Rheumatic disease and cystic fibrosis

Merkel, Peter A.

doi:10.1002/1529-0131(199908)42:8<1563::aid-anr1>3.0.co;2-h

Cited by 32 publications

(19 citation statements)

References 88 publications

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“…Osteoporosis is a common health problem as patients with cystic fibrosis survive longer (6)(7)(8)(9)(10)(11), and one study of patients with this disease showed that decreased bone density was more prevalent in the most malnourished patients (8). A subnormal body mass index (BMI), among other factors, can compromise bone density (6,11,12).…”

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confidence: 99%

Nutrient Status of Adults with Cystic Fibrosis

Gordon¹,

Anderson²,

Herlyn³

et al. 2007

Journal of the American Dietetic Association

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Nutrition is thought to influence disease status in patients with cystic fibrosis (CF). This crosssectional study sought to evaluate nutrient intake and anthropometric data from 64 adult outpatients with cystic fibrosis. Nutrient intake from food and supplements was compared with the Dietary Reference Intakes for 16 nutrients and outcomes influenced by nutritional status. Attention was given to vitamin D and calcium given potential skeletal implications due to cystic fibrosis. Measurements included weight, height, body composition, pulmonary function, and serum metabolic parameters. Participants were interviewed about dietary intake, supplement use, pulmonary function, sunlight exposure, and pain. The participants' mean body mass index (±standard deviation) was 21.8±4.9 and pulmonary function tests were normal. Seventy-eight percent used pancreatic enzyme replacement for malabsorption. Vitamin D deficiency [25-hydroxyvitamin D (25OHD)<37.5 nmol/L] was common: 25 (39%) were deficient despite adequate vitamin D intake. Lipid profiles were normal in the majority, even though total and saturated fat consumption represented 33.0% and 16.8% of energy intake, respectively. Reported protein intake represented 16.9% of total energy intake (range 10%-25%). For several nutrients, including vitamin D and calcium, intake from food and supplements in many participants exceeded recommended Tolerable Upper Intake Levels. Among adults with cystic fibrosis, vitamin D deficiency was common despite reported adequate intake, and lipid profiles were Copyright © 2007 Significant advances in the management of cystic fibrosis (CF) have increased the lifespan for patients with this common disease (1 in 3,900 white live births) (1). Previous research has suggested that patients with cystic fibrosis who have a good nutritional status have the best chance of maintaining and improving health (1-3). Nutritional therapy itself may also contribute to a better pulmonary status (4,5). A 1992 consensus report (1) emphasized the importance of detecting malnutrition in these patients to ensure optimal therapeutic management. A 2003 expert report on bone disease in cystic fibrosis cited both malnutrition and physical inactivity as risk factors for poor bone health (6).Osteoporosis is a common health problem as patients with cystic fibrosis survive longer (6-11), and one study of patients with this disease showed that decreased bone density was more prevalent in the most malnourished patients (8). A subnormal body mass index (BMI), among other factors, can compromise bone density (6,11,12). A high fracture risk and kyphosis are also documented in these patients (12). Serum vitamin D concentrations may be an important predictor of skeletal health, but the relationship between vitamin D status and bone density remains unclear in this disease (6,9-15).Most reports on nutrition in cystic fibrosis have been reviews, or include data collected in children (16)(17)(18)(19)(20)(21)(22)(23). In this study, anthropometric, nutritional, and lifestyle va...

