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1.
Pediatr Rep ; 13(3): 483-489, 2021 Aug 07.
Artigo em Inglês | MEDLINE | ID: mdl-34449684

RESUMO

Common variable immunodeficiency (CVID) is a primary immune deficit (PID) mainly characterized by hypogammaglobulinemia. In addition to increased susceptibility to infections and several immune-mediated manifestations, patients with CVID frequently develop bronchiectasis because of recurrent respiratory infections. This occurrence could be more likely if the diagnosis of CVID is delayed, as it often happens in less resourced clinical settings. A 15-year-old female patient was admitted to a tertiary hospital in Kazakhstan for consultation regarding a previous and established diagnosis of bronchiectasis. The clinical history was characterized by recurrent respiratory infections for several years, in addition to the development of a mixed restrictive-obstructive respiratory syndrome. Therefore, she underwent chest computerized tomography, which confirmed the presence of multiple and bilateral bronchiectasis. The clinical discussion on this patient highlighted that serum immunoglobulins were never measured previously and, thus, their assessment was strongly recommended. Based on that, a diagnosis of CVID was finally achieved, and the patient started the appropriate immunoglobulin replacement therapy. To our knowledge, this report is the first English-language publication on CVID and bronchiectasis from Central Asia. Bronchiectasis is currently an important medical problem in developing countries and populations with low socioeconomic status, where the diagnosis of the underlying cystic fibrosis and non-cystic fibrosis comorbidities can be delayed and more difficult than in countries with more accessible health care systems and facilities. This case report emphasized this important clinical issue in Central Asia and should raise the medical attention and awareness of this health problem, in order to improve the diagnostic timing and rate.

2.
World J Gastroenterol ; 27(25): 3825-3836, 2021 Jul 07.
Artigo em Inglês | MEDLINE | ID: mdl-34321847

RESUMO

Ordinary chronic pancreatitis is a well-known risk factor for pancreatic cancer, whereas such an association with autoimmune pancreatitis (AIP) is widely debated. Due to the rarity of the latter disorder, there are few specific clinical and epidemiological studies investigating the relation between AIP and pancreatic cancer, which do not seem to support it. However, these studies are affected by several limitations and, therefore, a link between AIP (and, specifically, type 1 AIP) and pancreatic cancer cannot be ruled out definitively on this basis. Moreover, several immunopathological aspects of type 1 AIP and, in general, immunoglobulin G4-related disease can create an immunological context that may impair the tumoral immunosurveillance and promote the pancreatic carcinogenesis and its progression. In detail, Th2 immunological dominance, type 2 macrophage polarization and basophil infiltration observed in type 1 AIP, may play a permissive role in creating a favorable immunological environment for pancreatic carcinogenesis, in addition to the immunosuppressive therapies that can be used in these patients.


Assuntos
Doenças Autoimunes , Pancreatite Autoimune , Neoplasias Pancreáticas , Pancreatite Crônica , Doenças Autoimunes/diagnóstico , Doenças Autoimunes/epidemiologia , Diagnóstico Diferencial , Humanos , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/epidemiologia , Pancreatite Crônica/diagnóstico , Pancreatite Crônica/epidemiologia
3.
World J Gastroenterol ; 27(19): 2251-2256, 2021 May 21.
Artigo em Inglês | MEDLINE | ID: mdl-34040319

RESUMO

Celiac Disease (CD) had been considered uncommon in Asia for a long time. However, several studies suggested that, in the Indian subcontinent and Middle East countries, CD is present and as prevalent as in Western countries. Outside these Asian regions, the information about the epidemiology of CD is still lacking or largely incomplete for different and variable reasons. Here, we discuss the epidemiological aspects and the diagnostic barriers in several Asian regions including China, Japan, Southeast Asia and Russia/Central Asia. In some of those regions, especially Russia and Central Asia, the prevalence of CD is very likely to be underestimated. Several factors may, to a different extent, contribute to CD underdiagnosis (and, thus, underestimation of its epidemiological burden), including the poor disease awareness among physicians and/or patients, limited access to diagnostic resources, inappropriate use or interpretation of the serological tests, absence of standardized diagnostic and endoscopic protocols, and insufficient expertise in histopathological interpretation.


