Gastrointestinal manifestations in children with primary immunodeficiency diseases

No Abstrac

Common variable immunodeficiency

Egypt J Pediatr Allergy Immunol 2006; 4(1): 3-1

Plasma concentration of thymus and activation-regulated chemokine in childhood asthma

Background: Thymus and activation-regulated chemokine (TARC) is responsible for trafficking of T helper 2 lymphocytes into sites of allergic inflammation. However, its role in assessing the severity of acute asthma in children is still unclear. Objective: We sought to evaluate plasma TARC as a marker for monitoring asthma exacerbation in terms of asthma attack severity. Methods: Plasma TARC concentration was estimated in 24 asthmatic children aged between 2 and 17 years attending the Pediatric Allergy and Immunology Unit of Children’s Hospital, Ain Shams University, and 23 age and sex-matched healthy children using a sandwich enzyme immunoassay technique. For asthmatic patients, the measurement was performed during and after the resolution of acute asthma attack. In addition, complete hemogram and plasma total IgE were evaluated and peak expiratory flow rate was assessed in asthmatic patients during and after acute asthma exacerbation. Results: Plasma TARC mean concentration was significantly higher during acute asthma (839.2 ± 453.6 pg/ml) than after resolution of symptoms (416.5 ± 324.8 pg/ml) and both were statistically higher than the control value (165.7 ± 135.2 pg/ml). During acute attacks of asthma, plasma TARC level was significantly elevated among patients with severe attacks of wheezing (1336.3 ± 431.2 pg/ml) than in those with moderate (743.8 ± 91.8 pg/ml) and mild (437.5 ± 66.1 pg/ml) attacks and inversely related to PEFR measurements during attacks (r = -98, P < 0.001). Meanwhile, no significant relationship was found between plasma TARC levels and either plasma total IgE levels or the absolute eosinophil count. Neither the history of other atopic symptoms nor family history of atopy influenced plasma TARC levels. A significant reduction in plasma TARC level was observed after treatment with inhaled ß2 agonist drugs either alone or in conjunction with inhaled glucocorticoids. Conclusion: Our findings support the concept that TARC may be implicated in the pathogenesis of asthma. Plasma TARC is a useful marker in monitoring the severity of asthma exacerbation and in assessing the degree of allergic inflammation in the asthmatic airway. This would help physicians to design appropriate therapy in terms of dose and duration of treatment especially among children with quiescent asthma. Future studies should focus on using TARC antagonists as a new approach to asthma immunotherapy.Keywords: bronchial asthma, acute attacks, remission, TARC, atopy, inhaled glucocorticoids, ß2 agonistsEgypt J Pediatr Allergy Immunol 2003; 1(2): 86-9

Antinucleosome antibodies as early predictors of lupus nephritis

Background: The role of the nucleosome in the induction of antibody response in lupus mediated tissue damage especially glomerulonephritis, may provide a new insight in the early diagnosis and alternative therapeutic developments in systemic lupus erythematosus (SLE). Objectives: To evaluate the frequency and specificity of antinucleosome antibody expression in SLE patients in relation to disease activity. Also, to assess their predictive value in subclinical lupus nephritis. Methods: This study included 26 patients with SLE and 52 control subjects (26 were healthy and 26 had juvenile rheumatoid arthritis "JRA"). Among lupus patients, 15 had clinical evidence of renal involvement. After clinical evaluation to calculate the SLE disease activity index (SLEDAI), measurements of urinary microalbumin and serum antinucleosome antibodies (antinucleosome specific, antihistone and anti ds-DNA antibodies by ELISA) were performed. Patients without clinical evidence of renal involvement were followed up for one year and measurement of urinary microalbumin was repeated at the end of the study period. Those who later developed microalbuminurea were categorized as patients with subclinical lupus nephritis. Results: The expression of the 3 studied antinucleosome antibodies was significantly higher among lupus patients as compared to JRA patients and healthy controls. Seropositivity for one or more antinucleosome antibodies was elicited in 84.5% of lupus patients. Serum levels of the 3 antinucleosome antibodies were significantly higher among lupus patients with clinical nephritis than those without nephritis. ANSAb had higher sensitivity, specificity and positive and negative predictive values for subclinical lupus nephritis (100%) than antihistone and anti ds-DNA antibodies (43%, 100%, 100% and 50% respectively for either antibodies). All patients with lupus nephritis were seropositive for at least one of the antinucleosome antibodies, while those without clinical or subclinical nephritis were seronegative for the 3 antinucleosome antibodies. In 27.3% of patients with lupus nephritis, ANSAB was positive while both antihistone and ds-DNA antibodies were negative. Antinucleosome antibodies correlated positively with SLEDAI and cumulative steroid dose and negatively with corrected creatinine clearance. Conclusions: The observed sensitivity and specificity of antinucleosome specific antibodies as early indicators of subclinical lupus nephritis appear encouraging and deserve further analysis on a large scale in order to confirm their validity, especially in the anti ds-DNA seronegative lupus patients.Keywords: antinucleosome antibodies, antinucleosome specific antibodies, anti ds-DNA antibodies, antihistone antibodies, SLE, lupus nephritisEgypt J Pediatr Allergy Immunol 2005; 3(2):54-6

