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The study aimed to assess loneliness experiences among athletes with disabilities (AWD) during the different phases of the pandemic and to understand its relationship with coping styles. Ninety-one AWD participated in a longitudinal study spanning 10 months, covering three time points: April (A), June (Time B), and November (Time C) 2021. The study used the Coping Inventory for Stressful Situations and the revised University of California Los Angeles Loneliness Scale-Revised (UCLA-R) to measure coping mechanisms and feelings of loneliness, respectively. Loneliness levels peaked during the most intense phase of the pandemic, showing significant differences between consecutive time points (χ2 = 20.29, p < 0.001, d = 0.24). The most robust regression models were built in Time B, using the "intimate contacts" dimension of loneliness as the dependent variable and "emotion-oriented coping", "avoidance-oriented coping", and "impairment" as independent variables, explaining 44% of the variance (p < 0.001). Loneliness among Paralympic athletes fluctuated during various phases of the pandemic. The findings highlight the significant influence of coping styles, particularly emotional styles which heightened perceived loneliness, and task-oriented styles which reduced it, on athletes' experiences of loneliness. These results underscore the need for longitudinal studies to delve deeper into the relationship between loneliness and mental health. Moreover, they stress the importance of developing tailored interventions that promote effective coping mechanisms in AWD during challenging times amid to COIVID-19.
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Adaptación Psicológica , COVID-19 , Soledad , Humanos , COVID-19/psicología , Soledad/psicología , Masculino , Femenino , Estudios Longitudinales , Adulto , Personas con Discapacidad/psicología , Atletas/psicología , SARS-CoV-2 , Adulto Joven , Pandemias , Deportes para Personas con Discapacidad/psicología , Paratletas/psicologíaRESUMEN
INTRODUCTION: Hereditary factor XI (FXI) deficiency is a rare coagulation disorder that may result in excessive bleeding requiring intervention to restore haemostasis. AIM: The aim of this review was to report the current knowledge of the worldwide incidence and prevalence of FXI deficiency. METHODS: A targeted PubMed search using terms related to FXI deficiency was conducted to identify studies published from April 2002 through April 2022. A manual search supplemented the electronic search. Studies were eligible for data abstraction if they reported population-based incidence proportions/rates or prevalence proportions for FXI deficiency. RESULTS: The electronic and manual searches returned 253 publications. After applying exclusion criteria, seven publications were included in the analysis, including a global report from the World Federation of Haemophilia (WFH). Six publications provided information on the prevalence of FXI deficiency that included 74 countries and regions. The estimated prevalence of FXI in the WFH report ranged from 0/100,000 in several countries to 55.85/100,000 individuals in the United Kingdom. Prevalence estimates in the PubMed findings ranged from .1 to 246.2/1,000,000 inhabitants with varying methods of case identification and time periods of analysis. One study estimated the incidence of FXI deficiency in Yecla, Spain at 2% of blood donors and .09% of hospital inpatients/outpatients with activated partial thromboplastin time (aPTT) tests. CONCLUSION: FXI deficiency is rare across the world, but additional steps could be taken to improve incidence and prevalence estimation, for example, development of a consistent FXI deficiency definition and incorporating genetic testing into a clinical routine to better identify and characterise cases.
