 |
|
АкушерствоАнатомияАнестезиологияВакцинопрофилактикаВалеологияВетеринарияГигиенаЗаболеванияИммунологияКардиологияНеврологияНефрологияОнкологияОториноларингологияОфтальмологияПаразитологияПедиатрияПервая помощьПсихиатрияПульмонологияРеанимацияРевматологияСтоматологияТерапияТоксикологияТравматологияУрологияФармакологияФармацевтикаФизиотерапияФтизиатрияХирургияЭндокринологияЭпидемиология |
ГЕННАЯ ТЕРАПИЯЕще одной надеждой больных МВ и их врачей является генная терапия. При помощи генно-инженерных технологий клонирована комплементарная ДНК, кодирующая белок МВТР, и ведутся активные поиски метода доставки этого гена в эпителиальные клетки бронхов. Доказано, что 10% уровень нормального МВТР достаточен, чтобы защитить дыхательные пути от развития патологического процесса. Проведены клинические испытания с использованием вирусных векторов и невирусных носителей (катионные липосомы, полимеросомы) [144]. Клинически значимого результата пока получено не было, однако исследования продолжаются, так как в случае успеха наступит новый этап в лечении МВ - этап этиологического подхода к лечению этого заболевания. Таким образом, МВ является генетически детерминированным заболеванием, основной патогенетический механизм которого связан с увеличением вязкости секрета экзокринных желез. При этом патология легких определяет клиническую картину и тяжесть течения и является одной из наиболее частых причин смерти при МВ у взрослых. Патоморфология легких определяется наличием множественных бронхо- и бронхиолоэктазов, выраженным воспалением, приводящим к структурным изменениям легочной ткани с развитием буллезной эмфиземы. Лечение больных МВ основано на комплексной терапии с применением антибактериальных, муко- и бронхолитических препаратов в сочетании с ферменто- и кинезитерапией. Перспективы лечения связаны с дальнейшей разработкой антибактериальной терапии, методов трансплантации и генетической коррекции основного дефекта МВ.
СПИСОК ЛИТЕРАТУРЫ 1.Andersen DH: Cystic fibrosis of the pancreas and its relation to celiac disease: A clinical and pathologic study.// Am J Dis Child 56:344-399, 1938. 2.Farber S: Some organic digestive disturbances in early life. //J Mich Med Sci 44:587-594, 1945. 3.J. Littlewood. Looking back over 40 years.// Part I. CFW Newsletter 2004, edition 1, Issue 4. - P.39-50 4..Andersen DH, Hodges RG: Celiac syndrome. V. Genetics of cystic fibrosis of the pancreas with a consideration of etiology.// Am J Dis Child 72:62-80, 1946 5.Gibson LE, Cooke RE: A test for concentration of electrolytes in sweat in cystic fibrosis of the pancreas utilizing pilocarpine by iontophoresis.// Pediatrics 23:545-549, 1959. 6.Di Sant'Agnese PA, Darling RC, Perera GA, Shea E: Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas. //Pediatrics 12:549-563, 1953 7.Di Sant'Agnese PA, Andersen DH. Cystic fibrosis of the pancreas in young adults.// Ann Intern Med. 1959 May;50(5):1321 - 1330 8.Schlesinger, Edward R.; Bahlke, Anne M. Cohen, Alan R. Development of a State-Wide Program for the Care of Children with Long-Term Illness.// Am J Public Health Nations Health. 1965 Jul;55(7):973 - 977. 9.Kramm E.R, Crane M.M, Sirken M.G, Brown M.L. A cystic fibrosis pilot survey in three New England states. //Am J Public Health. 1962 Dec;52:2041 - 2057. 