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1.
J Contemp Dent Pract ; 25(4): 326-330, 2024 Apr 01.
Article in English | MEDLINE | ID: mdl-38956846

ABSTRACT

AIM: To conduct a scientometric analysis on black stains, and dental caries, with a focus on identifying emergent patterns, collaborative networks, and thematic evolution. MATERIALS AND METHODS: A retrospective, descriptive, observational study was conducted using a scientometric approach. The Scopus database was utilized for literature search, selecting articles published between 1976 and 2023. The literature search was carried out on February 8, 2024, using AND and OR Boolean operators which were adapted to the search strategy. Finally, SciVal used different scientometric indicators such as "Scholarly Output", "Views Count", "Field-Weighted Citation Impact" and the "Citation Count". RESULTS: A total of 13 documents from 10 different sources were analyzed, covering a period from 1976 to 2023. Despite an annual growth rate of 0%, each document has an average of 21.08 citations, indicating their relevance in the field. The documents have an average age of 12.9 years and contain 336 references. A total of 143 additional keywords and 26 author keywords were identified. The documents were written by 62 different authors, with an average of 5 coauthors per document and 30.77% international coauthorships. CONCLUSION: The results indicate a significant relevance of the documents in the field, a notable collaboration among authors, and a significant thematic evolution in the research topics. The mentioned institutions and sources produced documents with notable results, indicating a significant interest and impact in the field. CLINICAL SIGNIFICANCE: This study provides a detailed view of the trends and patterns in the research of dental caries and black stains. The findings can assist dental health professionals in better understanding the prevalence and impact of these conditions. Furthermore, it can guide future research and treatment strategies in this field. How to cite this article: Mayta-Tovalino F, Espinoza-Carhuancho F, Reyes-Carmona J, et al. Emergent Patterns, Collaborative Networks and Thematic Evolution on Black Stain and Dental Caries: A Scientometric Study. J Contemp Dent Pract 2024;25(4):326-330.


Subject(s)
Dental Caries , Humans , Retrospective Studies , Bibliometrics , Cooperative Behavior , Dental Research
2.
Int J Mycobacteriol ; 13(1): 83-90, 2024 Jan 01.
Article in English | MEDLINE | ID: mdl-38771284

ABSTRACT

BACKGROUND: The convergence of type 2 diabetes mellitus (DM2) and tuberculosis (TB) may lead to increased mortality and complications, so the underlying mechanism is under investigation. Therefore, a bibliometric analysis was performed to describe the bibliometric indicators of publications evaluating the relationship between TB and DM2. METHODS: A descriptive and observational bibliometric study was conducted using the Scopus database to identify documents published from 2016-2023, for which free and controlled terms (Medical subject headings and Emtree) were used. The variables collected comprised the number of published documents, institutions, countries, authors, journals, and type of collaboration, which were exported to Excel 2016 and analyzed with SciVal. RESULTS: A total of 456 documents, 1624 authors, and 2173 citations were identified, with Medicine and Immunology-Microbiology being the subcategories with the highest and lowest number of documents (367 and 80 documents), respectively, with a strong decreasing trend correlation (R2: 0.95; P < 0.5) between the number and year of publication. While the country with the highest production was China (71 papers), the country with the highest citation was the United States (952 citations). In terms of authors, the highest production was by the American Venketaraman, and the highest impact was by the Asian Kimberly To. The institution with the highest number of papers was the Western University of Health Sciences, while Stellenbosch had the highest impact. CONCLUSION: Although the scientific productivity of DM2 and TB have reported growth rates of 158.75% and 7.3%, respectively, our results found a decreasing trend in publications associating these two diseases. The thematic evolution of the concepts in both diseases suggests that the relationship between them is not yet known, so future studies evaluating the underlying mechanisms of this comorbidity are suggested.


