Publications by authors named "Lucia Leonardi"

21 Publications

  • Page 1 of 1

Is a strict cow's milk-free diet necessary to treat milk oral immunotherapy-related eosinophilic esophagitis?

Acta Biomed 2021 07 1;92(3):e2021033. Epub 2021 Jul 1.

Dipartimento di Pediatria, Università Sapienza, Roma, Italia .

We report the case of a 15-year-old boy who developed eosinophilic esophagitis (EoE) during cow's milk oral immunotherapy (CM-OIT). In order to not completely invalidate OIT benefits, baked milk-containing foods were allowed instead of a strict CM-free diet. However, histological remission of EoE was reached only after a strict cow's milk-free diet, not associated to pharmacological treatment. Nevertheless, given the limited data on real incidence of food OIT related EoE and potential beneficial treatment for this condition, we highlight the need of prospective studies aimed to evaluate if a strict CM free diet in OIT related EoE is always necessary to obtain remission of the disease or similarly to CM EoE, baked milk-containing foods" diet could be abeneficial treatment also in these patients.
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http://dx.doi.org/10.23750/abm.v92i3.10050DOI Listing
July 2021

Inter-society consensus for the use of inhaled corticosteroids in infants, children and adolescents with airway diseases.

Ital J Pediatr 2021 Apr 21;47(1):97. Epub 2021 Apr 21.

Pediatric Unit, Department of Mother and Child, AUSL-IRCCS, Reggio Emilia, Italy.

Background: In 2019, a multidisciplinary panel of experts from eight Italian scientific paediatric societies developed a consensus document for the use of inhaled corticosteroids in the management and prevention of the most common paediatric airways disorders. The aim is to provide healthcare providers with a multidisciplinary document including indications useful in the clinical practice. The consensus document was intended to be addressed to paediatricians who work in the Paediatric Divisions, the Primary Care Services and the Emergency Departments, as well as to Residents or PhD students, paediatric nurses and specialists or consultants in paediatric pulmonology, allergy, infectious diseases, and ear, nose, and throat medicine.

Methods: Clinical questions identifying Population, Intervention(s), Comparison and Outcome(s) were addressed by methodologists and a general agreement on the topics and the strength of the recommendations (according to the GRADE system) was obtained following the Delphi method. The literature selection included secondary sources such as evidence-based guidelines and systematic reviews and was integrated with primary studies subsequently published.

Results: The expert panel provided a number of recommendations on the use of inhaled corticosteroids in preschool wheezing, bronchial asthma, allergic and non-allergic rhinitis, acute and chronic rhinosinusitis, adenoid hypertrophy, laryngitis and laryngospasm.

Conclusions: We provided a multidisciplinary update on the current recommendations for the management and prevention of the most common paediatric airways disorders requiring inhaled corticosteroids, in order to share useful indications, identify gaps in knowledge and drive future research.
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http://dx.doi.org/10.1186/s13052-021-01013-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8058583PMC
April 2021

Inborn errors of immunity with atopic phenotypes: A practical guide for allergists.

World Allergy Organ J 2021 Feb 22;14(2):100513. Epub 2021 Feb 22.

Department of Pediatrics, Giovanni XXIII Pediatric Hospital, Bari, Italy.

Inborn errors of immunity (IEI) are a heterogeneous group of disorders, mainly resulting from mutations in genes associated with immunoregulation and immune host defense. These disorders are characterized by different combinations of recurrent infections, autoimmunity, inflammatory manifestations, lymphoproliferation, and malignancy. Interestingly, it has been increasingly observed that common allergic symptoms also can represent the expression of an underlying immunodeficiency and/or immune dysregulation. Very high IgE levels, peripheral or organ-specific hypereosinophilia, usually combined with a variety of atopic symptoms, may sometimes be the epiphenomenon of a monogenic disease. Therefore, allergists should be aware that severe and/or therapy-resistant atopic disorders might be the main clinical phenotype of some IEI. This could pave the way to target therapies, leading to better quality of life and improved survival in affected patients.
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http://dx.doi.org/10.1016/j.waojou.2021.100513DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7907539PMC
February 2021

Immunological basis of virus-host interaction in COVID-19.

Pediatr Allergy Immunol 2020 11;31 Suppl 26:75-78

Department of Pediatrics, Giovanni XXIII Pediatric Hospital, Bari, Italy.