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Deficiencia del Factor XI , Humanos , Coagulación Sanguínea , Factor XI/genética , Deficiencia del Factor XI/epidemiología , Deficiencia del Factor XI/genética , Hemorragia , Tiempo de Tromboplastina Parcial , InternacionalidadRESUMEN
INTRODUCTION: Recent technological innovations in haemophilia have advanced at an astounding pace, including gene therapy programmes and bioengineered molecules for prophylaxis, products that reduce treatment burden through half-life extension, unique mechanisms of action, and subcutaneous administration. Additional technological advancements have emerged that are anticipated to further transform haemophilia care. AIM: Review new and emerging haemophilia therapies, including replacement and bypassing products, digital applications, utilisation of big data, and personalised medicine. METHODS: Data were obtained from peer-reviewed presentations/publications, and ongoing studies in haemophilia, ultrasonography, and artificial intelligence (AI). RESULTS: Available treatments include new recombinant factors VIII (FVIII) and IX (FIX), extended half-life FVIII/IX products, a new FVIIa product for inhibitor patients, and a FVIIIa-mimetic. Several novel therapeutics are in clinical trials, including FVIIIa mimetics and inhibitors of naturally-occurring anticoagulants. Ongoing gene therapy trials suggest that a single vector infusion using an optimised construct can produce factor activity that reduces bleeding to near zero for years. Today, persons with haemophilia (PwH) approach a lifespan comparable to that of the general population, presenting treatment challenges for age-related co-morbidities. Technological innovations have broadened beyond therapeutics to include large database analyses utilising remote data collection with handheld devices, and to tailor AI applications. Current development efforts include patient-performed ultrasonography, algorithms for scan interpretation, and point-of-care haemostatic testing devices. CONCLUSIONS: We have entered a golden age for haemophilia treatment and care with wide-ranging advancements targeting improved quality of life (QoL). Future-focused efforts by clinical and patient communities may provide equitable access and care for people impacted by haemophilia worldwide.
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Hemofilia A , Inteligencia Artificial , Factor IX/uso terapéutico , Factor VIII/uso terapéutico , Semivida , Hemofilia A/tratamiento farmacológico , Humanos , Calidad de VidaRESUMEN
INTRODUCTION: Surgery is frequently required in persons with haemophilia A (PwHA). Emicizumab, a bispecific, humanized monoclonal antibody, bridges activated factor (F) IX and FX. Management of patients undergoing surgery while receiving emicizumab is of clinical interest due to paucity of data. AIM: Review real-world experience of PwHA with/without FVIII inhibitors who required surgery while receiving emicizumab prophylaxis. METHODS: Data regarding peri-operative management, including type of surgery, haemostatic agent use and bleeding complications, were collected for PwHA receiving emicizumab undergoing surgery between 25/10/18 and 31/12/19 at the Indiana Hemophilia and Thrombosis Center. Analyses were exploratory and descriptive. RESULTS: Twenty minor and five major surgeries were performed in 17 and five patients, respectively. Overall, 9/20 minor surgeries were planned to occur with emicizumab as the sole haemostatic agent; of these, four required additional coagulation factor (2 due to haematomas following port removals, 1 due to oozing at port removal site, 1 due to bleeding following squamous cell carcinoma removal). Three of the 11 minor surgeries with planned additional coagulation factor resulted in non-major bleeds; all were safely managed with additional coagulation factor. All five major surgeries were planned with additional haemostatic agents; there was 1 bleed in a patient undergoing elbow synovectomy with nerve transposition, likely triggered by physical/occupational therapy. There were no major bleeds, thrombotic events or deaths. CONCLUSIONS: Additional haemostatic agent use is safe in PwHA undergoing surgery while receiving emicizumab. Additional data are needed to determine the optimal dosing/length of treatment of additional haemostatic agents to lower bleeding risk.
