Standards of care for patients with cystic fibrosis: a European consensus

Kerem, Eitan; Conway, S.P.; Elborn, J. Stuart; Heijerman, H.G.M.

doi:10.1016/j.jcf.2004.12.002

Cited by 396 publications

(383 citation statements)

References 66 publications

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“…The Annual Assessment should include biochemical liver function tests, complete blood count [31,32] and liver ultrasound [33]. Management of chronic hepatobiliary disease includes early treatment with ursodeoxycholic acid (UDCA) with correction of malnutrition, specific treatment of portal hypertension and liver failure, and liver transplantation [32].…”

Section: Livermentioning

confidence: 99%

“…A severe consequence of BMD can be rib or vertebral bone fractures which interfere with chest physiotherapy and are associated with increased morbidity and mortality. Preventive strategies include annual measurement of calcium and vitamin D levels, high milk diet, weight bearing exercise and treatment with biphosphonate [32,45].…”

Section: Bone Diseasementioning

confidence: 99%

“…While pregnancy is more difficult in those with an FEV 1 less than 50% predicted [32], it is well tolerated by women in good health maintaining adequate nutrition, with good outcomes for mother and baby. Some of the mothers develop gestational diabetes [65].…”

Section: Females and Pregnancymentioning

confidence: 99%

“…The CF centre needs to arrange appropriate inpatient and out-patient care, including annual assessments and home treatment [32,78]. Both inpatients and those receiving intravenous antibiotic therapy at home should be discussed at least once weekly in a multidisciplinary meeting with all the members of the CF team [32].…”

Section: Facilitiesmentioning

confidence: 99%

“…To ensure appropriate treatment a multidisciplinary team at the CF centre is needed [5], although in many European countries there are no specialised adult CF centres and only 15 of 34 European countries have a national CF patient registry [6], which is essential for monitoring patient numbers, treatments and outcomes. Recently there has been a major initiative with EuroCareCF Workpackage 1 to improve this situation [6].…”

Section: Introductionmentioning

confidence: 99%

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Adult cystic fibrosis care in the 21st century

Garattini

Bilton

Cremona

et al. 2015

Monaldi Arch Chest Dis

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Cystic fibrosis (CF) is the most common autosomal recessive inherited disease of Caucasian populations. As a result of a variety of diagnostic and therapeutic strategies there has been a dramatic increase in the life expectancy of patients with CF in the last decades and 50% of patients are now adults. This review will focus on the disease in adults and the provision of appropriate care. The complex care required to improve the survival and quality of life in the adult patients can best be provided in a dedicated adult cystic fibrosis unit. These units currently exist in many European countries, but more are needed in Italy.

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Section: Livermentioning

confidence: 99%

Section: Bone Diseasementioning

confidence: 99%

Section: Females and Pregnancymentioning

confidence: 99%

Section: Facilitiesmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Adult cystic fibrosis care in the 21st century

Garattini

Bilton

Cremona

et al. 2015

Monaldi Arch Chest Dis

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Predictors of Timing of Transfer From Pediatric- to Adult-Focused Primary Care

