performance for diagnosing VCFS using the most efficient face recognition model. A total of 98 VCFS patients (920 facial photographs) and 91 non-VCFS controls (463 facial photographs) were randomly divided into training and test sets. Additionally, we analyzed whether the classification results matched the known facial phenotype of VCFS.

Results:
The face recognition model demonstrated high accuracy, ranging from 94% to 99%, depending on the training dataset. The accuracy of the binary classification diagnostic model varied from 81% to 88% when evaluating with photographs taken at various angles, but reached 95% evaluating with frontal photographs only. Gradient-weighted class activation mapping heatmap revealed the high importance level of perinasal and periorbital areas, exhibiting consistency with the conventional facial phenotypes of VCFS.

Conclusion:
This study shows the feasibility and effectiveness of MTCNN-based model for detecting VCFS solely from facial photographs. The high accuracy underscores the potential of deep learning in aiding early diagnosis of rare genetic diseases, facilitating timely interventions for patient care.