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Anticuerpos Biespecíficos , Hemofilia A , Anticuerpos Biespecíficos/uso terapéutico , Anticuerpos Monoclonales Humanizados/uso terapéutico , Factores de Coagulación Sanguínea , Factor VIII/uso terapéutico , Hemofilia A/complicaciones , Hemofilia A/tratamiento farmacológico , HumanosRESUMEN
Glioblastoma (GBM) is the most common and most aggressive primary tumor of the central nervous system. Current GBM treatments have low effectiveness. This is mainly due to the high degree of heterogeneity of GBM tumors. Despite similarities in the classic microscopic image, these tumors differ significantly in molecular terms. The aim of the study was to classify GBM tumors into one of four molecular types based on the immunohistochemical expression of EGFR, PDGFRA, NF1, IDH1, p53 and PTEN proteins and find the association between individual glioma molecular types and prognostic clinical and morphological parameters. From the group of 162 patients the classical molecular type of tumor was observed in 17 (10%) patients, in 23 (14%) the tumor was mesenchymal, in 32 (20%) proneural, and in 90 (56%) neural. No significant relationship was observed between the molecular type of GBM tumors and the studied clinical and morphological parameters of prognostic significance. There were also no statistically significant correlations between the GBM tumor molecular type and survival, both in terms of overall survival and relapse-free survival. Analyzing the impact of all prognostic variables and molecular type of GBM on the probability of overall survival, statistically significant relationships were found.
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Neoplasias Encefálicas , Glioblastoma , Neoplasias Encefálicas/genética , Receptores ErbB/genética , Receptores ErbB/metabolismo , Glioblastoma/genética , Humanos , Isocitrato Deshidrogenasa/genética , Fosfohidrolasa PTEN/genética , Proteína p53 Supresora de Tumor/genéticaRESUMEN
The aim of this study was to assess the: 1) clinicopathologic features of papillary early gastric cancer (PEGC) (13 cases) compared to tubular early gastric cancer (TEGC) (41 cases); 2) efficiency of endoscopic submucosal dissection (ESD) in treatment of PEGC. From January 2007 to February 2016, a total of 54 consecutive patients with early gastric cancer (EGC) underwent ESD at the Department of Gastroenterology of the Pomeranian Medical University in Poland. The histologic type of carcinoma was assessed according to the WHO histological classification of GC. The extension of GC into the submucosa was measured using the Aperio Scan Scope image analysis system tools. PEGCs were diagnosed in 24.1% of the cases of EGC. PEGCs were significantly more elevated in macroscopic examination and better demarcated tumors than TEGC. There were no significant differences between gender, tumor location, ulceration, tumor size, depth of invasion (T), presence of intestinal metaplasia and lymphocytic infiltrate. Curative resection was achieved in 87.1% of patients with EGCs treated with ESD. The lower rate of curative resection (R0) observed in PEGC (76.9%) vs TEGC (90.2%) was not statistically significant. Further studies will be necessary to confirm the clinical and morphological presentation of PEGCs.
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Adenocarcinoma/patología , Neoplasias Gástricas/patología , Adulto , Anciano , Anciano de 80 o más Años , Resección Endoscópica de la Mucosa , Femenino , Humanos , Masculino , Persona de Mediana EdadRESUMEN
OBJECTIVE: Early gastric cancer (EGC) is defined as cancer invasion confined to the mucosa or submucosa, irrespective of lymph node metastasis. Recently endoscopic submucosal dissection (ESD) has been widely accepted for the treatment for dysplasia and EGC without lymph node metastases. While the method has been advanced in Far East countries, ESD is still being developed in Europe and has not gained enough popularity although it has been recommended as the treatment of choice for superficial gastric neoplastic lesions by European Society of Gastrointestinal Endoscopy (ESGE) in 2015. METHODS: The aim of the study was to perform a retrospective analysis of clinical and histomorphologic features of 58 cases of EGCs removed by ESD in a university hospital in Western Pomerania in Poland and to evaluate factors related to the efficiency of ESD resection. RESULTS: With univariate analysis, indications for ESD with the highest R0 rate were found in EGCs limited to mucosa (T1a, small mucosal, M), without muscularis mucosa invasion, localised in the middle/lower part of stomach and intestinal type in histological examination. The R0 complete resection rate was significantly (p < 0.0001) lower for T1b than that for T1a tumours (21.4% vs. 100%). Tumours with submucosal involvement were associated with lower efficiency of ESD procedure. CONCLUSIONS: Our data showed that in EGCs with favourable histomorphologic characteristics, ESD seemed to be a totally efficient and safe method of treatment in a European small-volume centre. R0 resection rate reached 81.1% of cases and median time of the ESD procedure was 61.5 min.
