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BACKGROUND: 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, noninfectious complications are a major challenge among CVID patients. METHODS: All CVID patients registered in the national database were included in this retrospective cohort study. Patients were divided into 2 groups based on the presence of B-cell lymphopenia. Demographic characteristics, laboratory findings, noninfectious organ involvement, autoimmunity, and lymphoproliferative diseases were evaluated. RESULTS: Among 387 enrolled patients, 66.4% were diagnosed with noninfectious complications and 33.6% with isolated 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 more frequent among patients with B-cell lymphopenia. As for organ involvement, the 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 than that of other types of autoimmunity not associated with B cell-lymphopenia. Furthermore, hematological cancers, particularly lymphoma, were the most common type of malignancy. The mortality rate was 24.5%, and respiratory failure and malignancies were the most common causes of death, with no significant differences between the 2 groups. CONCLUSIONS: Considering that some of the noninfectious complications might be associated with B-cell lymphopenia, regular patient monitoring and follow-up with proper medication (in addition to immunoglobulin replacement therapy) are highly recommended to prevent sequelae and increase patient quality of life.
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Linfócitos B , Imunodeficiência de Variável Comum , Linfopenia , Humanos , Imunodeficiência de Variável Comum/complicações , Imunodeficiência de Variável Comum/imunologia , Feminino , Masculino , Adulto , Estudos Retrospectivos , Linfócitos B/imunologia , Pessoa de Meia-Idade , Linfopenia/imunologia , Adulto Jovem , Autoimunidade , Adolescente , Idoso , CriançaRESUMO
BACKGROUND: Increased psychological pressure on oral healthcare professionals (OHP) due to COVID-19 has been shown, yet little is known about the long-term psychological impacts. We aimed to study the psychological impact of COVID-19 and associated factors including perceived risk and preparedness and vaccination status among OHP in the first year after the lockdown period in Norway. METHODS: A structured questionnaire sent electronically to dentists, dental hygienists and dental assistants inquired experiences and perceptions during the second year following the outbreak in Norway. The questionnaire comprised a COVID-19 fear scale and questions about risk perception, preparedness and vaccination status. Exploratory factor analysis (EFA) and Structural Equation Modeling (SEM) were used to assess psychological impact, perception of risk and preparedness according to vaccination status of the respondents. RESULTS: The majority of the 708 respondents were female (92.8%), had ten or more years of work experience (67.1%), and worked in public dental clinics (95.9%). Fears and concerns related to COVID-19 were common, 72.6% feared getting infected and 85.4% feared infecting others. Of the 642 respondents who agreed that their workplaces handled the situation well, 55.6% were fully vaccinated. Three factors were retrieved from EFA: Insecurity, Instability and Infection. SEM showed that females were more concerned with Infection, and respondents with long clinical experience were less likely to express fear about Instability. Fully vaccinated individuals felt more insecure about becoming infected, and those agreeing that their workplaces handled the current situation well were concerned with Insecurity. CONCLUSIONS: Despite widespread perception of adequate preparedness and high vaccine coverage, a considerable psychological impact and high levels of fear of COVID-19 were observed among the majority of OHP. Fully vaccinated individuals had a larger psychological burden than not fully vaccinated and those with unknown vaccination status. These findings can inform means and interventions to reduce negative impacts of fear in populations with a high psychological burden.
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COVID-19 , Serviços de Saúde Bucal , Humanos , Feminino , Masculino , COVID-19/epidemiologia , COVID-19/prevenção & controle , Controle de Doenças Transmissíveis , Noruega/epidemiologia , VacinaçãoRESUMO
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.
