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• W2001893668 abstract "The life expectancy for individuals with cysticfibrosis (CF) has dramatically improved over the past 50years.1Davis PB Drumm M Konstan MW Cystic fibrosis.Am J Respir Crit Care Med. 1996; 154: 1229-1256Crossref PubMed Scopus (826) Google Scholar The median length of survival has increased fromroughly 1 year in 1950 to > 30 years today. Children born with CFtoday have an estimated life expectancy of approximately 40years.2Elborn JS Shale DJ Britton JR Cystic fibrosis: current survival and population estimates to the year 2000.Thorax. 1991; 46: 881-885Crossref PubMed Scopus (314) Google Scholar There are multiple factors that have contributedto this progress. The development of pancreatic enzyme replacements totreat malabsorption and of effective antipseudomonal antibiotics tosuppress airway infection has been crucial. The consolidation of careat Cystic Fibrosis Care Centers sponsored by the US CysticFibrosis Foundation, early diagnosis, aggressive disease management, and a host of new therapeutic interventions also have been integral inenhancing quality of life and increasing life expectancy.The increasing longevity of CF patients has resulted in marked changesin the epidemiology of CF and an aging CF population.3FitzSimmons SC The changing epidemiology of cystic fibrosis.J Pediatr. 1993; 122: 1-9Abstract Full Text PDF PubMed Scopus (804) Google Scholar Themost recent Cystic Fibrosis Foundation Patient Registry reportindicates a significant increase in the percentage of CF patientsclassified as adults (≥ 18 years), with the total percentage risingfrom 29.5% in 1988 to 37% in 1998.4Cystic Fibrosis Foundation Patient registry annual data report 1998. Cystic Fibrosis Foundation, Bethesda, MD1999Google Scholar As a result of aging, CF patients are developing a number of CF-related complicationsas well as “adult” medical problems (eg, reproductiveissues) not routinely treated by pediatric pulmonologists. The CysticFibrosis Foundation has long recognized the need for internalmedicine-trained pulmonologists to care for the growing number of adultCF patients and has recommended, in a 1990 consensus document, theincorporation of adult-care specialists in center programs, theestablishment of inpatient services in internal medicine wards, and thecreation of transition teams or parallel adult-careteams.5Cystic Fibrosis Foundation Center Committee and Guidelines Subcommittee Cystic Fibrosis Foundation guidelines for patient services, evaluation, and monitoring in cystic fibrosis centers.Am J Dis Child. 1990; 144: 1311-1312Crossref PubMed Scopus (64) Google Scholar Clearly, there is a strong and growing need foradult-care providers in the nationwide network of CF Care Centers.Adult CF patients are at increased risk for a number of CF-relatedcomplications.6Schidlow DV Taussig LM Knowles MR Cystic Fibrosis Foundation consensus conference report on pulmonary complications of cystic fibrosis.Pediatr Pulmonol. 1993; 5: 87-198Google Scholar7Rolfe MW Schnapf BM Management of the adult patient with cystic fibrosis.Clin Pulm Med. 1995; 2: 75-87Crossref Scopus (3) Google Scholar These include CF-related diabetes, ototoxicity and nephrotoxicity due to repeated therapy withaminoglycoside antibiotics, the development of multiresistantPseudomonas aeruginosa and other organisms, liver disease, osteoporosis and arthropathy, pneumothorax, and majorhemoptysis.4Cystic Fibrosis Foundation Patient registry annual data report 1998. Cystic Fibrosis Foundation, Bethesda, MD1999Google Scholar Other CF-related disorders prevalent in theolder CF population include a variety of GI and genitourinary problems. Additionally, older CF patients encounter the same types of ailments asage-matched peers. Thus, as McCallum and colleagues point out in thisissue of CHEST (see page 1059), CF is now a disease of theadult population with many adult-specific issues. As such, adult CFpatients must be treated by an interdisciplinary team of adult-careproviders within the environment of the expanding CF Care Centernetwork.Infertility has long been recognized among men and women withCF.8Wood RE Boat TF Doershuk CF Cystic fibrosis.Am Rev Respir Crit Care Med. 1976; 113: 833-878PubMed Google Scholar Men are almost universally infertile due to thecongenital bilateral absence of the vas deferens (CBAVD) with resultantobstructive azoospermia, whereas women show normal anatomy but thick, tenacious cervical mucus that fails to thin duringmenses.9Phillipson G Cystic fibrosis and reproduction.Reprod Fertil Dev. 1998; 10: 113-119Crossref PubMed Scopus (27) Google Scholar Although the specific reasons for these findingsare unclear, it may relate to the degree of expression of the CFtransmembrane conductance regulator protein in reproductivetissues.10Tizzano EF Silver MM Chitayat D et al.Differential cellular expression of cystic fibrosis transmembrane conductance regulator in human reproductive tissues.Am J Pathol. 