Reliability of candida skin test in the evaluation of T-cell function in infancy

Background: Both standardized and non-standardized candida skin tests are used in clinical practice for functional in-vivo assessment of cellular immunity with variable results and are considered not reliable under the age of 1 year. We sought to investigate the reliability of using manually prepared candida intradermal test in the evaluation of T cell function in infants during their second year of life.Methods: Twenty-five healthy infants were tested with manually prepared intradermal candida test. Cultured lymphocytes were stimulated with phytohemagglutinin (PHA) and gamma interferon (IFN-γ) levels were measured in the culture supernatant of stimulated and non-stimulated samples using ELISA.Results: The enrolled infants were 14 to 24 months old (mean 19.2 ± 3.13 months). They were 17 boys (68 %) and 8 girls (32 %). Candida skin test was positive in 17 out of the 25 infants (68%). All infants showed increased IFN-γ levels after PHA stimulation (mean ± SD: 0.83±0.29 ng/ml) compared to basal levels (mean ± SD = 0.16 ± 0.16 ng/ml). The increase of IFN γ levels after PHA stimulation ranged from 1.54 to 38 folds. Infants with positive and negative candida tests showed comparable results in terms of clinical and immunological assessment except for weight percentiles for age that were higher among candida positive group.Conclusion: Candida intradermal test is a cost-effective simple test for evaluation of T cell function with 70 % sensitivity in healthy infants above the age of one year.Keywords: Candida, IFN-γ, infants, intradermal, lymphocyte proliferation, PHA stimulation, T cell, Tuberculi

Emerging Infections and Pertinent Infections Related to Travel for Patients with Primary Immunodeficiencies

Erratum: J Clin Immunol. 2017 Oct;37(7):693-694. doi: 10.1007/s10875-017-0436-0.In today's global economy and affordable vacation travel, it is increasingly important that visitors to another country and their physician be familiar with emerging infections, infections unique to a specific geographic region, and risks related to the process of travel. This is never more important than for patients with primary immunodeficiency disorders (PIDD). A recent review addressing common causes of fever in travelers provides important information for the general population Thwaites and Day (N Engl J Med 376:548-560, 2017). This review covers critical infectious and management concerns specifically related to travel for patients with PIDD. This review will discuss the context of the changing landscape of infections, highlight specific infections of concern, and profile distinct infection phenotypes in patients who are immune compromised. The organization of this review will address the environment driving emerging infections and several concerns unique to patients with PIDD. The first section addresses general considerations, the second section profiles specific infections organized according to mechanism of transmission, and the third section focuses on unique phenotypes and unique susceptibilities in patients with PIDDs. This review does not address most parasitic diseases. Reference tables provide easily accessible information on a broader range of infections than is described in the text.Peer reviewe

Controlo químico de infestantes

Uma planta é considerada infestante quando nasce espontaneamente num local e momento indesejados, podendo interferir negativamente com a cultura instalada. As infestantes competem com as culturas para o espaço, a luz, água e nutrientes, podendo atrasar e prejudicar as operações de colheita, depreciar o produto final e assegurarem a reinfestação nas culturas seguintes. Dado o modo de propagação diferenciado das diversas espécies de infestantes, com as anuais a propagarem-se por semente e as perenes ou vivazes a assegurarem a sua propagação através de órgãos vegetativos (rizomas, bolbos, tubérculos, etc.), assim, também o seu controlo quer químico, quer mecânico terá que ser diferenciado, ou seja, para controlar infestantes anuais será suficiente destruir a sua parte aérea, enquanto para controlar infestantes perenes teremos que destruir os seus órgãos reprodutivos. O controlo de infestantes poderá ser químico, através da utilização de herbicidas, ou mecânico pela utilização de alfaias agrícolas, tais como a charrua de aivecas, a charrua de discos, a grade de discos, o escarificador e a fresa. Quando a técnica utilizada na instalação das culturas é a sementeira directa, o controlo das infestantes terá que ser obrigatoriamente químico, enquanto se o recurso à mobilização do solo for a técnica mais utilizada (sistema de mobilização tradicional ou sistema de mobilização reduzida), o controlo das infestantes tanto poderá ser químico como mecânico. Neste trabalho iremos abordar apenas, o controlo químico de infestantes

