DIAGNOSTIC PROBLEMS OF MUCOVISCIDOSIS AND WAYS OF SOLUTION IN RUSSIA
Педиатрическая фармакология, 2014 Mucoviscidosis is a monogenic autosomal recessive caused by the CFTR gene mutations and characterized by pronounced genetic heterogeneity and clinical polymorphism, which emphasizes the need in comprehensive diagnosis and molecular-genetic verification ...
A. A. Baranov +10 more
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Native small airways secrete bicarbonate. [PDF]
, 2014 Since the discovery of Cl(-) impermeability in cystic fibrosis (CF) and the cloning of the responsible channel, CF pathology has been widely attributed to a defect in epithelial Cl(-) transport. However, loss of bicarbonate (HCO3(-)) transport also plays
Quinton, Paul M, Shamsuddin, AKM
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HISTORICAL AND MODERN ASPECTS OF CYSTIC FIBROSIS IN RUSSIA
Педиатрическая фармакология, 2013 The article is dedicated to historical and modern trials in the sphere of mucoviscidosis in Russia, peculiarities of clinical manifestations, pathophysiological and pathomorphological disorders and of the disease course with the analysis of the first ...
N. I. Kapranov +4 more
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Assembly and functional analysis of an S/MAR based episome with the cystic fibrosis transmembrane conductance regulator gene [PDF]
, 2018 Improving the efficacy of gene therapy vectors is still an important goal toward the development of safe and efficient gene therapy treatments. S/MAR (scaffold/matrix attached region)-based vectors are maintained extra-chromosomally in numerous cell ...
Ascenzioni, Fiorentina +10 more
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PECULIARITIES OF DORNASE ALFA AND KINESITHERAPY USE IN CHILDREN WITH CYSTIC FIBROSIS
Педиатрическая фармакология, 2013 The article is dedicated to modern treatment of mucoviscidosis. The article covers issues of pathogenesis and emphasizes importance of mucociliary clearance and anatomic-physiological peculiarities of respiratory tract’s structure in childhood in the ...
O. I. Simonova
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Tüdőtranszplantáció magyar betegek számára [PDF]
, 2013 When conservative treatment fails, lung transplantation often remains the only therapeutic option for patients with end stage parenchymal or vascular lung diseases.
Czebe K +3 more
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PRIMARY HEMOSTATIC SYSTEM CONDITION IN MUCOVISCIDOSIS IN CHILDREN
Педиатрическая фармакология, 2014 The article presents results of a study of platelet hemostasis in children with mucoviscidosis. Motionless and viscous mucus becomes infected with various pathogenic and opportunistic microbes in the setting of disturbed mucociliary clearance; when ...
O. B. Gordeeva +7 more
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Severe episodes of extra cellular dehydration : an atypical adult presentation of cystic fibrosis [PDF]
, 2008 Cystic fibrosis (CF) is usually diagnosed during childhood by respiratory or gastro-intestinal symptoms. Hyponatremic hypochloremic dehydration with metabolic alkalosis is a rare but typical presentation of CF in infants.
F. Illouz +5 more
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Distal intestinal obstruction syndrome as unusual manifestation of cystic fibrosis. Report of a case
Acta Pediátrica de México, 2014 Introduction: Distal intestinal obstruction syndrome (DIOS) or “meconium ileus equivalent” is a blockage of the terminal ileum or of the ascending colon caused by inspissated stools in patients with cystic fibrosis (CF) beyond the neonatal period.
Gómez Ruiz Alejandro +2 more
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Memorias y recomposiciones familiares [PDF]
, 2009 Based on a series of interviews from selected informants belonging to a wide three-generation survey, this paper questions the specificity of memories in recomposed families.
Segalen , Martine
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