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Adenocarcinoma/patología , Adenocarcinoma/cirugía , Resección Endoscópica de la Mucosa/métodos , Neoplasias Gástricas/patología , Neoplasias Gástricas/cirugía , Anciano , Anciano de 80 o más Años , Disección/métodos , Resección Endoscópica de la Mucosa/efectos adversos , Femenino , Mucosa Gástrica/patología , Humanos , Metástasis Linfática/patología , Persona de Mediana Edad , Polonia , Estudios RetrospectivosRESUMEN
BACKGROUND Mutations in DNA of mismatch repair (MMR) genes result in failure to repair errors that occur during DNA replication in microsatellites, resulting in accumulation of frameshift mutations in these genes and leading to DNA mismatch replication errors and microsatellite instability. Gastric cancers (GCs) with high MSI (MSI-H) are a well-defined subset of carcinomas showing distinctive clinicopathological features. In this study we investigated the rate of MSI and the correlation between MSI status and clinicopathological features of GC. MATERIAL AND METHODS The study included 107 patients with GCs: 61 with advanced gastric cancers (AGC) and 46 with early gastric cancer (EGC). MSI deficiency in GCs was assessed by the immunohistochemical analysis of expression of MMR proteins - MLH1, MSH2, MSH6, and PMS2 - using formalin-fixed and paraffin-embedded tissue. RESULTS A total of 6 (5.6%) MSI-H were observed. The loss of MMR proteins expression was associated with the intestinal type of GC in Lauren classification, and tubular and papillary architecture in WHO classification. There was no statistically significant association between negative MMR expression and other selected clinical parameters: age, sex, tumor location, depth of invasion (EGC and AGC), lymph nodes status, presence of the ulceration, and lymphocytic infiltrate. CONCLUSIONS In the present era of personalized medicine, the histological type of GC and MMR proteins status in cancer cells are very important for the proper surveillance of patients with familial GC and sporadic GCs, as well as for selecting the proper follow-up and treatment. Larger collaborative studies are needed to verify the features of MSI-H GCs in Poland.
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Reparación de la Incompatibilidad de ADN , Inestabilidad de Microsatélites , Neoplasias Gástricas/genética , Anciano , Proteínas de Unión al ADN/genética , Proteínas de Unión al ADN/metabolismo , Femenino , Regulación Neoplásica de la Expresión Génica , Humanos , Inmunohistoquímica , Masculino , Persona de Mediana Edad , Endonucleasa PMS2 de Reparación del Emparejamiento Incorrecto/genética , Endonucleasa PMS2 de Reparación del Emparejamiento Incorrecto/metabolismo , Homólogo 1 de la Proteína MutL/genética , Homólogo 1 de la Proteína MutL/metabolismo , Proteína 2 Homóloga a MutS/genética , Proteína 2 Homóloga a MutS/metabolismo , Polonia , Neoplasias Gástricas/metabolismo , Transcriptoma/genéticaRESUMEN
INTRODUCTION: Neuroblastic tumors can be characterized by three features: spontaneous regression, maturation and aggressive proliferation. The most common and routinely used method of assessing tumor cell proliferation is to determine the Ki-67 index in the tumor tissue. Despite numerous studies, neuroblastoma biology is not fully understood, which makes treatment results unsatisfactory. MCM 2 is a potential prognostic factor in the neuroblastoma group. MATERIAL/METHODS: The study is based on retrospective analysis of 35 patients treated for neuroblastic tumors in the Department of Pediatric Surgery and Oncology of the Medical University of Lodz, during the period 2001-2011. The material comprised tissues of 16 tumors excised during the operation and 19 biopsy specimens. Immunohistochemical examinations were performed with immunoperoxidase using mouse monoclonal anti-MCM 2 and anti-Ki-67 antibodies. RESULTS: We observed that MCM 2 expression ranged from 2% to 98% and the Ki-67 index ranged from 0 to 95%. There was a statistically significant correlation between expression of MCM 2 and the value of the Ki-67 index and a correlation close to statistical significance between expression of MCM 2 and unfavorable histopathology. There was no statistical relationship between expression of MCM 2 and age over 1 year and N-myc amplification. DISCUSSION: The presented research shows that MCM 2 may have prognostic significance in neuroblastic pediatric tumors and as a potential prognostic factor could be the starting point of new individualized therapy.
