Cystic fibrosis in neonates and infants
Cystic fibrosis (CF) is a common autosomal recessive disorder, characterized by chronic bronchopulmonary infection, pancreatic insufficiency, and subsequently, other multisystem complications. Most children are diagnosed before school age. Here we review the pathophysiology of the condition, the age...
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| Veröffentlicht in: | Early human development 2005-12, Vol.81 (12), p.997-1004 |
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| container_title | Early human development |
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| creator | Minasian, Caro McCullagh, Angela Bush, Andrew |
| description | Cystic fibrosis (CF) is a common autosomal recessive disorder, characterized by chronic bronchopulmonary infection, pancreatic insufficiency, and subsequently, other multisystem complications. Most children are diagnosed before school age. Here we review the pathophysiology of the condition, the age-related presentations of CF up until school age, and the appropriate use of diagnostic tests. A specialist centre should supervise treatment. There are exciting new advances in monitoring techniques in the preschool years, including CT scanning, bronchoscopy and gas mixing indices. Recent advances in the knowledge of the molecular biology of CF hold out the hope of specific therapies which can reverse the underlying defect causing death from CF lung disease. |
| doi_str_mv | 10.1016/j.earlhumdev.2005.10.001 |
| format | Article |
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Most children are diagnosed before school age. Here we review the pathophysiology of the condition, the age-related presentations of CF up until school age, and the appropriate use of diagnostic tests. A specialist centre should supervise treatment. There are exciting new advances in monitoring techniques in the preschool years, including CT scanning, bronchoscopy and gas mixing indices. Recent advances in the knowledge of the molecular biology of CF hold out the hope of specific therapies which can reverse the underlying defect causing death from CF lung disease.</description><identifier>ISSN: 0378-3782</identifier><identifier>EISSN: 1872-6232</identifier><identifier>DOI: 10.1016/j.earlhumdev.2005.10.001</identifier><identifier>PMID: 16278058</identifier><identifier>CODEN: EHDEDN</identifier><language>eng</language><publisher>Lausanne: Elsevier Ireland Ltd</publisher><subject>Biological and medical sciences ; Care and treatment ; Child, Preschool ; Cystic fibrosis ; Cystic Fibrosis - diagnosis ; Cystic Fibrosis - genetics ; Cystic Fibrosis - physiopathology ; Cystic fibrosis transmembrane regulator ; Female ; Gastroenterology. Liver. Pancreas. Abdomen ; Humans ; Infant ; Infant Nutritional Physiological Phenomena ; Infant, Newborn ; Infant, Newborn, Diseases - diagnosis ; Infant, Newborn, Diseases - physiopathology ; Infants ; Liver. Biliary tract. 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Most children are diagnosed before school age. Here we review the pathophysiology of the condition, the age-related presentations of CF up until school age, and the appropriate use of diagnostic tests. A specialist centre should supervise treatment. There are exciting new advances in monitoring techniques in the preschool years, including CT scanning, bronchoscopy and gas mixing indices. Recent advances in the knowledge of the molecular biology of CF hold out the hope of specific therapies which can reverse the underlying defect causing death from CF lung disease.</description><subject>Biological and medical sciences</subject><subject>Care and treatment</subject><subject>Child, Preschool</subject><subject>Cystic fibrosis</subject><subject>Cystic Fibrosis - diagnosis</subject><subject>Cystic Fibrosis - genetics</subject><subject>Cystic Fibrosis - physiopathology</subject><subject>Cystic fibrosis transmembrane regulator</subject><subject>Female</subject><subject>Gastroenterology. Liver. Pancreas. Abdomen</subject><subject>Humans</subject><subject>Infant</subject><subject>Infant Nutritional Physiological Phenomena</subject><subject>Infant, Newborn</subject><subject>Infant, Newborn, Diseases - diagnosis</subject><subject>Infant, Newborn, Diseases - physiopathology</subject><subject>Infants</subject><subject>Liver. Biliary tract. Portal circulation. Exocrine pancreas</subject><subject>Lung