The musculoskeletal complications of cystic fibrosis

Massie, John; Towns, Susan; Bernard, E; Chaitow, Jeffrey; Howman‐Giles, Robert; Asperen, Peter P Van

doi:10.1046/j.1440-1754.1998.00277.x

Cited by 26 publications

(19 citation statements)

References 19 publications

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“…Uma incidência inferior, em torno de 15,1% casos, havia sido publicada em estudo da década de 1980 22 . A alta incidência de complicações musculoesqueléticas na FC tem sido associada ao aumento da idade e piora da função pulmonar 4,18,23 . Especificamente sobre a postura, sugere-se que a presença de alterações posturais apresente correlação com reduções na função pulmonar e na capacidade de exercício 5,21,24 .…”

Section: Discussionunclassified

“…Alguns artigos apontam para o declínio da função pulmonar mediante comprometimentos posturais importantes 21 , principalmente do aumento da cifose torácica 23 . Massie et al 23 evidenciaram que esse tipo de alteração associa-se a idade aumentada e baixa função pulmonar, o que ainda é controverso, visto que o estudo de Erkilla et al não encontrou resultado semelhante.…”

Section: Discussionunclassified

“…Massie et al 23 evidenciaram que esse tipo de alteração associa-se a idade aumentada e baixa função pulmonar, o que ainda é controverso, visto que o estudo de Erkilla et al não encontrou resultado semelhante. Nele, a deformidade torácica não se correlacionou com a gravidade da doença nem com a função pulmonar, assim como na presente pesquisa, porém a prevalência da cifose aumentou com a idade 4 .…”

Section: Discussionunclassified

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Influence of thoracic spine postural disorders on cardiorespiratory parameters in children and adolescents with cystic fibrosis

Okuro¹,

Côrrea²,

Conti³

et al. 2012

J Pediatr (Rio J)

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Objectives: To assess the impact of increased thoracic kyphosis on pulmonary function and functional capacity in children and adolescents with cystic fibrosis (CF) and to verify the influence of disease severity, age and nutritional status on this deformity.Method: This was a cross-sectional, analytical study conducted at a university hospital. It included CF patients with confirmed diagnosis and without pulmonary exacerbation. The sample was submitted to postural assessment, spirometry (FEV 1 , FVC and FEV 1 /FVC) and 6-minute walk test distance (6-MWT distance). Data were analyzed using the Mann Whitney test, Spearman correlation and logistic regression.Results: Forty-two patients were enrolled, 61.9% presented increase of thoracic kyphosis. There was no difference in values of FEV 1 , FVC, FEV 1 /FVC and 6-MWT distance between the groups with or without thoracic kyphosis (p = 0.407; p = 0.756; p = 0.415; p = 0.294). In the group without alteration, patients with more disease severity had a mean FEV 1 of 74.1±21.9% and FVC of 79.8±18.7% while in those of lesser severity higher values were found (95.6±12.2% and 97.6±13.2%, respectively) (p = 0.027 and p = 0.027). The presence of kyphosis was correlated with age (p = 0.048) but not with severity (p = 0.151) and body mass index (p = 0.088). Conclusions:There was a high prevalence of increased thoracic kyphosis in children and adolescents with CF. The deformity did not affect pulmonary function and functional capacity and there was no relationship with disease severity. Regardless of posture, worsening of disease severity determined worsening of pulmonary function.J Pediatr (Rio J). 2012;88(4):310-6: Posture, cystic fibrosis, pulmonary function. ResumoObjetivos: Avaliar a repercussão do aumento da cifose torácica na função pulmonar e na capacidade funcional de crianças e adolescentes com fibrose cística e verificar a influência da gravidade da doença, idade e aspectos nutricionais sobre essa deformidade.Método: Estudo analítico de corte transversal, realizado em um hospital universitário. Incluiu fibrocísticos com diagnóstico confirmado e fora de exacerbação pulmonar. A amostra foi submetida à avaliação postural, registro de parâmetros espirométricos -volume expiratório forçado no primeiro segundo (VEF 1 ), capacidade vital forçada (CVF) e VEF 1 /CVF -e da distância percorrida pelo teste de caminhada de 6 minutos. Para análise dos dados, utilizou-se teste de Mann-Whitney, correlação de Spearman e regressão logística, considerando-se nível de significância de 5%. Resultados:No total, 42 pacientes foram incluídos, e 61,9% apresentaram aumento da cifose torácica. Não houve diferença nos valores de VEF 1 , CVF, VEF 1 /CVF e distância percorrida pelo teste de caminhada de 6 minutos entre os grupos com e sem cifose torácica (p = 0,407; p = 0,756; p = 0,415; p = 0,294). No grupo sem alteração postural, fibrocísticos mais graves apresentaram média de VEF 1 de 74,1±21,9% e CVF de 79,8±18,7%, enquanto naqueles de menor gravidade foram verificados valores superiores...