1994; 144: 906-914PubMed Google Scholar As the life expectancy for CF patients hasrisen and greater numbers of patients are reaching adulthood, theissues of fertility and reproductive health have become increasinglyimportant.The myriad reproductive health and fertility issues facing male andfemale CF patients have been addressed in numerouspublications.9Phillipson G Cystic fibrosis and reproduction.Reprod Fertil Dev. 1998; 10: 113-119Crossref PubMed Scopus (27) Google Scholar11Seale TW Flux M Rennert OM Reproductive defects in patients of both sexes with cystic fibrosis: a review.Ann Clin Lab Sci. 1985; 15: 152-158PubMed Google Scholar12Kotloff RM FitzSimmons SC Fiel SB Fertility and pregnancy in patients with cystic fibrosis.Clin Chest Med. 1992; 13: 623-635PubMed Google Scholar Additionally, a substantial body of medical literature addresses the issue of fertility and the approach toinfertility in the general population. McCallum and colleagues nowprovide a timely discussion of male infertility and an update on thecurrent surgical approaches to male infertility in patients with CF. They review their experience with eight couples in which the malepartner was infertile due to CBAVD, providing detailed clinicalinformation and therapeutic results (including pregnancy outcomes) of sperm retrieval and intracytoplasmic sperm injection.This article provides important information for all clinicianscaring for adolescent and adult CF patients. Its publication inCHEST represents a growing awareness of the importance of CFamong adult-care providers and the significance of the exchange of information across disciplines in medical care. The life expectancy for individuals with cysticfibrosis (CF) has dramatically improved over the past 50years.1Davis PB Drumm M Konstan MW Cystic fibrosis.Am J Respir Crit Care Med. 1996; 154: 1229-1256Crossref PubMed Scopus (826) Google Scholar The median length of survival has increased fromroughly 1 year in 1950 to > 30 years today. Children born with CFtoday have an estimated life expectancy of approximately 40years.2Elborn JS Shale DJ Britton JR Cystic fibrosis: current survival and population estimates to the year 2000.Thorax. 1991; 46: 881-885Crossref PubMed Scopus (314) Google Scholar There are multiple factors that have contributedto this progress. The development of pancreatic enzyme replacements totreat malabsorption and of effective antipseudomonal antibiotics tosuppress airway infection has been crucial. The consolidation of careat Cystic Fibrosis Care Centers sponsored by the US CysticFibrosis Foundation, early diagnosis, aggressive disease management, and a host of new therapeutic interventions also have been integral inenhancing quality of life and increasing life expectancy. The increasing longevity of CF patients has resulted in marked changesin the epidemiology of CF and an aging CF population.3FitzSimmons SC The changing epidemiology of cystic fibrosis.J Pediatr. 1993; 122: 1-9Abstract Full Text PDF PubMed Scopus (804) Google Scholar Themost recent Cystic Fibrosis Foundation Patient Registry reportindicates a significant increase in the percentage of CF patientsclassified as adults (≥ 18 years), with the total percentage risingfrom 29.5% in 1988 to 37% in 1998.4Cystic Fibrosis Foundation Patient registry annual data report 1998. Cystic Fibrosis Foundation, Bethesda, MD1999Google Scholar As a result of aging, CF patients are developing a number of CF-related complicationsas well as “adult” medical problems (eg, reproductiveissues) not routinely treated by pediatric pulmonologists. The CysticFibrosis Foundation has long recognized the need for internalmedicine-trained pulmonologists to care for the growing number of adultCF patients and has recommended, in a 1990 consensus document, theincorporation of adult-care specialists in center programs, theestablishment of inpatient services in internal medicine wards, and thecreation of transition teams or parallel adult-careteams.5Cystic Fibrosis Foundation Center Committee and Guidelines Subcommittee Cystic Fibrosis Foundation guidelines for patient services, evaluation, and monitoring in cystic fibrosis centers.Am J Dis Child. 