10. Sultz HA, Schlesinger ER, Mosher WE. The Erie County survey of long-term childhood illness. //I. Methodology.Am J Public Health Nations Health. 1966 Sep;56(9):1461-9 11.Kerem E, Conway S, Elborn S, Heijerman H; Consensus Committee. Standards of care for patients with cystic fibrosis: A European consensus.//J Cyst Fibros. 2005 Mar;4(1):7-26. 12.Littlewood JM. European cystic fibrosis society consensus on standards--a roadmap to "best care".// J Cyst Fibros. 2005 Mar;4(1):1-5. Epub 2005 Jan 25 13.Littlewood JM. Good care for people with cystic fibrosis.// Paediatr Respir Rev. 2000 Jun;1(2):179-89 14.Амемлина Е.Л.,Чучалин А.Г. Муковисцидоз, современный подход к диагностике и лечению.//Рус.Мед.Ж.., 1997. т.5, 17:1136-1142. 15.Dodge JA, Lewis PA, Stanton M, Wilsher J. Cystic fibrosis mortality and survival in the UK: 1947-2003.//Eur Respir J. 2007 Mar;29(3):522-6 16.Иващенко Т.Э., Баранов В.С. Биохимические и молекулярно-генетические основы патогенеза муковисцидозаю //Спб.: «Интермедика», 2002 - С.256. 17.Tsui L-C, Buchwald M: Biochemical and molecular genetics of cystic fibrosis. //Adv Hum Genet 20:153-266, 1991. 18.Riordan JR, Rommens JM, Kerem B-T, et al: Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA. //Science 245:1066-1073, 1989. 19.Kerem E, Corey M, Kerem B-S, et al: The relation between genotype and phenotype in cystic fibrosis: Analysis of the most common mutation (DF508).// N Engl J Med 323:1517-1522, 1990 20.. Cystic Fibrosis Foundation. Patient Registry Annual Data Report 2002. Bethesda, MD: Cystic Fibrosis Foundation, 2003Капранов Н.И., Каширская Н.Ю. Актуальные проблемы муковисцидоза на современном этапе в России.// Пульмонология, 1997 4:7-16. 22.Report of a Joint Meeting. The molecular Genetic Epidemioilogy of Cyctic Fibrosis//WHO/HGN/CF/WG/04.02. P.15 23.Davis PB. Cystic fibrosis since 1938.//Am J Respir Crit Care Med. 2006 Mar;173(5):475-82 24.Амелина Е.Л, Черняк А.В, Черняев А.Л.. Муковисцидоз: определение продолжительности жизни // Пульмонология - 2001 - 3 - С.61-64 25.. Zielenski J, Tsui LC: Cystic fibrosis: Genotypic and phenotypic variations.// Annu Rev Genet 29:777-807, 1995 26. Engelhardt JF, Yankaskas JR, Ernst SA, et al: Submucosal glands are the predominant site of CFTR expression in human bronchus.// Nat Genet 2:240-247, 1992 27.Gregory RJ, Cheng SH, Rich DP, et al: Expression and characterization of the cystic fibrosis transmembrane conductance regulator.// Nature 347:382-386, 1990. 28.Davis PB: Pathophysiology of the lung disease in cystic fibrosis. In Cystic Fibrosis (Lung Biology in Health and Disease), vol 64. New York: Marcel Dekker, 1993, pp 193-218. 29.Welsh M.J., Smith A.E.: Molecular mechanisms of CFTR chloride channel dysfunction in cys. tic fibrosis.// Cell., vol.73:1251-1254. 30. Knowles MR, Friedman KJ, Silverman L: Genetics, diagnosis, and clinical phenotype. In Cystic Fibrosis in Adults. Philadelphia: Lippincott-Raven, 1999, pp 27-42 31.Gradient of distribution in Europe of the major CF mutation and of its associated haplotype. European Working Group on CF Genetics (EWGCFG).//Hum Genet. 1990 Sep;85(4):436-45. 32.Baranov VS, Ivaschenko TE, Gorbunova VN,et al. Frequency of the F508 deletion in cystic fibrosis patients from the European part of the USSR.