作者: Hemiparesis 時(shí)間: 2025-3-21 22:30
Rare Diseases of the Immune Systemhttp://image.papertrans.cn/h/image/429955.jpg作者: MURKY 時(shí)間: 2025-3-22 03:19 作者: 撫育 時(shí)間: 2025-3-22 05:05 作者: CON 時(shí)間: 2025-3-22 08:43
https://doi.org/10.1007/978-3-319-91785-6B-cell; Antibodies; Gammaglobulines; Common Variable Immune Deficiency; Clinical Phenotypes; PID genes; Au作者: 臭了生氣 時(shí)間: 2025-3-22 14:32
Book 2019dy production due to defects intrinsic to B cells or defective interaction between B and T cells. There is extensive coverage of both basic science discoveries and the latest clinical advances in the field. The book is structured in accordance with the most recent classification of PIDs and also cov作者: Individual 時(shí)間: 2025-3-22 19:49
TWEAK Deficiency,T-dependent (diphtheria and tetanus toxoid) and independent antigens (pneumococcal polysaccharides). Total B-cell numbers are reduced with a selective deficiency in CD27+ memory B cells. CD8+ T cells and TCR a/b+ double-negative T cells are increased in numbers.作者: Mnemonics 時(shí)間: 2025-3-23 00:55 作者: Wallow 時(shí)間: 2025-3-23 05:05
Mucosal B Cells,tion, inhalation, or sexual contact. This chapter focuses on the mechanisms that coordinate B-cell development as well as on the mechanisms used by mucosal B cells and mucosal IgA to give protection to the host.作者: 驚呼 時(shí)間: 2025-3-23 08:55 作者: VICT 時(shí)間: 2025-3-24 06:26
ICOS Deficiency,ic infections. Immunoglobulin substitution and antibiotic treatment of breakthrough infections are the mainstays of treatment in ICOS deficiency. In cases of conventional treatment failure, hematopoietic stem cell transplantation may be considered.作者: Lignans 時(shí)間: 2025-3-24 07:39
, Defects,riable immunodeficiency phenotype, while gain-of-function mutations in . have been associated with a combined immunodeficiency phenotype. Patients can also develop endocrinopathies including adrenal insufficiency, as well as autoimmune disease manifestations.作者: 愚笨 時(shí)間: 2025-3-24 12:12 作者: 現(xiàn)任者 時(shí)間: 2025-3-24 17:05
CVID,quate therapy of complications. Allogenic peripheral stem cell therapy is considered today in patients with severe hematological changes (chronic transfusion-dependent anemia, leukopenia, thrombocytopenia), malignancies, and overall prognostically poor outcome despite consistent immunoglobulin substitution.作者: 以煙熏消毒 時(shí)間: 2025-3-24 20:22 作者: hauteur 時(shí)間: 2025-3-25 02:34
TACI Deficiency,forms of antibody deficiency. The management of CVID patients carrying a TACI mutation does not differ from conventional CVID. However, one should take into account the higher rates of autoimmune and lymphoproliferative complications seen in these individuals.作者: glucagon 時(shí)間: 2025-3-25 05:37 作者: colostrum 時(shí)間: 2025-3-25 09:24
l of processes, but also at the planetary scale of the whole system. Here, we describe the dynamics of the Earth system as the consequence of sequences of energy conversions that are constrained by thermodynamics. We first describe the well-established Carnot limit and show how it results in a maxim作者: 伸展 時(shí)間: 2025-3-25 13:47
Laura Patrussi,Nagaja Capitani,Mario Milco D’Elios,Cosima T. Baldarie calculations. Firstly, the principles of control volume analysis are enunciated and applied to flows of conserved quantities (e.g. mass, momentum, energy) through a control volume, giving integral (Reynolds transport theorem) and differential forms of the conservation equations. Several definition作者: Eeg332 時(shí)間: 2025-3-25 18:38 作者: Clinch 時(shí)間: 2025-3-25 20:11 作者: bronchiole 時(shí)間: 2025-3-26 00:22