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BACKGROUND: The SARS-CoV-2 pandemic put a pressure on all healthcare professionals and has affected the delivery of health care services globally. There is a need to understand the impact on different health care professionals in different countries. The aim of the present study was to explore the psychological impact of the pandemic among dental staff in Norway in relation to background characteristics, work situation and preparedness of the service. METHODS: A structured questionnaire sent electronically to dentists, dental hygienists and dental assistants inquired information about the lockdown period in Norway (13 March-17 April 2020). Distributions of background characteristics, perceptions of preparedness and psychological impact were calculated. Exploratory factor analysis was performed, and Structural Equation Models (SEMs) were used to compare psychological impact between dental professionals treating patients versus not during lockdown. RESULTS: Among the 1237 respondents, 58.8% worked clinically with patients. The majority were concerned of becoming infected (71.9%), of infecting others (85.4%) and/or of their family becoming infected (76.9%). Respondents who treated patients felt significantly more insecure about whether having become infected or not. The minority felt discriminated (6.7%), worried about death (11.7%), felt that life was threatening (9.8%) or felt loss of control of their lives (8.9%). More than 80% agreed that their workplace handled the situation well. Four factors were retrieved from the factor analysis. SEMs showed that gender and work experience had a significant effect on the factors Instability, Infection and Concerns. Respondents with work experience ≥10 years were less likely to express fear about Instability and Infection. Personnel reporting that their workplace had adequate equipment were also less concerned, however having adequate equipment did not reduce the factor Loss of control. CONCLUSION: The present study showed a considerable psychological impact of the COVID-19 pandemic on dental personnel in Norway regardless of working clinically with patients or not. However, working with patients increased the insecurity about own infection status and of infecting people close to them. A safe working environment and adequate infection control measures are associated with less fear of infection and feeling of instability.
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COVID-19 , Pandemias , Controle de Doenças Transmissíveis , Estudos Transversais , Pessoal de Saúde , Humanos , Noruega/epidemiologia , SARS-CoV-2 , Inquéritos e QuestionáriosRESUMO
Elastic electron-proton scattering (e-p) and the spectroscopy of hydrogen atoms are the two methods traditionally used to determine the proton charge radius, rp. In 2010, a new method using muonic hydrogen atoms1 found a substantial discrepancy compared with previous results2, which became known as the 'proton radius puzzle'. Despite experimental and theoretical efforts, the puzzle remains unresolved. In fact, there is a discrepancy between the two most recent spectroscopic measurements conducted on ordinary hydrogen3,4. Here we report on the proton charge radius experiment at Jefferson Laboratory (PRad), a high-precision e-p experiment that was established after the discrepancy was identified. We used a magnetic-spectrometer-free method along with a windowless hydrogen gas target, which overcame several limitations of previous e-p experiments and enabled measurements at very small forward-scattering angles. Our result, rp = 0.831 ± 0.007stat ± 0.012syst femtometres, is smaller than the most recent high-precision e-p measurement5 and 2.7 standard deviations smaller than the average of all e-p experimental results6. The smaller rp we have now measured supports the value found by two previous muonic hydrogen experiments1,7. In addition, our finding agrees with the revised value (announced in 2019) for the Rydberg constant8-one of the most accurately evaluated fundamental constants in physics.
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OBJECTIVES: Little is known about tissue changes underlying bone marrow lesions (BMLs) in non-weight-bearing joints with osteoarthritis (OA). Our aim was to characterize BMLs in OA of the hand using dynamic histomorphometry. We therefore quantified bone turnover and angiogenesis in subchondral bone at the base of the thumb, and compared the findings with control bone from hip OA. METHODS: Patients with OA at the base of the thumb, or the hip, underwent preoperative MRI to assess BMLs, and tetracycline labelling to determine bone turnover. Three groups were compared: trapezium bones removed by trapeziectomy from patients with thumb base OA (n = 20); femoral heads with (n = 24); and those without (n = 9) BMLs obtained from patients with hip OA who underwent total hip arthroplasty. RESULTS: All trapezium bones demonstrated MRI-defined BMLs. Compared with femoral heads without BMLs, the trapezia demonstrated significantly higher bone turnover (mean sd 0.2 (0.1) versus 0.01 (0.01) µm3/µm2/day), mineralizing surface (18.5% (13.1) versus 1.4% (1.3)) and vascularity (5.2% (1.1) versus 1.2% (0.6)). Femoral heads with BMLs exhibited higher bone turnover (0.3 (0.2) versus 0.2 (0.1) µm3/µm2/day), a higher mineralization rate (26.6% (10.6) versus 18.6% (11.9)) and greater trabecular thickness (301.3 µm (108) versus 163.6 µm (24.8)) than the trapezia. CONCLUSION: Bone turnover and angiogenesis were enhanced in BMLs of both the thumb base and hip OA, of which the latter exhibited the highest bone turnover. Thus, the increase in bone turnover in weight-bearing joints like the hip may be more pronounced than less mechanically loaded osteoarthritic joints demonstrating BMLs. The histological changes observed may explain the water signal from BMLs on MRI.Cite this article: M. Shabestari, N. J. Kise, M. A. Landin, S. Sesseng, J. C. Hellund, J. E. Reseland, E. F. Eriksen, I. K. Haugen. Enhanced angiogenesis and increased bone turnover characterize bone marrow lesions in osteoarthritis at the base of the thumb. Bone Joint Res 2018;7:406-413. DOI: 10.1302/2046-3758.76.BJR-2017-0083.R3.
