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1.
BMC Public Health ; 24(1): 1117, 2024 Apr 23.
Article in English | MEDLINE | ID: mdl-38654278

ABSTRACT

BACKGROUND: Despite nearly a quarter of Venezuelans remaining unvaccinated against coronavirus disease 2019 (COVID-19), the factors contributing to vaccine hesitancy in the country have not been thoroughly investigated. METHODS: A cross-sectional study was conducted from October 15th to 30th, 2022, using a knowledge, attitudes, and practices (KAP) survey to identify factors associated with COVID-19 vaccine hesitancy. RESULTS: The study analyzed data from 1,930 participants from all 24 states of Venezuela. The majority (93.4%) were vaccinated. The mean age was 40 years, predominantly female (67.3%), and held a university degree (70.6%). The mean KAP score was significantly higher among vaccinated individuals compared to unvaccinated ones (7.79 vs. 3.94 points for knowledge, 40 vs. 24 points for attitudes, and 16 vs. 10 points for practices, all p < 0.001). Increases in the scores for KAP were associated with increased odds of being vaccinated (84.6%, 25.6%, and 33% respectively for each one-point increase, all p < 0.001). Certain demographic factors such as marital status, occupation, religious beliefs, monthly income, and location influence COVID-19 vaccine knowledge. Higher income and certain occupations decrease the odds of low knowledge, while residing in specific states increases it. Attitudes towards the COVID-19 vaccine are influenced by age, health status, vaccination status, and location. Higher income and absence of certain health conditions decrease the odds of negative attitudes. Lastly, age, occupation, monthly income, and location affect vaccine practices. Advanced age and higher income decrease the odds of inappropriate practices, while residing in La Guaira state increases them. CONCLUSION: Factors such as age, education level, occupation, monthly income, and location were found to be associated with knowledge and attitudes towards COVID-19 vaccine among the surveyed Venezuelans.


Subject(s)
COVID-19 Vaccines , COVID-19 , Health Knowledge, Attitudes, Practice , Vaccination Hesitancy , Humans , Venezuela , Female , Male , Adult , Vaccination Hesitancy/statistics & numerical data , Vaccination Hesitancy/psychology , Cross-Sectional Studies , COVID-19 Vaccines/administration & dosage , COVID-19/prevention & control , COVID-19/psychology , Middle Aged , Young Adult , Surveys and Questionnaires , Adolescent , Aged
2.
J Natl Cancer Inst ; 116(2): 239-248, 2024 Feb 08.
Article in English | MEDLINE | ID: mdl-38059798

ABSTRACT

INTRODUCTION: The addition of taxanes to anthracycline-based chemotherapy is considered standard of care in the treatment of breast cancer. However, there are insufficient data regarding the safety of taxanes during pregnancy. The aim of this study was to describe the incidence of obstetric and neonatal adverse events associated with the use of taxane-containing chemotherapy regimens for the treatment of breast cancer during pregnancy. METHODS: This is a multicenter, international cohort study of breast cancer patients treated with taxanes during pregnancy. A descriptive analysis was undertaken to synthetize available data. RESULTS: A total of 103 patients were included, most of whom were treated with paclitaxel and anthracyclines given in sequence during gestation (90.1%). The median gestational age at taxane initiation was 28 weeks (range = 12-37 weeks). Grade 3-4 adverse events were reported in 7 of 103 (6.8%) patients. The most common reported obstetric complications were intrauterine growth restriction (n = 8 of 94, 8.5%) and preterm premature rupture of membranes (n = 5 of 94, 5.3%). The live birth rate was 92 of 94 (97.9%), and the median gestational age at delivery was 37 weeks (range = 32-40 weeks). Admission to an intensive care unit was reported in 14 of 88 (15.9%) neonates, and 17 of 70 (24.3%) live births resulted in small for gestational age neonates. Congenital malformations were reported in 2 of 93 (2.2%). CONCLUSION: Obstetric and neonatal outcomes after taxane exposure during pregnancy were generally favorable and did not seem to differ from those reported in the literature with standard anthracycline-based regimens. This study supports the use of taxanes during gestation when clinically indicated.