COVID-19 is a complex new viral disease, in which a strict balance between anti-viral immune response and the ensuing organ inflammation has a critical role in determining the clinical course. In adults, compelling evidence exists indicating that an uncontrolled inflammatory response ("cytokine storm") is pivotal in determining disease progression and mortality. Children may rarely present with severe disease. Modulating factors related to the host's genetic factors, age-related susceptibility, and the capability to mount appropriate immune responses might play a role in control virus load at an early stage and regulating the inflammatory reaction. Elucidating these mechanisms seems crucial in developing target therapies according to patient's age, immunologic status, and disease evolution in COVID-19.
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http://dx.doi.org/10.1111/pai.13363DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7753582PMC
November 2020

Case Report: A Case of X-Linked Agammaglobulinemia With High Serum IgE Levels and Allergic Rhinitis.

Front Immunol 2020 5;11:582376. Epub 2020 Nov 5.

Department of Pediatrics, Policlinico Umberto I, Sapienza University of Rome, Rome, Italy.

X-linked Agammaglobulinemia (XLA) is a rare genetic disorder of B-lymphocyte differentiation, characterized by the absence or paucity of circulating B cells, markedly reduced levels of all serum immunoglobulin isotypes and lack of specific antibody production. Bruton Tyrosine Kinase () gene encodes a cytoplasmic tyrosine kinase involved in the B cell maturation and its mutation, blocking B cell differentiation at the pre-B cell stage, and is responsible for XLA. All domains may be affected by the mutation, and the many genotypes are associated with a wide range of clinical presentations. Little is known about genotype-phenotype correlation in this disorder, and factors influencing the phenotype of XLA are not clearly understood. In this report we present a unique case of a young patient affected by XLA. The disease was genetically diagnosed at birth due to a family history of XLA, but during follow up, it was characterized by a CD19+ B cell percentage consistently greater than 2%. He never suffered severe infections, but at two years of age, he developed persistent rhinitis. Thus, total serum IgE levels were measured and detected over the normal range, and specific allergic investigations showed sensitization to dust mites. Further immunological tests (BTK expression, functional "" B cell proliferation upon CpG stimulation, B cell subset analysis) explained these findings as possible manifestations of a mild XLA phenotype. XLA patients rarely present with allergic manifestations, which could warrant further investigation. High serum IgE levels could be a sign of a mild phenotype, but their role and the mechanisms underlying their production in XLA need to be clarified.
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http://dx.doi.org/10.3389/fimmu.2020.582376DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7674281PMC
June 2021

Update in Primary Immunodeficiencies.

Acta Biomed 2020 09 15;91(11-S):e2020010. Epub 2020 Sep 15.

Department of Pediatrics and Emergency, Pediatric Allergy and Pulmunology Unit, Azienda Ospedaliera-Universitaria Consorziale-Policlinico, Ospedale Pediatrico Giovanni XXIII, Bari, Italy.

Primary immunodeficiencies (PIDs) are inherited disorders classically characterized by increased susceptibility to infections. Nevertheless, in the last two decades, genomic analysis (such as NGS) coupled with biochemical and cellular studies led to a more accurate definition for a growing number of novel genetic disorders associated with PIDs. This revealed new aspects of the immune system and its function and regulation within these diseases. In particular, it has been clarified that the clinical features of PIDs are much broader that originally thought and extend beyond an increased susceptibility to infections. More specifi- cally, immune dysregulation is very often described in novel characterized PIDs and can lead to multiple autoimmune diseases, lymphoproliferation and malignancies. If not promptly diagnosed, these could negatively impact patient's prognosis. The aim of this review is to increase the awareness of recently discovered PIDs, characterized predominantly by immune dysregulation phenotypes. Findings highlighted in this review  suggest screening for immunodeficiency in patients with lymphoproliferation or early onset/multiple autoimmune diseases. Prompt diagnosis would potentially allow most successful treatment and clinical outcome for patients with PIDs.
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http://dx.doi.org/10.23750/abm.v91i11-S.10314DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8023064PMC
September 2020

Polychlorinated biphenyls (PCBs) and risk of dementia and Parkinson disease: A population-based cohort study in a North Italian highly polluted area.

Chemosphere 2020 Dec 4;261:127522. Epub 2020 Jul 4.

ATS Brescia (Brescia Health Protection Agency), Italy.

