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1.
Clin Oncol (R Coll Radiol) ; 35(9): 576-585, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37301719

RESUMO

Reirradiation of the spine is carried out in 42% of patients who do not respond to treatment or have recurrent pain. However, there are few studies and data on the effect of reirradiation of the spine and the occurrence of acute and chronic side-effects caused by reirradiation, such as myelopathy, in these patients. This meta-analysis aimed to determine the safe dose in terms of biological effective dose (BED), cumulative dose and dose interval between BED1 and BED2 to decrease or prevent myelopathy and pain control in patients undergoing radiation therapy in the spinal cord. A search was carried out using EMBASE, MEDLINE, PUBMED, Google Scholar, Cochrane Collaboration library electronic databases, Magiran, and SID from 2000 to 2022 to recognise qualified studies. In total, 17 primary studies were applied to estimate the pooled effect size. The random effects model showed that the pooled BED in the first stage, the BED in the second stage and the cumulative BED1 and BED2 were estimated at 77.63, 58.35 and 115.34 Gy, respectively. Studies reported on dose interval. The results of a random effects model showed that the pooled interval was estimated at 13.86 months. The meta-analysis revealed that using appropriate BED1 and/or BED2 in a safe interval between the first and second phases of treatment can have an influential role in preventing or reducing the effects of myelopathy and regional control pain in spinal reirradiation.


Assuntos
Reirradiação , Doenças da Medula Espinal , Humanos , Reirradiação/efeitos adversos , Manejo da Dor , Doenças da Medula Espinal/etiologia , Bases de Dados Factuais , Dor
2.
Artigo em Inglês | MEDLINE | ID: mdl-37103527

RESUMO

BACKGROUND AND OBJECTIVE: Common variable immunodeficiency (CVID) is considered the most symptomatic type of inborn errors of immunity in humans. Along with infectious complications, which have numerous consequences, non-infectious complications are also a major challenge among CVID patients. METHODS: All registered CVID patients in the national database were included in this retrospective cohort study. Patients were divided into two groups based on the presence of B-cell lymphopenia. Demographic characteristics, laboratory findings, non-infectious organ involvements, autoimmunity, and lymphoproliferative diseases were evaluated. RESULTS: Among 387 enrolled patients, 66.4% were diagnosed with non-infectious complications; however, 33.6% had only infectious presentations. Enteropathy, autoimmunity, and lymphoproliferative disorders were reported in 35.1%, 24.3%, and 21.4% of patients, respectively. Some complications, including autoimmunity and hepatosplenomegaly, were reported to be significantly higher among patients with B-cell lymphopenia. Among organ involvement, dermatologic, endocrine and musculoskeletal systems were predominantly affected in CVID patients with B-cell lymphopenia. Among autoimmune manifestations, the frequency of rheumatologic, hematologic, and gastrointestinal autoimmunity was reported to be higher compared to other types of autoimmunity independent from the B cell-lymphopenia. Furthermore, hematological cancers, particularly lymphoma, were slightly introduced as the most common type of malignancy. Meanwhile, the mortality rate was 24.5%, and respiratory failure and malignancies were reported as the most common cause of death in our patients without significant differences between the two groups. CONCLUSION: Considering that some of the non-infectious complications might be associated with B-cell lymphopenia, therefore, regular patient monitoring and follow-up along with proper medications (besides immunoglobulins replacement therapy) are highly recommended to prevent further sequels and increase the patients' quality of life.