//Hum Genet. 1991 May;87(1):61-4. 33.Lerer I, Cohen S, Chemke M,et al.. The frequency of the delta F508 mutation on cystic fibrosis chromosomes in Israeli families: correlation to CF haplotypes in Jewish communities and Arabs.//Hum Genet. 1990 Sep;85(4):416-7. 34.Watson EK, Mayall ES, Simova L, et al. The incidence of delta F508 CF mutation, and associated haplotypes, in a sample of English CF families. //Hum Genet. 1990 Sep;85(4):435-6 35.Koch C, Cuppens H, Rainisio M, et al.. European Epidemiologic Registry of Cystic Fibrosis (ERCF): comparison of major disease manifestations between patients with different classes of mutations.//Pediatr Pulmonol. 2001 Jan;31(1):1-12. 36.Капранов Н.И., Шабалова Л.А., Каширская Н.Ю. и др. Муковисцидоз (современные достижения и проблемы). Методические рекомендации. М.: Медпрактика 2005. С.76 37.Dulhanty AM, Chang XB, Riordan JR., Mutation of potential phosphorylation sites in the recombinant R domain of the cystic fibrosis transmembrane conductance regulator has significant effects on domain conformation.//Biochem Biophys Res Commun. 1995 Jan 5;206(1):207-14. 38.Chang XB, Hou YX, Jensen TJ, Riordan JR., Mapping of cystic fibrosis transmembrane conductance regulator membrane topology by glycosylation site insertion.//J Biol Chem. 1994 Jul 15;269(28):18572-5. 39.Davis PB., Molecular and cell biology of cystic fibrosis.//,J Appl Physiol. 1991 May;70(5):2331-3. 40.Knowles MR, Robinson JM, Wood RE, et al: Ion composition of airway surface liquid of patients with cystic fibrosis as compared to normal and disease-control subjects. //J Clin Invest 100:2588-2595, 1997. 41.Berger HA, Travis SM, Welsh MJ: Regulation of the cystic fibrosis transmembrane conductance regulator Cl- channel by specific protein kinases and protein phosphatases.// J Biol Chem 268:2037-2047, 1993 42.Smith J.J., Travis S.M., Greenberg E.P., Welsh M.J. Cystic fibrosis airway epithelia fail to kill bacteria because of abnormal airway surface fluid. //Cell. 196; 85: 229-236. 43.Bonfeld T.L., Konstan M.W., Burfeind P. et al. Normal bronchial epitheliual cells constitutely produce the anti-inflammatory cytokine IL-10 which is down-regulated in cystic fibrosis.//Am. J. Resp. Cell Mol. Biol. 1995; 13: 257-261. 44.Sommerhof C.P., Nadel J.A., Basbaum C.B., Caughey G.H. Neutrophil elastase and catepsin G stimulate secretion from cultures bovine airway glands.// J. Clin. Invest. 1990; 85: 682-689. 45.Berger M., Sorensen R.U., Tosi M.F. et al. Complement receptor expression on neutrophils at the inflammatory site, The Pseudomonas-infected lung in cystic fibrosis.// J. Clin. Invest. 1989; 84: 1302-1313. 46.De Rose V. Mechanisms and markers of airway inflammation in cystiv fibrosis.//Eur. Resp. J. 2002; 19: 333-340. 47.Matsui H., Grubb B.R., Tarran R.et al. Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease. //Cell. 1998; 95: 1005-1015. 48.Guggino W.B. Cystic fibrosis and the salt conroversy. //Cell. 1999; 96: 607-610. 49.Кучкина Н.В., Самсонова М.В. Влияние осмотичности среды на морфофункциональное состояние нейтрофилов крови при муковисцидозе. //Пульмонология. 1996; 4: 77-79. 50.Leid JG, Willson CJ, Shirtliff ME, Hassett DJ, Parsek MR, Jeffers AK. The exopolysaccharide alginate protects Pseudomonas aeruginosa biofilm bacteria from IFN-gamma-mediated macrophage killing.