Cystic Fibrosis

MacLusky I , McLaughlin FJ , Levison H. Cystic fibrosis: Part I. Curr Probl Pediatr 1985;XV:1–49.

Andersen DH . Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathological study. AJDC 1938;56:344–399.

Knowles MR , Stutts MJ , Yankaskas JR , Gatzy JT , Boucher RC . Abnormal respiratory epithelial ion transport in cystic fibrosis. Clin Chest Med 1986;7:285–297.

Klinger KW . Genetics of cystic fibrosis. Semin Respir Med 1985;6: 243–251.

Riordan JR , Rommens JM , Kerem B-S , Alon N , Rozmahel R , Grzelczak Z , . Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA. Science 1989;245:1066–1073.

Kerem B-S , Rommens JM , Buchanan JA , Markiewicz D , Cox TK , Chakravarti A , . Identification of the cystic fibrosis gene: Genetic analysis. Science 1989;345:1073–1080.

Tsui L-C . International cystic fibrosis genetics consortium update, May 1991. Bethesda MD: Cystic Fibrosis Foundation.

Lemna WK , Feldman GL , Kerem B-S , Fernbach SD , Zevkovich EP , O'Brien WE , . Mutation analysis for heterozygote detection and the prenatal diagnosis of cystic fibrosis. N Engl J Med 1990;322:291–296.

Park RW , Grand RJ . Gastrointestinal manifestations of cystic fibrosis: A review. Gastroenterol 1981;81:1143–1161.

Holsclaw D , Eckstein H , Nixon H. Meconium ileus: A 20 year review of 109 cases. AJDC 1965;109:101–113.

Stern RC , Izant RJ Jr , Boat TF , Wood RE , Matthews LW , Doershuk CF . Treatment and prognosis of rectal prolapse in cystic fibrosis. Gastroenterol 1982;82:707–710.

Holsclaw D , Rocmans C , Shwachman H. Intussesception in patients with cystic fibrosis. Pediatrics 1971;48:51–58.

Roy CC , Weber AM , Morin CL , LePage G , Brisson G , Yousef I , . Hepatobiliary disease in cystic fibrosis: A survey of current issues and concepts. J Pediatr Gastroenterol Nutr 1982;1:469–478.

Orenstein DM , Henke KG , Costill DL , Doershuk CF , Lemon PJ , Stern RC . Exercise and heat stress in cystic fibrosis patients. Pediatr Res 1983;17:267–269.

Beckerman RC , Taussig LM . Hypoelectrolytemia and metabolic alkalosis in infants with cystic fibrosis. Pediatrics 1979;63:580–583.

Stern RC , Boat TF , Wood RE , Matthews LW , Doershuk CF . Treatment and prognosis of nasal polyps in cystic fibrosis. AJDC 1982; 136:1067–1070.

Corey M , Levison H , Crozier D. Five- to seven-year course of pulmonary function in cystic fibrosis. Am Rev Respir Dis 1976;114:1085–1092.

Pattishall EN . Longitudinal response of pulmonary function to bronchodilators in cystic fibrosis. Pediatr Pulmonol 1990;9:80–85.

Thomassen MJ , Demko CA , Doershuk CF . Cystic fibrosis: A review of pulmonary infections and interventions. Pediatr Pulmonol 1987;3: 334–351.

Marks MI . The pathogenesis and treatment of pulmonary infections in patients with cystic fibrosis. J Pediatr 1981;98:173–179.

Wood RE , Wanner A , Hirsch J , Farrell PM . Tracheal mucociliary transport in patients with cystic fibrosis and its stimulation by terbutaline. Am Rev Respir Dis 1975;111:733–738.

Thomassen MJ , Boxerbaum B , Demko CA , Kuchenbrod PJ , Dearborn DG , Wood RE . Inhibitory effect of cystic fibrosis serum on pseudomonas phagocytosis by rabbit and human alveolar macrophages. Pediatr Res 1979;13:1085–1088.

Tosi MF , Zakem H , Berger M. Neutrophil elastase cleaves c3bi on opsonized pseudomonas as well as CRI on neutrophils to create a functionally important opsonin receptor mismatch. J Clin Invest 1990;86:300–308.

Kaplan E , Schwachman H , Perlmutter A , Rule A , Khaw KT , Holsclaw D. Reproductive failure in males with CF. N Engl J Med 1968;279:65–69.

Kopito LE , Kosasky HJ , Schwachman H. Water and electrolytes in cervical mucus from patients with cystic fibrosis. Fertility Sterility 1973;24:512–516.

Orenstein DM . Diagnosis of cystic fibrosis. Semin Respir Med 1985; 6:252–260.

Rosenstein BJ , Langbaum TS , Gordes D , Brusilow SW . Cystic fibrosis: Problems encountered with sweat testing. JAMA 1978;240:1987–1988.

Wood RE , Boat TF , Doershuk CF . State of the art: Cystic fibrosis. Am Rev Respir Dis 1976;113:833–878.

Wilcken B , Brown ARD , Urwin R , Brown DA . Cystic fibrosis screening by dried spot trypsin assay: Results in 75,000 newborn infants. J Pediatr 1983;102:383–387.

Orenstein DM , Boat TF , Stern RC , Tucker AS , Charnock EL . The effect of early diagnosis and treatment in cystic fibrosis: A seven year study of 16 sibling pairs. AJDC 1977;131:973–975.