Background: Serum polychlorinated biphenyls (PCBs) have been associated with some chronic diseases, but little evidence exists on their possible relationship with neurodegenerative diseases. We aimed to assess the relationship between PCB exposure and the occurrence of dementia and Parkinson disease in a prospective cohort study in a highly polluted area (Brescia-Caffaro).

Methods: PCB exposure was assessed by measuring serum levels of 24 congeners. Data on the onset of dementia and Parkinson disease were retrieved by the Brescia Health Protection Agency Database. We used Poisson regression models adjusted for possible confounders to calculate rate ratios (RRs). A mediation analysis was performed to evaluate the mediatory role of cardiovascular diseases.

Results: 699 subjects without neurologic diseases at baseline were enrolled (48.1% males, 63.2 years of mean age) in 2001-2013 and followed up to 2018. During a mean follow-up of 8.8 years, 36 and 20 subjects developed dementia and Parkinson disease. Subjects in the 2nd and 3rd tertiles of the total PCBs distribution, compared with those in the 1st tertile, had a higher risk of dementia (RR = 2.30 and RR = 4.35). The estimates for Parkinson disease included the null value with wide confidence intervals. In the mediation analysis, the association between PCB exposure and dementia was dominated by the direct pathway and not by the hypertension-mediated pathway.

Conclusions: We observed a positive association between total PCBs serum levels and the onset of dementia not mediated by hypertension. For Parkinson, the unstable risk estimates did not allow to draw a conclusion on a possible association.
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http://dx.doi.org/10.1016/j.chemosphere.2020.127522DOI Listing
December 2020

Efficacy of Pidotimod use in treating allergic rhinitis in a pediatric population.

Ital J Pediatr 2020 Jul 7;46(1):93. Epub 2020 Jul 7.

Department of Pediatrics, Policlinico Umberto I, Sapienza University, Rome, Italy.

Background: Allergic rhinitis (AR) and adenoidal hypertrophy (AH) are the most frequent causative disorders of nasal obstruction in children, leading to recurrent respiratory infections. Both nasal cavities are colonized by a stable microbial community susceptible to environmental changes and Staphylococcus aureus seems to play the major role. Furthermore, nasal microbiota holds a large number and variety of viruses with upper respiratory tract infections. This local microbiota deserves attention because its modification could induce a virtuous cross-talking with the immune system, with a better clearance of pathogens. Although AR and AH present a different etiopathogenesis, they have in common a minimal chronic inflammation surrounding nasal obstruction; hence it would be challenging to evaluate the effect of an immunomodulator on this minimal chronic inflammation with possible clinical and microbiological effects. The aim of this study is therefore to evaluate the efficacy of an immunomoldulator (Pidotimod) on nasal obstruction in children with AR and/or AH and whether its action involves a variation of nasal microbiota.

Methods: We enrolled 76 children: those with allergic rhinitis (AR) sensitized to dust mites entered the AR group, those with adenoidal hypertrophy (AH) the AH group, those with both conditions the AR/AH group and those without AR ± AH as controls (CTRL). At the first visit they performed: skin prick tests, nasal fiberoptic endoscopy, anterior rhinomanometry, nasal swabs. Children with. AR ± AH started treatment with Pidotimod. After 1 month they were re-evaluated performing the same procedures. The primary outcome was the evaluation of nasal obstruction after treatment and the secondary outcome was the improvement of symptoms and the changes in nasal microflora.

Results: All patients improved their mean nasal flow (mNF) in respect to the baseline. In AR children mNF reached that one of CTRL. In AH children±AR the mNF was lower in respect to CTRL and AR group. We did not find any differences among all the groups at the two different time points in nasal microflora.

Conclusions: Pidotimod is able to give an improvement in nasal obstruction, especially in AR children but this effect seems to be not mediated by changes in nasal microbiota.
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http://dx.doi.org/10.1186/s13052-020-00859-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7341603PMC
July 2020

Relationship between quality of life and behavioural disorders in children with persistent asthma: a Multiple Indicators Multiple Causes (MIMIC) model.

Sci Rep 2020 04 24;10(1):6957. Epub 2020 Apr 24.

Unit of Epidemiology, 'Anna Meyer' Children's University Hospital, Viale Pieraccini 24, 50139, Florence, Italy.