Subject(s)
Breast Neoplasms , Bridged-Ring Compounds , Pregnancy , Infant, Newborn , Female , Humans , Infant , Breast Neoplasms/drug therapy , Breast Neoplasms/epidemiology , Breast Neoplasms/chemically induced , Cohort Studies , Taxoids/adverse effects , Antibiotics, Antineoplastic , Anthracyclines/adverse effects
3.
J Clin Med ; 12(23)2023 Nov 28.
Article in English | MEDLINE | ID: mdl-38068424

ABSTRACT

BACKGROUND: Infantile cerebral palsy is a neurological pathology that causes great morbidity, mortality, and disability in people who suffer from it, mainly affecting motor development. There are a multitude of non-pharmacological methods or therapies for its treatment. One of the main methods is Vojta therapy. This methodology acts on ontogenetic postural function and automatic postural control. OBJECTIVE: This study aims to demonstrate that there are changes in the motor development of children with cerebral palsy with the application of Vojta therapy. METHODS AND ANALYSIS: This is a randomized controlled trial on the effectiveness of two neurorehabilitation techniques in patients with cerebral palsy conducted at the Physical Medicine and Rehabilitation Service of the Teresa Herrera Maternal and Child Hospital of the A Coruña and Cee Health Area. The study will be conducted from January 2023 to December 2024. There will be two groups: the Vojta therapy group (n = 30) and the conventional physiotherapy group (n = 30). The measurement variables will be gross motor function as measured by the Gross Motor Function Measure (GMFM) and Infant Motor Profile (IMP) scales. ETHICS AND DISSEMINATION: The study was approved by the Research Ethics Committee of the University of Murcia (1823/2018) and Comité de Ética de la Investigación de A Coruña-Ferrol (2022/099). TRIAL REGISTRATION NUMBER: ClinicalTrials.gov; identifier: NCT06092619.

4.
Arthrosc Tech ; 12(10): e1827-e1836, 2023 Oct.
Article in English | MEDLINE | ID: mdl-37942102

ABSTRACT

The elbow is one of the most commonly dislocated joints. While conservative management is frequently performed for simple elbow dislocations, the importance of primary surgical treatment is still undetermined. However, promising results have been reached after surgical repair. We propose an arthroscopic surgical repair of the lateral ligament complex (LCL), performed with a horizontal suture and 2 Fibertak Knotless implants (Arthrex) placed on the LCL origin, one anterior and the other posterior. Operative treatment should be performed in patients with moderate and gross elbow laxity to avoid post-traumatic sequelae and decrease revision rates. Arthroscopic techniques create fewer complications. This procedure allows one to address intra-articular elbow joint pathology with less chance of wound complications and the ability to use bone anchors if desired.

5.
Sensors (Basel) ; 23(19)2023 Sep 26.
Article in English | MEDLINE | ID: mdl-37836921

ABSTRACT

Recent advances allow the use of Augmented Reality (AR) for many medical procedures. AR via optical navigators to aid various knee surgery techniques (e.g., femoral and tibial osteotomies, ligament reconstructions or menisci transplants) is becoming increasingly frequent. Accuracy in these procedures is essential, but evaluations of this technology still need to be made. Our study aimed to evaluate the system's accuracy using an in vitro protocol. We hypothesised that the system's accuracy was equal to or less than 1 mm and 1° for distance and angular measurements, respectively. Our research was an in vitro laboratory with a 316 L steel model. Absolute reliability was assessed according to the Hopkins criteria by seven independent evaluators. Each observer measured the thirty palpation points and the trademarks to acquire direct angular measurements on three occasions separated by at least two weeks. The system's accuracy in assessing distances had a mean error of 1.203 mm and an uncertainty of 2.062, and for the angular values, a mean error of 0.778° and an uncertainty of 1.438. The intraclass correlation coefficient was for all intra-observer and inter-observers, almost perfect or perfect. The mean error for the distance's determination was statistically larger than 1 mm (1.203 mm) but with a trivial effect size. The mean error assessing angular values was statistically less than 1°. Our results are similar to those published by other authors in accuracy analyses of AR systems.


Subject(s)
Augmented Reality , Surgery, Computer-Assisted , Reproducibility of Results , Femur/surgery , Surgery, Computer-Assisted/methods , Osteotomy
6.
Pediatric Health Med Ther ; 14: 267-280, 2023.
Article in English | MEDLINE | ID: mdl-37691881

ABSTRACT

Anemia is the most common hematologic abnormality identified in children and represents a major global health problem. A delay in diagnosis and treatment might place patients with anemia at risk for the development of rare but serious complications, including chronic and irreversible cognitive impairment. Identified risk factors contributing to the development of anemia in children include the presence of nutritional deficiencies, environmental factors, chronic comorbidities, and congenital disorders of hemoglobin or red blood cells. Pediatricians, especially those in the primary care setting, serve a particularly critical role in the identification and care of those children affected by anemia. Prompt recognition of these risk factors is crucial for developing appropriate and timely therapeutic interventions and prevention strategies.