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Biomarcadores de Tumor/análisis , Antígeno Ki-67/análisis , Componente 2 del Complejo de Mantenimiento de Minicromosoma/análisis , Neuroblastoma/diagnóstico , Adolescente , Animales , Niño , Preescolar , Femenino , Humanos , Inmunohistoquímica , Lactante , Recién Nacido , Masculino , Neuroblastoma/metabolismo , Pronóstico , Estudios RetrospectivosRESUMEN
Angiosarcoma is a rare form of sarcoma which may be either a primary tumor or it may result from previous irradiation because of another tumor. In this paper, we present a case of a female patient diagnosed as having peritoneal disseminated angiosarcoma 20 years after ovarian cancer treatment (surgery, chemotherapy and radiotherapy). The case was very atypical because of an extremely rare peritoneal location and disseminated nature of the changes. Based on the initial histological picture, poorly differentiated cancer metastasis was diagnosed, suggesting a recurrence of the ovarian cancer that had been diagnosed earlier. The time elapsed from the ovarian cancer diagnosis, history of the previous irradiation and concentration of tumor markers were the only additional clinical data provided to the pathologists, which ultimately contributed to a correct diagnosis. The case we present herein shows and emphasizes the importance of proper communication between a clinician and a pathologist, which is a prerequisite for a correct diagnosis and, consequently, for proper treatment of patients. It also confirms the high specificity of the HE4 (human epididymis protein 4) marker in the monitoring of ovarian cancer, which was within normal limits in spite of peritoneal tumor dissemination.
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Hemangiosarcoma/diagnóstico , Recurrencia Local de Neoplasia/diagnóstico , Neoplasias Ováricas/diagnóstico , Neoplasias Peritoneales/diagnóstico , Antineoplásicos/uso terapéutico , Biomarcadores de Tumor/sangre , Diagnóstico Diferencial , Resultado Fatal , Femenino , Hemangiosarcoma/tratamiento farmacológico , Hemangiosarcoma/patología , Humanos , Inmunohistoquímica , Persona de Mediana Edad , Recurrencia Local de Neoplasia/patología , Neoplasias Ováricas/patología , Neoplasias Ováricas/radioterapia , Neoplasias Peritoneales/tratamiento farmacológico , Neoplasias Peritoneales/patología , Proteínas/metabolismo , Tomografía Computarizada por Rayos X , Proteína 2 de Dominio del Núcleo de Cuatro Disulfuros WAPRESUMEN
This study aimed to adapt the Multisource Assessment of Social Competence Scale for the Polish population. The population examined in the study included only Polish participants of European (Caucasian) ancestry. The tool is composed of two scales, Prosocial and Antisocial, and four subscales. For the purpose of adaptation, children's social competence was evaluated based on their own and their parents' perspective. The sample consisted of children aged 9-15 (n = 253) and their parents (n = 248), with boys and girls accounting for 43% (n = 109) and 57% (n = 144) of child participants, respectively. All the participants originated from the western-central Greater Poland Voivodeship. The data analyzed in this study were collected in 2019. Internal consistency of the subscales and correlation between them were measured using Cronbach's Alpha reliability coefficient and Spearman's correlation coefficient, respectively. Confirmatory factor analysis was conducted for both groups (children and parents) in two-, three- and four-factor models. The confirmatory factor analysis for both groups (children and parents) attributed the four-factor model with the highest goodness-of-fit, fulfilling the criteria of a good-fitting model. The results show that the Multisource Assessment of Children's Social Competence Scale is an appropriately adapted tool for the evaluation of the social competence of children in Poland, taking different rater perspectives into account.