1990; 144: 1311-1312Crossref PubMed Scopus (64) Google Scholar Clearly, there is a strong and growing need foradult-care providers in the nationwide network of CF Care Centers. Adult CF patients are at increased risk for a number of CF-relatedcomplications.6Schidlow DV Taussig LM Knowles MR Cystic Fibrosis Foundation consensus conference report on pulmonary complications of cystic fibrosis.Pediatr Pulmonol. 1993; 5: 87-198Google Scholar7Rolfe MW Schnapf BM Management of the adult patient with cystic fibrosis.Clin Pulm Med. 1995; 2: 75-87Crossref Scopus (3) Google Scholar These include CF-related diabetes, ototoxicity and nephrotoxicity due to repeated therapy withaminoglycoside antibiotics, the development of multiresistantPseudomonas aeruginosa and other organisms, liver disease, osteoporosis and arthropathy, pneumothorax, and majorhemoptysis.4Cystic Fibrosis Foundation Patient registry annual data report 1998. Cystic Fibrosis Foundation, Bethesda, MD1999Google Scholar Other CF-related disorders prevalent in theolder CF population include a variety of GI and genitourinary problems. Additionally, older CF patients encounter the same types of ailments asage-matched peers. Thus, as McCallum and colleagues point out in thisissue of CHEST (see page 1059), CF is now a disease of theadult population with many adult-specific issues. As such, adult CFpatients must be treated by an interdisciplinary team of adult-careproviders within the environment of the expanding CF Care Centernetwork. Infertility has long been recognized among men and women withCF.8Wood RE Boat TF Doershuk CF Cystic fibrosis.Am Rev Respir Crit Care Med. 1976; 113: 833-878PubMed Google Scholar Men are almost universally infertile due to thecongenital bilateral absence of the vas deferens (CBAVD) with resultantobstructive azoospermia, whereas women show normal anatomy but thick, tenacious cervical mucus that fails to thin duringmenses.9Phillipson G Cystic fibrosis and reproduction.Reprod Fertil Dev. 1998; 10: 113-119Crossref PubMed Scopus (27) Google Scholar Although the specific reasons for these findingsare unclear, it may relate to the degree of expression of the CFtransmembrane conductance regulator protein in reproductivetissues.10Tizzano EF Silver MM Chitayat D et al.Differential cellular expression of cystic fibrosis transmembrane conductance regulator in human reproductive tissues.Am J Pathol. 1994; 144: 906-914PubMed Google Scholar As the life expectancy for CF patients hasrisen and greater numbers of patients are reaching adulthood, theissues of fertility and reproductive health have become increasinglyimportant. The myriad reproductive health and fertility issues facing male andfemale CF patients have been addressed in numerouspublications.9Phillipson G Cystic fibrosis and reproduction.Reprod Fertil Dev. 1998; 10: 113-119Crossref PubMed Scopus (27) Google Scholar11Seale TW Flux M Rennert OM Reproductive defects in patients of both sexes with cystic fibrosis: a review.Ann Clin Lab Sci. 1985; 15: 152-158PubMed Google Scholar12Kotloff RM FitzSimmons SC Fiel SB Fertility and pregnancy in patients with cystic fibrosis.Clin Chest Med. 1992; 13: 623-635PubMed Google Scholar Additionally, a substantial body of medical literature addresses the issue of fertility and the approach toinfertility in the general population. McCallum and colleagues nowprovide a timely discussion of male infertility and an update on thecurrent surgical approaches to male infertility in patients with CF. They review their experience with eight couples in which the malepartner was infertile due to CBAVD, providing detailed clinicalinformation and therapeutic results (including pregnancy outcomes) of sperm retrieval and intracytoplasmic sperm injection. This article provides important information for all clinicianscaring for adolescent and adult CF patients. Its publication inCHEST represents a growing awareness of the importance of CFamong adult-care providers and the significance of the exchange of information across disciplines in medical care." @default.
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