Subject(s)
Bibliometrics , Diabetes Mellitus, Type 2 , Tuberculosis , Humans , Tuberculosis/epidemiology
3.
Biomed Res Int ; 2024: 3279588, 2024.
Article in English | MEDLINE | ID: mdl-38726291

ABSTRACT

Objective: The aim of this study was to examine the characteristics of scientific production related to the use of filtered water in the field of dentistry. Material and Methods. A quantitative and descriptive observational study was carried out with a scientometric approach. Data were collected from the Web of Science (WOS) database during the period January 1991 to December 2023. A search strategy incorporating a combination of MeSH terms, including terms and thesauri related to "filtered water" and "dentistry", was used. R Studio version 4.3.2 and CiteSpace 6.2.R7 were used for data analysis. Results: Over the 32-year study period, 227 scholarly papers from 134 different sources were reviewed. The literature in this field has shown an annual growth rate of 10.44%. During the year 2010, a steady movement in the number of publications and authors was observed, with considerable collaborative interaction. In the year 2020, a large interaction between publications and their citations was found. The "Citation Burst" graph identified three references that have experienced the largest "burst" of citations in an evaluated period. Lotka's law described the productivity of authors, finding that most authors have published only one paper, while a smaller number of authors have published two papers. Most authors contributed a small number of articles, while a few authors contributed a large amount of the existing literature. Conclusion: A comprehensive overview of the scientific production related to the use of filtered water in dentistry over a span of 32 years is provided. The results highlight the growing interdisciplinarity and international collaboration in this field. Finally, the importance of filtered water in dentistry and its growing relevance in the scientific literature are emphasized.


Subject(s)
Dentistry , Humans , Bibliometrics , Water , Spatio-Temporal Analysis , Publications
4.
Vet World ; 17(3): 666-671, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38680158

ABSTRACT

Background and Aim: Understanding dental care in dogs has made remarkable progress in veterinary medicine. Therefore, this study aimed to analyze the academic literature published in veterinary dentistry from 1990 to 2023. Materials and Methods: A descriptive study was conducted using a scientometric approach and metadata from the Web of Science database. A search strategy adapted for this database was developed using MeSH and Emtree terms and the Boolean operators AND and OR. Using Bibliometrix, different metrics were evaluated to assess the scientific production of researchers and institutions and the impact of authors based on their publications. CiteSpace was also used for co-citation analysis and visualization of citation networks, trends, and patterns in this field of study over time. Results: The bibliometric study analyzed 211 documents from 50 different sources from 1990 to 2023, with an annual growth rate of 6.5%, covering the period 1990-2023. A total of 474 authors were identified, with an average of 2.82 coauthors per paper and 11.85% international coauthorships. The average age of the papers was 12.4 years and 4.55 citations per paper. The most common types of documents were articles (154 documents). Conclusion: Research in veterinary dentistry has shown steady growth from 1990 to 2023. Although there have been fluctuations in article production over the years, there has been a steady growth in article production in veterinary dentistry in general. The annual average number of citations per article has varied over the years, reaching 45 in 2015. However, the average number of citations per article has decreased significantly from 2021 to 2023.

6.
Article in English | MEDLINE | ID: mdl-37872063

ABSTRACT

INTRODUCTION: Acute myeloid leukemia is a challenging disease, due to a poor prognosis in developing countries. Herein, we aim to describe the clinical characteristics and outcomes after chemotherapy and transplantation. METHODS: A retrospective analytic observational study was performed with patients under 18 years of age with newly diagnosed acute myeloid leukemia treated at a referral center in Colombia. Two groups were compared: induction therapy (IT) and induction therapy plus consolidation (IT + C). The survival analysis was performed using the Kaplan-Meier method. RESULTS: We analyzed 34 patients diagnosed with acute myeloid leukemia; 20 received hematopoietic stem cell transplantation. Most were French-American-British (FAB) classification types M1, M5 and M0. The transplantation was haploidentical in 65%, conditioning was myeloablative in 67% and graft-versus-host disease prophylaxis was performed with post-transplant cyclophosphamide in 70%. Overall, the 5-year survival was 52% and the overall 5-year survival in the transplanted group was 80%. There were 16 deaths; in the IT group, n = 12, and in the IT + C group, n = 4. In the former, the main cause of death was septic shock and in the latter, it was relapse. CONCLUSION: Transplantation is a safe option. Receiving treatment and supportive measures in hematopoietic stem cell transplantation units is necessary to avoid infections, especially during induction cycles.

