{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T19:15:44Z","timestamp":1790709344874,"version":"4.1.0"},"reference-count":89,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2018,11,12]],"date-time":"2018-11-12T00:00:00Z","timestamp":1541980800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology"],"published-print":{"date-parts":[[2019,2]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Cystic fibrosis (CF) is an autosomal recessive genetic disease caused by mutations in the\n                    <jats:italic>CFTR<\/jats:italic>\n                    gene, with various clinical manifestations that affect pulmonary, digestive, exocrine and male reproductive functions as well as the bones and kidneys. This study aimed to reveal the spectrum of\n                    <jats:italic>CFTR<\/jats:italic>\n                    gene mutations in Arab CF patients and their corresponding clinical phenotypes among the 22 Arab countries. We searched four literature databases (PubMed, Science Direct, Web of Science and Scopus) from their times of inception to January 2018. All possible search terms were used to encompass the different clinical phenotypes, disease incidences,\n                    <jats:italic>CFTR<\/jats:italic>\n                    mutations, ages and consanguinity rates of CF patients in the 22 Arab countries. Our search strategy identified 678 articles; of these, 72 were eligible for this systematic review. We retrieved data from 18 Arab countries; only 1766 Arab patients with CF were identified, even after additional searches using Google and Google Scholar. The search uncovered a wide spectrum of mutations, some of which are shared with other ethnic groups and some unique to Arab patients. Although the clinical phenotypes of Arab patients were typical of CF, several distinct phenotypes were reported. Despite the rarity of genetic epidemiological studies of CF patients among the 22 Arab nations, the disease is frequently reported in Arab countries where consanguineous marriage is common. Therefore, significant attention should be paid to this problem by implementing carrier and premarital screening, newborn screening and genetic counselling.\n                  <\/jats:p>","DOI":"10.1111\/resp.13437","type":"journal-article","created":{"date-parts":[[2018,11,12]],"date-time":"2018-11-12T19:50:05Z","timestamp":1542052205000},"page":"127-136","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["Spectrum of mutations of cystic fibrosis in the 22 Arab countries: A systematic review"],"prefix":"10.1111","volume":"24","author":[{"given":"Duaa","family":"Al\u2010Sadeq","sequence":"first","affiliation":[{"name":"Biomedical Science Department, College of Health Sciences Qatar University Doha Qatar"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Taghreed","family":"Abunada","sequence":"additional","affiliation":[{"name":"Biomedical Science Department, College of Health Sciences Qatar University Doha Qatar"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rajaa","family":"Dalloul","sequence":"additional","affiliation":[{"name":"Biomedical Science Department, College of Health Sciences Qatar University Doha Qatar"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Fahad","sequence":"additional","affiliation":[{"name":"Biomedical Science Department, College of Health Sciences Qatar University Doha Qatar"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sara","family":"Taleb","sequence":"additional","affiliation":[{"name":"Biomedical Science Department, College of Health Sciences Qatar University Doha Qatar"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kholoud","family":"Aljassim","sequence":"additional","affiliation":[{"name":"Biomedical Science Department, College of Health Sciences Qatar University Doha Qatar"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fatima Alzahra","family":"Al Hamed","sequence":"additional","affiliation":[{"name":"Biomedical Science Department, College of Health Sciences Qatar University Doha Qatar"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8838-6638","authenticated-orcid":false,"given":"Hatem","family":"Zayed","sequence":"additional","affiliation":[{"name":"Biomedical Science Department, College of Health Sciences Qatar University Doha Qatar"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,11,12]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.2475911"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00018-016-2391-y"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc1502191"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI73469"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00018-016-2393-9"},{"key":"e_1_2_7_7_1","doi-asserted-by":"crossref","first-page":"46","DOI":"10.4037\/ccn2005.25.4.46","article-title":"Pathophysiology of cystic fibrosis: implications for critical care nurses","volume":"25","author":"Grossman S","year":"2005","journal-title":"Crit. Care