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Over the past two decades, single-molecule techniques have evolved into robust tools to study many fundamental biological processes. The combination of optical tweezers with fluorescence microscopy and microfluidics provides a powerful single-molecule manipulation and visualization technique that has found widespread application in biology. In this combined approach, the spatial (~nm) and temporal (~ms) resolution, as well as the force scale (~pN) accessible to optical tweezers is complemented with the power of fluorescence microscopy. Thereby, it provides information on the local presence, identity, spatial dynamics, and conformational dynamics of single biomolecules. Together, these techniques allow comprehensive studies of, among others, molecular motors, protein-protein and protein-DNA interactions, biomolecular conformational changes, and mechanotransduction pathways. In this chapter, recent applications of fluorescence microscopy in combination with optical trapping are discussed. After an introductory section, we provide a description of instrumentation together with the current capabilities and limitations of the approaches. Next we summarize recent studies that applied this combination of techniques in biological systems and highlight some representative biological assays to mark the exquisite opportunities that optical tweezers combined with fluorescence microscopy provide.
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DNA/isolamento & purificação , Microscopia de Fluorescência/métodos , Pinças Ópticas , Proteínas/isolamento & purificação , Imagem Individual de Molécula/métodos , DNA/química , Mecanotransdução Celular , Microfluídica/métodos , Microscopia de Fluorescência/tendências , Nanotecnologia/tendências , Proteínas/química , Imagem Individual de Molécula/tendênciasRESUMO
BACKGROUND: The Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive immunodeficiency disorder, caused by mutations in the WAS protein (WASP) gene and characterised by thrombocytopenia, small platelets, eczema, and recurrent infections associated with increased risk of autoimmunity and malignancy disorders. The gene for WAS has been mapped to the short arm of the X chromosome at Xp 11.22-23 and early detection of patients and diagnosis of new mutation might reduce related complications and increase their life expectancy. Method and result: We found a novel mutation by sequence analysis of genomic DNA coding of a 9-month old boy suffering from WAS. The mutation was insertion G in exon 10 of WASP gene. The consequence of the G insertion is a premature stop immediately at amino acid 335 (N335X or p.G334GfsX1) and truncated protein. CONCLUSION: The mutation analysis is helpful for the diagnosis of WAS patients and also expanding the spectrum of WASP mutations for carrier detection and prenatal diagnosis
No disponible
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Humanos , Masculino , Lactente , Síndrome de Wiskott-Aldrich/complicações , Síndrome de Wiskott-Aldrich/imunologia , Eczema/complicações , Eczema/imunologia , Trombocitopenia/complicações , Trombocitopenia/imunologia , Genômica/métodos , Recidiva , Rinite/epidemiologia , Rinite/imunologia , Sinusite/epidemiologia , Sinusite/imunologia , Imunoglobulina G/análiseRESUMO
OBJECTIVE: Bone marrow lesions (BML), previously denoted bone marrow edema, are detected as water signals by magnetic resonance imaging (MRI). Previous histologic studies were unable to demonstrate any edematous changes at the tissue level. Therefore, our aim was to investigate the underlying biological mechanisms of the water signal in MRI scans of bone affected by BML. METHODS: Tetracycline labeling in addition to water sensitive MRI scans of 30 patients planned for total hip replacement surgery was undertaken. Twenty-one femoral heads revealed BML on MRI, while nine were negative and used as controls (CON). Guided by the MRI images cylindrical biopsies were extracted from areas with BML in the femoral heads. Tissue sections from the biopsies were subjected to histomorphometric image analyses of the cancellous bone envelope. RESULTS: Patients with BML exhibited an average 40- and 18-fold increase of bone formation rate and mineralizing surface, respectively. Additionally, samples with BML demonstrated 2-fold reduction of marrow fat and 28-fold increase of woven bone. Immunohistochemical analysis showed a 4-fold increase of angiogenesis markers CD31 and von Willebrand Factor (vWF) in the BML-group compared to CON. CONCLUSION: This study indicates that BML are characterized by increased bone turnover, vascularity and angiogenesis in keeping with it being a reparatory process. Thus, the water signal, which is the hallmark of BML on MRI, is most probably reflecting increased tissue vascularity accompanying increased remodeling activity.
