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1.
Chaos ; 34(7)2024 Jul 01.
Article in English | MEDLINE | ID: mdl-38985964

ABSTRACT

In this paper, we investigate the time evolution of the rank correlation coefficients of the prestige indicators of the journals (such as Scimago Journal Rank) over 21 years, for 9856 journals in various disciplines. The observed dependencies do not differ qualitatively from those observed for the time evolution of the ranking correlations in wealth rheology among the richest people recently found by Burda et al. [Entropy 23, 842 (2021)], but quantitatively the changes in rank correlations for human wealth are much stronger than in the coefficients measuring the prestige of journals. In terms of Kendall's rank correlation coefficients, disciplines "Agricultural and Biological Sciences" and "Earth and Planetary Science" are the most typical, meaning that the time evolution of their rank correlation coefficient based on Scimago Journal Rank is closest to the time evolution of an average over all disciplines. The time evolution of the mean value of the overlap ratio is closest to the time evolution of the overlap ratio for "Psychology" and "Physics and Astronomy" disciplines. The Kendall rank correlation coefficients and the overlap ratio decrease nearly linearly over time, except for the first year, where their decay is significantly faster. The analogy between human wealth and the prestige indicators of journals is strengthened by performing a Monte Carlo simulation of the time evolution of the probability distribution function of Scimago Journal Rank. The simulation is based on a simple model of economic systems. Due to the different coverage of the journals in Scimago Journal & Country Rank and Web of Science, the identically defined measures based on the data in these databases are different.

2.
Acta Psychiatr Scand ; 2024 Jul 20.
Article in English | MEDLINE | ID: mdl-39032040

ABSTRACT

OBJECTIVE: The goals of this article are as follows. First, to investigate the possibility of detecting autism spectrum disorder (ASD) from text data using the latest generation of machine learning tools. Second, to compare model performance on two datasets of transcribed statements, collected using two different diagnostic tools. Third, to investigate the feasibility of knowledge transfer between models trained on both datasets and check if data augmentation can help alleviate the problem of a small number of observations. METHOD: We explore two techniques to detect ASD. The first one is based on fine-tuning HerBERT, a BERT-based, monolingual deep transformer neural network. The second one uses the newest, multipurpose text embeddings from OpenAI and a classifier. We apply the methods to two separate datasets of transcribed statements, collected using two different diagnostic tools: thought, language, and communication (TLC) and autism diagnosis observation schedule-2 (ADOS-2). We conducted several cross-dataset experiments in both a zero-shot setting and a setting where models are pretrained on one dataset and then training continues on another to test the possibility of knowledge transfer. RESULTS: Unlike previous studies, the models we tested obtained average results on ADOS-2 data but reached very good performance of the models in TLC. We did not observe any benefits from knowledge transfer between datasets. We observed relatively poor performance of models trained on augmented data and hypothesize that data augmentation by back translation obfuscates autism-specific signals. CONCLUSION: The quality of machine learning models that detect ASD from text data is improving, but model results are dependent on the type of input data or diagnostic tool.

3.
Nutrients ; 16(9)2024 Apr 28.
Article in English | MEDLINE | ID: mdl-38732574

ABSTRACT

"Managing Undernutrition in Pediatric Oncology" is a collaborative consensus statement of the Polish Society for Clinical Nutrition of Children and the Polish Society of Pediatric Oncology and Hematology. The early identification and accurate management of malnutrition in children receiving anticancer treatment are crucial components to integrate into comprehensive medical care. Given the scarcity of high-quality literature on this topic, a consensus statement process was chosen over other approaches, such as guidelines, to provide comprehensive recommendations. Nevertheless, an extensive literature review using the PubMed database was conducted. The following terms, namely pediatric, childhood, cancer, pediatric oncology, malnutrition, undernutrition, refeeding syndrome, nutritional support, and nutrition, were used. The consensus was reached through the Delphi method. Comprehensive recommendations aim to identify malnutrition early in children with cancer and optimize nutritional interventions in this group. The statement underscores the importance of baseline and ongoing assessments of nutritional status and the identification of the risk factors for malnutrition development, and it presents tools that can be used to achieve these goals. This consensus statement establishes a standardized approach to nutritional support, aiming to optimize outcomes in pediatric cancer patients.