7.
Mar Drugs ; 21(5)2023 Apr 29.
Article in English | MEDLINE | ID: mdl-37233478

ABSTRACT

Nocuolin A (1), an oxadiazine, was isolated from the cyanobacterium Nostoc sp. Its chemical structure was elucidated using NMR and mass spectroscopic data. From this compound, two new oxadiazines, 3-[(6R)-5,6-dihydro-4,6-dipentyl-2H-1,2,3-oxadiazin-2-yl]-3-oxopropyl acetate (2) and 4-{3-[(6R)-5,6-dihydro-4,6-dipentyl-2H-1,2,3-oxadiazin-2-yl]-3-oxopropoxy}-4-oxobutanoic acid (3), were synthesised. The chemical structures of these two compounds were elucidated by a combination of NMR and MS analysis. Compound 3 showed cytotoxicity against the ACHN (0.73 ± 0.10 µM) and Hepa-1c1c7 (0.91 ± 0.08 µM) tumour cell lines. Similarly, compound 3 significantly decreased cathepsin B activity in ACHN and Hepa-1c1c7 tumour cell lines at concentrations of 1.52 ± 0.13 nM and 1.76 ± 0.24 nM, respectively. In addition, compound 3 showed no in vivo toxicity in a murine model treated with a dose of 4 mg/kg body weight.


Subject(s)
Cathepsin B , Nostoc , Animals , Mice , Molecular Docking Simulation , Cell Line, Tumor , Molecular Structure
8.
Allergol Immunopathol (Madr) ; 51(3): 108-110, 2023.
Article in English | MEDLINE | ID: mdl-37169567

ABSTRACT

INTRODUCTION: Wilson disease is characterized by an alteration in copper metabolism that causes its accumulation in different tissues. Its diagnosis is established by the combination of clinical manifestations and paraclinical and genetic studies. Bruton agammaglobulinemia is an X-linked recessive hereditary disease belonging to the group of primary immunodeficiencies and is produced by mutation in the Bruton tyrosine kinase (BTK) gene. CASE REPORT: A 14-year-old Colombian patient with clinical characteristics of Bruton agammaglobulinemia presented with liver disease and clinically and molecularly diagnosed with Wilson disease. DISCUSSION: Bruton agammaglobulinemia and Wilson disease are considered rare diseases because of their low prevalence. We report for the first time a pediatric patient from southwestern Colombia presenting with both entities, and diagnosed clinically and molecularly, an association so far not reported in the literature.


Subject(s)
Agammaglobulinemia , Genetic Diseases, X-Linked , Hepatolenticular Degeneration , Adolescent , Humans , Agammaglobulinaemia Tyrosine Kinase/genetics , Agammaglobulinemia/diagnosis , Agammaglobulinemia/genetics , Genetic Diseases, X-Linked/diagnosis , Genetic Diseases, X-Linked/genetics , Hepatolenticular Degeneration/diagnosis , Hepatolenticular Degeneration/genetics , Mutation/genetics , Protein-Tyrosine Kinases/genetics
9.
Allergol. immunopatol ; 51(3): 108-110, 01 mayo 2023.
Article in English | IBECS | ID: ibc-219827

ABSTRACT

Introduction: Wilson disease is characterized by an alteration in copper metabolism that causes its accumulation in different tissues. Its diagnosis is established by the combination of clinical manifestations and paraclinical and genetic studies. Bruton agammaglobulinemia is an X-linked recessive hereditary disease belonging to the group of primary immunodeficiencies and is produced by mutation in the Bruton tyrosine kinase (BTK) gene. Case report: A 14-year-old Colombian patient with clinical characteristics of Bruton agammaglobulinemia presented with liver disease and clinically and molecularly diagnosed with Wilson disease. Discussion: Bruton agammaglobulinemia and Wilson disease are considered rare diseases because of their low prevalence. We report for the first time a pediatric patient from southwestern Colombia presenting with both entities, and diagnosed clinically and molecularly, an association so far not reported in the literature (AU)