// J Immunol. 2005 Dec 1;175(11):7512-8 51.Ornoy A., Arnon J., Katznelson D. et al. Pathological confirmation of cystic fibrosis in the fetus following prenatal diagnosis.// Am. J. Med. Genet. 1987; 28: 935-947. 52.Oppenheimer E.H., Esterly J.R. Pathology of cystic fibrosis; review of literature and comparison of 146 autopsied cases.// Perspect. Pediatr. Pathol. 1975; 2: 241-278. 53.Sheppard M.N. The pathology of cystic fibrosis. In: Cystic fibrosis./ Eds. M.E. Hodson and D.M. Geddes. Chapman & Hall Medical. London. 1997: 131-149. 54.Bedrossian C.W., Greenberg S.D., Singer D.B. et al. The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age groups.// Hum. Pathol. 1976; 7: 195-204. 55.Tomashefski J.F., Dahms B., Bruce M. Pleura in pneumothorax. Comparison of patients with cystic fibrosis and idiopatic spontaneous pneumothorax. //Arch. Patholl. Lab. Med. 1985. 109: 910-916. 56.Зашихин А.Л., Агафонов Ю.В., Заостровская Л.А., Черняев А.Л., Быков В.П. Морфофункциональная организация гладкой мышечной ткани бронхов различных генераций у человека // Пульмонология.- 1997.- 3.- С.61-64. 57.Guidotti T.L., Luetzeker J., di Sant’Agnese P.A. et al. Fatal disseminated aspergollosis in a previously well young adult with cystic fibrosis.// Am. J. Med. Sci. 1982; 282: 157-160. 58.Bhargava V., Tomashefski J.F., Stern R.C., Abramovsky C.R. The pathology of fungal infection and colonization in patients with cystic fibrosis.// Hum. Pathol. 1989; 20: 977 59.Brueton M.J., Ormerod L.P., Shah K.J., Anderson C.M. Allergic bronchopulmonary aspergillosis cimplicating cystic fibrosis in childhood.//Arch. Dis. Child. 1980; 55: 348-353. 60.Zeaske R., Bruns W.T., Fink J.N. et al. Immune response to Aspergillus in cystic fibrosis.// J. Allergy Clin. Immunol. 1988; 82: 73-77. 61.Bosken C.H., Myers J.L., Greenberger P.A., Katzenstein A.L. Pathologic features of allergic bronchopulmonary aspergillosis.//Am. J. Surg. Pathol. 1988; 12: 216-222. 62.Boat T.F., di Sat’Agnese P., Warwick W.J., Handwerger S.A. Pneumotorax in cystic fibrosis. //J. Am. Med. Assoc. 1969. 209: 1498-1504. 63.-Fellows K.E., Stigol L., Schuster S., et al. Selective bronchila arterigraphy in patients with cystic fibrosis and massive hemoptysis.// Radiology. 1975; 114: 551-555. 64.Ryland D., Reid L. The pulmonary circulation in cystic fibrosis. //Thorax; 1975: 285-292. 65.McGlennen R.C., Burke B.A., Dehner L.P. Systemic amyloidosis complicating cystic fibrosis. //Arch. Pathol. Lab. Med. 1968; 110: 879-884. 66.Di Sant'Agnese PA, Hubbard VS: The pancreas. Cystic Fibrosis. New York: Thieme-Stratton, 1984, pp 230-295 67.Handwerger S, Roth J, Gorden P, et al: Glucose intolerance in cystic fibrosis. //N Engl J Med 281:451-461, 1969 68.Di Sant'Agnese PA, Hubbard VS: The hepatobiliary system. Cystic Fibrosis. New York: Thieme-Stratton, 1984, pp 296-322 69.Di Sant'Agnese PA, Hubbard VS: The gastrointestinal tract. Cystic Fibrosis. New York: Thieme-Stratton, 1984, pp 212-229 70.Neutra MR, Trier JS: Rectal mucosa in cystic fibrosis: Morphological features before and after short term organ culture.//Gastroenterology 75:701-710, 1978 71.Brugman SM, Taussig LM: The reproductive system.: Cystic Fibrosis. New York: Thieme-Stratton, 1984, pp 323-337 72.