Eduardo López-Granadosa nonequilibrium process to be quantified for systems arbitrarily close to or far from equilibrium. For a system out of equilibrium, the average dissipation over a period, ., will be positive. For field driven flow in the thermodynamic and small field limits, the dissipation function becomes proport作者: Melodrama 時(shí)間: 2025-3-26 04:34 作者: incarcerate 時(shí)間: 2025-3-26 08:50
Jens Thieltions and EC/EU membership. The dynamic approach adopted for the study of the Spanish case has made it possible to look beyond the role of the central state and examine new constellations of actors and interactive processes which previous academic work on Spain has tended to underplay. The conclusio作者: 賭博 時(shí)間: 2025-3-26 13:13 作者: 四指套 時(shí)間: 2025-3-26 20:31 作者: 安撫 時(shí)間: 2025-3-27 00:37 作者: prick-test 時(shí)間: 2025-3-27 04:04 作者: 四指套 時(shí)間: 2025-3-27 06:33 作者: Commission 時(shí)間: 2025-3-27 12:36
Giuliana Roselli,Marinos Kallikourdis,Antonella Viola secondary ‘superstructures’. According to many radicals, the capitalist ‘mode of production’ transcended individual nations and became a single ‘world capitalist mode of production’ with a ‘centre’ and a ‘periphery’; this ‘world capitalism’ or ‘world-system’ is then interpreted as having distinct ‘作者: Diuretic 時(shí)間: 2025-3-27 14:50
,The B-Side of the?Immune Response,r ability to take advantage of interactions with the other immune cells to become antibody-producing plasma cells or memory B cells. This chapter focuses on the mechanisms by which B cells encounter, manage, and present antigens to T cells, to finally integrate both humoral and cellular immune respo作者: FLING 時(shí)間: 2025-3-27 18:51 作者: 爭吵 時(shí)間: 2025-3-28 01:51
CVID,s immunological features. Clinical hallmarks are hypogammaglobulinemia and severe and/or frequent, chronic-recurring bacterial infections especially affecting the respiratory tract. Given that immunological and clinical presentations are by far more diverse and—since CVID per se is not a monogenetic作者: 彩色的蠟筆 時(shí)間: 2025-3-28 02:18 作者: athlete’s-foot 時(shí)間: 2025-3-28 09:34 作者: 公社 時(shí)間: 2025-3-28 13:30
ICOS Deficiency,n common variable immunodeficiency. ICOS-deficient patients have high rates of immunedysregulatory complications, and bacterial, viral and opportunistic infections. Immunoglobulin substitution and antibiotic treatment of breakthrough infections are the mainstays of treatment in ICOS deficiency. In c作者: 輕浮思想 時(shí)間: 2025-3-28 16:54 作者: 噱頭 時(shí)間: 2025-3-28 21:02
Genetic CD21 Deficiency,e been reported. CD21 deficiency is caused by compound heterozygous or homozygous mutations in the . gene. The CD21-deficient patients may have a relatively mild clinical phenotype with recurrent bacterial infections and autoimmune-mediated myalgia or a more severe phenotype with recurrent upper and作者: 魯莽 時(shí)間: 2025-3-28 23:55
TACI Deficiency,cules which regulate B cell homeostasis, differentiation, and function. Heterozygous, compound heterozygous, or homozygous alterations in the TNFRSF13B gene are associated with various forms of antibody deficiency including IgA deficiency and CVID. These genetic alterations have a low penetrance and作者: Initiative 時(shí)間: 2025-3-29 04:04 作者: 責(zé)問 時(shí)間: 2025-3-29 07:55 作者: LEERY 時(shí)間: 2025-3-29 12:22 作者: 來這真柔軟 時(shí)間: 2025-3-29 18:22 作者: patella 時(shí)間: 2025-3-29 20:42 作者: BILK 時(shí)間: 2025-3-30 02:17
Class-Switch Recombination Defects,) from one type to the other (i.e., from IgM to IgG, IgA or IgE). This confers to the antibodies specific effector function and tissue distribution. Class-switch recombination deficiencies are a heterogeneous group of primary immunodeficiencies characterized by normal or increased levels of serum Ig作者: Pericarditis 時(shí)間: 2025-3-30 07:16