Assuntos
Doença Celíaca , Ásia/epidemiologia , Ásia Sudeste , Doença Celíaca/diagnóstico , Doença Celíaca/epidemiologia , China/epidemiologia , Humanos , Japão , Oriente Médio/epidemiologia , Prevalência , Federação Russa
4.
Front Cell Infect Microbiol ; 11: 625162, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33680992

RESUMO

The human salivary microbiota includes hundreds of bacterial species. Alterations in gut microbiota have been explored in Celiac Disease (CD), but fewer studies investigated the characteristics of salivary microbiome in these patients, despite the potential implications in its pathogenesis. Indeed, some recent studies suggested that the partial digestion of gluten proteins by some bacteria may affect the array of gluten peptides reaching the gut and the way by which those are presented to the intestinal immune system. The available clinical studies investigating the salivary microbiota in children and adults, are insufficient to make any reliable conclusion, even though some bacterial species/phyla differences have been reported between celiac patients and controls. However, the salivary microbiome could correlate better with the duodenal microbiota, than the fecal one. Therefore, further clinical studies on salivary microbiome by different and independent research groups and including different populations, are advisable in order to explore the usefulness of the salivary microbiome analysis and understand some aspects of CD pathogenesis with potential clinical and practical implications.


Assuntos
Doença Celíaca , Microbioma Gastrointestinal , Microbiota , Adulto , Criança , Duodeno , Glutens , Humanos
6.
Intern Emerg Med ; 16(2): 325-331, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32472479

RESUMO

Behçet's disease (BD) is a systemic vasculitis whose prevalence varies according to the ethnicity. HLA-B*51 is a well-known genetic factor predisposing to BD: populations with higher HLA-B*51 allelic frequencies are also those with a greater disease prevalence, as occurs in the Silk Road countries. However, the BD prevalence is unknown in many countries, including Kazakhstan and, in general, Central Asia, despite the location on or near the Silk Road. Due to the lack of clinical studies from this area, we analyzed the bone marrow donors registry in Kazakhstan, in order to assess the HLA-B*51 allelic and carrier frequencies, which resulted to be 8.14 and 15.28%, respectively. Through a comparison with the same parameters in other countries (by performing a data search in the medical literature and in the Allele Frequency Net Database), we might speculate a BD prevalence in Kazakhstan of 10-15/100.000 at least. This result should prompt a greater clinical consideration of this disease in Central Asia, as well as the publication of clinical data from this geographic area.

7.
Infez Med ; 28(4): 551-557, 2020 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-33257630

RESUMO

This article reports the largest nosocomial outbreak of B. cepacia-related Hospital Acquired Infectios (HAIs) and the epidemiological investigations leading to identification of ultrasound gel as a direct means of infection transmission. Multiple environmental sampling was conducted to identify the source and route of infection. The samples were collected from all sources considered to be potential reservoirs of B. cepacia. Standard methods for pathogen isolation and antibiotic sensitivity testing were used. In all, 61 patients developed B. cepacia-related sepsis and this agent was isolated only from ultrasonography gel. All patients required the placement of a central venous line to receive the chemotherapy for the underlying hematologic disease. The hospital outbreak persisted after identification of the source of infection and it took more than four months to be completely eradicated after the first cases. B. cepacia is a serious threat for hospitalized patients needing invasive procedures, including the central line placement for chemotherapy, regardless of the need of any intensive care. Implementation of protocols for active surveillance of HAIs should also target this opportunistic agent and include periodic sterility control of commonly used medical materials, including ultrasonography gel and equipment.

8.
Cell Immunol ; 358: 104215, 2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-33137647

RESUMO

Omalizumab is an anti-IgE humanized monoclonal antibody approved for the treatment of severe asthma and chronic spontaneous urticaria. Omalizumab binds free serum IgE and antagonizes its interaction with FcεRI, which is considered the main pharmacodynamic mechanism responsible for the clinical response to the treatment. The reduction of IgE serum concentration down-regulates the cellular expression of FcεRI on basophils. However, the biological events occurring on basophils during the therapy with omalizumab are multiple and complex. Here we review the current evidence regarding the specific biological effects of omalizumab on basophils in patients with asthma and chronic spontaneous urticaria. In addition to the modulation of IgE receptors, omalizumab may affect basophils homeostasis, intra-cellular signaling, cellular responsiveness/activation and cytokine release. These effects may be partially responsible for the clinical success of omalizumab and potentially provide useful biological markers for future assessment of the clinical response to the treatment. However, further investigation is required to better elucidate the role of basophils during the treatment with omalizumab.