Амелина Е.Л., Марченков Я.В., Черняк А.В., Красовский С.А. Количественная оценка результатов компьютерной томографии высокого разрешения (КТВР) органов грудной клетки взрослых больных муковисцидозом.//Сборник статей и тезисов VIIIНационального конгресса по муковисцидозу "Муковисцидоз у детей и взрослых". Ярославль, 5-6 июня 2007 г. 2007. 41-42. 73.Vilozni D, Bentur L, Efrati O, et al., Spirometry in early childhood in cystic fibrosis patients.//Chest. 2007 Feb;131(2):356-61 74.Kraemer R, Baldwin DN, Ammann RA,. Progression of pulmonary hyperinflation and trapped gas associated with genetic and environmental factors in children with cystic fibrosis.//Respir Res. 2006 Nov 30;7:138 75.Amelina E. Cherniak A. Chuchalin \A Comparison of lung volumes measured by different methods in adult patients with cystic fibrosis\\ The Netherlands J. of Medicine, 1999: 54; suppl, s51 (110) 76.Науменко Ж.К., Неклюдова Г.В., Черняк А.В., Амелина Е.Л., Калманова Е.Н., Чучалин А.Г. - Cостояние кардио-респираторной системы у взрослых больных муковисцидозом. //Тер.архив. 2002. 74 (3): 52-55. 77.Bell SC, Bowerman AR, Davies CA, et al. Nutrition in adults with cystic fibrosis.// Clin Nutr 1998; 17:211 - 215 78.Черняк А.В., Амелина Е.Л., Чучалин А.Г. - Нутритивный статус и выживаемость у взрослых больных муковисцидозом.// Сборник статей и тезисов 6-го Национального конгресса по муковисцидозу. Санкт-Петербург, 11-12 сентября 2003 г. 2003. 58 79.Burns JL, Emerson J, Stapp JR, et al. Microbiology of sputum from patients at cystic fibrosis centers in the United States.//Clin Infect Dis 1998; 27:158 - 163 80.Saiman L, Mehar F, Niu WW, et al. Antibiotic susceptibility of multiply resistant Pseudomonas aeruginosa isolated from patients with cystic fibrosis, including candidates for transplantation. //Clin Infect Dis 1996; 23:532 - 537 81.Davis P.B.et al. Cystic fibrosis.//Am. J. Resp. Crit. Care Med.,1996.Vol 154; 1220-1256. 82.Yankaskas JR, Marshall BC et al. Cystic fibrosis adult care: consensus conference report.// Chest.2004 Jan;125(1Suppl):11-39. 83.Doring G.Chronic lung infection in cystic fibrosis. //International congress and symposium series,2002.Vol.254; 5-15. 84.Livermore D. Current thinking on resistance mechanisms of Pseudomonas aeruginosa in cystic fibrosis. //International congress and symposium series,2002.Vol.254; 15-23. 85. Anonymous. Cystic fibrosis foundation patient registry 1997 annual data report. Bethesda, MD, USA. Cystic Fibrosis Foundation 1998 86. Koch C. Early Infection and Progression of Cystic Fibrosis Lung Disease // Pediatric Pulmonology. - 2002. - Vol.34. - P.232 - 236 87.Cystic Fibrosis Foundation. Consensus conference: microbiology and infectious diseases in cystic fibrosis. Bethesda, MD: Cystic Fibrosis Foundation, 2004; 1 - 26. 88.Döring G., Conway SP, Heijerman H.G.M., Hodson M.E. et al. Antibiotic therapy against Pseudomonas aeruginosa in cystic fibrosis: a European consensus // Eur Respir J. - 2000. - Vol.16. - P.749-767 89.Gharib R, Allen RP, Joos HA, Bravo LR: Paranasal sinuses in cystic fibrosis: Incidence of roentgen abnormalities.// Am J Dis Child 108:499-502, 1964 90.Stern RC, Jones K. Nasal and sinus disease: Cystic Fibrosis in Adults. Philadelphia: Lippincott-Raven, 1999, pp 221-231 91.Shwachman H, Kowalski M, Shaw K-T: Cystic fibrosis: An otolaryngologic perspective.