Knowledge on multiple interdependences between quality of life (QoL) and behavioural problems in relation to asthma severity and control is undetermined. The aims of the study were: (i) to assess the relationship of QoL and behavioural problems with asthma severity and control (ii) to predict children's "abnormal/borderline" status with variation in QoL. For these purposes a multicenter case-control study on 47 Severe Asthma (SA) and 94 Moderate Asthma (MA) children was performed. The MIMIC approach was applied to investigate the effect of SA and non-controlled asthma (NC) on QoL and behavioural disorders. Logistic regression was used to estimate probabilities of having an "abnormal/borderline" status with variation in QoL. The MIMIC model showed that the magnitude of the effect of SA and NC was larger on QoL (β = -0.37 and β = -0.30, respectively) than on behavioural problems (β = 0.27). With regards to the probability of having a borderline status, in MA a QoL of 1 returned a probability of 0.81, whereas in SA a QoL of 1 returned a probability of 0.89. In conclusion, SA children are highly affected by impaired QoL and behavioural problems. The MIMIC model allowed us to obtain a comprehensive assessment of QoL and behavioural problems with asthma severity and control.
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http://dx.doi.org/10.1038/s41598-020-62264-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7181655PMC
April 2020

Long-term follow-up of 168 patients with X-linked agammaglobulinemia reveals increased morbidity and mortality.

J Allergy Clin Immunol 2020 08 10;146(2):429-437. Epub 2020 Mar 10.

University Department of Pediatrics, Unit of Immune and Infectious Diseases, Bambino Gesù Children's Hospital, University of Rome Tor Vergata, and the Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.

Background: X-linked agammaglobulinemia (XLA) is the prototype of primary humoral immunodeficiencies. Long-term follow-up studies regarding disease-related complications and outcome are scarce.

Objective: Our aim was to describe the natural history of XLA.

Methods: A nationwide multicenter study based on the Italian Primary Immunodeficiency Network registry was established in 2000 in Italy. Affected patients were enrolled by documenting centers, and the patients' laboratory, clinical, and imaging data were recorded on an annual base.

Results: Data on the patients (N = 168) were derived from a cumulative follow-up of 1370 patient-years, with a mean follow-up of 8.35 years per patient. The mean age at diagnosis decreased after establishment of the Italian Primary Immunodeficiency Network registry (84 months before vs 23 months after). Respiratory, skin, and gastrointestinal manifestations were the most frequent clinical symptoms at diagnosis and during long-term follow-up. Regular immunoglobulin replacement treatment reduced the incidence of invasive infections. Affected patients developed chronic lung disease over time (47% after 40 years of follow-up) in the presence of chronic sinusitis (84%). Malignancies were documented in a minority of cases (3.7%). Overall survival for affected patients was significantly reduced when compared with that for the healthy male Italian population, and it further deteriorated in the presence of chronic lung disease.

Conclusions: This is the first detailed long-term follow-up study for patients with XLA, revealing that although immunoglobulin replacement treatment reduces the incidence of invasive infections, it does not appear to influence the development of chronic lung disease. The overall survival of affected patients is reduced. Further studies are warranted to improve patients' clinical management and increase awareness among physicians.
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http://dx.doi.org/10.1016/j.jaci.2020.03.001DOI Listing
August 2020

Polychlorinated biphenyls (PCBs) and risk of hypertension: A population-based cohort study in a North Italian highly polluted area.

Sci Total Environ 2020 Apr 11;714:136660. Epub 2020 Jan 11.

ATS Brescia (Brescia Health Protection Agency), Brescia, Italy. Electronic address:

This study aimed to assess the longitudinal association between PCB exposure and the subsequent occurrence of hypertension in a highly polluted area. The study subjects had at least one PCB serum measurement between 2003 and 2014 and were followed up to the end of 2017. Hypertension cases were obtained from the Brescia Health Protection Agency database. Risk ratios of hypertension for total PCBs, high-, middle- and low-chlorinated groups and some common congeners were estimated using Poisson regression models. 1031 subjects were included (43.4% males, mean age 45.3 years). 229 subjects developed hypertension during the follow-up. Total PCBs, middle- and high-chlorinated PCBs, and particularly PCB congeners 138, 153 and 180 were associated with an about 2-fold increased risk of hypertension comparing the third with first tertile of distribution. After lipid-standardization of PCB serum values, the excess risk showed only a slight attenuation. A dose-response linear relationship of the risk of hypertension by PCB serum concentrations as continuous variables was also observed. The PCBs-hypertension association was stronger in overweight/obese subjects than those with normal body mass index. In this prospective cohort study the serum levels of total and middle to high chlorinated (≥6 Cl) PCBs were associated with an increased risk of hypertension, particularly among overweight/obese subjects.
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http://dx.doi.org/10.1016/j.scitotenv.2020.136660DOI Listing
April 2020

Update on rhinitis and rhinosinusitis.