Assuntos
Asma/tratamento farmacológico , Basófilos/efeitos dos fármacos , Omalizumab/farmacologia , Urticária/tratamento farmacológico , Animais , Antialérgicos/farmacologia , Antiasmáticos/farmacologia , Asma/metabolismo , Asma/patologia , Basófilos/imunologia , Basófilos/metabolismo , Basófilos/patologia , Biomarcadores/metabolismo , Urticária Crônica/tratamento farmacológico , Urticária Crônica/metabolismo , Urticária Crônica/patologia , Citocinas/metabolismo , Humanos , Ensaios Clínicos Controlados Aleatórios como Assunto , Urticária/metabolismo , Urticária/patologia
9.
Toxins (Basel) ; 12(11)2020 10 28.
Artigo em Inglês | MEDLINE | ID: mdl-33126405

RESUMO

Chronic rhinosinusitis (CRS) is thought to be a multifactorial disease that includes a direct involvement of bacteria that trigger inflammation and contribute to CRS pathogenesis. Staphylococcus aureus infection and persistence is associated with chronic rhinosinusitis (CRS), and it may be particularly relevant in the form with nasal polyps (CRSwNP). The large array of exotoxins deployed by S. aureus is instrumental for the bacterium to warrant its infection and dissemination in different human body districts. Here, we analyze the common Th2 environment in CRSwNP and prospect a possible dynamic role played by S. aureus leukocidins in promoting this chronic inflammation, considering leukocidin ED (LukED) as a strong prototype candidate worth of therapeutic investigation. CCR5 is an essential target for LukED to exert its cytotoxicity towards T cells, macrophages and dendritic cells. Therefore, CCR5 blockade might be an interesting therapeutic option for CRS and, more specifically, persistent and relapsing CRSwNP. In this perspective, the arsenal of CCR5 antagonists being developed to inhibit HIV-1 entry (CCR5 being the major HIV-1 co-receptor) could be easily repurposed for CRS therapeutic investigation. Finally, direct targeting of LukED by neutralizing antibodies could represent an important additional solution to S. aureus infection.

10.
Biology (Basel) ; 9(10)2020 Sep 23.
Artigo em Inglês | MEDLINE | ID: mdl-32977704

RESUMO

Basophils are the rarest cell population in the blood. Even though basophils are known to participate in some allergic reactions and immune responses to parasitic infections, their immunological role is still largely elusive. Recent evidence has suggested that in some murine models of systemic lupus erythematosus and lupus-like nephritis, basophils may also be implicated in autoimmunity processes by promoting autoantibody production and tissue injury. We conducted a systematic search to collect the available evidence on basophils' potential immunomodulatory role in autoimmunity and, particularly, systemic lupus erythematosus. We identified several articles investigating basophils' role in murine models of lupus (n = 3) and in patients affected with systemic lupus erythematosus (n = 8). Even though the alteration of the "adaptive" immune response is considered the main immunopathological event in systemic lupus erythematosus, the contribution from the mechanisms of "innate" immunity and, particularly, basophils may be relevant as well, by modulating the activation, polarization, and survival of lymphocytes.

11.
Antivir Chem Chemother ; 28: 2040206620961712, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32972196

RESUMO

Macrolides are a large group of antibiotics characterised by the presence of a macro-lactone ring of variable size. The prototype of macrolide antibiotics, erythromycin was first produced by Streptomyces and associated species more than half a century ago; other related drugs were developed. These drugs have been shown to have several pharmacological properties: in addition to their antibiotic activity, they possess some anti-inflammatory properties and have been also considered against non-bacterial infections. In this review, we analysed the available clinical evidences regarding the potential anti-viral activity of macrolides, by focusing on erythromycin, clarithromycin and azithromycin. Overall, there is no significant evidences so far that macrolides might have a direct benefit on most of viral infections considered in this review (RSV, Influenza, coronaviruses, Ebola and Zika viruses). However, their clinical benefit cannot be ruled out without further and focused clinical studies. Macrolides may improve the clinical course of viral respiratory infections somehow, at least through indirect mechanisms relying on some and variable anti-inflammatory and/or immunomodulatory effects, in addition to their well-known antibacterial activity.