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Osteoartrite do Quadril , Medula Óssea , Doenças da Medula Óssea , Remodelação Óssea , Humanos , Imageamento por Ressonância Magnética , Osteoartrite do JoelhoRESUMO
BACKGROUND: The Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive immunodeficiency disorder, caused by mutations in the WAS protein (WASP) gene and characterised by thrombocytopenia, small platelets, eczema, and recurrent infections associated with increased risk of autoimmunity and malignancy disorders. The gene for WAS has been mapped to the short arm of the X chromosome at Xp 11.22-23 and early detection of patients and diagnosis of new mutation might reduce related complications and increase their life expectancy. METHOD AND RESULT: We found a novel mutation by sequence analysis of genomic DNA coding of a 9-month old boy suffering from WAS. The mutation was insertion G in exon 10 of WASP gene. The consequence of the G insertion is a premature stop immediately at amino acid 335 (N335X or p.G334GfsX1) and truncated protein. CONCLUSION: The mutation analysis is helpful for the diagnosis of WAS patients and also expanding the spectrum of WASP mutations for carrier detection and prenatal diagnosis.
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Eczema/genética , Mutagênese Insercional/genética , Trombocitopenia/genética , Proteína da Síndrome de Wiskott-Aldrich/genética , Síndrome de Wiskott-Aldrich/genética , Análise Mutacional de DNA , Éxons/genética , Humanos , Lactente , Irã (Geográfico) , Masculino , LinhagemRESUMO
New results are reported from a measurement of π^{0} electroproduction near threshold using the p(e,e^{'}p)π^{0} reaction. The experiment was designed to determine precisely the energy dependence of s- and p-wave electromagnetic multipoles as a stringent test of the predictions of chiral perturbation theory (ChPT). The data were taken with an electron beam energy of 1192 MeV using a two-spectrometer setup in Hall A at Jefferson Lab. For the first time, complete coverage of the Ï_{π}^{*} and θ_{π}^{*} angles in the pπ^{0} center of mass was obtained for invariant energies above threshold from 0.5 up to 15 MeV. The 4-momentum transfer Q^{2} coverage ranges from 0.05 to 0.155 (GeV/c)^{2} in fine steps. A simple phenomenological analysis of our data shows strong disagreement with p-wave predictions from ChPT for Q^{2}>0.07 (GeV/c)^{2}, while the s-wave predictions are in reasonable agreement.