4.
Eur Ann Allergy Clin Immunol ; 55(1): 19-28, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-34918886

RESUMO

Summary: Background. Inborn errors of immunity (IEIs) are a group of heterogeneous disorders with inherited faults in the immune system that increase susceptibility to infections, malignancies, lymphoproliferation, and autoimmune/autoinflammatory disorders. Methods. We retrospectively studied the demographic characteristics, clinical features, and immunological profiles of the 90 IEIs patients, who were diagnosed and classified according to the European Society for Immunodeficiencies (ESID) and International Union of Immunological Societies (IUIS) criteria from July 2010 to June 2021. The study was carried out in the Non-communicable Diseases Research Center, Imam Ali Hospital, Alborz, Iran. Results. Within a period of 11 years, 53 (58.9%) males and 37 (41.1%) females were diagnosed and followed up for 20 IEI disorders. The median (IQR) age of onset, age of clinical diagnosis and diagnostic delay was 0.7 (0.08-2.0), 3.18 (1.0-8.0) and 1.5 (0.17-5.0) years, respectively. Twelve patients (36.4%) had a positive family history of IEI, and the majority of patients (84.5%) had recurrent infections. Pneumonia (51.7%) was the most common clinical manifestation among IEI patients, followed by skin complications (46.2%). The most frequently diagnosed IEI was immunoglobulin A deficiency (IgAD) (14.4%) and severe combined immunodeficiency (SCID) (11.1%). Predominantly antibody deficiencies group (36.7%) was the most common category, followed by combined immunodeficiencies with associated or syndromic features group (27.8%). Conclusions. IEIs have different patterns within populations with high consanguinity. There is a need to search for underlying genetic and epigenetic factors in most common IEIs in Alborz.


Assuntos
Síndromes de Imunodeficiência , Doenças da Imunodeficiência Primária , Masculino , Feminino , Humanos , Estudos Retrospectivos , Irã (Geográfico)/epidemiologia , Diagnóstico Tardio , Síndromes de Imunodeficiência/diagnóstico , Síndromes de Imunodeficiência/epidemiologia , Síndromes de Imunodeficiência/genética
5.
Actas Dermosifiliogr ; 113(2): 157-165, 2022 Feb.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-35244561

RESUMO

Coronavirus disease-19 (COVID-19) is an emerging health situation caused by the "severe acute respiratory syndrome coronavirus 2" (SARS-CoV-2). The ongoing COVID-19 pandemic which emerged from the Chinese city of Wuhan in December 2019 has spread to over 188 countries and infected over 100 million people across the globe in over one year. Most common symptoms of COVID-19 include fever and respiratory illness. Among extrapulmonary signs associated with COVID-19, dermatological manifestations have been increasingly reported from different geographical regions. The exact incidence or prevalence of COVID-19 associated skin manifestation remains largely unknown and the pathophysiological mechanisms are still unclear. In this article, we have attempted to give a comprehensive overview of what has been learned an year into the pandemic on the epidemiology, clinical and histopathological features, pathophysiological mechanisms and clinical management of COVID-19 associated cutaneous manifestations.

6.
Actas Dermosifiliogr ; 113(2): T157-T165, 2022 Feb.
Artigo em Espanhol | MEDLINE | ID: mdl-34580541

RESUMO

Coronavirus disease-19 (COVID-19) is an emerging health situation caused by the "severe acute respiratory syndrome coronavirus 2" (SARS-CoV-2). The ongoing COVID-19 pandemic which emerged from the Chinese city of Wuhan in December 2019 has spread to over 188 countries and infected over 100 million people across the globe in over one year. Most common symptoms of COVID-19 include fever and respiratory illness. Among extrapulmonary signs associated with COVID-19, dermatological manifestations have been increasingly reported from different geographical regions. The exact incidence or prevalence of COVID-19 associated skin manifestation remains largely unknown and the pathophysiological mechanisms are still unclear. In this article, we have attempted to give a comprehensive overview of what has been learned a year into the pandemic on the epidemiology, clinical and histopathological features, pathophysiological mechanisms and clinical management of COVID-19 associated cutaneous manifestations.

7.
Clin Transl Oncol ; 24(5): 757-769, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-34839457

RESUMO

Colorectal cancer (CRC) is one of the leading causes of mortality among cancers. Many aspects of this cancer are under investigation to find established markers of diagnosis, prognosis, and also potential drug targets. In this review article, we are going to discuss the possible solution to all these aims by investigating the literature about cancer-associated fibroblasts (CAFs) involved in CRC. Moreover, we are going to review their interaction with the tumor microenvironment (TME) and vitamin D and their role in tumorigenesis and metastasis. Moreover, we are going to expand more on some markers produced by them or related to them including FAP, a-SMA, CXCL12, TGF- ß, POSTN, and ß1-Integrin. Some signaling pathways related to CAFs are as follows: FAK, AKT, activin A, and YAP/TAZ. Some genes related to the CAFs which are found to be possible therapeutic targets include COL3A1, JAM3, AEBP1 and, CAF-derived TGFB3, WNT2, and WNT54.