Assuntos
Antibacterianos/farmacologia , Antivirais/farmacologia , Infecções por Coronavirus/tratamento farmacológico , Macrolídeos/farmacologia , Pneumonia Viral/tratamento farmacológico , Animais , Antibacterianos/farmacocinética , Antibacterianos/uso terapêutico , Antivirais/farmacocinética , Antivirais/uso terapêutico , Betacoronavirus/efeitos dos fármacos , COVID-19 , Humanos , Macrolídeos/farmacocinética , Macrolídeos/uso terapêutico , Pandemias , SARS-CoV-2
13.
World J Gastroenterol ; 26(12): 1365-1381, 2020 Mar 28.
Artigo em Inglês | MEDLINE | ID: mdl-32256023

RESUMO

BACKGROUND: Celiac Disease (CD) is an immune-mediated disorder, in which the HLA immunogenetic background (DQ2 and DQ8 heterodimers) and environmental trigger (gluten) are well established. Indeed, both factors are necessary - but not sufficient - to develop CD. However, it is very likely that CD is underdiagnosed in both developing and developed countries, due to several aspects, including the fact that a lot of patients present mild and/or atypical symptoms, without the presence of any recognized risk factors. Therefore, the possibility and feasibility of widened screening strategies to identify CD patients are debated. AIM: To provide further evidence of the main epidemiological importance of HLA-DQB1*02 allele in the population of CD patients. METHODS: We performed a systematic search in PubMed, EMBASE, Cochrane, Web of Science and Scopus databases, in order to produce a systematic review assessing the carrier frequency of HLA-DQB1*02 allele in the celiac population. Following the PRISMA guidelines, we retrieved all the original articles describing CD patients' HLA-DQB1 genotype in such a way that could allow to assess the HLA-DQB1*02 carrier frequency among CD patients, along with the evidence of the appropriate diagnostic work-up to achieve a correct and final diagnosis of CD. RESULTS: The final output of this systematic search in the medical literature consisted of 38 studies providing the appropriate HLA-DQB1 genotype information of the respective CD population. According to this systematic review, including a pool of 4945 HLA-DQ genotyped CD patients, the HLA-DQB1*02 carrier frequency was 94.94%, meaning that only 5.06% of CD patients were completely lacking this allelic variant. Interestingly, if we consider only the studies whereby the prevalence of CD patients affected with type 1 diabetes mellitus was supposed or clearly established to be very low, the frequency of non-HLA-DQB1*02 carriers among CD patients dropped to 3.65%. CONCLUSION: Such a high carrier frequency of the HLA-DQB1*02 allelic variant (which is > 95%-96% in CD patients without risk factors, like type 1 diabetes mellitus comorbidity) might be exploited to consider a cost-effective and widened screening approach. If a sustainable strategy could be implemented through a low-cost targeted genetic test to detect the individual presence of HLA-DQB1*02 allele, an appropriate algorithm for serological screening in individuals resulting to be genetically predisposed to CD, might be considered.


Assuntos
Doença Celíaca/genética , Frequência do Gene/genética , Predisposição Genética para Doença/epidemiologia , Cadeias beta de HLA-DQ/análise , Adolescente , Adulto , Alelos , Criança , Pré-Escolar , Feminino , Predisposição Genética para Doença/genética , Testes Genéticos , Genótipo , Humanos , Lactente , Masculino , Adulto Jovem
14.
PLoS One ; 15(1): e0226546, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31895924

RESUMO

BACKGROUND: Celiac disease (CD) is a systemic immune-mediated disorder developing in HLA genetically predisposed individuals carrying HLA-DQ2 and/or HLA-DQ8 molecules. Recent evidences supported a predominant importance of HLA-DQB1 locus and, in particular, HLA-DQB1*02 alleles. This diagnosis is poorly considered in Kazakhstan, because of the assumption that CD is not prevalent in this population. OBJECTIVE: To demonstrate that the genetic predisposition to CD in Kazakhstan is not negligible and is actually comparable to Western populations. METHODS: Through the analysis of HLA-DQ genotypes of healthy bone marrow donors from Kazakhstan's national registry, we estimated the HLA-related genetic predisposition to CD in the country. RESULTS: We demonstrated that the frequency of CD-related HLA-DQB1 alleles and, as a consequence, of predisposed individuals to CD in Kazakhstan is significant and comparable to countries with the highest disease prevalence. CONCLUSION: Considering the dietary style in Kazakhstan, including wheat as a staple food, these results provided a preliminary background of knowledge to expect a significant CD prevalence in Kazakhstan and Central Asia by implementing appropriate and cost-effective diagnostic strategies.