et al. 2015

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IMPORTANCE A timely, well-coordinated transfer from pediatric-to adult-focused primary care is an important component of high-quality health care, especially for youths with chronic health conditions. Current recommendations suggest that primary-care transfers for youths occur between 18 and 21 years of age. However, the current epidemiology of transfer timing is unknown.OBJECTIVE To examine the timing of transfer to adult-focused primary care providers (PCPs), the time between last pediatric-focused and first adult-focused PCP visits, and the predictors of transfer timing. DESIGN, SETTING, AND PARTICIPANTS Retrospective cohort study of patients insured byHarvard Pilgrim Health Care (HPHC), a large not-for-profit health plan. Our sample included 60 233 adolescents who were continuously enrolled in HPHC from 16 to at least 18 years of age between January 2000 and December 2012. Pediatric-focused PCPs were identified by the following provider specialty types, but no others: pediatrics, adolescent medicine, or pediatric nurse practitioner. Adult-focused PCPs were identified by having any provider type that sees adult patients. Providers with any specialty provider designation (eg, gastroenterology or gynecology) were not considered PCPs. MAIN OUTCOMES AND MEASURESWe used multivariable Cox proportional hazards regression to model age at first adult-focused PCP visit and time from the last pediatric-focused to the first adult-focused PCP visit (gap) for any type of office visit and for those that were preventive visits.RESULTS Younger age at transfer was observed for female youths (hazard ratio [HR], 1.32 [95% CI, 1.29-1.36]) who had complex (HR, 1.06 [95% CI, 1.01-1.11]) or noncomplex (HR, 1.08 [95% CI, 1.05-1.12]) chronic conditions compared with those who had no chronic conditions. Transfer occurred at older ages for youths who lived in lower-income neighborhoods compared with those who lived in higher-income neighborhoods (HR, 0.89 [95% CI, 0.83-0.95]). The gap between last pediatric-focused to first adult-focused PCP visit was shorter for female youths than male youths (HR, 1.57 [95% CI, 1.53-1.61]) and youths with complex (HR, 1.35 [95% CI, 1.28-1.41]) or noncomplex (HR, 1.24 [95% CI, 1.20-1.28]) chronic conditions. The gap was longer for youths living in lower-income neighborhoods than for those living in higher-income neighborhoods (HR, 0.80 [95% CI, 0.75-0.85]). Multivariable models showed an adjusted median age at transfer of 21.8 years for office visits and 23.1 years for preventive visits and an adjusted median gap length of 20.5 months for office visits and 41.6 months for preventive visits. CONCLUSIONS AND RELEVANCEMost youths are transferring care later than recommended and with gaps of more than a year. While youths with chronic conditions have shorter gaps, they may need even shorter transfer intervals to ensure continuous access to care. More work is needed to determine whether youths are experiencing clinically important lapses in care or other negative health effects due to the delayed timing of tr...

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Prevalence and Incidence of Anxiety and Depression Among Children, Adolescents, and Young Adults With Life-Limiting Conditions

et al. 2019

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IMPORTANCE Children, adolescents, and young adults with life-limiting conditions experience various challenges that may make them more vulnerable to mental health problems, such as anxiety and depression. However, the prevalence and incidence of anxiety and depression among this population appears to be unknown. OBJECTIVE To conduct a systematic review and meta-analysis to estimate the prevalence and/or incidence of anxiety and depression in children, adolescents, and young adults with life-limiting conditions. DATA SOURCES Searches of MEDLINE (PubMed), PsycInfo, and Embase were conducted to identify studies published between January 2000 and January 2018. STUDY SELECTION Studies were eligible for this review if they provided primary data of anxiety or depression prevalence and/or incidence, included participants aged 5 to 25 years with a life-limiting condition, were conducted in an Organisation for Economic Cooperation and Development country, and were available in English. DATA EXTRACTION AND SYNTHESIS Random-effects meta-analyses were generated to provide anxiety and depression prevalence estimates. Meta-regression was conducted to analyze associations between study characteristics and each prevalence estimate. MAIN OUTCOMES AND MEASURES Prevalence of anxiety and depression. RESULTS A total of 14 866 nonduplicate articles were screened, of which 37 were included in the review. Of these, 19 studies reported anxiety prevalence, and 36 studies reported depression prevalence. The mean (range) age of participants was 15.4 (6-25) years. The meta-analysis of anxiety prevalence (n = 4547 participants) generated a pooled prevalence estimate of 19.1% (95% CI, 14.1%-24.6%). Meta-regression analysis found statistically significant differences in anxiety prevalence by assessment tool; diagnostic interviews were associated with higher anxiety prevalence (28.5% [95% CI, 13.2%-46.8%]) than self-reported or parent-reported measures (14.9% [95% CI, 10.9%-19.4%]). The depression meta-analysis (n = 5934 participants) found a pooled prevalence estimate of 14.3% (95% CI, 10.5%-18.6%). Meta-regression analysis revealed statistically significant differences in depression prevalence by the mean age of the sample (β = 0.02 [95% CI, 0.01-0.03]; P = .001). CONCLUSIONS AND RELEVANCE In this systematic review and meta-analysis, the prevalence of anxiety and depression among children, adolescents, and young adults with life-limiting conditions was high, highlighting the need for increased psychological assessment and monitoring. Further research is required to determine the prevalence and incidence of anxiety and depression in a larger sample of children, adolescents, and young adults with a broader range of life-limiting conditions.

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Standards of care for patients with cystic fibrosis: a European consensus

Cited by 396 publications

References 66 publications

Adult cystic fibrosis care in the 21st century

Adult cystic fibrosis care in the 21st century

Predictors of Timing of Transfer From Pediatric- to Adult-Focused Primary Care

Prevalence and Incidence of Anxiety and Depression Among Children, Adolescents, and Young Adults With Life-Limiting Conditions

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