Pediatr Allergy Immunol 2020 02;31 Suppl 24:32-33

Mother-Child, Urological Science Department, Sapienza University, Rome, Italy.

Rhinitis is a common, under-diagnosed condition in childhood and adolescence. Pathogenic classification of rhinitis includes allergic rhinitis (AR) and non-allergic rhinitis (NAR); the latter is characterized by non-infectious and infectious subtypes; non-infectious subtype is by far the most prevalent in infancy. Endotyping may help to predict chronic rhinosinusitis (CRS) risk factors, as well as disease progression and response to different treatments. The validation of pediatric algorithms and the implementation of novel biotherapeutics in the endotype-driven integrated pathways of CRS will lead in the next future to more successful disease management.
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http://dx.doi.org/10.1111/pai.13164DOI Listing
February 2020

Rare TACI Mutation in a 3-Year-Old Boy With CVID Phenotype.

Front Pediatr 2019 15;7:418. Epub 2019 Oct 15.

Division of Pediatric Immunology and Rheumatology, Department of Pediatrics, Sapienza University of Rome, Rome, Italy.

Common variable immunodeficiency (CVID) is the most common and clinically relevant primary immunodeficiency (PID). Genetic basis of CVID remains largely unknown. However, in a minority of CVID patients, a number of distinct genetic defects affecting the normal processes of B cell maturation and differentiation into memory B cells have now been identified, resulting in markedly reduced serum levels of immunoglobulin G (IgG) and low immunoglobulin A (IgA) or immunoglobulin M (IgM), with impaired antibody responses, despite the presence of normal levels of B cells. Patients with CVID develop recurrent and chronic infections of respiratory and gastrointestinal tracts, autoimmune diseases, lymphoproliferative complications, malignancies, and granulomatous disease. We report the case of a boy admitted to our unit for the first time at the age of three for reduced gamma globulin levels and a clinical history positive for two episodes of pneumonia. Our patient incompletely met ESID diagnostic criteria for CVID, but molecular genetic analysis, a NGS panel including 47 PID-associated genes was performed in the proband and in his parents, revealing the presence of a heterozygous nucleotide substitution in exon 4 (c.579C>A) of encoding TACI. This mutation has been described only in two CVID adult patients and in a child with selective IgA deficiency (sIgAD). We highlighted the same mutation in the asymptomatic mother and detected two extra heterozygous mutations of and . We promptly started intravenous immunoglobulin (IVIG) therapy with good tolerance. Despite the diagnosis of CVID remains clinical, in this case report we underline the importance of considering and planning genetic workup in all subjects with unclear diagnosis and of reporting new molecular diagnosis especially in case of rare mutations.
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http://dx.doi.org/10.3389/fped.2019.00418DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6803509PMC
October 2019

Serum levels of polychlorinated biphenyls and risk of non-Hodgkin lymphoma: A hospital-based case-control study.

Chemosphere 2019 Nov 1;235:969-975. Epub 2019 Jul 1.

Members of the Brescia NHL-PCB Working Group: Carmen Terraroli (Chief for coordination and clinical Research, ASST Spedali Civili), Arianna Coniglio (Surgical Clinic, Department of Experimental and Clinical Sciences, University of Brescia - Division of General Surgery, Brescia Hospital), Guido Zarattini (Orthopaedic Clinic University of Brescia, II Division of Orthopaedic and Traumatology Spedali Civili of Brescia, Italy), Giorgio Manca (Department of Plastic Surgery, ASST Spedali Civili, Brescia, Italy), Jacopo Fostinelli (Department of Medical and Surgical Specialties Radiological Sciences and Public Health, Institute of Occupational Health and Industrial Hygiene, University of Brescia), Roberto Festa, Maria Conti, Stefania Micheletti (Brescia Health Protection Agency), Italy.