7.
Eur J Haematol ; 111(5): 757-767, 2023 Nov.
Article in English | MEDLINE | ID: mdl-37587687

ABSTRACT

OBJECTIVES: We aimed to indirectly compare the efficacy of personalized prophylaxis with simoctocog alfa (Nuwiq®) versus three extended half-life (EHL) recombinant FVIII (rFVIII) concentrates. METHODS: Treatment effects were compared using matching-adjusted indirect comparisons after matching individual patient-level baseline characteristics for simoctocog alfa (pharmacokinetic [PK]-guided personalized prophylaxis) against published aggregate personalized prophylaxis data for efmoroctocog alfa, damoctocog alfa pegol, and rurioctocog alfa pegol. RESULTS: A higher percentage (p < .001) of patients with zero bleeds was found with simoctocog alfa compared with efmoroctocog alfa (75% vs. 45%), damoctocog alfa pegol (77% vs. 38%), and rurioctocog alfa pegol (target trough level 1%-3%; 78% vs. 42%). Similar efficacy was found comparing simoctocog alfa against rurioctocog alfa pegol 8%-12% (77% vs. 62%). The mean total annualized bleeding rate was lower (p < .001) with simoctocog alfa than damoctocog alfa pegol (1.5 vs. 4.9). Consistent with approved dosing, the mean FVIII weekly dose was higher (p < .001) for simoctocog alfa than efmoroctocog alfa, damoctocog alfa pegol, or rurioctocog alfa pegol 1%-3%, but lower (p < .001) than rurioctocog alfa pegol 8%-12%. CONCLUSIONS: Indirect comparisons demonstrated that PK-guided, personalized prophylaxis with simoctocog alfa can lead to higher zero bleed rates compared with personalized EHL rFVIII concentrate regimens, albeit with higher weekly doses, and a lower percentage of patients treated twice weekly or less.


Subject(s)
Factor VIII , Hemophilia A , Humans , Factor VIII/adverse effects , Factor VIII/therapeutic use , Half-Life , Hemophilia A/drug therapy , Hemorrhage/etiology , Hemorrhage/prevention & control , Hemorrhage/drug therapy , Recombinant Proteins , Treatment Outcome
8.
Curr Oncol ; 30(7): 6097-6110, 2023 06 24.
Article in English | MEDLINE | ID: mdl-37504314

ABSTRACT

(1) Background: recent evidence suggests that long low-dose capecitabine regimens have a synergistic effect with endocrine therapy as aromatase inhibitors (AIs), and might increase overall survival for hormone-receptor-positive, HER2-negative, metastatic breast cancer compared to both treatments. We performed a retrospective study to confirm the efficacy and expand the safety data for capecitabine plus AI (a combination henceforth named XELIA) for this indication. (2) We conducted a single-center retrospective cohort study of 163 hormone receptor-positive metastatic breast cancer patients who received either the XELIA regimen, capecitabine, or an aromatase inhibitor (AI) as single agents in first-line treatment. The primary endpoint was progression-free survival, and the secondary endpoints were overall survival, best objective response, and toxicity incidence. (3) Results: the median progression-free survival for patients receiving XELIA, AI, and capecitabine was 29.37 months (20.91 to 37.84; 95% CI), 20.04 months (7.29 to 32.80; 95% CI) and 10.48 (8.69 to 12.28; 95% CI), respectively. The overall response rate was higher in the XELIA group (29.5%) than in the AI (14.3%) and capecitabine (9.1%) groups. However, the differences in overall survival were not statistically significant. Apart from hand-foot syndrome, there were no statistically significant differences in adverse events between the groups. (4) Conclusions: this retrospective study suggests that progression-free survival and overall response rates improved with the XELIA regimen compared to use of aromatase inhibitors and capecitabine alone. Combined use demonstrated an adequate safety profile and might represent an advantageous treatment in places where CDK 4/6 is not available. Larger studies and randomized clinical trials are required to confirm the effects shown in our study.