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Nutrient Status of Adults with Cystic Fibrosis

Gordon¹,

Anderson²,

Herlyn³

et al. 2007

Journal of the American Dietetic Association

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“…Artropatía asociada FQ: Presente en un 2-9% de los pacientes y se manifiesta por cuadros de dolor e inflamación de articulaciones (20).…”

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Fibrosis Quística en El Adulto

Melo

Fernández

2015

Revista Médica Clínica Las Condes

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RESUMENLa Fibrosis Quística (FQ) es una enfermedad hereditaria autosómica recesiva. La detección precoz sumado a medidas de intervención temprana han modificado el curso de esta enfermedad con mejorías en su sobrevida, lo que ha llevado a una población creciente de pacientes de 18 años. La mutación genética determina una alteración en una Proteína Reguladora de Conductancia Transmembrana (CFTR) que afecta a numerosos órganos y sistemas, pero el compromiso pulmonar es el que causa mayor morbimortalidad. El germen más frecuente que infecta a adultos es Pseudomonas aeruginosa y si bien hay una serie de medidas para el manejo de la infección crónica por Pseudomonas las terapia dirigida a la restauración de la función de la proteína CFTR ha tomado relevancia. Cuando la falla respiratoria progresa, la única alternativa disponible es el trasplante pulmonar que mejora la sobrevida y la calidad de vida en estos pacientes.

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“…The prevalence of CFA is uncertain but it has been estimated as between 2% and 8.5% (Botton 2003;Johnson 1994). The mean age at onset has been reported as 13.6 years (Turner 1997) and 17 years (Merkel 1999); however, Merkel stated that this may have been overestimated if the arthritis had been present but undiagnosed for some years. As people with CF are living longer, the prevalence of CFA may be increasing (Merkel 1999); for example in France, life expectancy at birth has increased from 30.4 years for people born between 1994 and 1996 to 39.1 years for those born between 2001 and 2003 (Bellis 2007).…”

Section: Description Of the Conditionmentioning

confidence: 99%

“…As people with CF are living longer, the prevalence of CFA may be increasing (Merkel 1999); for example in France, life expectancy at birth has increased from 30.4 years for people born between 1994 and 1996 to 39.1 years for those born between 2001 and 2003 (Bellis 2007). There is no formal definition of CFA, but it consists of distinct symptoms (Merkel 1999;Turner 1997) such as recurrent episodes of joint pain, swelling, tenderness and limitation of movement which may be disabling (Johnson 1994;Merkel 1999;Rush 1986;Turner 1997). Joint pains develop over approximately 12 to 24 hours and episodes last less than one week (Turner 1997).…”

Section: Description Of the Conditionmentioning

confidence: 99%

“…In general, there are no di erences in lung function, respiratory pathogens, gastrointestinal symptoms or treatment between people with CF with and without arthritis, and exacerbations of joint symptoms are generally not associated with exacerbations of pulmonary disease (Johnson 1994;Turner 1997). It is possible that CFA may become persistent and disabling (Johnson 1994;Merkel 1999;Turner 1997). The pathogenesis and aetiology of CFA are unclear.…”

Section: Description Of the Conditionmentioning

confidence: 99%

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Disease modifying anti-rheumatic drugs in people with cystic fibrosis-related arthritis

Thornton

Rangaraj

2012

Cochrane Database of Systematic Reviews

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Although it is generally recognised that cystic fibrosis-related arthritis can be episodic and resolve spontaneously, treatment with analgesics and anti-inflammatory agents may be needed. But when episodic symptoms progress to persistent disease, disease-modifying anti-rheumatic drugs may be needed to limit the course of the disease. It is disappointing that no randomised controlled trials to rigorously evaluate these drugs could be found. This systematic review has identified the need for a well-designed adequately powered randomised controlled trial to assess the efficacy and safety of disease-modifying anti-rheumatic drugs for the management of cystic fibrosis-related arthropathy and hypertrophic osteoarthropathy in adults and children with cystic fibrosis. However, given the infrequency of cystic fibrosis-related arthritis and the range of symptoms and severity, randomised controlled trials may not be feasible and well-designed non-randomised observational studies may be more appropriate. Studies should also better define the two conditions.

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Rheumatic disease and cystic fibrosis

Cited by 32 publications

References 88 publications

Nutrient Status of Adults with Cystic Fibrosis

Nutrient Status of Adults with Cystic Fibrosis

Fibrosis Quística en El Adulto

Disease modifying anti-rheumatic drugs in people with cystic fibrosis-related arthritis

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