Reisman JJ , Rivington-Law B , Corey M , Marcotte J , Wannamaker E , Harcourt D , . Role of conventional physiotherapy in cystic fibrosis. J Pediatr 1988;113:632–636.

Orenstein DM , Franklin BA , Doershuk CF , Hellerstein HK , Germann KJ , Horowitz JG , . Exercise conditioning and cardiopulmonary fitness in cystic fibrosis. The effects of a three-month supervised running program. Chest 1981;80:392–398.

Orenstein DM , Henke KG , Cerny FJ . Exercise and cystic fibrosis. Physician Sports Med 1983;11:57–62.

Landau LI , Phelan PD . The variable effect of a bronchodilating agent on pulmonary function in cystic fibrosis. J Pediatr 1973;82:863–868.

Scanlin TF . Cystic fibrosis. In: Fishman AP , editor. Pulmonary diseases and disorders, 2nd ed. New York: McGraw Hill Book Co, 1998:1273–1294.

Knowles MR , Church NL , Waltner WE , Yanaskas JR , Gilligan P , King M , . A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis. N Engl J Med 1990;322:1189–1194.

Aitken ML , Burke W , McDonald G , Villalon M , Shak S , Montgomery AB , . Effect of inhaled recombinant human DNase on pulmonary function in normal and cystic fibrosis patients: Phase 1 study (abstract). Am Rev Respir Dis 1991;143:A298.

McElvaney NG , Hubbard RC , Birrer P , Chernick MS , Caplan DB , Frank MM , . Aerosol alpha-1 antitrypsin treatment for cystic fibrosis. Lancet 1991;1:392–394.

Auerbach HS , William M , Kirkpatrick JA , Colten HR . Alternate-day prednisone reduces morbidity and improves pulmonary function in cystic fibrosis. Lancet 1985;2:686–688.

Konstan MW , Vargo KM , Davis PB . Ibuprofen attenuates the inflammatory response to Pseudomonas aeruginosa in arat model of chronic pulmonary infection. Implications for antiinflammatory therapy in cystic fibrosis. Am Rev Respir Dis 1990;141:186–192.

Rosenstein BJ , Eigen H. Risks of alternate-day prednisone in patients with cystic fibrosis. Pediatrics 1991;87:245–246.

Grenier B. Use of the new quinolones in cystic fibrosis. Rev Infect Dis 1989;11 (suppl 5):S1245–S1251.

Scott J , Hutter J , Stewart S , Higenbottam T , Hodson M , Pemketh A , . Heart-lung transplantation for cystic fibrosis. Lancet 1988;2: 192–194.

McLaughlin RJ , Matthews WJ , Streider DJ , Khaw DT , Schuster S , Shwachman H. Pneumothorax in cystic fibrosis: Management and outcome. J Pediatr 1982;100:863–869.

Spector ML , Stern RC . Pneumothorax in cystic fibrosis: A 26-year experience. Ann Thorac Surg 1989;47:204–207.

Stern RC , Wood RE , Boat TF , Matthews LW , Tucker AS , Doershuk CF . Treatment and prognosis of massive hemoptysis in cystic fibrosis. Am Rev Respir Dis 1978;117:825–828.

Holsclaw DS , Grand RJ , Shwachman H. Massive hemoptysis in cystic fibrosis. J Pediatr 1970;76:829–838.

Fellows KE , Khaw KT , Schuster S , Shwachman H. Bronchial artery embolization in cystic fibrosis: Technique and long-term results. J Pediatr 1979;95:959–963.

Koletzko S , Stringer DA , Cleghorn GJ , Durie PR . Lavage treatment of distal intestinal obstruction syndrome in children with cystic fibrosis. Pediatrics 1989;83:727–733.

Psacharopoulos HT , Howard ER , Portmann B , Mowat AP , Williams R. Hepatic complications of cystic fibrosis. Lancet 1981;278–280.

Mieles LA , Orenstein D , Teperman L , Podesta L , Koneru B , Starzl T. Liver transplantation in cystic fibrosis. Lancet 1989;1:1073.

Burke P , Meyer V , Kocoshis S , Orenstein DM , Chandra R , Nord DJ , . Depression and anxiety in pediatric inflammatory bowel disease and cystic fibrosis. J Am Acad Child Adolesc Psychiatry 1989;28:948–951.

Finkelstein SM , Wielinski CL , Elliott GR , Warwick WJ , Barbosa J , Wu S-C , Klein DJ . Diabetes mellitus associated with cystic fibrosis. J Pediatr 1988;112:373–377.

Cystic Fibrosis Foundation Data Registry Report for 1989. Bethesda MD: Cystic Fibrosis Foundation, 1990.

MacLusky I , McLaughlin FJ , Levison H. Cystic fibrosis. Part II. Curr Probl Pediatr 1985;15:1–39.

Stern RC , Boat TF , Doershuk CF , Tucker AS , Primiano FP Jr , Matthews LW . Course of cystic fibrosis in 95 patients. J Pediatr 1976;89:406–411.

Drumm ML , Pope HA , Cliff WH , Rommens JM , Marvin SA , Tsui L-C , . Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer. Cell 1990;62:1227–1233.