Selective IgA Deficiency,estinal infections, autoimmunity, allergic conditions, and, in some cases, progression to the more severe antibody deficiency, common variable immunodeficiency (CVID). Genetics, intrinsic defects in lymphocytes, and impairment of the cytokine profile are all thought to contribute to IgAD pathogenesi作者: floaters 時(shí)間: 2025-3-30 08:12
IgG Subclass and Anti-polysaccharide Antibody Deficiency,the results of immunological laboratory investigations and potential underlying pathophysiology may differ greatly. Milder forms of PAD are often grouped together as hypogammaglobulinemia; this can be found at any age; however, the clinical significance may differ in different age groups. In some ch作者: 自由職業(yè)者 時(shí)間: 2025-3-30 13:05 作者: refine 時(shí)間: 2025-3-30 18:05 作者: Irrepressible 時(shí)間: 2025-3-30 21:25 作者: 割公牛膨脹 時(shí)間: 2025-3-31 04:21
Mirjam van der Burg,Andrew R. Gennery,Qiang Pan-Hammarstr?m作者: 拋媚眼 時(shí)間: 2025-3-31 06:18 作者: Bureaucracy 時(shí)間: 2025-3-31 11:13
Isabella Quinti,Cinzia Milito,Rossella Carello,Federica Pulvirenti作者: Canvas 時(shí)間: 2025-3-31 13:24
LRBA Deficiency,f CTLA-4 in Tregs, thereby controlling proinflammatory responses. However, the exact function of LRBA in other immune cells is not yet fully understood. Interestingly, LRBA-deficient mouse models do not mirror the human LRBA deficiency, as . null mice do not present any clinical or immunological sig作者: 挫敗 時(shí)間: 2025-3-31 20:02 作者: GIBE 時(shí)間: 2025-3-31 22:04 作者: 貴族 時(shí)間: 2025-4-1 03:01
IgG Subclass and Anti-polysaccharide Antibody Deficiency,cipation. In general, combinations of abnormal laboratory results seem to be associated with more symptoms and more sequelae. It is currently unknown how the different forms of milder hypogammaglobulinemia arise.作者: Cervical-Spine 時(shí)間: 2025-4-1 08:53
Differential Diagnosis in Hypogammaglobulinemia,ts with a low immunoglobulin serum level at the initial evaluation, defined causes of secondary hypogammaglobulinemias should be extensively ruled out. In particular, B-cell malignancies (mainly chronic lymphocytic leukemia) and plasma cell dyscrasias should be excluded in patients with low immunogl作者: escalate 時(shí)間: 2025-4-1 13:00
Book 2019 .Humoral Primary Immunodeficiencies. will be of high value for immunologists, pediatricians,rheumatologists, oncologists, internists, and infectious disease specialists and will also be informative for MD and PhD students..作者: 同義聯(lián)想法 時(shí)間: 2025-4-1 16:12
limit with a fast, positive and a slow, negative feedback that compensate each other at the maximum power state. We close with a discussion of the importance of a planetary, thermodynamic view of the whole Earth system, in which thermodynamics limits the intensity of the dynamics, interactions stro作者: 儲(chǔ)備 時(shí)間: 2025-4-1 20:01
Laura Patrussi,Nagaja Capitani,Mario Milco D’Elios,Cosima T. Baldari the effect of radiation, and the analysis of systems subdivided into compartments. A Reynolds decomposition of the entropy production equation then reveals an “entropy production closure problem” in fluctuating dissipative systems: even at steady state, the entropy production based on mean flow rat作者: abysmal 時(shí)間: 2025-4-2 01:16
Arianna Troilo,Nagaja Capitani,Laura Patrussi,Cosima T. Baldari,Mario Milco D’Eliosanded to regions wherein they become meta-stable, and eventually transition into more stable configurations. The maximally stable state is found to be that which maximises the rate of heat transfer, which is only equivalent to maximum internal entropy production in a strong forcing regime, while it 作者: 和平主義 時(shí)間: 2025-4-2 06:15 作者: Obliterate 時(shí)間: 2025-4-2 08:17 作者: 明確 時(shí)間: 2025-4-2 13:31 作者: 減去 時(shí)間: 2025-4-2 15:54