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Padres , Habilidades Sociales , Masculino , Femenino , Humanos , Niño , Polonia , Reproducibilidad de los Resultados , Encuestas y Cuestionarios , PsicometríaRESUMEN
Small cell cancer (SCC) is a neuroendocrine neoplasm, which is most frequently found in the lungs. Extrapulmonary location of SCC is rare and may involve 2.5-5% of SCCs. We present a case of a 31-year-old male patient with an extremely uncommon subglottic SCC. The patient was qualified for a radical sequential chemoradiotherapy. After treatment, patient's condition suggested complete remission. Recurrence was detected one year later, and the disease rapidly progressed, despite a second line chemotherapy. The patient died 29 months after initial diagnosis. This case aims to raise awareness on the aggressive laryngeal SCC and its good response to first line chemotherapy composed of cisplatin and etoposide, followed by radiotherapy.
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BACKGROUND: It has been speculated that higher concentrations of 25-hydroxy-vitamin D (25OHD) provide some protection against COVID-19. We assessed whether there is any relationship between 25OHD concentrations and the subsequent development of COVID-19 infection. MATERIALS AND METHODS: Concentrations of 25OHD were measured in March-April 2020 in 134 healthy subjects (57 males), age range 6-50, from a single urban general practice in central Poland. Data on COVID-19 infection during the subsequent 12 months (prior to the vaccination program) were obtained from the national database of COVID-19 cases. None of the subjects received any 25OHD supplements. RESULTS: The average 25OHD concentrations were 18.1 ± 7.39 ng/mL (37.3% had 25OHD above 20 ng/mL). Thirty-one (23.1%) patients developed COVID-19 infection, but an increased risk was only observed in individuals with 25OHD concentrations below 12 ng/mL (COVID-19 infection in 11 out of 25 patients (44%) with 25OHD < 12 ng/mL versus 20 out of 109 (18.3%) for those with 25OHD above 12 ng/mL, p = 0.0063). Such a relationship was no longer observed for subjects with 25OHD concentrations above 20 ng/mL (p = 0.2787). CONCLUSIONS: Although only a minority of healthy subjects had 25OHD concentrations above 20 ng/mL in spring, an increased risk of subsequent COVID-19 infection was only observed in those with severe 25OHD deficiency (<12 ng/mL).
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COVID-19 , Deficiencia de Vitamina D , Masculino , Humanos , Niño , Adolescente , Adulto Joven , Adulto , Persona de Mediana Edad , Deficiencia de Vitamina D/epidemiología , COVID-19/epidemiología , Vitamina D , Vitaminas , Suplementos DietéticosRESUMEN
Apolipoprotein B (apoB)-lipoproteins initiate and promote atherosclerotic cardiovascular disease. Plasma tissue plasminogen activator (tPA) activity is negatively associated with atherogenic apoB-lipoprotein cholesterol levels in humans, but the mechanisms are unknown. We found that tPA, partially through the lysine-binding site on its Kringle 2 domain, binds to the N terminus of apoB, blocking the interaction between apoB and microsomal triglyceride transfer protein (MTP) in hepatocytes, thereby reducing very-low-density lipoprotein (VLDL) assembly and plasma apoB-lipoprotein cholesterol levels. Plasminogen activator inhibitor 1 (PAI-1) sequesters tPA away from apoB and increases VLDL assembly. Humans with PAI-1 deficiency have smaller VLDL particles and lower plasma levels of apoB-lipoprotein cholesterol. These results suggest a mechanism that fine-tunes VLDL assembly by intracellular interactions among tPA, PAI-1, and apoB in hepatocytes.