Nurse"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1469-1809.2003.00028.x"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2017.11.013"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.prrv.2006.04.083"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2007.04.004"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1159\/000022849"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1093\/tropej\/48.6.348"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1093\/tropej\/44.1.35"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.15537\/smj.2016.3.13476"},{"key":"e_1_2_7_16_1","first-page":"157","article-title":"Incidence of cystic fibrosis in Bahrain","volume":"8","author":"Al Arrayed SS","year":"1996","journal-title":"J. Bahrain Med. Soc."},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2006.04.004"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1186\/s40064-016-2338-7"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jare.2013.07.005"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.30823"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1097\/GIM.0b013e31818efd59"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.4103\/1319-2442.109579"},{"key":"e_1_2_7_23_1","first-page":"185","article-title":"Clinical profile of cystic fibrosis. Atypical presentation","volume":"31","author":"Najada AS","year":"2010","journal-title":"Saudi Med. J."},{"key":"e_1_2_7_24_1","first-page":"1526","article-title":"The use of sweat chloride test for screening cystic fibrosis among malnourished children suffering from frequent respiratory infections","volume":"30","author":"Abu\u2010Alshiekh NK","year":"2009","journal-title":"Saudi Med. J."},{"key":"e_1_2_7_25_1","doi-asserted-by":"publisher","DOI":"10.1080\/02724936.2000.11748148"},{"key":"e_1_2_7_26_1","first-page":"157","article-title":"Cystic fibrosis in Jordan: clinical and genetic aspects","volume":"23","author":"Kakish KS","year":"2001","journal-title":"Bahrain Med. Bull."},{"key":"e_1_2_7_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(81)91260-5"},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2010.08.001"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004390050505"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2008.03.003"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1155\/2015\/138103"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.3109\/03014460.2011.557090"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2007.12.006"},{"key":"e_1_2_7_34_1","doi-asserted-by":"crossref","first-page":"e323","DOI":"10.18295\/2075-0528.1594","article-title":"Defining a mutational panel and predicting the prevalence of cystic fibrosis in Oman","volume":"14","author":"Fass UW","year":"2014","journal-title":"Sultan Qaboos Univ. Med. J."},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1399-0004.2000.570312.x"},{"key":"e_1_2_7_36_1","first-page":"1","article-title":"Novel mutation in the CFTR gene of cystic fibrosis patients in Oman","volume":"5","author":"Al\u2010Kindy H","year":"2014","journal-title":"J. Mol. Biomark. Diagn."},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.12816\/0026946"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0133890"},{"key":"e_1_2_7_39_1","doi-asserted-by":"publisher","DOI":"10.1155\/2015\/458653"},{"key":"e_1_2_7_40_1","doi-asserted-by":"publisher","DOI":"10.1155\/2014\/465395"},{"key":"e_1_2_7_41_1","first-page":"39","article-title":"Antimicrobial resistance of bacterial strains isolated from respiratory tract of cystic fibrosis patients with CFTR I1234V mutation","volume":"2","author":"Elshafie SS","year":"2007","journal-title":"J. Pediatr. Infect. Dis."},{"key":"e_1_2_7_42_1","doi-asserted-by":"publisher","DOI":"10.1080\/02724936.2000.11748135"},{"key":"e_1_2_7_43_1","doi-asserted-by":"publisher","DOI":"10.1038\/gim.2014.4"},{"key":"e_1_2_7_44_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2004.11.002"},{"key":"e_1_2_7_45_1","doi-asserted-by":"crossref","first-page":"69","DOI":"10.4103\/1817-1737.146892","article-title":"Two Qatari siblings with cystic fibrosis and apparent mineralocorticoid excess","volume":"10","author":"Zahraldin K","year":"2015","journal-title":"Ann. Thorac. Med."},{"key":"e_1_2_7_46_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1569-1993(10)60459-6"},{"key":"e_1_2_7_47_1","doi-asserted-by":"publisher","DOI":"10.1111\/crj.12335"},{"key":"e_1_2_7_48_1","doi-asserted-by":"publisher","DOI":"10.1080\/02724939992671"},{"key":"e_1_2_7_49_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.34.12.996"},{"key":"e_1_2_7_50_1","first-page":"97","article-title":"Sickle cell disease and cystic fibrosis","volume":"24","author":"Banjar HH","year":"2003","journal-title":"Saudi Med. J."},{"key":"e_1_2_7_51_1","doi-asserted-by":"publisher","DOI":"10.5144\/0256-4947.2003.416"},{"key":"e_1_2_7_52_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijpam.2016.09.001"},{"key":"e_1_2_7_53_1","first-page":"39","article-title":"Cystic fibrosis in Sudanese children: first report of 35 cases","volume":"14","author":"Ibrahim SA","year":"2014","journal-title":"Sudan. J. Paediatr."