Subject(s)
Humans , Male , Adolescent , Hepatolenticular Degeneration/diagnosis , Hepatolenticular Degeneration/genetics , Agammaglobulinemia/diagnosis , Agammaglobulinemia/genetics , Mutation
10.
Acta Cardiol ; 78(1): 55-63, 2023 Feb.
Article in English | MEDLINE | ID: mdl-34979883

ABSTRACT

BACKGROUND: Distal transradial access (dTRA) as a refinement of the conventional transradial access (TRA) has advantages in terms of risk of radial artery occlusion (RAO). In order to evaluate the real-world feasibility and safety of dTRA as the default access site for routine coronary angiography (CAG) and percutaneous coronary intervention (PCI) in a Latin-American centre, this prospective observational registry was conducted. METHODS: Consecutive patients with a prior assessment for CAG and/or PCI were enrolled in this single-centre prospective registry from October 2018 to March 2019. The primary endpoints were the success rate of CAG and PCI. Secondary endpoints included the success rate of puncture of the distal radial artery, complications at the puncture site and puncture time. RESULTS: The success rates of CAG and PCI were 100% (155/155) and 97% (69/71), respectively. Puncture time and fluoroscopic time were 52 ± 19 seconds and 16.3 ± 35.4 minutes, respectively. Haemostasis time was 142 ± 45 min. A total of 19 (12.5%) puncture site complications occurred, including 18 (11.6%) minor haematomas and one (0.6%) arterial perforation, in which the artery was patent at the one-month follow-up. Five patients complained of left thumb numbness at a one-month follow-up. No distal radial artery occlusion, pseudoaneurysm, or arteriovenous fistula occurred. CONCLUSIONS: The success and complication rates of ldTRA support the feasibility and safety of this procedure using the appropriate materials in previously selected patients.


Subject(s)
Arterial Occlusive Diseases , Percutaneous Coronary Intervention , Humans , Coronary Angiography/adverse effects , Coronary Angiography/methods , Percutaneous Coronary Intervention/adverse effects , Percutaneous Coronary Intervention/methods , Prospective Studies , Feasibility Studies , Radial Artery , Treatment Outcome
11.
Rev. cuba. med ; 61(4)dic. 2022.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1441700

ABSTRACT

Introducción: La asociación entre la retinopatía hipertensiva y cardiopatías coronarias es un tema de una larga controversia. La retinopatía hipertensiva ha sido definida como un predictor de mortalidad y morbilidad en pacientes hipertensos desde hace mucho tiempo. Además, estudios recientes han demostrado que la microvasculatura retiniana refleja la patología en los pequeños vasos sistémicos, incluida la microcirculación coronaria. Objetivos: El objetivo fue realizar una revisión sistemática y un análisis cualitativo y cuantitativo mediante un metanálisis para determinar la asociación entre la retinopatía hipertensiva y cardiopatías coronarias. Métodos: Se realizó la búsqueda sistemática de estudios relacionados con el tema. La fuente de búsqueda fue PubMed y Google Scholar. La revisión sistemática y meta-análisis se desarrollaron con las pautas Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA). Resultados: Se demostró que existe una asociación entre la retinopatía hipertensiva y la enfermedad de la arteria coronaria. (P=0,01; RR 1,29; IC 95 %: 1,06 a 1,56), se evaluó la hipertrofia ventricular izquierda como desenlace, se encontró un efecto estadísticamente significativo que asocia a la retinopatía hipertensiva con la hipertrofia ventricular izquierda (p=0,03; RR: 1,71; IC 95 %: 1,31 a 2,24). Conclusiones: Se encontró asociación entre retinopatía hipertensiva y las cardiopatías coronarias más frecuentes (Enfermedad coronaria e HVI).