// Otolaryngol Head Neck Surg 97:356-360, 1987. 92.Jaffe BF, Strome M, Khaw K-T, Shwachman H: Nasal polypectomy and sinus surgery for cystic fibrosis: A 10 year review. Otolaryngol Clin North Am 10:81-90, 1977. 93. Sinaasappel M., Stern M., Littlewood J., et al. Nutrition in patients with cystic fibrosis: a European Consecsus // Journal of cystic Fibrosis. - 2002. - V.1. -P.51-75 94.Di Sant'Agnese PA, Hubbard VS: The gastrointestinal tract. In Cystic Fibrosis. New York: Thieme-Stratton, 1984,Stern RC, Izant RJ Jr, Boat TF, et al: Treatment and prognosis of rectal prolapse in cystic fibrosis.// Gastroenterology 82:707-710, 1982 96.Sokol RJ, Durie PR: Recommendations for management of liver and biliary tract disease in cystic fibrosis; Cystic Fibrosis Foundation Hepatobiliary Disease Consensus Group.// J Pediatr Gastroenterol Nutr 28:S1-S13, 1999 97.Jebbink MC, Heijerman HG, Masclee AA, Lamers CB: Gallbladder disease in cystic fibrosis.// Neth J Med 41:123-126, 1992 98.Nussbaum E, Boat TF, Wood RE, Doershuk CF: Cystic fibrosis with acute hypoelectrolytemia and metabolic alkalosis in infancy.// Am J Dis Child 133:965-966, 1979 99.Flume PA, Yankaskas JR. Reproductive issues. Cystic Fibrosis in Adults. Philadelphia: Lippincott-Raven, 1999, pp 449-464 100. FitzSimmons SC, Fitzpatrick S, Thompson B, et al: A longitudinal study of the effects of pregnancy on 325 women with cystic fibrosis.// Pediatr Pulmonol (Suppl 13):99-101, 1996 101.Karczeski BA, Cutting GR Diagnosis of Cystic Fibrosis: CFTR-Related Disease and Screening // Cystic Fibrosis in the 21st Century. Karger, 2006, pp 69-76 102.Amelina E.L. Chernjak A.V. Kashirskaya N.J. Kapranov N.I. Chuchalin A.G. Factors affecting adult cystic fibrosis patients' survival. Abstracts 9th United European Gastrenterology Week, 6-10 October 2001, Amsterdam. 103.Каширская Н.Ю., Капранов Н.И. Коррекция экзокринной недостаточности поджелудочной железы микрогранулированными панкреатическими ферментными препаратами у больных муковисцидозом детей. //Вопросы современной педиатрии 2002: 1,5: 74 - 78. 104.Heijerman HG, Lamers CB, Bakker W. Omeprazole enhances the efficacy of pancreatin (pancrease) in cystic fibrosis.// Ann Intern Med 1997; 114:200 - 201 105.Williams SG, Ashworth F, McAlweenie A, et al. Percutaneous endoscopic gastrostomy feeding in patients with cystic fibrosis.// Gut 1999; 44:87 - 90 106.Lanng S, Thorsteinson B, Nerup J, et al. Diabetes mellitus in cystic fibrosis: effect of insulin therapy on lung function and infections.// Acta Paediatr 1998; 83:849 - 853 107.Milla CE, Warwick WJ, Moran A. Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at baseline.// Am J Respir Crit Care Med 2000; 162:891 - 895 108.Konig P, Poehler J, Barbero G. A placebo-controlled, double blind trial of the long-term effects of albuterol administration in patients with cystic fibrosis.// Pediatr Pulmonol 1998; 25:32 - 36 109.Hordvik NL, Sammut PH, Judy CG, et al. The effects of albuterol on the lung function of hospitalized patients with cystic fibrosis.// Am J Respir Crit Care Med 1996; 154:156 - 160 110.Zach MS, Oberwaldner B, Forche G, et al. Bronchodilators increase airway instability in cystic fibrosis.