Polychlorinated biphenyls (PCB) have been classified by the International Agency for Research on Cancer (IARC) in Group 1 as carcinogenic to human, based on sufficient evidence in humans of an increased risk of cutaneous malignant melanoma and limited evidence for non-Hodgkin lymphoma (NHL). However present findings on the association of PCB exposure and NHL are still controversial. This study examined the relationship between PCB serum levels and risk of NHL in a Northern Italy area (Brescia province), where a chemical factory produced PCBs from 1938 to 1984, causing human contamination. A case-control study of 215 NHL patients and 215 control subjects was conducted. Cases and controls were assayed for serum levels of 33 PCB congeners. No associations were found between risk of NHL and serum levels of total PCBs (OR = 0.51; 95% CI: 0.25-1.04 for highest vs lowest quartile) or specific PCB congeners. The study confirmed a strong association of NHL with HCV infection (OR = 3.60; 95% CI: 1.30-10.02). This case-control study does not support the hypothesis of an association between current serum levels of PCBs and NHL development in the general population.
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http://dx.doi.org/10.1016/j.chemosphere.2019.06.193DOI Listing
November 2019

Polychlorinated biphenyl serum levels, thyroid hormones and endocrine and metabolic diseases in people living in a highly polluted area in North Italy: A population-based study.

Heliyon 2019 Jun 4;5(6):e01870. Epub 2019 Jun 4.

Department of Medical and Surgical Specialties Radiological Sciences and Public Health, Unit of Epidemiology, Hygiene and Public Health, University of Brescia, Italy.

Polychlorinated biphenyls (PCBs) are persistent organic pollutants produced until the 1980s, which they are still present worldwide. They have been associated with metabolic and endocrine diseases and hypertension in humans, but definite evidence is lacking. A chemical factory producing PCBs caused a heavy pollution in an urban area in Northern Italy. We aimed to evaluate present PCBs serum levels according to demographic and lifestyle variables and their associations with endocrine and metabolic diseases and hypertension in the resident general population. A random sample of 816 adults aged 20-79 years (mean ± SD: 49.1 ± 16.5 years) was enrolled in a cross-sectional population-based study. The participants provided a fasting blood sample for laboratory analysis and were face-to-face interviewed about the presence of chronic diseases. The serum level of total PCBs was computed as the sum of 33 PCB congeners. The median serum level of lipid-adjusted total PCBs was 435.2 and 95 centile was 2154.9 ng/g lipid. Medium and high chlorinated PCBs with immunotoxic and endocrine disrupting activity contributed most to total PCB serum levels, particularly PCBs 138, 153, 170, 180 and 194. The serum levels of total PCBs and of PCB functional groups were positively associated with age and negatively with female gender, education, smoking habit and BMI, and not associated with serum levels of thyroid hormones and TSH and glycaemia and with presence of endocrine diseases, diabetes and hypertension by multivariable analysis. Subgroup analyses according to gender, age and BMI provided similar results. In conclusion, this study shows a long-term persistence of past exposure to PCBs and does not support the hypothesis of an association between PCB exposure and prevalence of endocrine and metabolic diseases and hypertension.
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http://dx.doi.org/10.1016/j.heliyon.2019.e01870DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6551472PMC
June 2019

Immunophenotype Anomalies Predict the Development of Autoimmune Cytopenia in 22q11.2 Deletion Syndrome.

J Allergy Clin Immunol Pract 2019 Sep - Oct;7(7):2369-2376. Epub 2019 Mar 26.

University Department of Pediatrics, Unit of Immune and Infectious Diseases, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy; Department of Systems Medicine, University of Rome Tor Vergata, Rome, Italy.

Background: Patients with 22q11.2 deletion syndrome (22q11.2DS) may develop severe thrombocytopenic purpura and hemolytic anemia. There are no reliable predictors for the development of hematologic autoimmunity (HA) in these patients.

Objective: To describe the peculiar B and T subpopulation defects in patients with 22q11DS who have developed HA and test if these defects precede the development of HA.

Methods: We performed a case-control multicenter study. Patients with HA were compared with a control population of 22q11.2DS without HA (non-HA). A complete immunological evaluation was performed at diagnosis and at the last follow-up including extensive T and B phenotypes.

Results: Immunophenotype at the last follow-up was available in 23 HA and 45 non-HA patients. HA patients had significantly decreased percentage of naïve CD4 cells (26.8% vs 43.2%, P = .003) and recent thymic emigrants (48.6% vs 80.5%, P = .046); decreased class-switched B cells (2.0% vs 5.9%, P = .04) and increased naive B cells (83.5% vs 71.4%, P = .02); increased CD16+/56+ both in absolute number (312 vs 199, P = .009) and percentage (20.0% vs 13.0%, P = .03). Immunophenotype was performed in 36 patients (11 HA and 25 non-HA) at diagnosis. Odds ratio (OR) of immune cytopenia were estimated for both CD4 naïve ≤30% (OR 14.0, P = .002) and switched memory B cells ≤2% (OR 44.0, P = .01). The estimated survival curves reached statistical significance, respectively, P = .0001 and P = .002.