Subject(s)
Malnutrition , Neoplasms , Child , Child, Preschool , Humans , Child Nutrition Disorders/therapy , Child Nutrition Disorders/diagnosis , Child Nutrition Disorders/diet therapy , Child Nutrition Disorders/prevention & control , Consensus , Delphi Technique , Malnutrition/diagnosis , Malnutrition/therapy , Malnutrition/etiology , Malnutrition/prevention & control , Medical Oncology/standards , Neoplasms/complications , Neoplasms/therapy , Nutrition Assessment , Nutritional Status , Nutritional Support/methods , Pediatrics/standards , Pediatrics/methods , Poland , Societies, Medical
4.
Cancers (Basel) ; 16(5)2024 Feb 28.
Article in English | MEDLINE | ID: mdl-38473329

ABSTRACT

BACKGROUND/AIM: The role of immune checkpoint inhibitors (ICIs; anti-PD1) in the treatment of childhood cancers is still evolving. The aim of this nationwide retrospective study was to assess the safety and effectiveness of ICIs used in a group of 42 patients, with a median age of 13.6 years, with various types of advanced malignancies treated in pediatric oncology centers in Poland between 2015 and 2023. RESULTS: The indications for treatment with anti-PD1 were as follows: Hodgkin lymphoma (11); malignant skin melanoma (9); neuroblastoma (8); and other malignancies (14). At the end of follow-up, complete remission (CR) was observed in 37.7% (15/42) of children and disease stabilization in 9.5% (4/42), with a mean survival 3.6 (95% CI = 2.6-4.6) years. The best survival (OS = 1.0) was observed in the group of patients with Hodgkin lymphoma. For malignant melanoma of the skin, neuroblastoma, and other rare malignancies, the estimated 3-year OS values were, respectively, 0.78, 0.33, and 0.25 (p = 0.002). The best progression-free survival value (0.78) was observed in the group with malignant melanoma. Significantly better effects of immunotherapy were confirmed in patients ≥ 14 years of age and good overall performance ECOG status. Severe adverse events were observed in 30.9% (13/42) patients.

5.
Pigment Cell Melanoma Res ; 37(4): 453-461, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38509752

ABSTRACT

Pediatric melanomas are rare tumors that have clinical and histological differences from adult melanomas. In adult melanoma, the immunohistochemical marker PRAME is increasingly employed as a diagnostic adjunct. PRAME is also under investigation as a target structure for next-generation immunotherapies including T-cell engagers. Little is known about the characteristics of PRAME expression in pediatric melanoma. In this retrospective study, samples from 25 pediatric melanomas were compared with control groups of melanomas in young adults (18-30 years; n = 32), adult melanoma (>30 years, n = 30), and benign melanocytic nevi in children (0-18 years; n = 30) with regard to the immunohistochemical expression of PRAME (diffuse PRAME expression >75%/absolute expression). Pediatric melanomas show lower diffuse PRAME expression (4%) and lower absolute PRAME expression (25%) compared to young adult melanomas (15.6%/46.8%) and adult melanomas (50%/70%). A significant age-dependent expression could be observed. An analysis of event-free survival shows no prognostic role for PRAME in pediatric melanoma and young adult melanoma, but a significant association with diffuse PRAME expression in adulthood. The age dependency of PRAME expression poses a potential pitfall in the diagnostic application of melanocytic tumors in young patients and may limit therapeutic options within this age group. The immunohistochemical expression of the tumor-associated antigen PRAME is an increasingly important diagnostic marker for melanocytic tumors and is gaining attention as a possible immunotherapeutic target in melanoma. As the available data primarily stem from adult melanoma, and given the clinical and histological distinctions in pediatric melanomas, our understanding of PRAME expression in this specific patient group remains limited. The age-dependent low PRAME expression shown here constrains the use of this marker in pediatric melanoma and may also limit the use of immunotherapeutic strategies against PRAME in young patients.


