scholarly journals Alpha-1-Antitrypsin Deficiency in Serbian Adults with Lung Diseases

2012 ◽  
Vol 16 (11) ◽  
pp. 1282-1286 ◽  
Author(s):  
Aleksandra Topic ◽  
Marija Stankovic ◽  
Aleksandra Divac-Rankov ◽  
Natasa Petrovic-Stanojevic ◽  
Marija Mitic-Milikic ◽  
...  
Keyword(s):  
Lung Diseases ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

scholarly journals ALPHA-1-ANTITRYPSIN DEFICIENCY IN CHILDREN

2017 ◽  
pp. 166-170
Author(s):  
S. I. MELNIK ◽  
M. V. PINEVSKAYA ◽  
E. A. ORLOVA ◽  
S. V. STAREVSKAYA ◽  
I. Y. MELNIKOVA ◽  
...  
Keyword(s):  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Lung Diseases ◽  
Hereditary Disease ◽  
Diagnosis And Treatment ◽  
Enzyme Replacement ◽  
Antitrypsin Deficiency ◽  
Leading Position ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

Hereditary deficiency of the alpha-1-antitrypsin occupies a leading position among the causes of chronic nonspecific lung diseases with emphysema formation. Deficiency of alpha-1-antitrypsin is a potentially fatal hereditary disease, under-diagnosed by physicians of various specialities. The authors familiarize pediatricians with this hereditary disease, difficulties of its diagnosis and treatment. There are presented own author`s data on the diagnosis and the experience of the enzyme replacement therapy of alpha-1-antitrypsin deficiency with the drug INN «Alpha-1 antitrypsin human» (Respikam).

scholarly journals Rare Lung Diseases I – Lymphangioleiomyomatosis

2006 ◽  
Vol 13 (7) ◽  
pp. 375-380 ◽  
Author(s):  
Stephen C Juvet ◽  
David Hwang ◽  
Gregory P Downey
Keyword(s):  
Lung Diseases ◽  
Focal Point ◽  
Lung Parenchyma ◽  
Great Promise ◽  
Childbearing Age ◽  
Antitrypsin Deficiency ◽  
Cystic Changes ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Rare Lung Diseases ◽  
Alpha 1 Antitrypsin

The present article is the first in a series that will review selected rare lung diseases. The objective of this series is to promote a greater understanding and awareness of these unusual conditions among respirologists. Each article will begin with a case that serves as a focal point for a discussion of the pathophysiology and management of the particular condition. The first article is on lymphangioleiomyomatosis (LAM); subsequent articles will focus on pulmonary alveolar proteinosis, alpha-1-antitrypsin deficiency and primary ciliary dyskinesia.LAM is a rare, progressive and (without intervention) often fatal interstitial lung disease that predominantly affects women of childbearing age. LAM is characterized by progressive interstitial infiltration of the lung by smooth muscle cells, resulting in diffuse cystic changes of the lung parenchyma. The molecular basis of this disorder has been delineated over the past five years and LAM is now known to be a consequence of mutations in the tuberous sclerosis genes. This knowledge, combined with advances in our understanding of the signalling pathways regulated by these genes, has given rise to potential molecular therapies that hold great promise for treating this devastating disease.

Sign in / Sign up

Export Citation Format

Share Document