Introduction: The association between hypertensive retinopathy and coronary heart disease is a subject of long-standing controversy. Hypertensive retinopathy has long been defined as a predictor of mortality and morbidity in hypertensive patients. In addition, recent studies have shown that the retinal microvasculature reflects pathology in small systemic vessels, including the coronary microcirculation. Objectives: The aim was to perform a systematic review and a qualitative and quantitative analysis by meta-analysis to determine the association between hypertensive retinopathy and coronary heart disease. Methods: A systematic search for studies related to the topic was performed. The search source was PubMed and Google Scholar. The systematic review and meta-analysis were developed using the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines. Results: An association between hypertensive retinopathy and coronary artery disease was demonstrated (P=0.01; RR 1.29; 95 % CI: 1.06 to 1.56), left ventricular hypertrophy was evaluated as an outcome, a statistically significant effect was found associating hypertensive retinopathy with left ventricular hypertrophy (P=0.03; RR: 1.71; 95 % CI: 1.31 to 2.24). Conclusions: An association was found between hypertensive retinopathy and the most frequent coronary heart diseases (coronary artery disease and LVH).

12.
Int J Mol Sci ; 23(20)2022 Oct 21.
Article in English | MEDLINE | ID: mdl-36293551

ABSTRACT

Central nervous system tumors are the most common solid neoplasia during childhood and represent one of the leading causes of cancer-related mortality. Tumors arising from astrocytic cells (astrocytomas) are the most frequently diagnosed, and according to their histological and pathological characteristics, they are classified into four categories. However, an additional layer of molecular classification considering the DNA sequence of the tumorigenesis-associated genes IDH1/2 and H3F3A has recently been incorporated into the classification guidelines. Although mutations in H3F3A are found exclusively in a subtype of grade IV pediatric astrocytoma, mutations in IDH1/2 genes are very rare in children under 14 years of age. The transcriptomic profiles of astrocytoma in adults and children have been extensively studied. However, there is scarce information on these profiles in pediatric populations considering the status of tumorigenesis-associated genes. Therefore, here we report the transcriptomic landscape of the four grades of pediatric astrocytoma by RNA sequencing. We found several well-documented biological functions associated with the misregulated genes in the four grades of astrocytoma, as well as additional biological pathways. Among the four grades of astrocytoma, we found shared misregulated genes that could have implications in tumorigenesis. Finally, we identified a transcriptional signature for almost all grades of astrocytoma that could be used as a transcription-based identification method.


Subject(s)
Astrocytoma , Brain Neoplasms , Adult , Child , Humans , Transcriptome , Brain Neoplasms/pathology , Astrocytoma/pathology , Mutation , Carcinogenesis
13.
Biomedica ; 42(3): 531-540, 2022 09 02.
Article in English, Spanish | MEDLINE | ID: mdl-36122292

ABSTRACT

Introduction: Hematopoietic stem cell transplantation is the curative option for some diseases and is increasing patient survival. The health-related quality of life in these patients is not systematically evaluated. Objectives: The present study sought to describe the health-related quality of life and complications in children who underwent hematopoietic stem cell transplantation. Materials and methods: A cross-sectional study was conducted on pediatric transplanted survivors. Health-related quality of life was measured using the KIDSCREEN-27 scale and Short Form-12 (SF-12) in patients between 8 and 14 years of age and those over 14 years, respectively. Statistical analysis was performed using STATA 12 software. We used the Rasch model person parameter estimates translated into T-values to score the questionnaire. Results: A total of 42 children answered the questionnaires. The most frequent adverse events were chronic graft Vs. host disease and endocrine complications. According to European norm data in the KIDSCREEN-12 scale, scores for the school dimension and social and peer support were below the 50th. percentile. The group administered immunosuppressants had lower scores on the physical component of the SF-12™ scale. Conclusions: In general, the KIDSCREEN-27 does appear to suggest some quality-of-life deficit in younger children. The scales showed reliability in this population.