Subject(s)
Antigens, Neoplasm , Immunohistochemistry , Melanoma , Skin Neoplasms , Humans , Melanoma/pathology , Melanoma/metabolism , Antigens, Neoplasm/metabolism , Adult , Adolescent , Young Adult , Child , Male , Female , Child, Preschool , Skin Neoplasms/pathology , Skin Neoplasms/metabolism , Skin Neoplasms/genetics , Biomarkers, Tumor/metabolism , Infant , Middle Aged , Retrospective Studies , Infant, Newborn , Prognosis , Aged
6.
Chaos ; 33(11)2023 Nov 01.
Article in English | MEDLINE | ID: mdl-37934184

ABSTRACT

Scientific cooperation on an international level has been well studied in the literature. However, much less is known about this cooperation on an intercontinental level. In this paper, we address this issue by creating a collection of approximately 13.8×106 publications around the papers by one of the highly cited authors working in complex networks and their applications. The obtained rank-frequency distribution of the probability of sequences describing continents and the number of countries-with which authors of papers are affiliated-follows the power law with an exponent -1.9108(15). Such dependence is known in the literature as Zipf's law, and it has been originally observed in linguistics; later, it turned out that it is very commonly observed in various fields. The number of distinct "continent (number of countries)" sequences in a function of the number of analyzed papers grows according to power law with exponent 0.527(14); i.e., it follows Heap's law.

7.
EBioMedicine ; 96: 104797, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37716236

ABSTRACT

BACKGROUND: Genomic characterisation has led to an improved understanding of adult melanoma. However, the aetiology of melanoma in children is still unclear and identifying the correct diagnosis and therapeutic strategies remains challenging. METHODS: Exome sequencing of matched tumour-normal pairs from 26 paediatric patients was performed to study the mutational spectrum of melanomas. The cohort was grouped into different categories: spitzoid melanoma (SM), conventional melanoma (CM), and other melanomas (OT). FINDINGS: In all patients with CM (n = 10) germline variants associated with melanoma were found in low to moderate melanoma risk genes: in 8 patients MC1R variants, in 2 patients variants in MITF, PTEN and BRCA2. Somatic BRAF mutations were detected in 60% of CMs, homozygous deletions of CDKN2A in 20%, TERTp mutations in 30%. In the SM group (n = 12), 5 patients carried at least one MC1R variant; somatic BRAF mutations were detected in 8.3%, fusions in 25% of the cases. No SM showed a homozygous CDKN2A deletion nor a TERTp mutation. In 81.8% of the CM/SM cases the UV damage signatures SBS7 and/or DBS1 were detected. The patient with melanoma arising in giant congenital nevus (CNM) demonstrated the characteristic NRAS Q61K mutation. INTERPRETATION: UV-radiation and MC1R germline variants are risk factors in the development of conventional and spitzoid paediatric melanomas. Paediatric CMs share genomic similarities with adult CMs while the SMs differ genetically from the CM group. Consistent genetic characterization of all paediatric melanomas will potentially lead to better subtype differentiation, treatment, and prevention in the future. FUNDING: Found in Acknowledgement.

8.
Int J Mol Sci ; 24(14)2023 Jul 12.
Article in English | MEDLINE | ID: mdl-37511103

ABSTRACT

Extracellular vesicles (EVs) are nanoparticles containing various bioactive cargos-e.g., proteins, RNAs, and lipids-that are released into the environment by all cell types. They are involved in, amongst other functions, intercellular communication. This article presents studies on EVs produced by the probiotic yeast Saccharomyces boulardii CNCM I-745. The size distribution and concentration of EVs in the liquid culture of yeast were estimated. Moreover, the vesicles of S. boulardii were tested for their cytotoxicity against three model human intestinal cell lines. This study did not show any significant negative effect of yeast EVs on these cells under tested conditions. In addition, EVs of S. boulardii were verified for their ability to internalize in vitro with human cells and transfer their cargo. The yeast vesicles were loaded with doxorubicin, an anticancer agent, and added to the cellular cultures. Subsequently, microscopic observations revealed that these EVs transferred the compound to human intestinal cell lines. A cytotoxicity test confirmed the activity of the transferred doxorubicin. Detailed information about the proteins present in EVs might be important in terms of exploring yeast EVs as carriers of active molecules. Thus, proteomic analysis of the EV content was also conducted within the present study, and it allowed the identification of 541 proteins after matching them to the Saccharomyces Genome Database (SGD). Altogether, this study provides strong evidence that the EVs of the probiotic CNCM I-745 strain could be considered a drug delivery system.