Subject(s)
Breast Neoplasms , Humans , Female , Breast Neoplasms/pathology , Capecitabine/therapeutic use , Aromatase Inhibitors/therapeutic use , Retrospective Studies , Receptor, ErbB-2 , Antineoplastic Combined Chemotherapy Protocols
9.
J Pediatr Hematol Oncol ; 45(6): e781-e783, 2023 08 01.
Article in English | MEDLINE | ID: mdl-37494615

ABSTRACT

Coagulation factor VII (FVII) deficiency is a congenital disorder with heterogeneous clinical phenotypes ranging from asymptomatic to life-threatening bleeding and/or thrombotic events. We present the case of an adolescent male who developed acute deep and superficial venous thromboses of the upper extremities in the setting of multiple peripheral venous line insertions and shortly after receiving his second coronavirus disease of 2019 immunization dose. A hemostatic work-up revealed low FVII activity levels associated with 4 different FVII genetic variants. We highlight the need to better understand the pathophysiologic mechanisms behind FVII deficiency-associated prothrombotic risk and the role that specific FVII genetic variants may play in the clinical presentation of these patients.


Subject(s)
Coronavirus Infections , Coronavirus , Factor VII Deficiency , Thrombosis , Male , Humans , Factor VII Deficiency/complications , Factor VII Deficiency/genetics , Factor VII/genetics , Immunization
10.
Gac Med Mex ; 159(3): 210-218, 2023.
Article in English | MEDLINE | ID: mdl-37494706

ABSTRACT

BACKGROUND: Heart valve replacement surgery with mechanical or biological prostheses entails a risk of thromboembolism and bleeding complications. OBJECTIVE: To determine the complications related to complementary anticoagulation therapy and the probability of risk. METHODS: One-hundred and sixty-three patients who underwent heart valve replacement between 2002 and 2016 with either mechanical or biological prostheses, and who received vitamin K antagonists after hospital discharge, were studied. Anticoagulation therapy was categorized into optimal and non-optimal according to INR values prior to the development of complications. Patients with comorbidities and other risk factors for thrombosis and/or bleeding were excluded. RESULTS: In total, 68.7 % of patients received mechanical prostheses, and 31.3 %, biological prostheses (p ≤ 0.001); 25.2 % experienced the complications that motivated the study (p ≤ 0.001), which were hemorrhagic in 48.8 %, thromboembolic in 26.8 %, and of both types in 24.4 % (relative risk = 4.229). Among the patients with complications, 95.1 % received mechanical prostheses, and 4.9 %, biological (p = 0.005); non-optimal INR was identified in 49.7 % (p ≤ 0.001). CONCLUSIONS: Given the high risk of thromboembolic and hemorrhagic complications, valve prostheses must be carefully chosen, and care priorities should include prevention and follow-up, especially in those patients who require anticoagulation therapy.


ANTECEDENTES: El reemplazo valvular por prótesis mecánicas o biológicas implica riesgo de tromboembolismo y complicaciones hemorrágicas. OBJETIVO: Determinar las complicaciones relacionadas con la terapia de anticoagulación complementaria y la probabilidad de riesgo en pacientes portadores de prótesis valvulares del corazón. MÉTODOS: Se estudiaron 163 pacientes entre 2002 y 2016, portadores de prótesis mecánicas y biológicas, quienes recibieron antagonistas de la vitamina K posterior al egreso hospitalario. La terapia de anticoagulación se categorizó en óptima y no óptima conforme a los valores de INR previos a las complicaciones. Fueron excluidos los pacientes con comorbilidades y otros factores de riesgo de trombosis y/o sangrado. RESULTADOS: a 68.7 % de los pacientes se les colocó prótesis mecánica y a 31.3 %, biológica (p ≤ 0.001); 25.2 % presentó las complicaciones motivo de estudio (p ≤ 0.001), hemorrágicas en 48.8 %, tromboembólicas en 26.8 % y de ambos tipos en 24.4 % (riesgo relativo = 4.229); a 95.1 % de los pacientes con complicaciones se les colocó prótesis mecánica y a 4.9 %, biológica (p = 0.005); 49.7 % presentó INR no óptimo (p ≤ 0.001). CONCLUSIONES: Ante riesgo alto de complicaciones tromboembólicas y hemorrágicas, la elección de las prótesis valvulares, la prevención y el seguimiento son prioridades, principalmente en quienes requieren terapia de anticoagulación.