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BACKGROUND: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders (PID), with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease. OBJECTIVES: The current study was performed to evaluate and compare demographic and clinical data in the most common types of PAD. MATERIALS AND METHODS: We performed a retrospective review of the medical records of all PAD patients with a confirmed diagnosis of common variable immunodeficiency (CVID), hyper IgM syndrome (HIgM), selective IgA deficiency (SIgAD), and X-linked agammaglobulinemia (XLA) who were diagnosed during the last 30 years at the Children's Medical Center, Tehran, Iran. RESULTS: A total number of 280 cases of PAD (125 CVID, 32 HIgM, 63 SIgAD, and 60 XLA) were enrolled in the study. The median (range) age at the onset of disease in CVID, HIgM, SIgAD, and XLA was 2 (0-46), 0.91 (0-9), 1 (0-26), and 1 (0-10) years, respectively. Gastrointestinal infections were more prevalent in CVID patients, as were central nervous system infections in XLA patients. Autoimmune complications were more prevalent in HIgM patients, malignancies in CVID patients, and allergies in SIgAD patients. The mortality rate for CVID, HIgM, and XLA was 27.2%, 28.1%, and 25%, respectively. No deaths were reported in SIgAD patients. CONCLUSIONS: SIgAD patients had the best prognosis. While all PAD patients should be monitored for infectious complications, special attention should be paid to the finding of malignancy and autoimmune disorders in CVID and HIgM patients, respectively.
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Síndromes de Imunodeficiência/complicações , Adolescente , Adulto , Criança , Pré-Escolar , Imunodeficiência de Variável Comum/complicações , Feminino , Humanos , Síndromes de Imunodeficiência/mortalidade , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Centros de Atenção TerciáriaRESUMO
Background: Primary antibody deficiency (PAD) is the most common group of primary immunodeficiency disorders (PID), with a broad spectrum of clinical features ranging from severe and recurrent infections to asymptomatic disease. Objectives: The current study was performed to evaluate and compare demographic and clinical data in the most common types of PAD. Materials and Methods: We performed a retrospective review of the medical records of all PAD patients with a confirmed diagnosis of common variable immunodeficiency (CVID), hyper IgM syndrome (HIgM), selective IgA deficiency (SIgAD), and X-linked agammaglobulinemia (XLA) who were diagnosed during the last 30 years at the Childrens Medical Center, Tehran, Iran. Results: A total number of 280 cases of PAD (125 CVID, 32 HIgM, 63 SIgAD, and 60 XLA) were enrolled in the study. The median (range) age at the onset of disease in CVID, HIgM, SIgAD, and XLA was 2 (0-46), 0.91 (0-9), 1 (0-26), and 1 (0-10) years, respectively. Gastrointestinal infections were more prevalent in CVID patients, as were central nervous system infections in XLA patients. Autoimmune complications were more prevalent in HIgM patients, malignancies in CVID patients, and allergies in SIgAD patients. The mortality rate for CVID, HIgM, and XLA was 27.2%, 28.1%, and 25%, respectively. No deaths were reported in SIgAD patients. Conclusions: SIgAD patients had the best prognosis. While all PAD patients should be monitored for infectious complications, special attention should be paid to the finding of malignancy and autoimmune disorders in CVID and HIgM patients, respectively (AU)
Antecedentes: Las inmunodeficiencias humorales primarias (PAD) es el grupo más frecuente de inmunodeficiencias primarias (IDP), y engloba un amplio espectro de características clínicas, que van desde los pacientes con infecciones graves y recurrentes a los casos asintomáticos. Objetivos: El presente estudio se realizó para evaluar y comparar los datos demográficos y clínicos de los tipos más comunes de PAD. Materiales y Métodos: Se revisaron retrospectivamente, las historias clínicas de todos los pacientes con PAD con un diagnóstico confirmado de: inmunodeficiencia variable común (CVID), síndrome de hiper IgM (HIgM), deficiencia selectiva de IgA (SIgAD),y de agammaglobulinemia ligada al cromosoma X (XLA), que fueron diagnosticados durante los últimos 30 años, en el Centro Médico de Niños, Teherán, Irán. Resultados: Se incluyeron en este estudio un total de 280 casos de PAD, englobando 125 pacientes con CVID, 32 HIgM, 63 SIgAD, y 60 pacientes con XLA. La mediana (rango) de edad al inicio de la enfermedad en la CVID, HIgM, SIgAD y XLA fue: 2 (0-46), 0,91 (0-9), 1 (0-26) y 1 (0-10) años, respectivamente. Las infecciones gastrointestinales fueron más frecuentes en los pacientes con CVID, mientras que las infecciones del sistema nervioso central lo fueron en la XLA. Las complicaciones autoinmunes fueron más prevalentes en los pacientes con HIgM, los tumores malignos en las CVID y las enfermedades alérgicas en las SIgAD. La tasa de mortalidad de CVID, HIgM y XLA fue 27,2%, 28,1% y 25%, respectivamente. No hubo mortalidad en el grupo de pacientes con SIgAD. Conclusiones: Los pacientes con SIgAD tuvieron el mejor pronóstico. Aunque todos los pacientes con PAD deben ser controlados estrechamente para evitar las complicaciones infecciosas, se debe prestar especial atención a la aparición de enfermedades malignas y autoinmunes en los pacientes con CVID y HIgM, respectivamente (AU)