Subject(s)
Extracellular Vesicles , Probiotics , Humans , Saccharomyces cerevisiae , Proteomics , Extracellular Vesicles/metabolism , Probiotics/pharmacology , Doxorubicin/pharmacology , Doxorubicin/metabolism
9.
Pharmaceutics ; 15(4)2023 Apr 12.
Article in English | MEDLINE | ID: mdl-37111712

ABSTRACT

Due to epirubicin's (EPI) narrow therapeutic index and risk of cardiotoxicity, it is critical to monitor concentrations of this drug when being used to treat cancer patients. In this study, a simple and fast magnetic solid-phase microextraction (MSPME) protocol for the determination of EPI in plasma and urine samples is developed and tested. Experiments were performed using prepared Fe3O4-based nanoparticles coated with silica and a double-chain surfactant-namely, didodecyldimethylammonium bromide (DDAB)-as a magnetic sorbent. All the prepared samples were analyzed via liquid chromatography coupled with fluorescence detection (LC-FL). The validation parameters indicated good linearity in the range of 0.001-1 µg/mL with a correlation coefficient > 0.9996 for plasma samples, and in the range of 0.001-10 µg/mL with a correlation coefficient > 0.9997 for urine samples. The limit of detection (LOD) and limit of quantification (LOQ) for both matrices were estimated at 0.0005 µg/mL and 0.001 µg/mL, respectively. The analyte recovery after sample pretreatment was 80 ± 5% for the plasma samples and 90 ± 3% for the urine samples. The developed method's applicability for monitoring EPI concentrations was evaluated by employing it to analyze real plasma and urine samples collected from a pediatric cancer patient. The obtained results confirmed the proposed MSPME-based method's usefulness, and enabled the determination of the EPI concentration-time profile in the studied patient. The miniaturization of the sampling procedure, along with the significant reduction in pre-treatment steps, make the proposed protocol a promising alternative to routine approaches to monitoring EPI levels in clinical laboratories.

10.
Pharmaceutics ; 15(4)2023 Apr 17.
Article in English | MEDLINE | ID: mdl-37111740

ABSTRACT

In recent years, therapeutic drug monitoring (TDM) has been applied in docetaxel (DOC)-based anticancer therapy to precisely control various pharmacokinetic parameters, including the concentration of DOC in biofluids (e.g., plasma or urine), its clearance, and its area under the curve (AUC). The ability to determine these values and to monitor DOC levels in biological samples depends on the availability of precise and accurate analytical methods that both enable fast and sensitive analysis and can be implemented in routine clinical practice. This paper presents a new method for isolating DOC from plasma and urine samples based on the coupling of microextraction and advanced liquid chromatography with tandem mass spectrometry (LC-MS/MS). In the proposed method, biological samples are prepared via ultrasound-assisted dispersive liquid-liquid microextraction (UA-DLLME) using ethanol (EtOH) and chloroform (Chl) as the desorption and extraction solvents, respectively. The proposed protocol was fully validated according to the Food and Drug Administration (FDA) and the International Council for Harmonization of Technical Requirements for Pharmaceuticals for Human Use (ICH) requirements. The developed method was then applied to monitor the DOC profile in plasma and urine samples collected from a pediatric patient suffering from cardiac angiosarcoma (AS) with metastasis to lungs and mediastinal lymph nodes, who was receiving treatment with DOC at a dose of 30 mg/m2 body surface area. Due to the rarity of this disease, TDM was carried out to determine the exact levels of DOC at particular time points to ascertain which levels were conducive to maximizing the treatment's effectiveness while minimizing the drug's toxicity. To this end, the concentration-time profiles of DOC in the plasma and urine samples were determined, and the levels of DOC at specific time intervals up to 3 days after administration were measured. The results showed that DOC was present at higher concentrations in the plasma than in the urine samples, which is due to the fact that this drug is primarily metabolized in the liver and then eliminated with the bile. The obtained data provided information about the pharmacokinetic profile of DOC in pediatric patients with cardiac AS, which enabled the dose to be adjusted to achieve the optimal therapeutic regimen. The findings of this work demonstrate that the optimized method can be applied for the routine monitoring of DOC levels in plasma and urine samples as a part of pharmacotherapy in oncological patients.

