Genetic stability of Campylobacter coli in patients with primary antibody deficiencies

In the Clinical Communication, Dion et al 1 reported that in patients with severe primary antibody deficiency (PAD), Campylobacter infection is a major cause (6.5%) of chronic or recurrent diarrhea. Moreover, by a molecular study performed in a subset of 18 strains from 5 patients with recurrent infections, they demonstrated that all strains were different, even when the episodes occurred closely over time. Thus, the authors hypothesized that reinfection would be more likely than persistent colonization, although colonization with multiple strains cannot be excluded. In a previous study, 2 our group showed that Campylobacter coli (C. coli) (6.7%) was the first cause of diarrhea in patients with symptomatic PAD with a positive stool culture, followed by Campylobacter jejuni (C. jejuni) (3.9%). Moreover, C. coli was also the most frequent isolate (5%) in patients with asymptomatic PAD, followed by C. jejuni (1.2%), whereas in immunocompetent individuals, C. jejuni is one of the most prevalent etiologic agents of gastroenteritis and C. coli has a low prevalence in diarrheal disease

The Composition and, in Particular, the Disinfectant Value of the Phenols in Coal Tars from Various Sources

The literature on the subject of disinfection with phenolic bodies is very extensive but much the greater part of it is concerned only with the lower boiling fractions of the phenols from which phenol and the cresols, in a pure state, may be isolated with comparative ease. The object of the present research is to examine the phenols of higher boiling points, from which few pure products have so far been isolated, in order to determine whether knowledge of their disinfectant value in addition to known physical constants will assist in distinguishing, or proving relationship, between phenols from different sources such as from low temperature or high temperature coal-tars. Owing to the low solubility of the high boiling phenols in water it was necessary to employ them in an emulsified form. Methods of emulsifying the phenols were studied and comparisons were made between the disinfectant value and the average size of the emulsion particles, in order to obtain the most efficient type of preparation and to determine the extent of variation of the disinfectant value with alteration in the emulsion particle size. The disinfectant values were determined by the Rideal-Walker method and, during the progress of the work, one cause for inconsistent results was traced to the relatively inaccurate method of standardising the broth culture medium. Slight variations in the acidity of the broth were found to change the phenol coefficient value of the same disinfectant preparation and, after publication of the results, the official method of standardising the broth was revised. The comparison of the phenols from different sources of tar was commenced after ascertaining the best methods to obtain consistent results for the disinfectant values of the phenols. In the comparison, identical fractions of the phenols from four tars were prepared and the disinfectant values of the fractions were compared with those of pure phenols. The results of the research work are presented under the following four headings: (1) Introductory review of literature. (2) Preparation of disinfectant emulsions and compar- ison of their germicidal power with emulsion particle size determinations. (3) Effect of slight variation in the adjustment of the acidity of Rideal-Walker broth upon the phenol coefficient value of coal-tar disinfectants. (4) Comparison of the disinfectant value of the phenols from different sources of tar

Clinical management of patients with primary immunodeficiencies during the COVID-19 pandemic

Introduction: Patients affected by Inborn Errors of Immunity (IEI) represent a potential group-at-risk in the current COVID-19 pandemic. Studies on large and small cohorts of IEI reported a huge variability clinical manifestations associated to SARS-Cov-2, ranging from asymptomatic, mild, moderate/severe to death. A great impulse to improve remote assistance programs and to switch to home-based treatment to reduce mobility and face to face contacts has been implemented. Areas covered: The authors completed a comprehensive review of the literature by searching the PubMed database for studies on large and small cohorts and case reports of IEI patients with COVID-19, with the aim to provide useful information for their clinical management during the COVID-19 pandemic. Expert opinion: Surprisingly, a low number of IEI patients affected by SARS-Cov-2 were reported with a risk to die for COVID-19 overlapping that of the general population. The low number might be explained by the choice of most physicians to inform early in the pandemic about safety measures, to switch most of the IEI patients to home therapy and to remote assistance. The guidelines issued by the scientific societies and periodically updated, represent the best tool for the clinical management of IEI patients

Lymphoma in common variable immunodeficiency: interplay between immune dysregulation, infection and genetics