Conclusions: Among patients with 22q11.2DS, those with HA have characteristic lymphocyte anomalies that appear considerably before HA onset. Systematic immunophenotyping of patients with 22q11.2DS at diagnosis is advisable for early identification of patients at risk for this severe complication.
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http://dx.doi.org/10.1016/j.jaip.2019.03.014DOI Listing
October 2020

Chronic urticaria in a child affected by atopic dermatitis: effective treatment with omalizumab.

J Dermatolog Treat 2018 ;29(sup3):17-19

a Pediatrics Department , Umberto I Hospital, Sapienza University , Roma , Italy.

This article describes a clinical experience in a 7-year-old girl with chronic spontaneous urticaria and atopic dermatitis, resistant to therapy with antihistamines and topical steroid. Omalizumab treatment not only allowed complete remission of chronic spontaneous urticaria but also significantly improved atopic dermatitis symptoms. The off-label use of omalizumab was well tolerated and no adverse events were reported.
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http://dx.doi.org/10.1080/09546634.2018.1543844DOI Listing
January 2018

Plasma levels of polychlorinated biphenyls and risk of cutaneous malignant melanoma: A hospital-based case-control study.

Environ Int 2018 04 28;113:20-25. Epub 2018 Jan 28.

Section of Dermatology, Department of Clinical and Experimental Sciences, University of Brescia, Italy.

Polychlorinated biphenyls (PCB) have been classified by the International Agency for Research on Cancer (IARC) in Group 1 as carcinogenic to human, based on sufficient evidence in humans of an increased risk of cutaneous malignant melanoma (CMM), however few studies have been done in the general population. This study examined the relationship between PCB plasma levels and risk of CMM adjusting for sun sensitivity and sun exposure in a province of Northern Italy (Brescia), where a chemical factory produced PCBs from 1938 to 1984 causing human contamination. A case-control study of 205 CMM patients and 205 control subjects was conducted. Cases and controls were assayed for plasma levels of 33 PCB congeners. No associations was found between risk of CMM and plasma levels of total PCB (OR = 0.81; 95% CI: 0.34-1.96 for highest vs lowest quartile) or specific congeners. The study confirmed the association with light skin colour (OR = 3.00; 95% CI: 1.91-4.73), cumulative lifetime UV exposure (OR = 2.56; 95% CI: 1.35-4.85) and high level of education (OR = 1.45; 95% CI: 1.03-2.05). This case-control study does not support the hypothesis of an association between current plasma levels of PCBs and CMM development in the general population.
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http://dx.doi.org/10.1016/j.envint.2018.01.018DOI Listing
April 2018

Temporal trends of polychlorinated biphenyls serum levels in subjects living in a highly polluted area from 2003 to 2015: a follow-up study.

Int J Hyg Environ Health 2017 04 16;220(2 Pt B):461-467. Epub 2017 Jan 16.

ATS Brescia (Brescia Health Protection Agency), Italy.

Introduction: A chemical factory located in Brescia, an industrialized town in North Italy, produced polychlorinated biphenyls (PCBs) since the 1930s to the 1980s, determining a relevant pollution of soil, food and people. Since 2001, following a survey carried out in the general population living in the town, the individuals with PCB serum levels higher than the reference entered a follow-up study.

Methods: We performed a prospective cohort study including subjects with at least three PCB measures during the period 2003-2015. We evaluated the temporal trends of total PCB serum levels, the most common PCB congeners (PCB 138,153, 170, 180, 194 and 209), and toxic equivalency of dioxin-like PCBs using univariate and multivariate mixed models with and without a cubic-spline term for time.

Results: A total of 128 subjects were enrolled (46.1% males, mean age at enrolment 57.7 years). The geometric mean of PCBs decreased from 35.4ng/ml at 0-3 years to 29.3ng/ml at 4-8 years and to 23.0ng/ml at 9-12 years. A 3.8% reduction of total PCB serum levels per year was estimated using both univariate and multivariate mixed models adjusted for age; a similar reduction was found for the most common PCB congeners and for toxic equivalency. Total PCB serum levels decreased over time for each age group: -3.9%, -4.0% and -3.4% for subjects aged ≤55 years, 56-65 and ≥66 years, respectively.