11.
Open Med (Wars) ; 18(1): 20230652, 2023.
Article in English | MEDLINE | ID: mdl-36874365

ABSTRACT

In recent years, off-label use of sirolimus (SIR) has been gaining attention in the clinical practice. However, since it is critical to achieve and maintain therapeutic blood levels of SIR during treatment, the regular monitoring of this drug in individual patients must be implemented, especially in off-label indications of this drug. In this article, a fast, simple, and reliable analytical method for determining SIR levels in whole blood samples is proposed. Sample preparation based on dispersive liquid-liquid microextraction (DLLME) followed by liquid chromatography-mass spectrometry (LC-MS/MS) was fully optimized toward the analysis of SIR and proposed as a fast, simple, and reliable analytical method for determining the pharmacokinetic profile of SIR in whole-blood samples. In addition, the practical applicability of the proposed DLLME-LC-MS/MS method was evaluated by analyzing the pharmacokinetic profile of SIR in whole blood samples obtained from two pediatric patients suffering from lymphatic anomalies, receiving this drug as off-label clinical indication. The proposed methodology can be successfully applied in routine clinical practice for the fast and precise assessment of SIR levels in biological samples, thus allowing SIR dosages to be adjusted in real time during pharmacotherapy. Moreover, the measured SIR levels in the patients indicate the need for monitoring between doses to ensure the optimal pharmacotherapy of patients.

12.
Arch Med Sci ; 18(5): 1253-1261, 2022.
Article in English | MEDLINE | ID: mdl-36160344

ABSTRACT

Introduction: Markers of inflammation such as neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and lymphocyte-to-monocyte ratio (LMR) have been found to be associated with survival in cancer patients. The aim of the current study was to establish the prognostic significance of simple laboratory markers of systemic inflammation in paediatric patients diagnosed with Wilms tumour (WT). Additionally, we aimed to compare the complete blood count (CBC) parameters of WT patients and the non-oncological control group. Material and methods: The study group included 88 children diagnosed with WT. Clinicopathological data, as well as CBC, C-reactive protein (CRP) and lactate dehydrogenase (LDH) levels at diagnosis, were obtained. Additionally, the laboratory results of 62 healthy control paediatric patients were collected. Uni- and multivariate proportional Cox's hazard analyses were computed to create a model predicting relapse-free survival (RFS) and overall survival (OS) in the study group. Results: High CRP, LDH, and NLR were associated with a higher stage of WT and shorter RFS, whereas all parameters correlated with OS. In multivariate analysis, only LDH levels had adverse significance in predicting RFS. C-reactive protein and LMR retained their prognostic value in the multivariate model predicting OS. Comparing the WT group with controls, high LDH, high CRP, high NLR, and high PLR were associated with WT presence. Conclusions: Preoperative LDH, CRP, NLR, PLR, and LMR have significant prognostic value in patients with WT independently of age and stage. Combined low CRP and high LMR identified the group of patients with excellent OS. Patients with high LDH were characterized by the highest risk of relapse.

13.
Eur J Cancer ; 175: 19-30, 2022 11.
Article in English | MEDLINE | ID: mdl-36087394

ABSTRACT

BACKGROUND: Primary lung carcinoma is an exceptionally rare childhood tumour, as per definition of the European Cooperative Study Group on Paediatric Rare Tumours (EXPeRT), with an incidence of 0.1-0.2/1,000,000 per year. Little is known about the clinical characteristics of children with primary lung carcinoma, a gap which this joint analysis of the EXPeRT group aimed to fill. PATIENTS AND METHODS: We performed a retrospective case series of children (aged 0-18 years) with primary lung carcinoma, as collected through the EXPeRT databases between 2000 and 2021. We recorded relevant clinical characteristics including treatment and outcome. RESULTS: Thirty-eight patients were identified with a median age of 12.8 years at diagnosis (range: 0-17). Mucoepidermoid carcinoma (MEC) was the most frequent entity (n = 20), followed by adenocarcinoma (n = 12), squamous cell carcinoma (n = 4), adenosquamous carcinoma (n = 1) and small-cell lung cancer (n = 1). Patients with MEC presented rarely with lymph node metastases (2/20 cases). Overall, 19/20 patients achieved long-lasting remission by surgical resection only. Patients with other histologies often presented in advanced stages (14/18 TNM stage IV). With multimodal treatment, 3-year overall survival was 52% ± 13%. While all patients with squamous cell carcinoma died, the 12 patients with adenocarcinoma had a 3-year overall survival of 64% ± 15%. CONCLUSIONS: Primary lung carcinomas rarely occur in children. While the outcome of children with MEC is favourable with surgery alone, patients with other histotypes have a poor prognosis, despite aggressive treatment, highlighting the need to develop new strategies for these children, such as mutation-guided treatment.