Assuntos
Fibroblastos Associados a Câncer , Neoplasias Colorretais , Fibroblastos Associados a Câncer/metabolismo , Carboxipeptidases/metabolismo , Carcinogênese/patologia , Neoplasias Colorretais/patologia , Fibroblastos/metabolismo , Humanos , Prognóstico , Proteínas Repressoras/metabolismo , Fator de Crescimento Transformador beta/metabolismo , Microambiente Tumoral
8.
Actas Dermosifiliogr ; 2021 Nov 27.
Artigo em Inglês | MEDLINE | ID: mdl-34866636

RESUMO

Coronavirus disease-19 (COVID-19) is an emerging health situation caused by the "severe acute respiratory syndrome coronavirus 2" (SARS-CoV-2). The ongoing COVID-19 pandemic which emerged from the Chinese city of Wuhan in December 2019 has spread to over 188 countries and infected over 100 million people across the globe in over one year. Most common symptoms of COVID-19 include fever and respiratory illness. Among extrapulmonary signs associated with COVID-19, dermatological manifestations have been increasingly reported from different geographical regions. The exact incidence or prevalence of COVID-19 associated skin manifestation remains largely unknown and the pathophysiological mechanisms are still unclear. In this article, we have attempted to give a comprehensive overview of what has been learned an year into the pandemic on the epidemiology, clinical and histopathological features, pathophysiological mechanisms and clinical management of COVID-19 associated cutaneous manifestations.


La enfermedad por coronavirus de 2019 (COVID-19) es una situación sanitaria emergente causada por el "síndrome respiratorio agudo severo por coronavirus 2" (SARS-CoV-2). La pandemia por COVID-19 en curso, que surgió de la ciudad china de Wuhan en Diciembre de 2019, se ha propagado en 188 países, y ha infectado a más de 100 millones de personas a nivel mundial a lo largo de un año. Los síntomas más comunes de la COVID-19 incluyen fiebre y enfermedad respiratoria. Entre los signos extrapulmonares asociados a COVID-19 se han reportado cada vez más manifestaciones dermatológicas en las diferentes regiones geográficas. La incidencia o prevalencia exactas de las manifestaciones cutáneas asociadas a la COVID-19 son bastante desconocidas, y los mecanismos patofisiológicos siguen sin dilucidarse. En este artículo hemos tratado de aportar una visión general amplia de lo que hemos aprendido en un año de inmersión en la pandemia en cuanto a epidemiología y características clínicas e histopatológicas, mecanismos patofisiológicos y manejo clínico de las manifestaciones cutáneas asociadas a la COVID-19.

9.
Clin Transl Oncol ; 23(12): 2431-2447, 2021 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-34160771

RESUMO

Acute myeloid leukemia (AML), the most common form of leukemia amongst adults, is one of the most important hematological malignancies. Epidemiological data show both high incidence rates and low survival rates, especially in secondary cases among adults. Although classic and novel chemotherapeutic approaches have extensively improved disease prognosis and survival, the need for more personalized and target-specific methods with less side effects have been inevitable. Therefore, immunotherapeutic methods are of importance. In the following review, primarily a brief understanding of the molecular basis of the disease has been represented. Second, prior to the introduction of immunotherapeutic approaches, the entangled relationship of AML and patient's immune system has been discussed. At last, mechanistic and clinical evidence of each of the immunotherapy approaches have been covered.


Assuntos
Imunoterapia/métodos , Leucemia Mieloide Aguda/terapia , Humanos , Prognóstico
10.
Public Health ; 186: 44-51, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-32768623