Introducción. El trasplante de células madre hematopoyéticas es la opción curativa para algunas enfermedades y está aumentando el tiempo de supervivencia de los pacientes. La calidad de vida relacionada con la salud en estos pacientes no se evalúa de manera sistemática. Objetivos. Describir la calidad de vida relacionada con la salud y las complicaciones en niños con trasplante de células madre hematopoyéticas. Materiales y métodos. Es un estudio transversal en pacientes pediátricos sobrevivientes al trasplante. Se midió la calidad de vida relacionada con la salud, utilizando el cuestionario KIDSCREEN-27 en pacientes entre 8 y 14 años y la SF-12™ (Short Form-12) en pacientes mayores de 14 años. El análisis estadístico se realizó en el software Stata 12. Utilizamos el modelo de Rasch, trasladando estimación de parámetros a valores t para obtener el resultado de los cuestionarios. Resultados. En total, 42 pacientes respondieron alguno de los cuestionarios. Los eventos adversos más frecuentes fueron "enfermedad crónica de injerto Vs. contra huésped" y "complicaciones endocrinas". De acuerdo con la normalidad de datos del KIDSCREEN-27, los puntajes de las dimensiones "ambiente escolar" y "soporte social y pares" fueron inferiores al percentil 50. En el cuestionario SF-12™, el grupo que utilizaba inmunosupresores tuvo un menor puntaje en el componente físico. Conclusiones. En general, los resultados del KIDSCREEN-27 sugieren un cierto déficit de calidad de vida en pacientes entre 8 y 14 años. Los cuestionarios mostraron confiabilidad en la muestra.


Subject(s)
Hematopoietic Stem Cell Transplantation , Quality of Life , Child , Colombia , Cross-Sectional Studies , Humans , Immunosuppressive Agents , Reproducibility of Results
14.
Biomédica (Bogotá) ; 42(3): 531-540, jul.-set. 2022. tab, graf
Article in Spanish | LILACS | ID: biblio-1403604

ABSTRACT

Introducción. El trasplante de células madre hematopoyéticas es la opción curativa para algunas enfermedades y está aumentando el tiempo de supervivencia de los pacientes. La calidad de vida relacionada con la salud en estos pacientes no se evalúa de manera sistemática. Objetivos. Describir la calidad de vida relacionada con la salud y las complicaciones en niños con trasplante de células madre hematopoyéticas. Materiales y métodos. Es un estudio transversal en pacientes pediátricos sobrevivientes al trasplante. Se midió la calidad de vida relacionada con la salud, utilizando el cuestionario KIDSCREEN-27 en pacientes entre 8 y 14 años y la SF-12™ (Short Form-12) en pacientes mayores de 14 años. El análisis estadístico se realizó en el software Stata 12. Utilizamos el modelo de Rasch, trasladando estimación de parámetros a valores t para obtener el resultado de los cuestionarios. Resultados. En total, 42 pacientes respondieron alguno de los cuestionarios. Los eventos adversos más frecuentes fueron "enfermedad crónica de injerto Vs. contra huésped" y "complicaciones endocrinas". De acuerdo con la normalidad de datos del KIDSCREEN-27, los puntajes de las dimensiones "ambiente escolar" y "soporte social y pares" fueron inferiores al percentil 50. En el cuestionario SF-12™, el grupo que utilizaba inmunosupresores tuvo un menor puntaje en el componente físico. Conclusiones. En general, los resultados del KIDSCREEN-27 sugieren un cierto déficit de calidad de vida en pacientes entre 8 y 14 años. Los cuestionarios mostraron confiabilidad en la muestra.


Introduction: Hematopoietic stem cell transplantation is the curative option for some diseases and is increasing patient survival. The health-related quality of life in these patients is not systematically evaluated. Objectives: The present study sought to describe the health-related quality of life and complications in children who underwent hematopoietic stem cell transplantation. Materials and methods: A cross-sectional study was conducted on pediatric transplanted survivors. Health-related quality of life was measured using the KIDSCREEN-27 scale and Short Form-12 (SF-12) in patients between 8 and 14 years of age and those over 14 years, respectively. Statistical analysis was performed using STATA 12 software. We used the Rasch model person parameter estimates translated into T-values to score the questionnaire. Results: A total of 42 children answered the questionnaires. The most frequent adverse events were chronic graft Vs. host disease and endocrine complications. According to European norm data in the KIDSCREEN-12 scale, scores for the school dimension and social and peer support were below the 50th. percentile. The group administered immunosuppressants had lower scores on the physical component of the SF-12™ scale. Conclusions: In general, the KIDSCREEN-27 does appear to suggest some quality-of-life deficit in younger children. The scales showed reliability in this population.