Subject(s)
Heart Valve Prosthesis Implantation , Heart Valve Prosthesis , Thromboembolism , Humans , Tertiary Care Centers , Thromboembolism/epidemiology , Thromboembolism/etiology , Thromboembolism/prevention & control , Heart Valve Prosthesis/adverse effects , Anticoagulants/therapeutic use , Hemorrhage/epidemiology , Hemorrhage/etiology , Heart Valves , Heart Valve Prosthesis Implantation/adverse effects
11.
Gac. méd. Méx ; 159(3): 215-223, may.-jun. 2023. tab
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1448279

ABSTRACT

Resumen Antecedentes: El reemplazo valvular por prótesis mecánicas o biológicas implica riesgo de tromboembolismo y complicaciones hemorrágicas. Objetivo: Determinar las complicaciones relacionadas con la terapia de anticoagulación complementaria y la probabilidad de riesgo en pacientes portadores de prótesis valvulares del corazón. Métodos: Se estudiaron 163 pacientes entre 2002 y 2016, portadores de prótesis mecánicas y biológicas, quienes recibieron antagonistas de la vitamina K posterior al egreso hospitalario. La terapia de anticoagulación se categorizó en óptima y no óptima conforme a los valores de INR previos a las complicaciones. Fueron excluidos los pacientes con comorbilidades y otros factores de riesgo de trombosis y/o sangrado. Resultados: a 68.7 % de los pacientes se les colocó prótesis mecánica y a 31.3 %, biológica (p ≤ 0.001); 25.2 % presentó las complicaciones motivo de estudio (p ≤ 0.001), hemorrágicas en 48.8 %, tromboembólicas en 26.8 % y de ambos tipos en 24.4 % (riesgo relativo = 4.229); a 95.1 % de los pacientes con complicaciones se les colocó prótesis mecánica y a 4.9 %, biológica (p = 0.005); 49.7 % presentó INR no óptimo (p ≤ 0.001). Conclusiones: Ante riesgo alto de complicaciones tromboembólicas y hemorrágicas, la elección de las prótesis valvulares, la prevención y el seguimiento son prioridades, principalmente en quienes requieren terapia de anticoagulación.


Abstract Background: Heart valve replacement surgery with mechanical or biological prostheses entails a risk of thromboembolism and bleeding complications. Objective: To determine the complications related to complementary anticoagulation therapy and the probability of risk. Methods: One-hundred and sixty-three patients who underwent heart valve replacement between 2002 and 2016 with either mechanical or biological prostheses, and who received vitamin K antagonists after hospital discharge, were studied. Anticoagulation therapy was categorized into optimal and non-optimal according to INR values prior to the development of complications. Patients with comorbidities and other risk factors for thrombosis and/or bleeding were excluded. Results: In total, 68.7 % of patients received mechanical prostheses, and 31.3 %, biological prostheses (p ≤ 0.001); 25.2 % experienced the complications that motivated the study (p ≤ 0.001), which were hemorrhagic in 48.8 %, thromboembolic in 26.8 %, and of both types in 24.4 % (relative risk = 4.229). Among the patients with complications, 95.1 % received mechanical prostheses, and 4.9 %, biological (p = 0.005); non-optimal INR was identified in 49.7 % (p ≤ 0.001). Conclusions: Given the high risk of thromboembolic and hemorrhagic complications, valve prostheses must be carefully chosen, and care priorities should include prevention and follow-up, especially in those patients who require anticoagulation therapy.

12.
J Pers Med ; 13(5)2023 Apr 25.
Article in English | MEDLINE | ID: mdl-37240897

ABSTRACT

Computer technologies play a crucial role in orthopaedic surgery and are essential in personalising different treatments. Recent advances allow the usage of augmented reality (AR) for many orthopaedic procedures, which include different types of knee surgery. AR assigns the interaction between virtual environments and the physical world, allowing both to intermingle (AR superimposes information on real objects in real-time) through an optical device and allows personalising different processes for each patient. This article aims to describe the integration of fiducial markers in planning knee surgeries and to perform a narrative description of the latest publications on AR applications in knee surgery. Augmented reality-assisted knee surgery is an emerging set of techniques that can increase accuracy, efficiency, and safety and decrease the radiation exposure (in some surgical procedures, such as osteotomies) of other conventional methods. Initial clinical experience with AR projection based on ArUco-type artificial marker sensors has shown promising results and received positive operator feedback. Once initial clinical safety and efficacy have been demonstrated, the continued experience should be studied to validate this technology and generate further innovation in this rapidly evolving field.