// Am Rev Respir Dis 1985; 131:537 - 543 111.Salvatore D, D’Andria M. Effects of salmeterol on arterial oxyhemoglobin saturations in patients with cystic fibrosis.//Pediatr Pulmonol 2002; 34:11 - 15 112.Hordvik NL, Sammut PH, Judy CG, et al. Effectiveness and tolerability of high-dose salmeterol in cystic fibrosis.// Pediatr Pulmonol 2002; 34:287 - 296 113.Weintraub SJ, Eschenbacher WL. The inhaled bronchodilators ipratropium bromide and metaproterenol in adults with // CF. Chest 1989; 95:861 - 864 114.Aмелина Е.Л., Черняк А.В. - Эффективность тиотропия бромида у больных муковисцидозом. // Сборник тезисов 15-го Национального конгресса по болезням органов дыхания. Москва, 29 ноября-2 декабря 2005 г. Пульмонология. Приложение. 2005. 49 (160). 115.Stafanger G, Koch C. N-acetylcysteine in cystic fibrosis and Pseudomonas aeruginosa infection: clinical score, spirometry and ciliary motility. // Eur Respir J 1989; 2:234 - 237 116.Ratjen F, Wonne R, Posselt HG, et al. A double-blind placebo controlled trial with oral ambroxol and N-acetylcysteine for mucolytic treatment in cystic fibrosis. // Eur J Pediatr 1985; 144:374 - 378 117.Ranasinha C, Assoufi B, Shak S et al Effecacy and safety of short-term administration of aerosolised recombinant human DNase in adult whit stable stage cystic fibrosis. // Lancet. - 1993. - V.342. - P.199-202. 118.Shah PL, Bush A, Canny GJ et al. Recombinant human Dnase 1 (rhDNase) in cystic fibrosis patients whit severe pulmonary disease: short-term, double-blind stady followed by a six month open-label triatment. // Eur Respir J. - 1995. - V.8. - P.954-958. 119.Amelina E., Cherniak A., Voznesenskiy N., Cherniaev A., Chuchalin A. - The effect of daily inhaled DNAse in adult patients with cystic fibrosis. The Netherlands J. of Medicine. 1999. 54; suppl. s81 (228). 120.Shah PL, Convey SP, Scott SF, Rainisio M, Wildman M, Stableforth D, Hodson ME. A case-controlled stady with Dornase Alfa to evaluate impact on disease progression over a 4-year period. // Respiration. - 2001. - P.68 121.Hodson M E, Shah P L. Dnase trials in cystic fibrosis. // Eur Resp J. - 1995. - V.8. - P.1786-1791 122.D Desmond KJ, Schwenk WF, Thomas E, et al. Immediate and long-term effects of chest physiotherapy in patients with cystic fibrosis. J Pediatr 1983; 103:538 - 542 123.Reisman JJ, Rivington-Law B, Corey M, et al. Role of conventional physiotherapy in cystic fibrosis. // J Pediatr 1988; 113:632 - 636 124.MMcIlwaine PM, Wong LT, Peacock D, et al. Long-term comparative trial of conventional postural drainage and percussion versus positive expiratory pressure physiotherapy in the treatment of cystic fibrosis. // J Pediatr 1997; 131:570 - 574 125.McIlwaine PM, Wong LT, Peacock D, et al. Long-term comparative trial of positive expiratory pressure versus oscillating positive expiratory pressure (flutter) physiotherapy in the treatment of cystic fibrosis. // J Pediatr 2001; 138:845 - 850 126. Enright S, Chatham K, Ionescu AA, Unnithan VB, Shale DJ.2004;126(2):405-11 Inspiratory muscle training improves lung function and exercise capacity in adults with cystic fibrosis. // Chest. 2004;126(2):405-11 127.de, van Aalderen WM, Kraan J, Koeter GH, van der Schans CP., Inspiratory muscle training in patients with cystic fibrosis. // Respir Med. 2001;95(1):31-6. 