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Humanos , Imunodeficiência de Variável Comum/epidemiologia , Deficiência de IgA/epidemiologia , Hipergamaglobulinemia/epidemiologia , Agamaglobulinemia/epidemiologia , /estatística & dados numéricos , Infecções/imunologia , Síndromes de Imunodeficiência/epidemiologiaRESUMO
Estrogen deficiency promotes bone loss and skeletal muscle dysfunction. Peroxisome proliferator-activated receptors (PPARs) have 3 subtypes (α, δ, and γ). PPARγ agonists induce bone loss, whereas PPARα agonists increase bone mass. Although PPARδ agonists are known to influence skeletal muscle metabolism, the skeletal effects are unsettled. This study investigated the musculoskeletal effects of the PPARδ agonist GW501516 in ovariectomized (OVX) rats. Female Sprague Dawley rats, 12 weeks of age, were allocated to a sham-operated group and 3 OVX groups; high-dose GW501516 (OVX-GW5), low-dose GW501516 (OVX-GW1), and a control group (OVX-CTR), respectively (n = 12 per group). Animals received GW501516 or vehicle (methylcellulose) daily for 4 months by gavage. Bone mineral density (BMD) was assessed by dual x-ray absorptiometry at the femur, spine, and whole body. Bone microarchitecture at the proximal tibia was assessed by microcomputed tomography, and dynamic histomorphometry was performed. Quadriceps muscle morphology and the relative expression of mitochondrial proteins were analyzed. Bone metabolism markers and metabolic markers were measured in plasma. After 4 months, the OVX-GW5 group displayed lower femoral BMD than OVX-CTR. Trabecular separation was higher in the GW-treated groups, compared with OVX-CTR. The OVX-GW5 group also exhibited lower cortical area fraction and a higher structure model index than OVX-CTR. These effects coincided with impaired bone formation in both GW groups. The OVX-GW5 group displayed elevated triglyceride levels and reduced adiponectin levels, whereas no effects on muscle morphology or mitochondrial gene expression appeared. In summary, the PPARδ agonist GW501516 negatively affected bone properties in OVX rats, whereas no effects were detected in skeletal muscle.
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Músculo Esquelético/efeitos dos fármacos , Osteoblastos/efeitos dos fármacos , PPAR delta/agonistas , Tiazóis/farmacologia , Tíbia/efeitos dos fármacos , Absorciometria de Fóton , Animais , Composição Corporal/efeitos dos fármacos , Diferenciação Celular/efeitos dos fármacos , Linhagem Celular , Proliferação de Células/efeitos dos fármacos , Feminino , Imunoensaio , Ratos , Ratos Sprague-Dawley , Reação em Cadeia da Polimerase em Tempo RealRESUMO
OBJECTIVES: Tonsil and adenoid are part of waldeyers ring; the basic function of which are antibody formation, which later react against a grat variety of antigens. The Adenotonsillectomy is the most common operation in small children but the exact reasons of adenotonsillar hypertrophy remains unknown, some researches have shown that allergy may be at risk factor for adenotonsillar hypertrophy. METHODS: Thorough one year two separated groups of children at the ENT and allergy ward of childrens hospital was enrolled in the study. The study group consisted of 117 children between 1 and 14 years old (with average of 6) who had adenotonsillar hypertrophy. The control group consisted of 100 children in the similar age that had not adenotonsillar hypertrophy. Both groups were examined for the incidence of allergic disease, results of skin prick test, serum IgE levels and close contact to smoke. RESULTS: In the study group 70.3% of children with adenotonsillar hypertrophy had positive skin prick test. But only 10% of children in control group had positive skin prick test. Increased serum total IgE level was confirmed in 48% of children with positive skin prick test in study group were in close contact with smoker parents. CONCLUSION: Allergy and sensitivity to different kinds of allergens are important risk factors for adenotonsillar hypertrophy in children. Allergy control may have role in reducing the rate of adenotonsillectomy in children suffering allergic reactions with adenotonsillar hypertrophy.