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Section: Discussionunclassified

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Influence of thoracic spine postural disorders on cardiorespiratory parameters in children and adolescents with cystic fibrosis

Okuro¹,

Côrrea²,

Conti³

et al. 2012

J Pediatr (Rio J)

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“…In a more recent study by the same group, pain (assessed with a retrospective interview) was associated with decreased HRQOL as measured by several domains on the CFQ-R (85). Four additional studies have evaluated pain in patients with CF (86)(87)(88)(89). Two studies focused on pain in the setting of severe and end-stage disease (86,87), whereas the other focused on musculoskeletal pain (88).…”

Section: Painmentioning

confidence: 99%

“…Four additional studies have evaluated pain in patients with CF (86)(87)(88)(89). Two studies focused on pain in the setting of severe and end-stage disease (86,87), whereas the other focused on musculoskeletal pain (88). One study focused solely on adults (89).…”

Section: Painmentioning

confidence: 99%

Patient-reported Outcomes in Cystic Fibrosis

Goss¹,

Quittner²

2007

Proceedings of the American Thoracic Society

116

107

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Over the past 20 years, there has been tremendous progress in the area of patient-reported outcomes (PROs). A PRO instrument is defined as any measure of a patient's health status that is elicited directly from the patient and assesses how the patient "feels or functions with respect to his or her health condition." The advances seen in clinical research regarding PROs has been mirrored in research in cystic fibrosis (CF). A large number of instruments have been used for both therapeutic and nontherapeutic clinical research for many chronic conditions. This review will summarize a history of the development of PROs and how PROs are viewed by the U.S. Food and Drug Administration. We will then review the current state of the art of patient-reported outcomes in CF, specifically addressing the evaluation of different PRO instruments in terms of their reliability and validity. Finally, we will delineate further areas for development of PROs in CF. We believe that the future of CF research will incorporate a more diverse selection of PRO outcome measures; these outcome measures ultimately may be incorporated into clinical care to standardize symptom assessment and provide information regarding the need for specific clinical interventions to improve the quality of care delivered to these patients.

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Anti-inflammatory drugs and analgesics for managing symptoms in people with cystic fibrosis-related arthritis

Thornton

Rangaraj²

2008

Cochrane Database of Systematic Reviews

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Although it is generally recognised that CFA may be episodic and resolve spontaneously, treatment with analgesics and anti-inflammatory agents may be needed. While this approach may be sufficient to manage symptoms, it is disappointing that no randomised controlled trials to rigorously evaluate these agents were found, nor could the authors identify any quasi-randomised. This systematic review has identified the need for a well-designed adequately-powered randomised controlled trial to assess the efficacy and safety of pharmacological agents for the symptomatic management of cystic fibrosis-related arthritis (CFA and HPO) in adults and children with cystic fibrosis. Studies should also better define the two conditions. A study has recently been conducted in CFA and may help fill this gap when analysed and published.

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The musculoskeletal complications of cystic fibrosis

Abstract: Increasing age with deteriorating clinical and pulmonary function were associated with a higher incidence of musculoskeletal involvement. The development of symptomatic HPOA is a marker of poor prognosis.

Cited by 26 publications

References 19 publications

Influence of thoracic spine postural disorders on cardiorespiratory parameters in children and adolescents with cystic fibrosis

Influence of thoracic spine postural disorders on cardiorespiratory parameters in children and adolescents with cystic fibrosis

Patient-reported Outcomes in Cystic Fibrosis

Anti-inflammatory drugs and analgesics for managing symptoms in people with cystic fibrosis-related arthritis

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