13.
PLoS One ; 18(5): e0284966, 2023.
Article in English | MEDLINE | ID: mdl-37141308

ABSTRACT

Information and communication technologies have significantly transformed the way advanced societies interact, produce, deliver services and consume resources. All walks of life are now touched by these technologies. However, compared to other areas of society, digital penetration is much lower in the development of and access to social services. The main objective of this paper was to find out what technological devices are used, how they are used and the way citizens interact with public bodies using technology to deliver social services. This has been part of a wider project on innovation in social services using participative methodologies centred on the development of local Hubs. The findings reveal a digital divide in technology-enabled access to social services that excludes the very people most in need of benefits and support.


Subject(s)
Communication , Social Work , Humans , Technology
14.
Res Pract Thromb Haemost ; 7(1): 100001, 2023 Jan.
Article in English | MEDLINE | ID: mdl-36970740

ABSTRACT

Background: Before the official US Food and Drug Administration approval in 2021, pediatric hematologists across the United States have used direct oral anticoagulants (DOACs) "off-label" and based on extrapolation from labeling for adults with venous thromboembolism (VTE) and interim results of pediatric-specific DOAC clinical studies. Objectives: The American Thrombosis and Hemostasis Network 15 (ATHN 15) study aimed to characterize the use of DOACs from 2015 to 2021 at 15 specialized pediatric hemostasis centers in the United States, with emphasis on safety and effectiveness. Methods: Eligible participants were those aged 0 to 21 years who had a DOAC included as part of their anticoagulation regimen for the treatment of acute VTE or secondary prevention of VTE. Data were collected for up to 6 months after initiation of the DOAC. Results: A total of 233 participants were enrolled, with a mean age of 16.5 years. Rivaroxaban was the most commonly prescribed DOAC (59.1%) followed by apixaban (38.8%). Thirty-one (13.8%) participants reported bleeding complications while on a DOAC. Major or clinically relevant nonmajor bleeding events occurred in 1 (0.4%) and 5 (2.2%) participants, respectively. Worsening menstrual bleeding was reported in 35.7% of females aged >12 years and occurred more frequently in those using rivaroxaban (45.6%) compared with apixaban (18.9%). The recurrent thrombosis rate was 4%. Conclusion: Pediatric hematologists at specialized hemostasis centers in the United States have been using DOACs for the treatment and prevention of VTEs, primarily in adolescents and young adults. Reported DOAC use showed adequate safety and effectiveness rates.

15.
Blood Rev ; 58: 101018, 2023 03.
Article in English | MEDLINE | ID: mdl-36210240

ABSTRACT

Von Willebrand disease (VWD) is an inherited bleeding disorder caused by quantitative or qualitative deficiencies in von Willebrand factor (VWF). People with VWD may experience excessive, recurrent or prolonged bleeding, particularly during menstruation, childbirth, surgery or following trauma. However, many VWD patients are undiagnosed, and therefore inadequately treated. Reasons for the underdiagnosis of VWD include its relatively mild symptoms, complex diagnosis, lack of awareness among non-specialist healthcare providers and the general population, and a lack of prioritisation of disorders disproportionately affecting females. The vwdtest.com platform was launched as part of a global initiative to raise awareness and improve diagnosis of VWD. Besides providing VWD-specific educational resources, the website includes an online bleeding self-assessment tool and offers diagnostic support for individuals, and their providers, who have a score suggestive of a bleeding disorder. vwdtest.com helps to address these unmet needs, especially in regions with limited access to educational and diagnostic resources.