RESUMO

BACKGROUND: Self-harm-related death is one of the most unfortunate, tragic, and regrettable types of death owing to injuries with a variety of socio-economic and cultural causes. The study aimed to determine the trend in the mortality of self-harm by sex and age at national and provincial levels in Iran over a period of 26 years. METHODS: The Iran Death Registration System (DRS), cemetery databanks in Tehran and Esfahan, and the national population and housing censuses of Iran were used for this study. Using a growth model, the population was estimated in the age groups. Incompleteness, misalignment, and misclassification in the DRS were all considered and addressed accordingly. We used a spatio-temporal and Gaussian process regression model to estimate mortality rates in children and adults. RESULTS: Over the study period, 67,670 deaths were estimated owing to self-harm across the country. The overall age-standardized mortality rate decreased from 4.32 per 100,000 (95% unit interface (UI): 3.25-5.75) to 2.78 (2.15-3.59) per 100,000 between 1990 and 2015, a reduction of approximately 35.65%. The M/F ratio was 2.03:1 with an annual percent change of -2.38% and -1.37% for women and men, respectively. The annual self-harm mortality rate was higher among individuals aged 15-24 years, as well as it was more in men during the study period. CONCLUSION: Mortality from self-harm has declined over the study period in Iran. Higher rates in men and in population aged 15-24 years, with considerable variation by province, were the distinguishing features of self-harm. Iran needs to improve monitoring through a comprehensive multisectoral strategy; and most importantly, provide timely, effective and low-cost preventive interventions.


Assuntos
Comportamento Autodestrutivo/mortalidade , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Censos , Criança , Bases de Dados Factuais , Feminino , Humanos , Irã (Geográfico)/epidemiologia , Masculino , Pessoa de Meia-Idade , Mortalidade/tendências , Comportamento Autodestrutivo/epidemiologia , Fatores Sexuais , Fatores de Tempo , Adulto Jovem
11.
Arch Osteoporos ; 15(1): 103, 2020 07 10.
Artigo em Inglês | MEDLINE | ID: mdl-32651719

RESUMO

Musculoskeletal diseases (MSDs) are the leading cause of disability and facing them demands updated reports on their burden for efficient policymaking. We showed Iran had the highest female-to-male ratio and highest increase in the burden of musculoskeletal diseases, in the past three decades, worldwide. We further confirmed the role of population aging as the main cause. PURPOSE: MSDs comprise most of the top causes of years lived with disability (YLDs) worldwide and are rapidly increasing in lower- and middle-income countries. Here, we present disability and mortality due to MSDs in Iran at the national level from 1990 to 2017. METHODS: We used Global Burden of Disease (GBD) 2017 Study data and standard methodology and presented the burden of MSDs in rates of years of life lost (YLLs), YLDs, and disability-adjusted life years (DALYs) during 1990-2017, for population aged ≥ 5 years old. We further explored attributable risk factors and decomposed the changing trend in DALYs to assess underlying causes. RESULTS: In Iran, MSDs were responsible for 1.82 million (95%uncertainty interval [UI] 1.3-2.4) DALYs, in 2017. During the past 28 years, with 1.75% annualized percentage change (APC), Iran had the highest percentage increase in the all-ages MSD DALYs rate worldwide, while the age-standardized DALYs APC was negligible. Low back pain was the greatest contributor to DALYs and caused 4.5% of total DALYs. The female population is experiencing considerably higher burden of MSDs, with 115% and 48% higher all-ages YLLs and YLDs rates per 100,000, respectively (YLLs 28.7; YLDs 2629.1), than males (YLLs 13.2; YLDs 1766.1). However, due to wide UIs, difference was not significant. Only 17.6% of MSD YLDs are attributable to assessed risk factors. CONCLUSION: Despite that MSDs are rising as an important cause of disability in Iran, these conditions are not sufficiently addressed in health policies. There is urgent need for cross-sectoral engagement, especially addressing the MSDs in females.


Assuntos
Carga Global da Doença , Doenças Musculoesqueléticas , Feminino , Saúde Global , Humanos , Irã (Geográfico)/epidemiologia , Expectativa de Vida , Masculino , Doenças Musculoesqueléticas/epidemiologia , Anos de Vida Ajustados por Qualidade de Vida
12.
Nat Commun ; 11(1): 2888, 2020 Jun 08.
Artigo em Inglês | MEDLINE | ID: mdl-32514054

RESUMO

Solids with competing interactions often undergo complex phase transitions with a variety of long-periodic modulations. Among such transition, devil's staircase is the most complex phenomenon, and for it, CeSb is the most famous material, where a number of the distinct phases with long-periodic magnetostructures sequentially appear below the Néel temperature. An evolution of the low-energy electronic structure going through the devil's staircase is of special interest, which has, however, been elusive so far despite 40 years of intense research. Here, we use bulk-sensitive angle-resolved photoemission spectroscopy and reveal the devil's staircase transition of the electronic structures. The magnetic reconstruction dramatically alters the band dispersions at each transition. Moreover, we find that the well-defined band picture largely collapses around the Fermi energy under the long-periodic modulation of the transitional phase, while it recovers at the transition into the lowest-temperature ground state. Our data provide the first direct evidence for a significant reorganization of the electronic structures and spectral functions occurring during the devil's staircase.