Subject(s)
Adenocarcinoma , Carcinoma, Adenosquamous , Carcinoma, Mucoepidermoid , Carcinoma, Squamous Cell , Adenocarcinoma/pathology , Adolescent , Carcinoma, Mucoepidermoid/pathology , Carcinoma, Squamous Cell/pathology , Child , Humans , Lung/pathology , Retrospective Studies , Survival Rate , Syndrome
14.
Phys Rev E ; 105(5-1): 054312, 2022 May.
Article in English | MEDLINE | ID: mdl-35706162

ABSTRACT

We discuss a cellular automaton simulating the process of reaching Heider balance in a fully connected network. The dynamics of the automaton is defined by a deterministic, synchronous, and global update rule. The dynamics has a very rich spectrum of attractors including fixed points and limit cycles, the length and number of which change with the size of the system. In this paper we concentrate on a class of limit cycles that preserve energy spectrum of the consecutive states. We call such limit cycles perfect. Consecutive states in a perfect cycle are separated from each other by the same Hamming distance. Also the Hamming distance between any two states separated by k steps in a perfect cycle is the same for all such pairs of states. The states of a perfect cycle form a very symmetric trajectory in the configuration space. We argue that the symmetry of the trajectories is rooted in the permutation symmetry of vertices of the network and a local symmetry of a certain energy function measuring the level of balance and frustration of triads.

15.
Cancers (Basel) ; 14(6)2022 Mar 10.
Article in English | MEDLINE | ID: mdl-35326572

ABSTRACT

An effective therapy for advanced melanoma, a skin cancer with the highest mortality, has not yet been developed. The endocannabinoid system is considered to be an attractive target for cancer treatment. The use of endocannabinoids, such as anandamide (AEA), is considered to be much greater than as a palliative agent. Thus, we checked its influence on various signaling pathways in melanoma cells. Our investigation was performed on four commercial cell lines derived from different progression stages (radial WM35 and vertical WM115 growth phases, lymph node WM266-4 metastasis, solid tumor A375-P metastasis). Cell viability, glucose uptake, quantification of reactive oxygen species production, expression of selected genes encoding glycosyltransferases, quantification of glycoproteins production and changes in the glycosylation profile and migration, as well as in cell elastic properties were analyzed. The cell glycosylation profile was investigated using the biophysical profiling method-the quartz crystal microbalance with dissipation monitoring (QCM-D). Anandamide treatment of only metastatic cells resulted in: an increase in the cell metabolism, a decrease in GFAT-1 and DPM1 expression, followed by a decrease in L1-CAM glycoprotein production, which further influenced the reduction in the cell glycosylation profile and migration. Considering our results, AEA usage is highly recommended in the combined therapy of advanced melanoma.

16.
Radiologe ; 62(4): 316-321, 2022 Apr.
Article in German | MEDLINE | ID: mdl-35290492

ABSTRACT

BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a rare demyelinating disease that occurs predominantly in children. According to the guidelines, ADEM belongs to the myelin oligodendrocyte glycoprotein (MOG)-associated diseases and usually manifests after febrile infections (also after SARS-CoV-2) or postvaccinally. OBJECTIVES: Incidence, course and clinical, and as well, as radiological features and new developments and treatment of ADEM. METHODS: Analysis and review of the literature on ADEM and of notable cases and guidelines. RESULTS: The first signs of ADEM include fever, nausea and vomiting, headache and meningism as well as, by definition, encephalopathy, which usually manifests as drowsiness and confusion. The radiological diagnosis is made by magnetic resonance imaging (MRI). Here, the asymmetrically distributed, diffuse and tumefactive lesions can be located supra- and infratentorially. In the acute phase, the lesions usually show contrast enhancement and restricted diffusion. Spinal involvement of the gray matter with the typical H­pattern with myelitis transversa is not uncommon. ADEM has mostly a monophasic course, with a recurrent form ("relapsing ADEM") in 1-20% of cases. For treatment, steroids and in severe cases immunosuppressive drugs are used. CONCLUSIONS: ADEM is generally a monophasic disease whose symptoms usually last for a few weeks or months. It is crucial to differentiate ADEM from other demyelinating diseases, like for example multiple sclerosis, in order not to delay the proper treatment.