Subject(s)
Quality of Life , Bone Marrow Transplantation , Child , Mental Health
15.
Pediatr Transplant ; 26(7): e14348, 2022 11.
Article in English | MEDLINE | ID: mdl-35781747

ABSTRACT

BACKGROUND: Fanconi anemia is a congenital disorder belonging to bone marrow syndromes, with a risk of developing malignancy. Hematopoietic stem cell transplantation is the only curative treatment in these cases. Here, we aimed to report our clinical experience in pediatric patients with Fanconi anemia treated with haploidentical stem cell transplantation and post-transplant cyclophosphamide, an alternative strategy. METHODS: We performed a case report based on clinical records of two patients who signed the informed consent form and were treated at Fundación Valle del Lili. RESULT: Two pediatric patients, both with reduced-intensity conditioning, prophylaxis for acute graft-versus-host disease with post-transplant cyclophosphamide. They achieved primary neutrophil/platelets engraftment, and 100% chimerism. Had grade I or II graft-versus-host disease resolved? Currently are alive and in complete remission. CONCLUSIONS: The use of mismatched related donors for haploidentical stem cell transplantation and post-transplant cyclophosphamide might be a promising option, and well-tolerated in pediatric patients. Serial chimerism can be useful during follow-up.


Subject(s)
Fanconi Anemia , Graft vs Host Disease , Hematopoietic Stem Cell Transplantation , Child , Cyclophosphamide/therapeutic use , Fanconi Anemia/complications , Fanconi Anemia/therapy , Graft vs Host Disease/drug therapy , Graft vs Host Disease/etiology , Graft vs Host Disease/prevention & control , Hematopoietic Stem Cell Transplantation/adverse effects , Humans , Transplantation Conditioning/adverse effects
16.
Allergol Immunopathol (Madr) ; 50(4): 17-22, 2022.
Article in English | MEDLINE | ID: mdl-35789399

ABSTRACT

INTRODUCTION: In recent decades, there has been a growing increase in the diagnosis of patients with inborn errors of the immune system, formerly known as primary immunodeficiency disorders (PIDs). Timely diagnosis remains a challenge due to low clinical suspicion and poor education on the subject. It is estimated that between 70% and 90% of these pathologies remain underdiagnosed in our environment. OBJECTIVE: The objective of this study is to characterize the demographic and clinical presentation of pediatric group patients with inborn errors of the immune system in a Colombian tertiary hospital. METHODS: Retrospective descriptive study of 306 patients with a diagnosis of innate errors of the immune system who consulted the PID clinic between 2011 and 2018 in a high-complexity institution in Cali, Colombia. RESULTS: Three-hundred and six patients were included. The median age was 4 years (IQR 2.3-7.7 years), and 59.5% of the patients were male. According to the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency classification for inborn errors of the immune system, the most common group was antibody deficiency in 74.8% (n˂229), especially in the age group between 1 and 5 years. The least frequent in our population was complement deficiency. Of the warning signs stipulated for these pathologies, the most frequent were the (1) need for intravenous antibiotics (32%), (2) difficulty growing (15.7%), (3) four or more episodes of ear infection (10.8%), and (4) abscesses in organs or cutaneous abscesses (12.7%). No patient reported two or more episodes of pneumonia or sinusitis, and only 5.8% of the patients received a bone marrow transplant. CONCLUSIONS: Innate errors of the immune system require an early diagnosis with follow-up from an early age to ensure adequate management and follow-up in order to reduce morbidity and mortality. It is imperative to sensitize the medical population about the existence of these pathologies so that early intervention can be carried out, which improves the quality of life of patients and their families.


Subject(s)
Abscess , Quality of Life , Child , Child, Preschool , Colombia/epidemiology , Female , Humans , Immune System , Infant , Male , Retrospective Studies , Tertiary Care Centers
18.
Blood Res ; 57(1): 34-40, 2022 Mar 31.
Article in English | MEDLINE | ID: mdl-35256547