13.
Allergol Immunopathol (Madr) ; 48(4): 332-338, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32475613

RESUMO

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disease caused by interaction of genetic, epigenetic, and environmental factors. One of the important epigenetic factors in SLE would be methylation of immune-related genes, such as FOXP3, which plays a role in activating the regulation and also the function of T cells. To date, the relationship between levels of serum bio-markers and the susceptibility to lupus in children has not been well-understood. In this study, the involvement of etiologic factors, such as methylation of FOXP3 gene, was investigated in children with SLE. METHOD: Twenty-four female children with SLE and 25 female healthy subjects without any history of autoimmune and inflammatory diseases were included in this study. Blood samples were obtained and DNA was extracted from the blood cells. The bisulphite method was used to convert the DNA using the MethylEdge™ Bisulfite Conversion System Kit. Then, methylation of the gene was investigated using Real Time methylation specific PCR. RESULTS: The FOXP3 DNA methylation in patients and healthy subjects was significantly different. While the median unmethylated DNA in patients was 0.57±0.43, it was 0.97±0.83 in healthy subjects (P=0.012). The Demethylation Index in patients was 0.007±0.003, significantly lower than in controls (0.014±0.013; P=0.012). CONCLUSIONS: The FOXP3 gene methylation in children with SLE was significantly higher than healthy subjects, which could possibly affect the level of gene expression. Therefore, one of the causes of increased immune response in SLE can be the lower expression of FOXP3 by hypermethylation of this gene.


Assuntos
Fatores de Transcrição Forkhead/genética , Lúpus Eritematoso Sistêmico/genética , Adolescente , Criança , Metilação de DNA/genética , Feminino , Fatores de Transcrição Forkhead/sangue , Regulação da Expressão Gênica/genética , Humanos , Lúpus Eritematoso Sistêmico/sangue
14.
Allergol Immunopathol (Madr) ; 48(6): 711-719, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32404246

RESUMO

INTRODUCTION: The prevalence of undiagnosed primary immunodeficiency diseases is remarkably high and contributes to increasing the rate of morbidity and mortality among this group of patients. OBJECTIVE: To examine the 10 warning sign scoring system in patients suspected of primary immune deficiency and also estimate the diagnostic delay in patients with proven disease. METHODS: This descriptive cross-sectional study was carried out during the years 2015-2016 in Ali Asghar (AS) Clinic and Hospital. Two hundred patients with suspected primary immune deficiency disease were eligible for inclusion in the study. Multivariable logistic regression analysis was used to determine the relation between findings. RESULTS: In this study, the majority of suspected cases of immunodeficiency were males (57%) with a mean age of 3.33±2.89 years. Twenty-one (10.5%) patients were diagnosed with immunodeficiency disease. The mean diagnostic delay among primary immune deficient patients was 2.05±1.7 years. There was a significant relationship between having parental consanguinity (OR=2.68, 95% CI: 1.07-6.70), allergies (OR=5.03, 95% CI: 1.13-22.31), vaccine adverse effects (OR=9.31, 95% CI: 1.24-69.96) and primary immune deficiency diagnosis. No association was observed between age (OR=0.98, 95% CI: 0.84-1.14), gender (OR=0.99, 95% CI: 0.39-2.47), immune deficiency scoring (OR=0.68, 95% CI: 0.31-1.45) and primary immune deficiency diagnosis. CONCLUSION: Ten warning sign scoring system is of less value to consider a patient suspected of having primary immune deficiency. There is a meaningful delay in diagnosis of primary immune deficiencies especially in antibody deficiency defects which seeks further upgrading of knowledge in physicians.


