Conţinutul numărului revistei |
Articolul precedent |
Articolul urmator |
787 6 |
Ultima descărcare din IBN: 2023-01-25 22:43 |
Căutarea după subiecte similare conform CZU |
616.23/24-097-022-053.2-036 (1) |
Patologie. Medicină clinică (6994) |
SM ISO690:2012 TOMACINSCHI, Cristina, SELEVESTRU, Rodica, ŞCIUCA, Svetlana. Manifestări bronhopulmonare în agamaglobulinemie. In: Buletin de Perinatologie, 2018, nr. 5(81), pp. 13-16. ISSN 1810-5289. |
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Buletin de Perinatologie | ||||||
Numărul 5(81) / 2018 / ISSN 1810-5289 | ||||||
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CZU: 616.23/24-097-022-053.2-036 | ||||||
Pag. 13-16 | ||||||
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Actuality: Bruton disease is a primary immunodefi ciency characterized by the absence of mature B cells with a severe antibody defi ciency. Clinical manifestations have their onset as soon as the protective eff ect of maternal immunoglobulins decreases, approximately at the age of six months. The first sign are frequent respiratory infections, which may have a severe progression, and in the absence of early diagnosis and substitution treatment may present a life-threatening risk. Material and methods. This article presents the clinical and exploratory results of 4 children diagnosed with agammaglobulinemia, focusing on determination of immunoglobulins and lymphocytic population and the polymorphism of bronchopulmonary manifestations. Results. For all children, the diagnosis of Bruton agammaglobulinaemia was suspected by the persistence of respiratory infections and severe purulent infections. The 4 patients primarily address with frequent respiratory infections and failure to thrive. In 2 of the children were present purulent infections with diff erent localizations (meningitis, empyema, sinusitis, osteomyelitis) with progressive severe progression. Gradually all patients involved chronic bronchopulmonary phenomena with bronchial deformities, pulmonary fi brosis, bronchiectasis. The immunological investigation in all patients indicated a reduction of serum immunoglobulin values: IgA - 1.78±0.9 mg/dl; IgM - 1.8±0.67 mg/dl; IgG - 6.75±0.54 mg/dl. Determination of lymphocyte populations in these patients resulted in normal T – 49±13.8% lymphocyte variation and a signifi cant reduction in B cells of 8.8±3.03% with a minimum of 2% and a maximum of 12%. One of the children underwent genetic testing, with a hemizigotic change of IVS1+5G>A in the BTK 1 intron 1. Conclusion. The study demonstrates the variety of bronchopulmonary manifestations in patients with agammaglobulinemia. Summarizing them may be helpful in setting the diagnosis as early as possible and initiating treatment to avoid further complications. |
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Cuvinte-cheie agammaglobulinemia, infections, immunodefi ciencies, агаммаглобулинемия, инфекции, иммунодефициты |
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