Subject(s)
Encephalomyelitis, Acute Disseminated , Encephalomyelitis, Acute Disseminated/diagnostic imaging , Encephalomyelitis, Acute Disseminated/therapy , Humans , Magnetic Resonance Imaging , Myelin-Oligodendrocyte Glycoprotein/therapeutic use
17.
J Clin Res Pediatr Endocrinol ; 14(2): 251-257, 2022 06 07.
Article in English | MEDLINE | ID: mdl-34544220

ABSTRACT

Nijmegen breakage syndrome (NBS) is a rare autosomal recessive disease, affecting mainly patients of Slavic origin. It is caused by a defect in the NBN gene, resulting in defective nibrin protein formation. This leads to chromosomal instability, which predisposes to cancer, with lymphoid malignancies predominating. Nibrin is also involved in gonadal development and its disfunction in females with NBS frequently results in a pure gonadal dysgenesis (PGD) causing hypergonadotropic hypogonadism. However, only a few ovarian tumors in NBS patients have been reported to date. We describe the first case of a girl with NBS with PGD, who developed metachronous bilateral ovarian germ cell tumors (dysgerminoma and gonadoblastoma). Pathogenesis of PGD, neoplastic transformation and therapeutic approach in females with NBS are discussed.


Subject(s)
Gonadal Dysgenesis , Gonadoblastoma , Hypogonadism , Nijmegen Breakage Syndrome , Ovarian Neoplasms , Female , Gonadal Dysgenesis/complications , Gonadal Dysgenesis/genetics , Gonadoblastoma/complications , Gonadoblastoma/genetics , Humans , Hypogonadism/genetics , Nijmegen Breakage Syndrome/complications , Nijmegen Breakage Syndrome/diagnosis , Nijmegen Breakage Syndrome/genetics , Ovarian Neoplasms/complications , Ovarian Neoplasms/genetics
18.
Diagnostics (Basel) ; 11(12)2021 Dec 14.
Article in English | MEDLINE | ID: mdl-34943596

ABSTRACT

We performed a multi-institutional analysis of 74 children with ependymoma to evaluate to what extent the clinical outcome of prospective trials could be reproduced in routine practice. The evaluation of factors that correlated with outcome was performed with a log rank test and a Cox proportional-hazard model. Survival was estimated with the Kaplan-Meier method. The majority of patients had brain tumours (89%). All had surgery as primary treatment, with adjuvant radiotherapy (RTH) and chemotherapy (CTH) applied in 78% and 57%, respectively. Median follow-up was 80 months and 18 patients died. Five- and 10-year overall survival (OS) was 83% and 73%. Progression was observed in 32 patients, with local recurrence in 28 cases. The presence of metastases was a negative prognostic factor for OS. Five- and 10-year progression-free survival (PFS) was 55% and 40%, respectively. The best outcome in patients with non-disseminated brain tumours was observed when surgery was followed by RTH (+/-CTH afterwards; p = 0.0001). Children under 3 years old who received RTH in primary therapy had better PFS (p = 0.010). The best outcome of children with ependymoma is observed in patients who received radical surgery followed by RTH, and irradiation should not be omitted in younger patients. The role of CTH remains debatable.

19.
Entropy (Basel) ; 23(11)2021 Oct 28.
Article in English | MEDLINE | ID: mdl-34828116

ABSTRACT

The concept of Heider balance, usually applied to interpersonal relations, is generalized here to opinions gathered in surveys. At first, we compare four algorithms, which drive a matrix dataset to a balanced state. The criterion is that the final state obtained with an algorithm should be as close as possible to the initial state. The result is that deterministic differential equations work better than their Monte Carlo counterparts. Next, we apply the winning algorithms to the matrix of correlations between opinions gathered in American states between 1974 and 1998. The results are interpreted in terms of the classic comfort hypothesis (E. Babbie, 2007).

20.
Entropy (Basel) ; 23(10)2021 Oct 14.
Article in English | MEDLINE | ID: mdl-34682069

ABSTRACT

The main goal of our work is to show how ideas change in social networks. Our analysis is based on three concepts: (i) temporal networks, (ii) the Axelrod model of culture dissemination, (iii) the garbage can model of organizational choice. The use of the concept of temporal networks allows us to show the dynamics of ideas spreading processes in networks, thanks to the analysis of contacts between agents in networks. The Axelrod culture dissemination model allows us to use the importance of cooperative behavior for the dynamics of ideas disseminated in networks. In the third model decisions on solutions of problems are made as an outcome of sequences of pseudorandom numbers. The origin of this model is the Herbert Simon's view on bounded rationality. In the Axelrod model, ideas are conveyed by strings of symbols. The outcome of the model should be the diversity of evolving ideas as dependent on the chain length, on the number of possible values of symbols and on the threshold value of Hamming distance which enables the combination.

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