},{"key":"e_1_2_7_54_1","doi-asserted-by":"publisher","DOI":"10.1179\/027249302125000157"},{"key":"e_1_2_7_55_1","doi-asserted-by":"publisher","DOI":"10.1515\/cclm-2013-1001"},{"key":"e_1_2_7_56_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.patbio.2015.04.002"},{"key":"e_1_2_7_57_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.patbio.2015.07.003"},{"key":"e_1_2_7_58_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.patbio.2015.07.004"},{"key":"e_1_2_7_59_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12041-015-0609-7"},{"key":"e_1_2_7_60_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12041-013-0208-4"},{"key":"e_1_2_7_61_1","first-page":"223","article-title":"Identification of a cystic fibrosis mutation W19X in Tunisia","volume":"71","author":"Boudaya M","year":"2013","journal-title":"Ann. Biol. Clin."},{"key":"e_1_2_7_62_1","doi-asserted-by":"publisher","DOI":"10.1177\/004947559502500307"},{"key":"e_1_2_7_63_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1399-3003.1999.13a19.x"},{"key":"e_1_2_7_64_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1098-1004(1998)11:5<412::AID-HUMU15>3.0.CO;2-O"},{"key":"e_1_2_7_65_1","doi-asserted-by":"publisher","DOI":"10.26719\/2010.16.6.699"},{"key":"e_1_2_7_66_1","doi-asserted-by":"publisher","DOI":"10.1186\/s13256-015-0620-x"},{"key":"e_1_2_7_67_1","doi-asserted-by":"publisher","DOI":"10.1093\/oxfordjournals.aje.a009172"},{"key":"e_1_2_7_68_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0012-3692(15)52709-8"},{"key":"e_1_2_7_69_1","doi-asserted-by":"publisher","DOI":"10.4046\/trd.2016.79.4.267"},{"key":"e_1_2_7_70_1","first-page":"1","article-title":"An Arab registry for type 1 diabetes: global benefits for type 1 diabetes patients","author":"Zayed H","year":"2016","journal-title":"Curr. Med. Res. Opin."},{"key":"e_1_2_7_71_1","doi-asserted-by":"crossref","first-page":"1230","DOI":"10.26719\/1999.5.6.1230","article-title":"Geographic distribution of cystic fibrosis transmembrane regulator gene mutations in Saudi Arabia","volume":"5","author":"Banjar H","year":"1999","journal-title":"East. Mediterr. Health J."},{"key":"e_1_2_7_72_1","doi-asserted-by":"publisher","DOI":"10.1002\/humu.21232"},{"key":"e_1_2_7_73_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jcf.2012.01.005"},{"key":"e_1_2_7_74_1","doi-asserted-by":"publisher","DOI":"10.4172\/2155-9929.1000168"},{"key":"e_1_2_7_75_1","doi-asserted-by":"publisher","DOI":"10.4254\/wjh.v5.i3.156"},{"key":"e_1_2_7_76_1","doi-asserted-by":"publisher","DOI":"10.1136\/adc.77.4.i370"},{"key":"e_1_2_7_77_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004310051277"},{"key":"e_1_2_7_78_1","doi-asserted-by":"publisher","DOI":"10.1186\/1742-4755-6-17"},{"key":"e_1_2_7_79_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0021932004007060"},{"key":"e_1_2_7_80_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.38982.704931.AE"},{"key":"e_1_2_7_81_1","doi-asserted-by":"publisher","DOI":"10.1038\/srep37284"},{"key":"e_1_2_7_82_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11011-017-0006-4"},{"key":"e_1_2_7_83_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11892-016-0736-4"},{"key":"e_1_2_7_84_1","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.31.3.224"},{"key":"e_1_2_7_85_1","doi-asserted-by":"publisher","DOI":"10.1093\/tropej\/49.1.54"},{"key":"e_1_2_7_86_1","doi-asserted-by":"publisher","DOI":"10.1007\/s004390000246"},{"key":"e_1_2_7_87_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.gene.2016.07.007"},{"key":"e_1_2_7_88_1","doi-asserted-by":"publisher","DOI":"10.1111\/ijcp.12871"},{"key":"e_1_2_7_89_1","doi-asserted-by":"publisher","DOI":"10.1093\/tropej\/38.3.113"},{"key":"e_1_2_7_90_1","doi-asserted-by":"publisher","DOI":"10.1038\/s10038-017-0402-y"}],"container-title":["Respirology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fresp.13437","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/resp.13437","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,4]],"date-time":"2026-04-04T01:29:52Z","timestamp":1775266192000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/resp.13437"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,11,12]]},"references-count":89,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2019,2]]}},"alternative-id":["10.1111\/resp.13437"],"URL":"https:\/\/doi.org\/10.1111\/resp.13437","archive":["Portico"],"relation":{},"ISSN":["1323-7799","1440-1843"],"issn-type":[{"value":"1323-7799","type":"print"},{"value":"1440-1843","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,11,12]]},"assertion":[{"value":"2018-03-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-10-22","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-11-12","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}