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Tonsila Faríngea/patologia , Hipersensibilidade/epidemiologia , Tonsila Palatina/patologia , Tonsila Faríngea/imunologia , Adolescente , Distribuição por Idade , Alérgenos , Estudos de Casos e Controles , Distribuição de Qui-Quadrado , Criança , Pré-Escolar , Intervalos de Confiança , Feminino , Seguimentos , Humanos , Hipersensibilidade/diagnóstico , Hipertrofia/diagnóstico , Hipertrofia/epidemiologia , Hipertrofia/imunologia , Incidência , Lactente , Masculino , Tonsila Palatina/imunologia , Valores de Referência , Medição de Risco , Distribuição por Sexo , Testes Cutâneos/métodosRESUMO
T-B-NK+ severe combined immunodeficiency (SCID) is an autosomal recessive disease that is caused mainly by a defect in the recombination activating genes (RAG). Patients with SCID usually experience life-threatening opportunistic infections in early infancy and complications after vaccination with bacille Calmette-Guérin (BCG). We report a patient of consanguineous parents who was referred to our center with subaxillary lymphadenitis and respiratory distress. Laboratory studies confirmed the diagnosis of T-B-NK+ SCID and molecular studies revealed homozygous mutations in the RAG2 gene. The patient died despite administration of antituberculosis drugs, antibiotics, and intravenous immunoglobulin. Inoculation of live vaccines such as BCG should be postponed in families with a positive history of SCID until screening tests rule out this condition.
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Vacina BCG , Proteínas de Ligação a DNA/genética , Proteínas Nucleares/genética , Infecções Oportunistas/genética , Imunodeficiência Combinada Severa/genética , Tuberculose/genética , Antibacterianos/uso terapêutico , Linfócitos B/imunologia , Vacina BCG/administração & dosagem , Contraindicações , Proteínas de Ligação a DNA/imunologia , Evolução Fatal , Testes Genéticos , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Lactente , Masculino , Mutação , Proteínas Nucleares/imunologia , Infecções Oportunistas/complicações , Infecções Oportunistas/fisiopatologia , Infecções Oportunistas/terapia , Linhagem , Insuficiência Respiratória , Imunodeficiência Combinada Severa/complicações , Imunodeficiência Combinada Severa/fisiopatologia , Imunodeficiência Combinada Severa/terapia , Linfócitos T/imunologia , Tuberculose/complicações , Tuberculose/fisiopatologia , Tuberculose/terapia , Tuberculose dos LinfonodosRESUMO
BTK deficiency is a primary immunodeficiency disease characterized by the absence of circulating B cells and agammaglobulinemia. While recurrent bacterial infections are the most common manifestations, symptoms of allergy and asthma are rare. We present the case of a 7-year-old boy who presented with asthma symptoms, allergic rhinitis, and severe papular urticaria. He had a positive skin prick test to aeroallergens and food allergens. However, further laboratory tests revealed a low number of B cells and decreased serum levels of all immunoglobulin isotypes. Molecular analysis revealed a mutation in the BTK gene. Although patients with BTK deficiency seem to be protected from atopy, our patient had allergic symptoms suggesting a bias toward a type 2 helper T cell pattern in this case. Primary antibody deficiency should be considered in the differential diagnosis of pediatric allergy and asthma when respiratory infection persists despite appropriate treatment.