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Development of an Expert-Based Scoring System for Early Identification of Patients with Inborn Errors of Immunity in Primary Care Settings - the PIDCAP Project.

Early diagnosis of inborn errors of immunity (IEIs) has been shown to reduce mortality, morbidity, and healthcare costs. The need for early diagnosis has led to the development of computational tools that trigger earlier clinical suspicion by physicians. Primary care professionals serve as the first line for improving early diagnosis. To this end, a computer-based tool (based on extended Jeffrey Modell Foundation (JMF) Warning Signs) was developed to assist physicians with diagnosis decisions for IEIs in the primary care setting. Two expert-guided scoring systems (one pediatric, one adult) wer

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… The following individuals were excluded: pediatric patients with selective IgA deficiency, usually asymptomatic; adult individuals with less than 5 diagnosis entries in the primary care registry (corresponding to p10 of the number of adult diagnosis distribution) were considered non-representative on …

Гут Микробиом и мальгический энцефаломилит/хронический синдром утомления (ME/CFS): Понимание механизмов борьбы с заболеваниями.

Myalgic Encephalomyelitis/Chronic Fatige (ME/CFS) — клиническая болезнь, характерная особенность которой заключается в постэкспертальной болезни (PEM). Она может влиять на многие органы и системы, что приводит к серьезному ухудшению качества жизни пациентов. Несмотря на многочисленные постинфекционные, иммунологические, неврологические, метаболические и эндокринные изменения, не существует ни окончательного диагностического маркера, ни утвержденного лечения. Этиология и патофизиология по-прежнему не до конца понимаются; однако появляющиеся данные свидетельствуют о том, что кишечная микробиомия

Электронная добыча медицинских карт свидетельствует о воздействии иммунного состояния пациента на клинические результаты имплантации биоматериалов после повреждения мышечной ткани скелета.

Имплантация медицинских устройств и биоматериалов может помочь восстановить форму и функцию отсутствующей или поврежденной ткани. Известно, что иммунная система играет важнейшую роль как в позитивном, так и в негативном исходе этих имплантированных материалов. Реакция иностранного организма характеризуется осаждением белка и свертыванием, за которым следуют макрофагенное воспаление, расстройство фагоцитоза, образование гигантских клеток и, в конечном счете, фиброз. Это может препятствовать функционированию имплантированных устройств (например. Инсулиновые насосы), а также космезис (например, к

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… B cell immunodeficiencies included Bruton's agammaglobulinemia, selective IgA deficiency, common variable immunodeficiency, etc . ECM- and hydrogel-treated patients demonstrated more variation based on immune compartment (T cell vs. …

Yellow nail syndrome: a review.

Yellow nail syndrome (YNS; OMIM 153300, ORPHA662) is a very rare disorder that almost always occurs after 50 years of age but a juvenile or familial form has also been observed. YNS is diagnosed based on a triad associating yellow nail discoloration, pulmonary manifestations (chronic cough, bronchiectasia, pleural effusion) and lower limb lymphedema. Chronic sinusitis is frequently associated with the triad. YNS etiology remains unknown but a role of lymphatic impairment is usually evoked. YNS is more frequently isolated but may be associated in rare cases with autoimmune diseases, other clini

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… Isolated case reports associated YNS with IgA deficiency [ 22 ] or hypogammaglobulinemia [ 80 ]. …

The Complexities of Diagnosis with Co-Existing Gaucher Disease and Hemato-Oncology-A Case Report and Review of the Literature.

Hematological abnormalities are the most common early symptoms of Gaucher disease (GD), with an increased risk of hematopoietic system malignancies reported in patients with GD. GD may be associated with monoclonal and polyclonal gammopathies; however, the mechanism of association of GD with multiple myeloma (MM) remains uncertain. Enzyme replacement therapy (ERT) has been shown to improve patients' cytopenia and it seems to facilitate anti-myeloma therapy in patients with co-occurring GD and MM. Although it is necessary to demonstrate the deficiency of enzymatic activity, as well as using gen

Complement C3a activates osteoclasts by regulating the PI3K/PDK1/SGK3 pathway in patients with multiple myeloma.

