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Badriashvili N., Giorgibiani M.,
Pheradze M., Giorgadze M., Kikalishvili L., Gvardjaladze N.
The chair of specialization in pediatric |
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Key words: cystic fibrosis of the
pancreas, cardiovascular system, children, erythrocyte, ATP-ase.
Summary: 42 patients of various ages with cystic fibrosis of the
pancreas were analyzed by authors. The bronchopulmonary forms of cystic
fibrosis were prevalensed, which determine the prognosis of disease.
Changes in red blood cell ultra structure and energy metabolism, as well
as haemostatic disorders were identified in children suffering from
cystic fibrosis of the pancreas. The results obtained contributed to the
study of the disease pathogenesis and were of great value for medical
practice providing a comprehensive assessment of multiple-modality
treatment.
Analysis of investigations allowed to find differences in the changes of
the level of adenylnukleotides and activity ATP-ase of erythrocytes at
mucoviscidosis defending upon the stage and the criticity of the injure
of the bronchopulmonary system resulting in the considerate decrease of
ATP, the increase of ADP, AMP concentration and activity of ATP-ase. The
decrease of the energy balance of erythron promotes the shortening of
the length of the life, hemolysis and anemia. Anemia is progressing with
the worsening of the state of health of patients and promotes the
increase of the tissue hypoxia. By the help of the data of ATP-ATP-asesystem
one can evaluate the dynamics of the disease and the effectiveness of
the therapy.
The studies of the morphology of erythrocytes revealed the change of the
shape and properties of the cellular membranes under this pathology, the
change of permeability, the decrease of the number of discocytes of
regular shape, the increase of the number of degenerated and appearance
of pathologic form of erythrocytes more expressed at mixed form of
cystic fibrosis, especially at disorder of cardiovascular system.
Cystic fibrosis of the pancreas is a severe disease with progressive
course and often with fatal outcome. But early diagnosis and right
tactic of treatment and management of patients influence on the outcome
of disease and prolong life of patients.
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