Pneumonologia i alergologia polska
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Pneumonol Alergol Pol · Jan 2013
Review[Genetic variants of alpha-1 antitrypsin: classification and clinical implications].
Inherited alpha-1 antitrypsin deficiency is listed among the three most common genetic disorders in Caucasians. It considerably increases the risk of progressive obstructive lung diseases, mostly chronic obstructive pulmonary disease, as well as chronic liver disorders, hepatitis, cirrhosis, and cancer. It is estimated that more than 5.5% of the Polish population carries one of the most common deficiency phenotypes, which might be relatively easily detected due to low alpha-1 antitrypsin serum concentration. ⋯ These dysfunctional alpha-1 antitrypsin variants are characterized by scarce antiproteolytic activity and quite often by fully effective protein production resulting in normal serum levels. Consequently, dysfunctional variant identification is possible only by means of pheno- or genotyping. This review presents clinically useful characteristics of main genetic alpha-1 antitrypsin variants.
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Pneumonol Alergol Pol · Jan 2013
Case Reports[Nodular lymphoid hyperplasia - a rare case of lymphoproliferative disorder of the lungs].
Nodular lymphoid hyperplasia (NLH) belongs to a very rare, mild, lymphoproliferative disease of unestablished aetiology historically included in the group of pseudolymphomas. Its existence was controversial for many years, until modern techniques of pathomorphological diagnosis approved it as a separate entity of lung disease. It manifests in the form of well limited nodules localized in the lungs, which are mostly identified accidentally. ⋯ The patient was qualified for right-sided videothoracoscopy and removal of the lung nodule. In classic HE staining of the histological material, the presence of lymphoid infiltration of the lungs was revealed, which formed lymph follicles with reactive germinal centres. In order to differentiate from the malignant lymphatic expansion, immunohistochemical designations were made, which showed positive expression of CD20 antigen in the B cell zone, positive expression of the CD3 antigen in the T cells zone, positive expression of CD23 antigen in the lymph follicles, negative expression of bcl-2 in the lymph follicles, and positive expression of MIB-1 in the germinal centres of lymph follicles. Such a histopathological and immunohistochemical picture provided the basis for diagnosis of nodular lymphoid hyperplasia of the lung.
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Pneumonol Alergol Pol · Jan 2013
Review Case ReportsSevere Legionella pneumophila pneumonia and non-invasive ventilation: presentation of two cases and brief review of the literature.
Legionella pneumophila is an agent also well known to be frequently responsible for severe community acquired pneumonia. Recent studies regarding severe community-acquired pneumonia have shown that Legionella pneumophila is the second most common cause of admission to ICU, not far behind pneumococcal pneumonia. The mortality of severe Legionella pneumonia is high (30%). ⋯ The use of non-invasive ventilation in severe community acquired pneumonia is controversial. However, after recent pandemics, the number of studies reporting good rates of success for NIV has increased. Both our patients were managed in a respiratory intermediate care unit, avoiding invasive ventilation and invasive monitoring, which lowered costs yet was equally effective in providing a good outcome when compared to intubation in the Intensive Care Unit.