Effects of alfa-1-antitripsin deficiency on lung disease

Овчаренко, С. И.; Сон, Е. А.

doi:10.18093/0869-0189-2011-0-5-79-86

Cited by 2 publications

(6 citation statements)

References 47 publications

(61 reference statements)

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“…Індукція продукції інгібіторів протеаз, цитокінів сімейства IL 1, кальцій зв'язуючих білків є початком розвитку запального процесу. Інгібітори протеаз SLPI і РІ3 нейтралізують найбільшою мірою нейтрофільну еластазу, а також катепсин G, трипсин нейтрофілів, хіма зу, триптазу опасистих клітин, трипсин і хімо трипсин панкреатичних клітин [1,13,24].…”

Section: ол цимбалиста ивано франковский национальный медицинский университет украинаunclassified

“…-SLPI забезпечує значною мірою природну антифунгальну активність слизових оболонок (фунгіцидний ефект проти Aspergillus fumiga tus, Candida albicans) [1,20,24,34].…”

Section: Slpi рі3 беруть участь у регуляції місцевого і системного запаленняunclassified

“…Часто пацієнти роблять видих при зімкнутих губах (підвищу ється внутрішньобронхіальний тиск, що перешкоджає колапсу бронхів). У зв'язку з редукцією судин МКК наростає гіпертензія в ньому, правошлуночкова недостатність [16,21,24,28].…”

Section: ураження дихальної системиunclassified

“…Метод КТ легень є вирішальним як для діагно стики емфіземи легень (переважно в базальних відділах легень і її панацинарний характер на ранніх стадіях захворювання), так і контро лю за її прогресуванням, діагностики бронхоек тазів, булл. Останні частіше виявляються у верхніх долях [24,28,31,32 [21,29,30,35].…”

Section: ураження дихальної системиunclassified

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Characteristics of diseases in children with inherited α1-antitrypsin deficiency

Tsymbalista¹

2021

MPU

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The article presents the current views on genetics, features of metabolic disorders, course of various clinical manifestations, diagnostic criteria, and treatment principles for monogenic enzymopathy — an inherited α1;antitrypsin (AAT) deficiency. Inherited AAT deficiency manifests itself as four clinical syndromes: respiratory disease (pulmonary emphysema, chronic obstructive pulmonary disease (COPD), bronchial asthma, spontaneous pneumothorax, bronchiectasis); liver disease and blood vessel damage. Respiratory disease manifests itself between the ages of 20–40 years. Chronic pulmonary emphysema is typical for homozygous PiZZ gene carriers. The most unfavorable clinical course variant including persistent cough, COPD development, bronchial asthma, pneumonia, co-existence of various respiratory diseases and severe disorders of external respiration is the PISZ phenotype. Bronchial asthma has severe clinical course with torpid purulent endobronchitis; is often uncontrolled despite background therapy. In COPD patients, exacerbations are frequent, severe, and prolonged. The reduction in the vessels of the pulmonary circulation results in pulmonary hypertension, right ventricular failure. In AAT deficiency, there is a high incidence of bronchiectasis, lung cancer. Liver diseases manifest themselves as neonatal hepatitis, biliary atresia, liver cirrhosis, infectious complications. Chronic form is characterized by subclinical changes in the biochemical parameters of liver function most of which (up to 70%) normalize before the age of 18 years. In patients until the age of 20 years, disease slowly progresses and leads to the development of liver cirrhosis in 30% of cases, while older patients develop liver cirrhosis in 30–50% of cases. Some patients have a benign course of liver disease and present with hepatomegaly. Hemorrhagic syndrome occurs when protease inhibitor gene product acquires the properties of antithrombin. Vascular disease manifests itself as necrotizing panniculitis, severe hemorrhagic vasculitis with serum antineutrophil cytoplasmic antibodies to proteinase (С-АNСА). AAT deficiency may coexist with systemic vasculitis, vascular aneurysm, many other diseases, as well as with the development of aplastic processes of various localization. In addition to clinical manifestations, diagnosis of inherited AAT deficiency includes screening programs, instrumental (X-ray, spirometry, ECG, ultrasound), laboratory (liver function, coagulation profile, immunogram, serum AAT concentration), genetic investigations, exclusion testing for cystic fibrosis, viral hepatitis, and other diseases. Treatment of patients with inherited AAT deficiency consists in adequate nutrition and physical activity; treatment of pre-existing conditions; prevention and timely therapy for respiratory infections; pathogenetic therapy. Pathogenetic therapy for AAT deficiency is a replacement (augmentation) therapy with exogenous α1-antitrypsin concentrate. Replacement therapy is indicated only in patients >18 years of age with the PiZZ phenotype or other rare AAT phenotypes. No conflict of interest was declared by the author. Key words: children, inherited α1-antitrypsin deficiency, diagnosis, therapy.

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Section: ол цимбалиста ивано франковский национальный медицинский университет украинаunclassified

Section: Slpi рі3 беруть участь у регуляції місцевого і системного запаленняunclassified

Section: ураження дихальної системиunclassified

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Characteristics of diseases in children with inherited α1-antitrypsin deficiency

Tsymbalista¹

2021

MPU

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“…[50][51][52] AAT is synthesized predominantly by liver hepatocytes and to a significantly smaller degree by mononuclear phagocytes, neutrophils, and epithelial cells of the lung, intestines and kidney parenchyma. 35,53,54 AAT binds to elastase, trypsin, chymotrypsin, thrombin, and bacterial proteases. Its most important physiological effect is NE inhibition.…”

Section: α1-antitrypsinmentioning

confidence: 99%

The functional status of neutrophils in patients with bronchial asthma, chronic obstructive pulmonary disease, bronchial asthma with chronic obstructive pulmonary disease, and community-acquired pneumonia

Gb¹

2018

JLPRR

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Effects of alfa-1-antitripsin deficiency on lung disease

Cited by 2 publications

References 47 publications

Characteristics of diseases in children with inherited α1-antitrypsin deficiency

Characteristics of diseases in children with inherited α1-antitrypsin deficiency

The functional status of neutrophils in patients with bronchial asthma, chronic obstructive pulmonary disease, bronchial asthma with chronic obstructive pulmonary disease, and community-acquired pneumonia

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