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Apolipoproteínas B , Aterosclerosis , Hepatocitos , Lipoproteínas VLDL , Inhibidor 1 de Activador Plasminogénico , Activador de Tejido Plasminógeno , Humanos , Apolipoproteínas B/sangre , Aterosclerosis/sangre , Aterosclerosis/metabolismo , Hepatocitos/metabolismo , Lipoproteínas VLDL/metabolismo , Inhibidor 1 de Activador Plasminogénico/sangre , Inhibidor 1 de Activador Plasminogénico/metabolismo , Activador de Tejido Plasminógeno/metabolismo , Animales , Ratones , Ratones Endogámicos C57BLRESUMEN
INTRODUCTION: The regulatory approval of medical products in countries with limited regulatory resources can be lengthy, which often compromises patients' timely access to much-needed medicines. To improve the efficiency of regulatory systems, reliance is being used. Reliance allows an authority to leverage the work performed by other authorities, such as scientific evaluations, to decide on medical products approval within their jurisdiction. This reduces duplication of regulatory efforts, resources and time, while maintaining national sovereignty. AREAS COVERED: This article analyzes the outcomes and stakeholders' experience of using medicines assessments performed by Stringent Regulatory Authorities (SRA) in the Collaborative Registration Procedures (CRP). Since its establishment in 2015, 59 approvals were granted to 16 medicines in 23 countries through SRA CRP. Results show that the procedure is delivering on the intended benefits of access and speed, with long-term positive impact for resource-limited countries. The article concludes with recommendations on the need for guidance on management of post-approval changes, wider promotion of the procedure, and increased collaboration between authorities. EXPERT OPINION: The SRA CRP provides a mechanism for the use of reliance by strengthening communication and promoting the exchange of information among regulators. This fosters faster regulatory approvals and, consequently, earlier access to medicines.
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Organización Mundial de la Salud , HumanosRESUMEN
Background: Raised parathormone (PTH) and normal calcium concentrations can be observed both in normocalcemic primary hyperparathyroidism (nPHPT) and in secondary hyperparathyroidism, e.g. due to vitamin D deficiency. We assessed the impact of season on the validity of diagnosis of nPHPT in terms of screening investigations to be performed in the primary care settings. Material and methods: On two occasions (March/April & September/October) we measured vitamin D (25OHD), PTH and total calcium in 125 healthy subjects, age range 6-50, not taking any vitamin D supplements. Results: In autumn there was an increase in 25OHD concentrations (from 18.1 ± 7.37ng/ml to 24.58 ± 7.72ng/ml, p<0.0001), a decline in PTH from 44.40 ± 17.76pg/ml to 36.63 ± 14.84pg/ml, p<0.001), without change in calcium levels. Only 45 subjects (36%) were vitamin D sufficient (25OHD>20/ml) in spring versus 83 (66.4%) in autumn, p<0.001. Elevated PTH concentrations were noted in 10 subjects in spring (8%) and in six subjects (4.8%) (p<0.05) in autumn. In spring, however, eight out of ten of these subjects (80%) had 25OHD<20 ng/ml, versus one in six (16.7%) in autumn (p<0.01). Normalization of PTH was observed in seven out ten subjects (70%), and all of them had 25-OHD<20 ng/ml in spring. Conclusions: In spring elevated PTH concentrations in the setting of normocalcemia are more likely to be caused by 25OHD deficiency rather by nPHPT. In contrast, in autumn, increased PTH concentrations are more likely to reflect nPHPT. We postulate that screening for nPHPT should be done in 25OHD replete subjects, i.e. in autumn rather than in spring.