ABSTRACT

Background: Antifungal prophylaxis is recommended for hematopoietic stem cell transplantation (HSCT) to decrease the incidence of invasive fungal infections (IFI). This study aimed to compare the two groups of antifungal prophylaxis in pediatric patients undergoing allogeneic HSCT. Methods: This observational, analytic, retrospective cohort study compared the incidence of IFI with antifungal prophylaxis with voriconazole vs. other antifungals in the first 100 days after allogeneic HSCT in patients aged <18 years between 2012 and 2018. The statistical analysis included univariate and multivariate analyses and determination of the cumulative incidence of invasive fungal infection by the Kaplan‒Meier method using STATA 14 statistical software. Results: A total of 139 allogeneic HSCT were performed. The principal diagnosis was acute leukemia (63%). The 75% had haploidentical donors, and 50% used an antifungal in the month before transplantation. Voriconazole (69%) was the most frequently administered antifungal prophylaxis. The cumulative incidence of IFI was 5% (7 cases). Of the patients with IFIs, four began prophylaxis with voriconazole, one with caspofungin, and one with fluconazole. Additionally, six were possible cases, one was proven (Candida parapsilosis), and 1/7 died. Conclusion: There were no differences in the incidence of IFI between patients who received prophylaxis with voriconazole and other antifungal agents.

19.
Polymers (Basel) ; 13(11)2021 Jun 02.
Article in English | MEDLINE | ID: mdl-34199390

ABSTRACT

ND1 subunit possesses the majority of the inhibitor binding domain of the human mitochondrial respiratory complex I. This is an attractive target for the search for new inhibitors that seek mitochondrial dysfunction. It is known, from in vitro experiments, that some metabolites from Annona muricata called acetogenins have important biological activities, such as anticancer, antiparasitic, and insecticide. Previous studies propose an inhibitory activity of bovine mitochondrial respiratory complex I by bis-tetrahydrofurans acetogenins such as annocatacin B, however, there are few studies on its inhibitory effect on human mitochondrial respiratory complex I. In this work, we evaluate the in silico molecular and energetic affinity of the annocatacin B molecule with the human ND1 subunit in order to elucidate its potential capacity to be a good inhibitor of this subunit. For this purpose, quantum mechanical optimizations, molecular dynamics simulations and the molecular mechanics/Poisson-Boltzmann surface area (MM/PBSA) analysis were performed. As a control to compare our outcomes, the molecule rotenone, which is a known mitochondrial respiratory complex I inhibitor, was chosen. Our results show that annocatacin B has a greater affinity for the ND1 structure, its size and folding were probably the main characteristics that contributed to stabilize the molecular complex. Furthermore, the MM/PBSA calculations showed a 35% stronger binding free energy compared to the rotenone complex. Detailed analysis of the binding free energy shows that the aliphatic chains of annocatacin B play a key role in molecular coupling by distributing favorable interactions throughout the major part of the ND1 structure. These results are consistent with experimental studies that mention that acetogenins may be good inhibitors of the mitochondrial respiratory complex I.

20.
World Allergy Organ J ; 14(3): 100527, 2021 Mar.
Article in English | MEDLINE | ID: mdl-33747343

ABSTRACT

BACKGROUND: The current literature describes the characteristics of some skin manifestations in the context of primary immunodeficiency diseases (PIDs), also known as inborn errors of the immune system. However, there are hardly any data on the epidemiological trends of skin manifestations and PIDs in Latin America (LA). We aimed to describe the characteristics of patients with skin manifestations and the diagnosis of a PID treated at a tertiary hospital in Colombia. METHODS: This was a retrospective observational study. Data were taken from the institutional database of pediatric PIDs, which includes 306 patients under 18 years of age who attended a tertiary care center in Cali, Colombia for inpatient or outpatient services between December 2013 and December 2018. A trained third-year dermatology resident reviewed the electronic clinical records of all the patients in the database and double-checked patients who presented with cutaneous signs and symptoms. RESULTS: A total of 83 patients out of the original 306 patients (27.1%) presented with some type of cutaneous manifestation. Of these patients, 56.6% had atopic dermatitis, 56.6% reported at least one episode of skin infection, and some of the patients had both of these manifestations. Infections were more frequent in the PID group of combined immunodeficiency associated with well-defined syndromes and atopic dermatitis in the group of antibody deficiencies. CONCLUSIONS: It is important to recognize dermatological clinical characteristics in patients with PIDs. More studies are necessary to establish recommendations regarding the approach of diagnosis and management of these patients.

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