PURPOSE OF REVIEW: Common variable immunodeficiency represents the largest group of primary immunodeficiency patients. The variable clinical manifestations include an increased susceptibility to chronic infections, granulomatous disease and the lymphoproliferative predisposition to develop lymphoma. This review discusses the latest insights into common variable immunodeficiency and uses common variable immunodeficiency as a model to examine the links between immunodeficiency and chronic infections in causing lymphoma. RECENT FINDINGS: Newly identified disease genes within the common variable immunodeficiency population, have advanced the understanding of human immunodeficiency and the molecular basis of B-cell biology. Refined laboratory techniques have better defined this heterogeneous condition by classifying the underlying B-cell and T-cell abnormalities. New sensitive methods have also identified the presence of persistent infections that may play a role in the development of lymphoma. SUMMARY: There are several reasons for an increased risk of lymphoma in common variable immunodeficiency patients. These include genetics, immune dysregulation, radiosensitivity and chronic infections such as Helicobacter pylori, human herpes virus type 8 and cytomegalovirus. Chronic infections may enhance the development of lymphoma in an antigen specific manner. The interaction between chronic infections and the development of lymphoma is still unclear but studies to clarify this may lead to prevention measures and lymphoma reduction strategies

Development and Initial Validation of a Questionnaire to Measure Health-Related Quality of Life of Adults with Common Variable Immune Deficiency: The CVID_QoL Questionnaire.

BACKGROUND: Generic health status quality of life (QoL) instruments have been used in patients with common variable immune deficiency (CVID). However, by their nature, these tools may over- or underestimate the impact of diseases on an individual's QoL. OBJECTIVE: The objective of this study was to develop and validate a questionnaire to measure specific-health-related QoL for adults with CVID (CVID_QoL). METHODS: The 32-item content of the CVID_QoL questionnaire was developed using focus groups and individual patient interviews. Validation studies included 118 adults with CVID who completed Short Form-36, Saint George Respiratory Questionnaire, General Health Questionnaire-12, and EuroQol-5D questionnaire in a single session. Principal component and factor analysis solutions identified 3 scores to be similar in number and content for each solution. Validation of 3 factor scores was performed by construct validity. Reproducibility, reliability, convergent validity, and discriminant validity were evaluated. Matrices consisting of correlations between the 32 items in the CVID_QOL were calculated. RESULTS: Factor analysis identified 3 dimensions: emotional functioning (EF), relational functioning (RF), and gastrointestinal and skin symptoms (GSS). The instrument had good internal consistency (Cronbach's alpha, min. 0.74 for GSS, max. 0.84 for RF, n = 118) and high reproducibility (intraclass correlation coefficient, min. 0.79 for RF, max 0.90 for EF, n = 27). EF and RF scores showed good convergent validity correlating with conceptually similar dimensions of other study scales. Acute and relapsing infections had a significant impact on EF and RF. CONCLUSIONS: This study provides evidence of the reliability and construct validity of the CVID_QoL to identify QoL issues in patients with CVID that may not be addressed by generic instruments

Arquiteturas de educação: O espaço do possível. A Cultura de vida na experiência da secretaria municipal de escolas de educação infantil da Reggio Emilia

En estrecha relación con el pensamiento de Loris Malaguzzi y la teoría de los 100 lenguajes de los niños, los espacios en la pedagogía de Reggio asumen un papel de “educadores centrales y necesarios”. Una cultura del habitar los lugares de la primera infancia que ha cobrado vida a través de muchas experimentaciones e investigaciones dentro de la idea de una escuela pública nueva, pensada como lugar de cultura y conocimiento. La valorización de los lenguajes expresivos y poéticos en los procesos de aprendizaje contribuye a redefinir la percepción estética como estrategia cognoscitiva, algo inherente tanto en niños como en adultos, y una modalidad perceptiva que contagia la calidad del espacio arquitectónicoIn close connection with the thinking of Loris Malaguzzi and the theory of the 100 languages of children, spaces in the pedagogy of Reggio assume a role of "central and necessary educators." A culture of living in the places of early childhood that has come to life through many experiments and research within the idea of a new public school, designed as a place of culture and knowledge. The valuation of expressive and poetic languages in the learning processes contributes to redefine the aesthetic perception as a cognitive strategy, something inherent in both children and adults, and a perceptive modality that infects the quality of the architectural spaceEm estrita relação com o pensamento de Loris Malaguzzi e com a teoria das 100 linguagens das crianças, os espaços na pedagogia de Reggio assumem um papel de "educadores centrais e necessários". Uma cultura do habitar os lugares da primeira infância que tem ganho vida através de muitos experimentos e pesquisas, baseadas na ideia de uma nova escola pública, concebida como lugar de cultura e conhecimento. A valorização das linguagens expressivas e poeticas no processo de aprendizagem ajuda a redefinir a percepção estética como estratégia cognitiva, algo presente tanto em crianças como adultos, e uma modalidade perceptiva que determina a qualidade do espaço arquitectónic