Assuntos
Doença Celíaca/epidemiologia , Predisposição Genética para Doença , Cadeias beta de HLA-DQ/genética , Polimorfismo Genético , Padrões de Prática Médica/estatística & dados numéricos , Adolescente , Adulto , Alelos , Estudos de Casos e Controles , Doença Celíaca/genética , Feminino , Genótipo , Humanos , Cazaquistão/epidemiologia , Masculino , Pessoa de Meia-Idade , Prevalência , Adulto Jovem
16.
J Microbiol Immunol Infect ; 53(1): 188-189, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31153829

RESUMO

The findings by Yang-TI et al. suggested that macrolide-resistance and/or delayed appropriate antimicrobial treatment may contribute to the development of Mycoplasma pneumoniae-related extra-pulmonary diseases in children. Indeed, these conditions can have variable clinical expression and severity, and may arise in immunologically predisposed children after recurrent/persistent exposure to Mycoplasma pneumoniae.


Assuntos
Anti-Infecciosos , Pneumonia por Mycoplasma , Antibacterianos , Criança , Humanos , Macrolídeos , Mycoplasma pneumoniae
17.
Rheumatol Int ; 40(5): 695-702, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-31529231

RESUMO

Macrophage activation syndrome (MAS) is a potentially fatal complication of a number of rheumatological conditions, but few studies assessed it in juvenile dermatomyositis (JDM). Indeed, MAS is not considered as a frequent complication of JDM, but its occurrence could be under-estimated. In order to address this issue, we performed a revision of the available medical literature, describing and assessing patients with both MAS and JDM. After retrieving 253 records initially, 11 papers were selected as appropriate for our research objective, which provided a total of 12 patients affected with both MAS and JDM. Our pooled case series suggested that MAS in JDM may not be very rare, even though no final conclusion about its incidence and mortality rate can be made. However, JDM-related MAS seems to be difficult to treat, since methylprednisolone pulse therapy alone was not sufficient in most cases. Moreover, MAS in JDM patients often occurred at the onset of the rheumatic disease, before the final diagnosis of JDM could be established. Finally, MAS criteria validated for systemic Juvenile Idiopathic Arthritis (sJIA) resulted to be a very useful guidance to diagnose MAS in JDM patients as well, but their reliability may not be absolute. Therefore, cohort and multicenter studies are needed to assess the incidence and improve the diagnostic criteria for MAS in JDM patients.


Assuntos
Dermatomiosite/complicações , Síndrome de Ativação Macrofágica/etiologia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Síndrome de Ativação Macrofágica/diagnóstico , Síndrome de Ativação Macrofágica/fisiopatologia , Masculino
18.
Microb Pathog ; 139: 103863, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31712120

RESUMO

Mycoplasma pneumoniae (M. pneumoniae) is a small bacterium characterized by the absence of cell wall. It is a human pathogen causing upper and lower respiratory infections, both in adults and children. However, it is also considered to be implicated in the pathogenesis of several types of extra-respiratory diseases, including some gastrointestinal disorders. The liver involvement in children during or after M. pneumoniae infections is analyzed and discussed in this review. Through a systematic literature search, it is evidenced that M. pneumoniae is not infrequently associated with alteration of liver function, but rarely causes acute and severe hepatitis in children. M. pneumoniae should be considered as an unusual cause of acute hepatitis in children, whenever the most common hepatotropic viruses have been excluded. The pathogenesis of M. pneumoniae-related hepatitis is likely to be immune-mediated: both the innate and adaptive immune responses may play a fundamental role. However, the exact pathological mechanisms have to be elucidated yet. Further clinical studies are needed in order to understand the actual relevance of this microorganism in liver disease and its pathogenesis.


Assuntos
Hepatite/complicações , Pneumonia por Mycoplasma/complicações , Pneumonia por Mycoplasma/microbiologia , Doença Aguda , Criança , Bases de Dados Factuais , Gastroenteropatias/complicações , Hepatite/imunologia , Hepatite/microbiologia , Humanos , Fígado , Mycoplasma pneumoniae , Pneumonia por Mycoplasma/imunologia
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