Assuntos
Consanguinidade , Diagnóstico Tardio/estatística & dados numéricos , Doenças da Imunodeficiência Primária/epidemiologia , Vacinas/efeitos adversos , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Lactente , Irã (Geográfico)/epidemiologia , Modelos Logísticos , Masculino , Análise Multivariada , Prevalência , Doenças da Imunodeficiência Primária/diagnóstico , Doenças da Imunodeficiência Primária/imunologia , Fatores de Risco
15.
Allergol Immunopathol (Madr) ; 48(1): 62-66, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31477400

RESUMO

INTRODUCTION AND OBJECTIVES: Considering the possible roles of interleukin-23 receptor (IL-23R) gene in the pathogenesis of juvenile systemic lupus erythematosus (JSLE), the objective of this study was to elucidate whether polymorphisms of the IL23R are associated with susceptibility to JSLE in an Iranian population. MATERIALS AND METHODS: A case-control study on 62 patients with JSLE and 78 healthy controls was performed to investigate the associations of four single nucleotide polymorphisms (SNPs) in IL-23R gene, namely, rs7517847, rs10489629, rs11209026, and rs1343151, with susceptibility to JSLE, using real-time polymerase chain reaction Taqman genotyping technique. RESULTS: Analysis of allele and genotype frequency of four selected SNPs revealed statistically significant positive association between homozygous variant of rs7517847 (TT) (P, 0.02) and T allele at the same position (P, 0.01) with JSLE vulnerability. There was no significant association between other evaluated SNPs and JSLE susceptibility. CONCLUSION: These findings suggest that particular IL-23R gene variants could affect individual susceptibility to JSLE.


Assuntos
Predisposição Genética para Doença/genética , Lúpus Eritematoso Sistêmico/genética , Receptores de Interleucina/genética , Adolescente , Alelos , Estudos de Casos e Controles , Criança , Pré-Escolar , Feminino , Frequência do Gene , Estudos de Associação Genética , Genótipo , Humanos , Irã (Geográfico)/epidemiologia , Masculino , Polimorfismo de Nucleotídeo Único
16.
Public Health ; 170: 78-88, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30978579

RESUMO

OBJECTIVES: Comprehensive and up-to-date data on fatal injury trends are critical to identify challenges and plan priority setting. This study provides a comprehensive assessment of poisoning mortality trends across Iran. STUDY DESIGN: The data were gathered from various resources, including death registration systems, cemetery databases of Tehran and Esfahan, the Demographic and Health Survey of 2000, and three rounds of national population and housing censuses. METHODS: After addressing incompleteness for child and adult death data separately and using a spatio-temporal model and Gaussian process regression, the level and trend of child and adult mortality were estimated. For estimating cause-specific mortality, the cause fraction was calculated and applied to the level and trend of death. RESULTS: From 1990 to 2015, 40,586 deaths due to poisoning were estimated across the country. The poisoning-related age-standardized death rate per 100,000 was estimated to have changed from 3.08 (95% uncertainty interval [UI]: 2.32-4.11) in 1990 to 0.96 (95% UI: 0.73-1.25) in 2015, and the male/female ratio was 1.35 during 25 years of study with an annual percentage change of -5.4% and -4.0% for women and men, respectively. The annual mortality rate was higher among children younger than 5 years and the elderly population (≥70 years) in the study period. CONCLUSIONS: This study showed that mortality from poisoning declined in Iran over the period from 1990 to 2015 and varied by province. Understanding the reasons for the differences of poisoning mortality by province will help in developing and implementing measures to reduce this burden in Iran.


Assuntos
Intoxicação/mortalidade , Adolescente , Adulto , Idoso , Criança , Pré-Escolar , Bases de Dados Factuais , Feminino , Inquéritos Epidemiológicos , Humanos , Lactente , Irã (Geográfico)/epidemiologia , Masculino , Pessoa de Meia-Idade , Mortalidade/tendências , Adulto Jovem
18.
Allergol Immunopathol (Madr) ; 47(1): 90-104, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-29703631

RESUMO

T helper 9 (TH9) cells are considered as newly classified helper T cells that have an important role in the regulation of immune responses. Since these cells preferentially produce IL-9, these cells are termed TH9 cells. Recently, the role of TH9 and its signature cytokine (IL-9) has been investigated in a wide range of diseases, including autoimmunity, allergy, infections, cancer and immunodeficiency. Herein, we review the most recent data concerning TH9 cells and IL-9 as well as their roles in disease. These insights suggest that TH9 cells are a future target for therapeutic intervention.