Gastric cancer is the leading cause of death in Italian adult patients with common variable immunodeficiency

An increased prevalence of malignant lymphoma and of gastric cancer has been observed in large cohorts of patients with common variable immunodeficiency (CVID), the most frequently symptomatic primary immunodeficiency. Surveillance strategies for cancers in CVID should be defined based on epidemiological data. Risks and mortality for cancers among 455 Italian patients with CVID were compared to cancer incidence data from the Italian Cancer Registry database. CVID patients showed an increased cancer incidence for all sites combined (Obs = 133, SIR = 2.4; 95%CI = 1.7\u20133.5), due to an excess of non-Hodgkin lymphoma (Obs = 33, SIR = 14.3; 95%CI = 8.4\u201322.6) and of gastric cancer (Obs = 25; SIR = 6.4; 95%CI = 3.2\u201312.5). CVID patients with gastric cancer and lymphoma had a worse survival in comparison to cancer-free CVID (HR: 4.8, 95%CI: 4.2\u201344.4 and HR: 4.2, 95%CI: 2.8\u201344.4). Similar to what observed in other series, CVID-associated lymphomas were more likely to be of B cell origin and often occurred at extra-nodal sites. We collected the largest case-series of gastric cancers in CVID subjects. In contrast to other reports, gastric cancer was the leading cause of death in CVID. Standardized mortality ratio indicated a 10.1-fold excess mortality among CVID patients with gastric cancer. CVID developed gastric cancer 15 years earlier than the normative population, but they had a similar overall survival. Only CVID diagnosed at early stage gastric cancer survived >24 months. Stomach histology from upper endoscopy performed before cancer onset showed areas of atrophic gastritis, intestinal metaplasia or dysplasia. CVID patients might progress rapidly to an advanced cancer stage as shown by patients developing a III-IV stage gastric cancer within 1 year from an endoscopy without signs of dysplasia. Based on high rate of mortality due to gastric cancer in Italian CVID patients, we hereby suggest a strategy aimed at early diagnosis, based on regular upper endoscopy and on Helicobacter pylori infection treatment, recommending an implementation of national guidelines

Risk factors for Haemophilus influenzae and pneumococcal respiratory tract colonization in CVID

To the Editor: Disease-specific studies focused on infection risk in common variable immune deficiencies (CVIDs) are needed to define strategies for controlling respiratory infections predominantly due to bacteria such as Streptococcus pneumoniae and Haemophilus influenzae.1 Little information is available on the rate of airway bacterial carriage and its consequence in hypogammaglobulinemias. Despite IgG replacement, recurrent respiratory infections are common in CVID, possibly leading to chronic lung damage2 and poor quality of life.3 Thus, patients are often prescribed antibiotics and/or long-term antimicrobial prophylactic regimens. Several regimens are used including rotation or periodically changing antibiotics.4 However, antibiotics influence antimicrobial resistance among airway microbiota. In a recent meta-analysis on patients with chronic lung diseases, 30% of S pneumoniae showed resistance to macrolides.

Health-Related Quality of Life in Patients with CVID Under Different Schedules of Immunoglobulin Administration: Prospective Multicenter Study

We assessed the health-related quality of life (HRQoL) in CVID adults receiving different schedules of immunoglobulin replacement therapy (IgRT) by intravenous (IVIG), subcutaneous (SCIG), and facilitated (fSCIG) preparations. For these patients, IgRT schedule was chosen after a period focused on identifying the most suitable individual option

Appropriate lung management in patients with primary antibody deficiencies

Introduction: Human primary immunodeficiency diseases (PIDs) include a broad spectrum of more than 350 disorders, involving different branches of the immune system and classified as 'rare diseases.' Predominantly antibody deficiencies (PADs) represent more than half of the PIDs diagnosed in Europe and are often diagnosed in the adulthood. Areas covered: Although PAD could first present with autoimmune or neoplastic features, respiratory infections are frequent and respiratory disease represents a relevant cause of morbidity and mortality. Pulmonary complications may be classified as infection-related (acute and chronic), immune-mediated, and neoplastic. Expert opinion: At present, no consensus guidelines are available on how to monitor and manage lung complications in PAD patients. In this review, we will discuss the available diagnostic, prognostic and therapeutic instruments and we will suggest an appropriate and evidence-based approach to lung diseases in primary antibody deficiencies. We will also highlight the possible role of promising new tools and strategies in the management of pulmonary complications. However, future studies are needed to reduce of diagnostic delay of PAD and to better understand lung diseases mechanisms, with the final aim to ameliorate therapeutic options that will have a strong impact on Quality of Life and long-term prognosis of PAD patients