Subject(s)
Hemorrhagic Disorders , von Willebrand Diseases , Female , Humans , von Willebrand Diseases/diagnosis , von Willebrand Diseases/therapy , von Willebrand Diseases/complications , von Willebrand Factor , Hemorrhage/diagnosis , Hemorrhage/etiology , Hemorrhage/therapy
16.
J Pediatr Adolesc Gynecol ; 35(6): 647-652, 2022 Dec.
Article in English | MEDLINE | ID: mdl-35917902

ABSTRACT

STUDY OBJECTIVE: To examine the clinical characteristics and prevalence of congenital bleeding disorders (CBDs), with emphasis on congenital factor VII (FVII) deficiency and other rare bleeding disorders, in adolescent and young adult females referred to a hemophilia treatment center (HTC) for evaluation and management of heavy menstrual bleeding (HMB) and iron deficiency anemia (IDA) DESIGN: In this single-center retrospective study, we reviewed the clinical characteristics and prevalence of CBDs in postmenarchal females, younger than 22 years of age, referred to an HTC from 2015 to 2021 for evaluation of HMB with or without IDA. RESULTS: One hundred females, with a mean age of 15 years (range 9-20 years), met initial study criteria, and 95 were included in the final analysis. Forty-five (47%) females were ultimately diagnosed with a CBD. The most prevalent diagnoses were FVII deficiency and type 1 von Willebrand disease (VWD) (42.3%, n = 19 each). Forty-two percent of patients with FVII deficiency had a low-for-age FVII activity level, 21.1% were only positive for the FVII R353Q variant associated with borderline FVII levels, whereas 36.8% had both a low-for-age FVII activity level and a positive R353Q variant. Eighty percent of patients with a CBD were found to have relatives with abnormal bleeding symptoms. CONCLUSION: Congenital FVII deficiency is prevalent among female adolescents experiencing HMB with or without IDA. In addition to VWD, evaluation for this specific factor deficiency should be considered as part of the initial CBD workup. Presence of abnormal bleeding history in the family could also help to predict presence of a CBD.


Subject(s)
Anemia, Iron-Deficiency , Factor VII Deficiency , Hemorrhagic Disorders , Iron Deficiencies , Menorrhagia , Adolescent , Child , Female , Humans , Young Adult , Anemia, Iron-Deficiency/epidemiology , Factor VII , Factor VII Deficiency/complications , Factor VII Deficiency/epidemiology , Hemorrhage , Hemorrhagic Disorders/complications , Menorrhagia/etiology , Menorrhagia/complications , Prevalence , Retrospective Studies
17.
Haemophilia ; 28 Suppl 5: 3-15, 2022 Jul.
Article in English | MEDLINE | ID: mdl-35861919

ABSTRACT

INTRODUCTION: The sixth Åland Islands Conference on von Willebrand disease (VWD) on the Åland Islands, Finland, was held from 20 to 22 September 2018. AIM: The meeting brought together experts in the field of VWD from around the world to share the latest advances and knowledge in VWD. RESULTS AND DISCUSSION: The topics covered both clinical aspects of disease management, and biochemical and laboratory insights into the disease. The clinical topics discussed included epidemiology, diagnosis and treatment of VWD in different countries, management of children with VWD, bleeding control during surgery, specific considerations for the management of type 3 VWD and bleeding control in women with VWD. Current approaches to the management of acquired von Willebrand syndrome were also discussed. Despite significant advances in the understanding and therapeutic options for VWD, there remain many challenges to be overcome in order to optimise patient care. In comparison with haemophilia A, there are very few registries of VWD patients, which would be a valuable source of data on the condition and its management. VWD is still underdiagnosed, and many patients suffer recurrent or severe bleeding that could be prevented. Awareness of VWD among healthcare practitioners, including non-haematologists, should be improved to allow timely diagnosis and intervention. Diagnosis remains challenging, and the development of fast, simple assays may help to facilitate accurate and rapid diagnosis of VWD.


Subject(s)
von Willebrand Disease, Type 3 , von Willebrand Diseases , Child , Congresses as Topic , Female , Finland , Hemorrhage , Humans , Registries , von Willebrand Diseases/complications , von Willebrand Diseases/diagnosis , von Willebrand Diseases/drug therapy , von Willebrand Factor/therapeutic use
18.
Expert Rev Med Devices ; 19(6): 489-497, 2022 Jun.
Article in English | MEDLINE | ID: mdl-35903900

ABSTRACT

INTRODUCTION: Patient-specific instrumentation (PSI) for total knee arthroplasty (TKA) surgery was initially developed to increase accuracy. The potential PSI benefits have expanded in the last decade, and other advantages have been published. However, different authors are critical of PSI and argue that the advantages are not such and do not compensate for the extra cost. This article aims to describe the recently published advantages and disadvantages of PSI. AREAS COVERED: Narrative description of the latest publications related to PSI in accuracy, clinical and functional outcomes, operative time, efficiency, and other benefits. EXPERT OPINION: We have published high accuracy of the system, with a not clinically relevant loss of accuracy, significantly higher precision with PSI than with conventional instruments, and a high percentage of cases in the optimal range and similar to that obtained with computer-assisted navigation, greater imprecision for tibial slope, a significant blood loss reduction, and time consumption, an acceptable and non-significant increase in the cost per procedure, and no difference in complications during hospital admission and at 90 days. We think that PSI will not follow the Scott Parabola and that it will continue to be a valuable type of device in some instances of TKA surgery.