Cabbage and fermented vegetables : From death rate heterogeneity in countries to candidates for mitigation strategies of severe COVID-19

Large differences in COVID-19 death rates exist between countries and between regions of the same country. Some very low death rate countries such as Eastern Asia, Central Europe, or the Balkans have a common feature of eating large quantities of fermented foods. Although biases exist when examining ecological studies, fermented vegetables or cabbage have been associated with low death rates in European countries. SARS-CoV-2 binds to its receptor, the angiotensin-converting enzyme 2 (ACE2). As a result of SARS-CoV-2 binding, ACE2 downregulation enhances the angiotensin II receptor type 1 (AT(1)R) axis associated with oxidative stress. This leads to insulin resistance as well as lung and endothelial damage, two severe outcomes of COVID-19. The nuclear factor (erythroid-derived 2)-like 2 (Nrf2) is the most potent antioxidant in humans and can block in particular the AT(1)R axis. Cabbage contains precursors of sulforaphane, the most active natural activator of Nrf2. Fermented vegetables contain many lactobacilli, which are also potent Nrf2 activators. Three examples are: kimchi in Korea, westernized foods, and the slum paradox. It is proposed that fermented cabbage is a proof-of-concept of dietary manipulations that may enhance Nrf2-associated antioxidant effects, helpful in mitigating COVID-19 severity.Peer reviewe

Nrf2-interacting nutrients and COVID-19 : time for research to develop adaptation strategies

There are large between- and within-country variations in COVID-19 death rates. Some very low death rate settings such as Eastern Asia, Central Europe, the Balkans and Africa have a common feature of eating large quantities of fermented foods whose intake is associated with the activation of the Nrf2 (Nuclear factor (erythroid-derived 2)-like 2) anti-oxidant transcription factor. There are many Nrf2-interacting nutrients (berberine, curcumin, epigallocatechin gallate, genistein, quercetin, resveratrol, sulforaphane) that all act similarly to reduce insulin resistance, endothelial damage, lung injury and cytokine storm. They also act on the same mechanisms (mTOR: Mammalian target of rapamycin, PPAR gamma:Peroxisome proliferator-activated receptor, NF kappa B: Nuclear factor kappa B, ERK: Extracellular signal-regulated kinases and eIF2 alpha:Elongation initiation factor 2 alpha). They may as a result be important in mitigating the severity of COVID-19, acting through the endoplasmic reticulum stress or ACE-Angiotensin-II-AT(1)R axis (AT(1)R) pathway. Many Nrf2-interacting nutrients are also interacting with TRPA1 and/or TRPV1. Interestingly, geographical areas with very low COVID-19 mortality are those with the lowest prevalence of obesity (Sub-Saharan Africa and Asia). It is tempting to propose that Nrf2-interacting foods and nutrients can re-balance insulin resistance and have a significant effect on COVID-19 severity. It is therefore possible that the intake of these foods may restore an optimal natural balance for the Nrf2 pathway and may be of interest in the mitigation of COVID-19 severity

La importancia de la vacunación y el tratamiento con inmunoglobulina para pacientes con inmunodeficiencias primarias

Las inmunodeficiencias primarias son un grupo heterogéneo de enfermedades genéticas raras que afectan el desarrollo o el funcionamiento de la inmunidad innata o la adaptativa. La mayoría de los pacientes que las padecen tienen mayor susceptibilidad a infecciones tanto comunes como raras, a veces con desenlace fatal. Por lo tanto es esencial ofrecer tratamiento rápido y eficaz para prevenir infecciones, proporcionando programas de vacunación adecuados y administrando regularmente el tratamiento con inmunoglobulina G (IgG) de reemplazo, en los trastornos con defecto en la producción o funcionamiento de los anticuerpos. En algunas condiciones también está indicada la quimioprofilaxis con antibióticos y antifúngicos