ObjectiveMyeloma bone disease (MBD) is the most common complication of multiple myeloma (MM). Our previous study showed that the serum levels of C3/C4 in MM patients were significantly positively correlated with the severity of bone disease. However, the mechanism of C3a/C4a in osteoclasts MM patients remains unclear.MethodsThe formation and function of osteoclasts were analyzed after adding C3a/C4a in vitro. RNA-seq analysis was used to screen the potential pathways affecting osteoclasts, and the results were verified by Western blot, qRT-PCR, and pathway inhibitors.ResultsThe osteoclast area

ИММУНОТРОПНАЯ ТЕРАПИЯ В ЛЕЧЕНИИ РЕЦИДИВИРУЮЩЕГО РИНОСИНУСИТА

Рецидивирующие риносинуситы протекают на фоне иммунодефицитных состояний, латентно, длительно, без болевого симптома.Цель. Определение показаний к назначению иммунотропной терапии у больных с рецидивирующим риносинуситом.Материалы и методы. Обследовано 85 больных с гнойным рецидивирующим риносинуситом без болевого симптома. Всем больным проведено полное клиническое обследование, иммунограмма, определение уровня IL-1β, IL-4, IL-6, IL-8, IL-10, TNFα, INFγ и субстанции Р (SP) в сыворотке крови. Для коррекции иммунной недостаточности в стандартную схему лечения гнойного риносинусита у части больны

The Role of Purported Mucoprotectants in Dealing with Irritable Bowel Syndrome, Functional Diarrhea, and Other Chronic Diarrheal Disorders in Adults.

Chronic diarrhea is a frequent presenting symptom, both in primary care medicine and in specialized gastroenterology units. It is estimated that more than 5% of the global population suffers from chronic diarrhea. and that about 40% of these subjects are older than 60 years. The clinician is frequently faced with the need to decide which is the best therapeutic approach for these patients. While the origin of chronic diarrhea is diverse, impairment of intestinal barrier function, dysbiosis. and mucosal micro-inflammation are being increasingly recognized as underlying phenomena characterizing

Помимо потери веса: изучение неврологических последствий изменённой микробиоты кишечника послекариатрической хирургии.

В настоящем обзоре обсуждаются результаты, связанные с неврологическими расстройствами, кишечной микробиотой и бариатрической хирургией, с уделением особого внимания нейротрансмиттерам, нейроэндокрину, патофизиологии бактерий, способствующих возникновению нарушений, и возможным терапевтическим вмешательствам. Исследования в области нейротрансмиттеров показывают, что их уровни сильно зависят от кишечного микробиота, которая может связать их с такими неврологическими расстройствами, как болезнь Альцгеймера, болезнь Паркинсона, множественный склероз, депрессия и расстройства спектра аутизма. Пато

Research Hotspots and Trends in Global Cancer immunometabolism:A Bibliometric Analysis from 2000 to 2023.

BackgroundCancer poses a major global health challenge, and immunotherapy, known as the third revolution in cancer treatment, has brought new hope to patients. The emerging field of immunometabolism has further enhanced the safety and efficacy of immunotherapy. Over the past two decades, this field has rapidly evolved in oncology, leading to numerous significant findings. This review systematically examines the literature on immunometabolism in cancer, visualizing research trends and identifying future directions.MethodsA comprehensive literature search was conducted in the Web of Science, Pub

The Use of Immune Checkpoint Inhibitors in Oncology and the Occurrence of AKI: Where Do We Stand?

Immune checkpoint inhibitors (ICIs) are a novel class of immunotherapy drugs that have improved the treatment of a broad spectrum of cancers as metastatic melanoma, non-small lung cancer or renal cell carcinoma. These humanized monoclonal antibodies target inhibitory receptors (e.g. CTLA-4, PD-1, LAG-3, TIM-3) and ligands (PD-L1) expressed on T lymphocytes, antigen presenting cells and tumor cells and elicit an anti-tumor response by stimulating immune system. Nevertheless, the improved overall survival is complicated by the manifestation of Immune-related Adverse Effects (irAEs). During treat

The multiple roles of interferon regulatory factor family in health and disease.

