Publications by authors named "S Ottaviani"

147 Publications

[Compliance with biologic agents: Current situation].

Rev Mal Respir 2021 Jun 14. Epub 2021 Jun 14.

Service de rhumatologie, hôpital Bichat-Claude Bernard, AP-HP, 46, rue Henri-Huchard, 75018 Paris, France.

Despite the fact that the prognosis of chronic inflammatory disorders is improved by biological agents, compliance with those therapeutics remains imperfect. Compliance corresponds to the measurable part of the follow-up of the medical prescription by the patient, whereas adherence is related to the acceptation of the treatment by the patient. The compliance rates of biologic agents are generally higher than those of conventional therapies. Compliance can be influenced by the real or experienced efficacity of the treatment, by patient-related factors or by the patient-physician relationship. An increase of compliance is associated with an improvement of adherence. To achieve this, the physician can use educational measures such as patient education, which allows the identification of poor adherence. Such programs have been shown to improve the patient's knowledge of the disease and treatment leading to better adherence and compliance.
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http://dx.doi.org/10.1016/j.rmr.2021.06.001DOI Listing
June 2021

The Importance of N186 in the Alpha-1-Antitrypsin Shutter Region Is Revealed by the Novel Bologna Deficiency Variant.

Int J Mol Sci 2021 May 26;22(11). Epub 2021 May 26.

Department of Molecular and Translational Medicine, University of Brescia, viale Europa 11, 25123 Brescia, Italy.

Alpha-1-antitrypsin (AAT) deficiency causes pulmonary disease due to decreased levels of circulating AAT and consequently unbalanced protease activity in the lungs. Deposition of specific AAT variants, such as the common Z AAT, within hepatocytes may also result in liver disease. These deposits are comprised of ordered polymers of AAT formed by an inter-molecular domain swap. The discovery and characterization of rare variants of AAT and other serpins have historically played a crucial role in the dissection of the structural mechanisms leading to AAT polymer formation. Here, we report a severely deficient shutter region variant, Bologna AAT (N186Y), which was identified in five unrelated subjects with different geographical origins. We characterized the new variant by expression in cellular models in comparison with known polymerogenic AAT variants. Bologna AAT showed secretion deficiency and intracellular accumulation as detergent-insoluble polymers. Extracellular polymers were detected in both the culture media of cells expressing Bologna AAT and in the plasma of a patient homozygous for this variant. Structural modelling revealed that the mutation disrupts the hydrogen bonding network in the AAT shutter region. These data support a crucial coordinating role for asparagine 186 and the importance of this network in promoting formation of the native structure.
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http://dx.doi.org/10.3390/ijms22115668DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8198886PMC
May 2021

Groin Pain After Gemcitabine for Lung Cancer.

J Clin Rheumatol 2021 Jan 19. Epub 2021 Jan 19.

From the Departments of *Rheumatology †Thoracic Oncology, Bichat-Claude Bernard Hospital, Paris, France.

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http://dx.doi.org/10.1097/RHU.0000000000001652DOI Listing
January 2021

COVID-19 infection in severe Alpha 1-antitrypsin deficiency: Looking for a rationale.

Respir Med 2021 07 30;183:106440. Epub 2021 Apr 30.

Centre for Diagnosis of Inherited Alpha-1 Antitrypsin Deficiency, Laboratory of Biochemistry and Genetics, Institute for Respiratory Disease, Department of Internal Medicine and Therapeutics, University of Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.

The clinical manifestations of COVID-19 are heterogeneous: 46.4% of patients admitted into hospital reported to have at least one comorbidity. Comorbidities such as COPD, diabetes, hypertension and malignancy predispose patients with Covid-19 to adverse clinical outcomes. Alpha 1-antitrypsin deficiency (AATD) is a genetic disorder caused by pathological mutation(s) in the SERPINA1 gene resulting in an imbalance in proteinase activity which may lead to premature emphysema and COPD. Our aim was to investigate whether people with severe AAT deficiency (AATD) have an increased risk of (severe) COVID-19 infection. We collected data on COVID-19 symptoms, laboratory-confirmed infection, hospitalization and treatment by means of a telephone survey, directly administered to Italian severe AATD subjects in May 2020. We then compared our findings with data collected by the Istituto Superiore di Sanità on the total population in Italy during the same period. We found an higher frequency of SARS-CoV-2 infection in our cohort (3.8%) compared to national data regarding infection, thus giving severe AATD a relative risk of 8. 8 (95%CI 5.1-20,0; p<0.0001) for symptomatic SARS-CoV-2 infection. Moreover, the relative risk (RR) was higher in AATD patients with pre-existing lung diseases (RR 13.9; 95%CI 8.0-33.6; p<0.001), but with a similar death rate (1 in 8, 12.5%) compared to the general population (13.9%; RR 0.9). These preliminary findings highlight the importance of close surveillance in the spread of COVID-19 in patients with severe AATD and underlines the need for further studies into the role of the antiprotease shield in preventing SARS-Cov-2 infection.
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http://dx.doi.org/10.1016/j.rmed.2021.106440DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8086383PMC
July 2021