Subject(s)
Arthroplasty, Replacement, Knee , Knee Prosthesis , Surgery, Computer-Assisted , Humans , Knee Joint/surgery , Operative Time , Tibia/surgery
19.
J Pediatr Hematol Oncol ; 44(4): 181-185, 2022 05 01.
Article in English | MEDLINE | ID: mdl-35446803

ABSTRACT

Retinoblastoma (RB) is the most common intraocular pediatric malignancy. Advancements in intra-arterial chemotherapy (IAC) for treatment of RB have resulted in dramatic improvement in eye salvage rates. Data regarding IAC outcomes and associated hematologic toxicities are limited. The objective of this retrospective study was to analyze baseline characteristics, efficacy, and hematologic complications associated with IAC treatment in children with RB at a single international referral institution. Ninety-five sessions of IAC were performed in 28 patients. Mean age at RB diagnosis was 12.5 months (SD, 9.2 mo). Fourteen patients had bilateral RB. IAC agents included melphalan, carboplatin, and topotecan. The most common regimens were triple-agent IAC and single-agent melphalan (66.3% and 15.8%, respectively). Median number of IAC sessions was 3 (mean: 3.39, range: 1 to 9). Eye salvage rate was 83.7% with an overall survival rate of 100% at a median follow-up of 29 months (mean: 29.8 mo, range: 1 to 63 mo). A total of 26 patients (92.9%) experienced at least 1 hematologic toxicity during their treatment course Prevalence of neutropenia, anemia, and thrombocytopenia were 89.3%, 85.7%, and 25%, respectively. While IAC is effective in salvaging most eyes with advanced intraocular RB, over half of patients experienced clinically significant neutropenia and anemia. Clinicians should be vigilant in monitoring patients for IAC-related complications.


Subject(s)
Neutropenia , Retinal Neoplasms , Retinoblastoma , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Child , Follow-Up Studies , Humans , Infant , Infusions, Intra-Arterial , Melphalan , Neutropenia/drug therapy , Retinal Neoplasms/diagnosis , Retinal Neoplasms/drug therapy , Retinoblastoma/diagnosis , Retinoblastoma/drug therapy , Retrospective Studies , Treatment Outcome
20.
Article in English | MEDLINE | ID: mdl-35457522

ABSTRACT

The Cobb angle value is a critical parameter for evaluating adolescent idiopathic scoliosis (AIS) patients. This study aimed to evaluate a software's validity and absolute reliability to determine the Cobb angle in AIS digital X-rays, with two different degrees of experienced observers. Four experts and four novice evaluators measured 35 scoliotic curves with the software on three separate occasions, one month apart. The observers re-measured the same radiographic studies on three separate occasions three months later but on conventional X-ray films. The differences between the mean bias errors (MBE) within the experience groups were statistically significant between the experts (software) and novices (manual) (p < 0.001) and between the novices (software) and novices (manual) (p = 0.005). When measured with the software, the intra-group error in the expert group was MBE = 1.71 ± 0.61° and the intraclass correlation coefficient (ICC (2,1)) = 0.986, and in the novice group, MBE = 1.9 ± 0.67° and ICC (2,1) = 0.97. There was almost a perfect concordance among the two measurement methods, ICC (2,1) = 0.998 and minimum detectable change (MCD95) < 0.4°. Control of the intrinsic error sources enabled obtaining inter- and intra-observer MDC95 < 0.5° in the two experience groups and with the two measurement methods. The computer-aided software TraumaMeter increases the validity and reliability of Cobb angle measurements concerning manual measurement.


Subject(s)
Scoliosis , Adolescent , Humans , Radiography , Reproducibility of Results , Research Design , Scoliosis/diagnostic imaging , Software , Spine
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