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Hiperparatiroidismo Primario , Adolescente , Adulto , Calcio , Niño , Humanos , Hiperparatiroidismo Primario/complicaciones , Hiperparatiroidismo Primario/diagnóstico , Persona de Mediana Edad , Hormona Paratiroidea , Estaciones del Año , Vitamina D , Adulto JovenRESUMEN
PURPOSE: to determine management problems of ovarian masses in girls in order to form a baseline for prospective randomized studies of the established topics and quality improvement of our management. MATERIALS AND METHODS: We performed a national analysis of clinical aspects of ovarian masses in girls operated on in Poland, analyzed retrospectively medical files of all consecutive patients aged 0-18 who underwent surgeries for ovarian lesions between 2012 and 2017 at 17 pediatric surgical departments and complemented the analysis with a scoping review of a recent primary research related to ovarian masses in children. RESULTS: The study group comprised 595 patients. Forty-four (7.39%) girls were diagnosed with malignant tumors. The overall preservation rate was 64.54%. The analysis revealed that positive tumor markers (OR = 10.3), lesions larger than 6 cm (OR = 4.17) and solid mass on ultrasound examination (OR = 5.34) are interdependent variables differentiating malignant tumors from non-malignant lesions (X42 = 79.1; p = 0.00000). Our scoping review revealed 10 major branches of research within the topic of ovarian masses in pediatric population. CONCLUSIONS: We have developed an overview of the field with the emphasis on the local environment. Our next step is a multi-institutional prospective study of a quality improvement project implementation based on the obtained knowledge.
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Quistes Ováricos , Neoplasias Ováricas , Biomarcadores de Tumor , Niño , Femenino , Humanos , Quistes Ováricos/diagnóstico , Quistes Ováricos/cirugía , Neoplasias Ováricas/diagnóstico , Neoplasias Ováricas/cirugía , Estudios Prospectivos , Estudios RetrospectivosRESUMEN
Factor XI (FXI) deficiency (hemophilia C or Rosenthal disease) was first described in the 1950s in a multigenerational family experiencing bleeding related to surgery and dental procedures. Managing patients with FXI deficiency presents several challenges, including a lack of correlation of bleeding symptoms with FXI activity levels, the large volume of fresh frozen plasma required to achieve hemostatic FXI levels, lack of availability of FXI concentrate in certain regions of the world, and the inherent thrombotic risk associated with replacement therapy. This article summarizes presentation, diagnosis, and management of patients with FXI deficiency in a variety of clinical settings.
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Deficiencia del Factor XI , Factor XI , Deficiencia del Factor XI/diagnóstico , Deficiencia del Factor XI/terapia , Hemorragia/etiología , Hemostasis , Humanos , TrombosisRESUMEN
BACKGROUND: There is no consensus regarding vitamin sufficiency status with either 20 ng/mL or 30 ng/mL sufficiency cut-off. We assessed the effects of summer sunshine exposure on vitamin D status. PARTICIPANTS: We measured vitamin D concentrations, PTH, creatinine, and total calcium in 132 healthy subjects, age 29.36 ± 13.57 years, in spring and autumn. RESULTS: There was an overall increase in vitamin D concentrations from spring to autumn from 18.1 ± 7.39 ng/mL to 24.58 ± 7.72 ng/mL, (p < 0.001), accompanied by a decrease in PTH from 44.4 ± 17.76 pg/mL to 36.6 ± 14.84 pg/mL, (p < 0.001). In spring, only 5.3% of individuals were vitamin D sufficient for a 30 ng/mL cut-off, increasing to 23.2% in autumn (p < 0.001). In contrast, when a 20 ng/mL cut-off was employed, vitamin D sufficiency was found in 34.1% in spring and 66.4% individuals in autumn, respectively, (p < 0.001). In multiple regression analysis, holiday leave was the only significant determinant of vitamin D increase (p < 0.001). CONCLUSIONS: Holiday leave is the strongest determinant of an increase in vitamin D. The majority of healthy individuals fail to reach a 30 ng/mL vitamin D cut-off after summer sunshine exposure. This raises the question, whether such a cut-off is indeed suitable for the Polish population.