128. 129.Meyer KC, Lewandoski JR, Zimmerman JJ et al. Human neutrophyl elastase elastase/alpha1-antiprotease complex in cystic fibrosis - comparison with interstitial lung disease and evaluation of the effect of intravenously administered antibiotic therapy.// Am Rev Respir Dis 1991;144(3): 580-585 130.PedersenSS, Hoiby N. Et al. Management of Pseudomonas aeruginosa lung infection in Danish cystic fibrosis patients.// Acta Paediat Scand. 1997; 76:955-961. 131.Marks MI, Antibiotic therapy of bronchopulmonary infections in cystic fibrosis: the American approach. // Antibiotic Chemother 1989; 42: 229-236. 132.Esmond G, Butler M, McCormack A, et al. Comparison of hospital and home intravenous antibiotic therapy in adults with cystic fibrosis. // J Cystic Fibrosis 2002;1(Suppl 1):P362. 133. Pond M, Newport M, Joanes D, et al. Home versus hospital intravenous antibiotic therapy in the treatment of young adults with cystic fibrosis. / Eur Respir J 1995;7:1640 - 4. 134.Thornton, R Elliott, M P Tully, M Dodd and A K Webb;Long term clinical outcome of home and hospital intravenous antibiotic treatment in adults with cystic fibrosis // Thorax 2004;59:242-246 135.Amelina E., Senkevich N., Cherniak A., Cherniaev A., Chuchalin A. - Home intravenous therapy in adult cystic fibrosis patients. The impact on lung function and quality of life. Abstracts World Congress on Lung Health and 10th ERS Annual Congress. Florence, Italy, August 30-September 3, 2000. // Eur.Respir.J. 2000. 16; suppl.31. 123s (P915). 136.Marco T, Asensio O, Bosque M, et al. Home intravenous antibiotics for cystic fibrosis (Cochrane Review). Issue 1. In: The Cochrane Library. Oxford, Update Software, 2001 137.Сенкевич Н.Ю, Амелина Е.Л. Качество жизни взрослых больных муковисцидозом: факты и гипотезы. // Пульмонология - 1999 - N3- С.51-57 138.Ramsey BW, Pepe MS, Quan JM, et al. Intermittent administration of inhaled tobramycin in patients with cystic fibrosis.// N Engl J Med 1999; 340:23 - 30 139.Nickerson B, Montgomery A, Kylstra J, et al. Safety and effectiveness of 2 years of treatment with intermittent inhaled tobramycin in CF patients [abstract].// Pediatr Pulmonol 1999; Suppl 19:243 - 244 140.Авдеев С.Н., Самойленко В.А., Амелина Е.Л., Чучалин А.Г. Интенсивная терапия при муковисцидозе у взрослых. //. Пульмология, 2000: 3; 87-99) 141.SpectorM., Stern R. Pneumothorax in cystic fibrosis: a 26-year experience. // Ann. Thorac. Surg. 1989; 47: 204-207. 142.Sweeney., Fellows K. Bronchial artery embolization for severe haemoptysis in cystic fibrosis.// Chest 1990; 97:1322-1326. 143.Hodson M.E., Madden B.P.,Steven M.H.et al. Non-invasive mechanical ventilation for cystic fibrosis-a potential bridge to transplanttion. // Eur. respir. J. 1991; 4:524-527. 144.. Johnson LG, Knowles MR: New therapeutic strategies for cystic fibrosis lung disease. Cystic Fibrosis in Adults. Philadelphia: Lippincott-Raven, 1999, 233-258
document: $pr: version: 01-2007.1 codepage: windows-1251 type: klinrek id: kli2049549 : 08.5. БРОНХОЭКТАЗЫ meta: author: fio[ru]: Я.Н. Шойхет, Н.М. Лепихин, Д.Н. Лепихина codes: next: type: dklinrek code: III.III type: dkli00181 Дата добавления: 2015-01-18 | Просмотры: 1056 | Нарушение авторских прав |