CYSTIC FIBROSIS: PRESENT DAY ASPECTS OF DIAGNOSTICS AND TREATMENT

Issues of etiology, pathogenesis, epidemiology, diagnostics and treatment are inspected from the present day point of view focusing on novel screening methods used for early diagnostics of the disease. The article gives grounded key principles of complex treatment including rational use of antibacterial and mucolytic agents.Key words: cystic fibrosis, diagnostics, treatment, children

CYSTIC FIBROSIS: PRESENT DAY ASPECTS OF DIAGNOSTICS AND TREATMENT

Issues of etiology, pathogenesis, epidemiology, diagnostics and treatment are inspected from the present day point of view focusing on novel screening methods used for early diagnostics of the disease. The article gives grounded key principles of complex treatment including rational use of antibacterial and mucolytic agents.Key words: cystic fibrosis, diagnostics, treatment, children

CLINICAL AND FUNCTIONAL EFFECTIVENESS AND SAFETY OF HIGH-FREQUENCY OSCILLATION OF CHEST WITH «THE VEST AIRWAY CLEARANCE SYSTEM» IN CHILDREN WITH CYSTIC FIBROSIS

Authors present results of treatment of 60 patients with cystic fibrosis 1–17 years old with «The Vest Airway Clearance System». Method of high-frequency oscillation of chest increases volume of moved off phlegm and improves functional abilities of lungs. This method can be used in younger children and in patients regardless of age in poor condition who can’t actively carry out drainage of phlegm and respiratory exercises. Key words: children, cystic fibrosis, high-frequency oscillation of chest, treatment.(Voprosy sovremennoi pediatrii — Current Pediatrics. 2010;9(5):67-71

PECULIARITIES OF ENT-DAMAGE IN CHILDREN WITH CYSTIC FIBROSIS

Traditional approach to cystic fibrosis patients treatment doesn’t involve upper respiratory tract assessment, though abnormal changes — consequences of the cystic fibrosis transmembrane conductivity regulator gene mutation- do affect nasal and paranasal mucosa to the same extent. Approximately half of cystic fibrosis patients suffer from chronic rhinosinusitis and/or nasal polyposis that worsens the clinical course of already severe disease. Chronic hyperplasia in paranasal cavities can be quite extensive, recurrent and can lead to destruction of osseous walls of the cavity and of nasal septum. Thus increasing the amount of hospital admissions and and their duration. Low awareness of ENT-specialists working in polyclinics and in hospitals of ENT-pathology in cystic fibrosis patients leads to belated diagnostics, excessive manipulations, ineffective treatment, including surgery. All these lays grounds to implication of the early screening diagnostic program and development of proper treatment methods of ENT-complications of cystic fibrosis — therapeutic as well as surgical, with strict specification of indications and contraindications. Key words: cystic fibrosis, chronic rhino sinusitis, nasal polyposis. (Voprosy sovremennoi pediatrii — Current Pediatrics. — 2011; 10 (5): 49–53.

PHARMACOECONOMIC EFFECTIVENESS OF SOME MODERN METHODS OF THERAPY IN CYSTIC FIBROSIS CHILDREN

In the last years interest has been aroused to pharmacoeconomics — the economic evaluation of treatment effectiveness. The investigation objective was the study of clinical effectiveness and the cost expediency of the inclusion of some modern drugs and medical technologies in the complex therapy of cystic fibrosis (CF) in children for the last decade in moscow. 105 patients during the period from 1993 to 2004 were investigated. Within this span of time some drugs were included: substitutive micros pheric pancreatic enzymes (Kreon) — 1993, mucolytic agent dornase alfa (Pulmozyme) and antibiotic ceftazidime — 1998, antibiotics cefepime and meropenem — 2003. since 1998 patients began to receive planned domiciliary intravenous therapy by antibiotics. The introduction of new drugs and medical technics (intravenous antibacterial therapy at home) caused increase of life expectancy of patients with CF from 16 to 27,9 years. The annual cost of therapy was in 1993 — 1809.8, in 1998 — 4266.7, and in 2003 — 23830.8 US dollars. Severity, age of patients, and pseudomonas infection were the decisive factors in the treatment cost.Key words: cystic fibrosis, pharmacoeconomics, therapy, life expectancy

PHARMACOECONOMIC EFFECTIVENESS OF SOME MODERN METHODS OF THERAPY IN CYSTIC FIBROSIS CHILDREN

In the last years interest has been aroused to pharmacoeconomics — the economic evaluation of treatment effectiveness. The investigation objective was the study of clinical effectiveness and the cost expediency of the inclusion of some modern drugs and medical technologies in the complex therapy of cystic fibrosis (CF) in children for the last decade in moscow. 105 patients during the period from 1993 to 2004 were investigated. Within this span of time some drugs were included: substitutive micros pheric pancreatic enzymes (Kreon) — 1993, mucolytic agent dornase alfa (Pulmozyme) and antibiotic ceftazidime — 1998, antibiotics cefepime and meropenem — 2003. since 1998 patients began to receive planned domiciliary intravenous therapy by antibiotics. The introduction of new drugs and medical technics (intravenous antibacterial therapy at home) caused increase of life expectancy of patients with CF from 16 to 27,9 years. The annual cost of therapy was in 1993 — 1809.8, in 1998 — 4266.7, and in 2003 — 23830.8 US dollars. Severity, age of patients, and pseudomonas infection were the decisive factors in the treatment cost.Key words: cystic fibrosis, pharmacoeconomics, therapy, life expectancy