Conclusion: A significant decrease of serum concentrations of PCBs was observed in subjects with high serum levels of these chemicals at enrolment over a twelve year period. The decrease was evident for each PCB congener and age group.
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http://dx.doi.org/10.1016/j.ijheh.2017.01.002DOI Listing
April 2017

B-cell activation with CD40L or CpG measures the function of B-cell subsets and identifies specific defects in immunodeficient patients.

Eur J Immunol 2017 01 25;47(1):131-143. Epub 2016 Nov 25.

B Cell Physiopathology Unit, Immunology Research Area, Ospedale Pediatrico Bambino Gesù IRCSS, Roma, Italy.

Around 65% of primary immunodeficiencies are antibody deficiencies. Functional tests are useful tools to study B-cell functions in vitro. However, no accepted guidelines for performing and evaluating functional tests have been issued yet. Here, we report our experience on the study of B-cell functions in infancy and throughout childhood. We show that T-independent stimulation with CpG measures proliferation and differentiation potential of memory B cells. Switched memory B cells respond better than IgM memory B cells. On the other hand, CD40L, a T-dependent stimulus, does not induce plasma cell differentiation, but causes proliferation of naïve and memory B cells. During childhood, the production of plasmablasts in response to CpG increases with age mirroring the development of memory B cells. The response to CD40L does not change with age. In patients with selective IgA deficiency (SIgAD), we observed that switched memory B cells are reduced due to the absence of IgA memory B cells. In agreement, IgA plasma cells are not generated in response to CpG. Unexpectedly, B cells from SIgAD patients show a reduced proliferative response to CD40L. Our results demonstrate that functional tests are an important tool to assess the functions of the humoral immune system.
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http://dx.doi.org/10.1002/eji.201646574DOI Listing
January 2017

Substantial decline of polychlorinated biphenyls serum levels 10years after public health interventions in a population living near a contaminated site in Northern Italy.

Environ Int 2016 Oct 10;95:69-78. Epub 2016 Aug 10.

Department of Medical and Surgical Specialties Radiological Sciences and Public Health, Institute of Occupational Health and Industrial Hygiene, University of Brescia, Italy.

Introduction: Polychlorinated biphenyls (PCBs) are persistent pollutants classified among endocrine disruptors and human carcinogens. In an urban area in Northern Italy (Brescia), a chemical factory producing PCBs from 1938 to 1984 caused soil and food pollution. Since the discovering of the environmental pollution in the area in 2001, various public health interventions have been implemented to avoid human contamination.

Methods: Two independent surveys were carried out in 2003 and 2013 using the same study design and methods. A random sample of the adult general population living in four areas of the town with different soil contamination level was enrolled in both surveys. Investigation included serum levels of 24 PCB congeners and of other common clinical-chemical parameters and questions about residential and occupational history as well as current and past diet and consumption of locally produced food. 537 subjects were tested in the 2003 and 488 in the 2013 survey.

Results: Total PCB serum levels showed a strong correlation with age (Spearman r=0.71 in 2003 and r=0.80 in 2013), but not with gender. Consumers of food produced in the most polluted area had higher total PCB serum levels (median 15.6 and 4.7ng/ml in 2003 and 2013, respectively) than non-consumers (4.1 and 2.3ng/ml) and consumers of food produced in non-polluted areas (6.5 and 1.9ng/ml), with increasing trend of PCB serum levels with cumulative local food intake. PCB serum levels from 2003 to 2013 survey declined by around half in all age groups, especially among the youngest people. The total PCB reduction was found to be due to a combination of a birth cohort effect (-41% every 10years) and survey period effect (-18% in 2013 versus 2003), with medium chlorinated CB congeners showing the most relevant reduction (-30%) between the two surveys. High chlorinated CBs were more strongly associated with birth cohort (-46% every 10years): in the 2013 survey they represented 58% of total PCBs in 60-79years old while they were 37% among the under 40s with a median value 20 times higher in the oldest than in the youngest (3.1 versus 0.16ng/ml).

Conclusions: PCBs serum levels declined from the 2003 to 2013 survey though they are still high in the elderly mainly because of past intake of locally produced contaminated food. Present findings suggest that PCBs food chain contamination and human absorption have been interrupted successfully in the area since discovery of the pollution in 2001.
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http://dx.doi.org/10.1016/j.envint.2016.07.021DOI Listing
October 2016
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