Assuntos
Doenças do Sistema Imunitário/imunologia , Imunoterapia/métodos , Interleucina-9/imunologia , Linfócitos T Auxiliares-Indutores/imunologia , Animais , Autoimunidade , Humanos
19.
Br J Dermatol ; 179(5): 1033-1048, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-29791718

RESUMO

BACKGROUND: Alopecia areata is a disorder that results in nonscarring hair loss. The psychological impact can be significant, leading to feelings of depression and social isolation. Objectives In this article, we seek to review the pathophysiological mechanisms proposed in recent years in a narrative fashion. METHODS: We searched MEDLINE and Scopus for articles related to alopecia areata, with a particular emphasis on its pathogenesis. RESULTS: The main theory of alopecia areata pathogenesis is that it is an autoimmune phenomenon resulting from a disruption in hair follicle immune privilege. What causes this breakdown is an issue of debate. Some believe that a stressed hair follicle environment triggers antigen presentation, while others blame a dysregulation in the central immune system entangling the follicles. Evidence for the latter theory is provided by animal studies, as well investigations around the AIRE gene. Different immune-cell lines including plasmacytoid dendritic cells, natural killer cells and T cells, along with key molecules such as interferon-γ, interleukin-15, MICA and NKG2D, have been identified as contributing to the autoimmune process. CONCLUSIONS: Alopecia areata remains incurable, although it has been studied for years. Available treatment options at best are beneficial for milder cases, and the rate of relapse is high. Understanding the exact mechanisms of hair loss in alopecia areata is therefore of utmost importance to help identify potential therapeutic targets.


Assuntos
Alopecia em Áreas/imunologia , Doenças Autoimunes/imunologia , Folículo Piloso/imunologia , Privilégio Imunológico , Fatores Imunológicos/uso terapêutico , Alopecia em Áreas/tratamento farmacológico , Alopecia em Áreas/patologia , Animais , Doenças Autoimunes/tratamento farmacológico , Doenças Autoimunes/patologia , Células Dendríticas/imunologia , Células Dendríticas/metabolismo , Modelos Animais de Doenças , Folículo Piloso/citologia , Folículo Piloso/patologia , Antígenos de Histocompatibilidade Classe I/metabolismo , Humanos , Interferon gama/imunologia , Interferon gama/metabolismo , Interleucina-15/imunologia , Interleucina-15/metabolismo , Queratinócitos/imunologia , Queratinócitos/patologia , Células Matadoras Naturais/imunologia , Células Matadoras Naturais/metabolismo , Subfamília K de Receptores Semelhantes a Lectina de Células NK/imunologia , Subfamília K de Receptores Semelhantes a Lectina de Células NK/metabolismo , Recidiva , Linfócitos T/imunologia , Linfócitos T/metabolismo , Fatores de Transcrição/genética , Fatores de Transcrição/imunologia , Resultado do Tratamento , Proteína AIRE
20.
Allergol Immunopathol (Madr) ; 46(6): 594-598, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-29739689

RESUMO

INTRODUCTION AND OBJECTIVES: Pediatric Systemic Lupus Erythematosus (pSLE) is an autoimmune disorder of children. Early disease onset raises the probability of genetic etiology and it is more severe than adult SLE. PATIENTS AND METHODS: Herein an eight-year-old girl with pSLE from consanguineous parents is reported. RESULTS: Although she was diagnosed as pSLE since the age of two years, Whole Exome Sequencing (WES) revealed a rare stop-gained C>T mutation in C1QA gene. The variant was validated and segregated in patient and the family. Furthermore, serum levels of the C1q protein were measured and found to be much lower than normal ranges. CONCLUSIONS: This study indicated that C1Q deficiency should be considered as a differential diagnosis of pSLE. Therefore, measurement of C1q should be recommended in all cases with pSLE.


Assuntos
Complemento C1q/genética , Genótipo , Lúpus Eritematoso Sistêmico/genética , Deleção de Sequência/genética , Criança , Pré-Escolar , Consanguinidade , Feminino , Homozigoto , Humanos , Linhagem , Sequenciamento do Exoma
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