Interferon Regulatory Factors (IRFs), a family of transcription factors, profoundly influence the immune system, impacting both physiological and pathological processes. This review explores the diverse functions of nine mammalian IRF members, each featuring conserved domains essential for interactions with other transcription factors and cofactors. These interactions allow IRFs to modulate a broad spectrum of physiological processes, encompassing host defense, immune response, and cell development. Conversely, their pivotal role in immune regulation implicates them in the pathophysiology of v

Solitary Plasmacytoma of the Breast: A Case of an Uncommon Breast Neoplasm.

Plasmacytoma is a rare cancer that originates from a single plasma cell and is characterized by the abnormal proliferation of monoclonal plasma cells. It is typically localized in a single area of the body, most commonly in the bone or soft tissue. Solitary plasmacytoma can be further classified as either solitary plasmacytoma of bone (SPB) or solitary extramedullary plasmacytoma (SEP or EMP). Diagnosis may be delayed in symptomatically silent plasmacytomas, but early diagnosis and prompt treatment are crucial for the management of this disease. The mean age for patients with plasmacytoma vari

Antibodies to watch in 2018.

The pace of antibody therapeutics development accelerated in 2017, and this faster pace is projected to continue through 2018. Notably, the annual number of antibody therapeutics granted a first approval in either the European Union (EU) or United States (US) reached double-digits (total of 10) for the first time in 2017. The 10 antibodies granted approvals are: brodalumab, dupilumab, sarilumab, guselkumab, benralizumab, ocrelizumab, inotuzumab ozogamicin, avelumab, duvalumab, and emicizumab. Brodalumab, however, had already been approved in Japan in 2016. As of December 1, 2017, nine antibody

Pernicious Anemia: The Hematological Presentation of a Multifaceted Disorder Caused by Cobalamin Deficiency.

Pernicious anemia is still a neglected disorder in many medical contexts and is underdiagnosed in many patients. Pernicious anemia is linked to but different from autoimmune gastritis. Pernicious anemia occurs in a later stage of autoimmune atrophic gastritis when gastric intrinsic factor deficiency and consequent vitamin B12 deficiency may occur. The multifaceted nature of pernicious anemia is related to the important role of cobalamin, which, when deficient, may lead to several dysfunctions, and thus, the proteiform clinical presentations of pernicious anemia. Indeed, pernicious anemia may l

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… Furthermore, PA associated with common variable immunodeficiency, low serum immunoglobulin concentrations, selective IgA deficiency, or the childhood PA, should be distinguished from classic PA due to both an earlier onset of symptoms and the absence of an association with gastric corpus atrophy of the …

Ход работы сессии 28th Европейский конгресс педиатрической ремуматологии (ПСР) 2022) Прага, Чешская Республика. 20-23 сентябрь 2022.

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KDIGO Clinical Practice Guideline on the Evaluation and Care of Living Kidney Donors.

The 2017 Kidney Disease: Improving Global Outcomes (KDIGO) Clinical Practice Guideline on the Evaluation and Care of Living Kidney Donors is intended to assist medical professionals who evaluate living kidney donor candidates and provide care before, during and after donation. The guideline development process followed the Grades of Recommendation Assessment, Development, and Evaluation (GRADE) approach and guideline recommendations are based on systematic reviews of relevant studies that included critical appraisal of the quality of the evidence and the strength of recommendations. However, m

Stevens–Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) : Immunologic Reactions

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Membranous nephropathy: pathogenesis and treatments.

Membranous nephropathy (MN), an autoimmune disease, can manifest at any age and is among the most common causes of nephrotic syndrome in adults. In 80% of cases, the specific etiology of MN remains unknown, while the remaining cases are linked to drug use or underlying conditions like systemic lupus erythematosus, hepatitis B virus, or malignancy. Although about one-third of patients may achieve spontaneous complete or partial remission with conservative management, another third face an elevated risk of disease progression, potentially leading to end-stage renal disease within 10 years. The i

Навигатор биомаркеров человека.

Биомаркеры играют ключевую роль в современных исследованиях медицины, особенно в ранней диагностике заболеваний и персонализированном лечении. Несмотря на то, что предыдущие исследования систематически обзоры маркеров в различных областях заболеваний, интегрированная система, которая связывает основные физиологические системы, охватывает множество органов и охватывает широкий спектр заболеваний, все еще отсутствует. В контексте современных вызовов здоровья, характеризующихся высокой распространенностью хронических заболеваний и широкими сопутствующими заболеваниями, создание панорамной системы