diseases</subject><subject>Meconium ileus</subject><subject>Medical sciences</subject><subject>Neonatal Screening - methods</subject><subject>Other diseases. Semiology</subject><subject>Screening</subject><issn>0378-3782</issn><issn>1872-6232</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2005</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkFtLAzEQhYMotlb_guyL-rTrJHtJ9lGLNxB80eeQzU40ZS-abAX_vVNb6KOEEGbyneHMYSzhkHHg1fUqQxO6j3Xf4ncmAEpqZwD8gM25kiKtRC4O2RxyqVK6YsZOYlwBgaqGYzbjlZCKijm7Wv7EydvE-SaM0cfED8mA42AmjIkZWqqdGaZ4yo6c6SKe7d4Fe7u_e10-ps8vD0_Lm-fUFgWfUgvkD-q8NI6DcLyCGkUJqgSXy9bJUgirTKPKqlAoG1mjsqJQuRC1w4bX-YJdbud-hvFrjXHSvY8Wu86Qq3XUlZK1UqIgMNuC76ZDTS7HKRhLp8Xe23FA56l_wwuQIEouSaC2AkuLxoBOfwbfm_CjOehNqnql96nqTaqbH0qVpOc7U-umx3Yv3MVIwMUOMNGazgUzWB_3nMyrQv4Nut1ySBF-eww6Wo-DxdYHtJNuR_-_m1_yXZfg</recordid><startdate>20051201</startdate><enddate>20051201</enddate><creator>Minasian, Caro</creator><creator>McCullagh, Angela</creator><creator>Bush, Andrew</creator><general>Elsevier Ireland Ltd</general><general>Elsevier</general><general>The Lancet Publishing Group, a division of Elsevier Science Ltd</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20051201</creationdate><title>Cystic fibrosis in neonates and infants</title><author>Minasian, Caro ; McCullagh, Angela ; Bush, Andrew</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c441t-c01010935af102f1609e250850f37df7522c8ab85648e7b79e8c2483229feb193</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2005</creationdate><topic>Biological and medical sciences</topic><topic>Care and treatment</topic><topic>Child, Preschool</topic><topic>Cystic fibrosis</topic><topic>Cystic Fibrosis - diagnosis</topic><topic>Cystic Fibrosis - genetics</topic><topic>Cystic Fibrosis - physiopathology</topic><topic>Cystic fibrosis transmembrane regulator</topic><topic>Female</topic><topic>Gastroenterology. Liver. Pancreas. Abdomen</topic><topic>Humans</topic><topic>Infant</topic><topic>Infant Nutritional Physiological Phenomena</topic><topic>Infant, Newborn</topic><topic>Infant, Newborn, Diseases - diagnosis</topic><topic>Infant, Newborn, Diseases - physiopathology</topic><topic>Infants</topic><topic>Liver. Biliary tract. Portal circulation. Exocrine pancreas</topic><topic>Lung diseases</topic><topic>Meconium ileus</topic><topic>Medical sciences</topic><topic>Neonatal Screening - methods</topic><topic>Other diseases. Semiology</topic><topic>Screening</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Minasian, Caro</creatorcontrib><creatorcontrib>McCullagh, Angela</creatorcontrib><creatorcontrib>Bush, Andrew</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Early human development</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Minasian, Caro</au><au>McCullagh, Angela</au><au>Bush, Andrew</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Cystic fibrosis in neonates and infants</atitle><jtitle>Early human development</jtitle><addtitle>Early Hum Dev</addtitle><date>2005-12-01</date><risdate>2005</risdate><volume>81</volume><issue>12</issue><spage>997</spage><epage>1004</epage><pages>997-1004</pages><issn>0378-3782</issn><eissn>1872-6232</eissn><coden>EHDEDN</coden><abstract>Cystic fibrosis (CF) is a common autosomal recessive disorder, characterized by chronic bronchopulmonary infection, pancreatic insufficiency, and subsequently, other multisystem complications. 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| identifier | ISSN: 0378-3782 |
| ispartof | Early human development, 2005-12, Vol.81 (12), p.997-1004 |
| issn | 0378-3782 1872-6232 |
| language | eng |
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| source | MEDLINE; Elsevier ScienceDirect Journals |
| subjects | Biological and medical sciences Care and treatment Child, Preschool Cystic fibrosis Cystic Fibrosis - diagnosis Cystic Fibrosis - genetics Cystic Fibrosis - physiopathology Cystic fibrosis transmembrane regulator Female Gastroenterology. Liver. Pancreas. Abdomen Humans Infant Infant Nutritional Physiological Phenomena Infant, Newborn Infant, Newborn, Diseases - diagnosis Infant, Newborn, Diseases - physiopathology Infants Liver. Biliary tract. Portal circulation. Exocrine pancreas Lung diseases Meconium ileus Medical sciences Neonatal Screening - methods Other diseases. Semiology Screening |
| title | Cystic fibrosis in neonates and infants |
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