Clinical features, survival rate, and prognostic factors in young adults with cystic fibrosis

Huang, Nancy Ν.; Schidlow, Daniel V.; Szatrowski, Ted H.; Palmer, Judy; Laraya-Cuasay, Lourdes R.; Yeung, William H.; Hardy, Karen; Quitell, Lynn; Fiel, Stanley B.

doi:10.1016/0002-9343(87)90147-1

Cited by 111 publications

(49 citation statements)

References 47 publications

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“…However, the present study provides an important extra dimension to published studies on predictors of mortality. The earliest, observational, studies recognised the association of poor nutritional status and low FEV1 with a worse outcome [15][16][17]. Since then, more robust epidemiological studies have confirmed this correlation, including a large population study of the Canadian Patient Data Registry [3].…”

Section: Discussionmentioning

confidence: 99%

Cystic fibrosis and survival to 40 years: a case–control study

Simmonds¹,

MacNeill²,

Cullinan³

et al. 2010

Eur Respir J

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The clinical course of patients with cystic fibrosis (CF) is variable and probably determined by many interacting factors. We aimed to examine the influence of early social and clinical factors on long-term survival.A case-control study of adult CF patients was used to compare long-term survivors (aged o40 yrs) with patients who died before reaching 30 yrs of age. Each case (n578) was matched by birth date with at least one control (n5152), after exclusion of ''late diagnosis'' patients. Probability-weighted logistic regression models were used to identify influences on survival.Factors resulting in increased probabilities of survival included high body mass index (OR 1.76, 95% CI 1.40-2.22), forced expiratory volume in 1 s (OR per 5% increase 1.54, 95% CI 1.32-1.80), and forced vital capacity (OR per 5% increase 1.54, 95% CI 1.33-1.78) at transfer to the adult clinic and the exclusive use of oral antibiotics (OR 8.31,. Factors resulting in decreased probabilities of survival were Pseudomonas aeruginosa acquisition (OR 0.18, 95% 0.05-0.65) or pneumothorax before transfer to the adult clinic (OR 0.02, 95% CI 0.004-0.08) and referral from a paediatric clinic in a deprived area (OR 0.13, 95% CI 0.04-0.38).Long-term survival is associated with the clinical features present by the time of referral to an adult clinic. Even ''early-diagnosis'' disease appears to have different phenotypes, possibly independent of CF gene function, that have different survival patterns.

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Section: Discussionmentioning

confidence: 99%

Cystic fibrosis and survival to 40 years: a case–control study

Simmonds¹,

MacNeill²,

Cullinan³

et al. 2010

Eur Respir J

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“…6,55,56 65 The links between more detailed measures of exercise performance including recovery parameters, disease severity and quality of life deserve more attention. Objective measures may be more accurate in predicting prognosis and mapping progress and response to treatments than more subjective scoring systems of well-being.…”

Section: Discussionmentioning

confidence: 99%

Early oxygen uptake recovery following exercise testing in children with chronic chest diseases

Stevens

Oades

Armstrong

et al. 2009

Pediatric Pulmonology

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“…A distribuição em relação a sexo, raça 4-6 e incidência de manifestações pulmonares 4,7,8 foi semelhante aos dados encontrados na literatura. A FC, apesar de não ter cura, apresenta melhora significativa com o tratamento sintomático 23 .…”

Section: Discussionunclassified

Cystic fibrosis at a Brazilian center of excellence: clinical and laboratory characteristics of 104 patients and their association with genotype and disease severity

et al. 2004

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Objective: To identify the clinical, laboratory and radiographic characteristics of the cystic fibrosis patients under care at Universidade Estadual de Campinas (UNICAMP) in the last decade of the twentieth century, and to investigate the association of these characteristics with genotype and severity of the disease as measured by the Shwachman score.Methods: Descriptive, retrospective and cross-sectional study of the patients assisted at UNICAMP hospitals Cystic Fibrosis Clinic from July 1990 to July 2000. Results:One hundred and four patients were studied; 53.8% male; 93.3% Caucasian; 89.4% presented with respiratory symptoms; 59.6% presented with digestive symptoms; 5.8% had meconium ileus; 4.8% had diabetes. The mean age at onset of symptoms was 3 months, and the mean age at diagnosis was 2 years and 4 months. At diagnosis, 69.9 and 56.6% of the patients had weight and height below 10 th percentile, respectively; in 10.6%, sweat chloride was < 60 mEq/l. Staphylococcus aureus was found in 80.2%, Pseudomonas aeruginosa in 76.0%, and Burkholderia cepacia in 5.2%. ∆F508 homozygosis was observed in 18.75%, whereas 62.50% of the patients were ∆F508 heterozygous. A moderate/severe Shwachman score was found in 15.7%. Eighteen patients died in that period (17.3%). The mean age at death was 7 years and 8 months; median survival after diagnosis was 18 years and 4 months. Patients who have at least one ∆F508 mutation have more frequent alterations in fecal fat levels when compared to patients who do not have this mutation (p < 0.05). There were no differences in any parameter between ∆F508 homozygous and heterozygous patients. Conclusions:The clinical and laboratory characteristics of the 104 patients studied were similar to the characteristics described for patients in other countries. Exceptions are the higher age at diagnosis and lower survival. Our results support the recommendation for early diagnosis and the need for more treatment opportunities in the population of cystic fibrosis patients. ResumoObjetivo: Estudar as características clínicas, laboratoriais e radiográficas de pacientes fibrocísticos acompanhados na última década do século 20 na UNICAMP e verificar se existe associação com o genótipo e a gravidade da doença medida pelo escore de Shwachman.Métodos: Estudo descritivo, retrospectivo e de corte transversal dos pacientes fibrocísticos acompanhados na UNICAMP, que tiveram atendimento entre julho de 1990 e julho de 2000.Resultados: Foram estudados 104 pacientes: sexo masculino -53,8%; raça caucasóide -93,3%; comprometimento pulmonar -89,4%, comprometimento digestivo -59,6%; íleo meconial -5,8%; diabetes melito -4,8%; mediana da idade de início dos sintomas -3 meses; mediana da idade no diagnóstico -2 anos e 4 meses; 69,9 e 56,6% apresentavam peso e estatura abaixo do percentil 10, respectivamente, na época do diagnóstico; dosagem de cloro no suor < 60 mEq/l -10,6%; colonização: S. aureus -80,2%, P. aeruginosa -76,0%, B. cepacia -5,2%; ∆F508 homozigoto -18,75%, ∆F508heterozigoto -62,5%; escore de ...

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Clinical features, survival rate, and prognostic factors in young adults with cystic fibrosis

Cited by 111 publications

References 47 publications

Cystic fibrosis and survival to 40 years: a case–control study

Cystic fibrosis and survival to 40 years: a case–control study

Early oxygen uptake recovery following exercise testing in children with chronic chest diseases

Cystic fibrosis at a Brazilian center of excellence: clinical and laboratory characteristics of 104 patients and their association with genotype and disease severity

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