Musculoskeletal manifestations in cystic fibrosis

Botton, Estelle; Saraux, Alain; Laselve, Hermine; Jousse, Sandrine; Goff, P. Le

doi:10.1016/s1297-319x(03)00063-0

Cited by 83 publications

(55 citation statements)

References 96 publications

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“…Entre os fatores que contribuem para essa deformidade, estão: redução da massa muscular 25 e da densidade mineral óssea 26 , trabalho respiratório excessivo 22 , osteopenia 24 e dor crônica 26 .…”

Section: Discussionunclassified

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

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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“…These changes tend to progress with the passing of the years, as per observed in our results: with an increase in age, posture adaptations intensify in order to make up for the drop in vital capacity, causing a progressive increase in head projection and neck lordosis (Table 1). Numerous studies have assessed the association between respiratory dysfunction and posture changes in other clinical situations; nonetheless, in such papers muscle-skeletal changes and posture balance changes are seen as consequence of the additional stress employed during normal ventilation 15,16,17 . The results from our study bring about an important contribution as they show changes to the respiratory function arising from posture changes.…”

Section: Discussionmentioning

confidence: 99%

Alterações posturais e função pulmonar de crianças respiradoras bucais

Silveira

Mello

Guimarães

et al. 2010

Braz. j. otorhinolaryngol. (Impr.)

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Mout h-breathing children have changes in their stomatognathic system, which result in head projection, stress increase in the scapular belt muscles and postural adaptations. Although thoracic shape and posture can influence ventilatory dynamics, we didn't find studies addressing pulmonary function of mouth-breathing children.Aims: this study aimed at analyzing the posture of mouth-breathing children, and studying the existence of correlations between posture and pulmonary volumes.Material and Methods: prospective, observational and cross-sectional study, where the posture and pulmonary function of 17 mouth-breathing children and of 17 nasal-breathing children were evaluated by means of photogrammetry and forced spirometry.Results: when compared to nasal-breathing, mouth-breathing subjects presented an increment in head projection and cervical lordosis, forwarded gravity center and reduced pulmonary volumes. There was an association between head projection and forced vital capacity, and between postural alterations and age.Conclusion: mouth-breathing children have postural alterations which increases with age and also reduced spirometry values. The vital capacity reduction correlates negatively with head projection. Braz J Otorhinolaryngol. 2010;76(6):683-6. BJORL ORIGINAL ARTICLE

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“…Laboratoriškai nustatomas teigiamas reumatoidinis faktorius. Ligai gydyti skiriami modifikuojantys ir imunomoduliaciniai vaistai [10].…”

Section: Kitos Artropatijosunclassified

Cistinės Fibrozės Artropatijos: Klinikinis Atvejis

Rauluševičiūtė

Radžiūnienė

Dumčius

2017

Medicinos Teorija Ir Praktika

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Reikšminiai žodžiai: cistinė fibrozė, sąnariai, artritas. Cistinė fibrozė yra reta, bet viena dažniausių ir letališkiausių autosominiu recesyviniu būdu paveldimų ligų tarp baltosios rasės žmonių. Sergant sutirštėjęs egzokrininių liaukų sekretas pažeidžia daugelio organų ir sistemų funkcijas. Dažniausiai tai pasireiškia kvėpavimo takų ir virškinamojo trakto pažeidimais, tačiau yra ir ekstrapulmoninių komplikacijų, su kuriomis susiduriama kur kas rečiau. Viena tokių cistinės fibrozės komplikacijų yra artropatija (sąnarių pažeidimo sindromas). Tai palyginti reta komplikacija tarp sergančių cistine fibroze ligonių, atsirandanti 15–20 gyvenimo metais. Dažniausiai pasitaiko dvi artropatijų formos: epizodinis artritas ir hipertrofinė plautinė osteoartropatija. Osteoartikuliarinės komplikacijos pirmą kartą buvo aprašytos 1979 m. Joms esant, kauliniame audinyje sumažėja mineralinių medžiagų kiekis. Artropatijoms gydyti svarbi ankstyva diagnostika, adekvatus gydymas ir prevencija. Artropatijos išsivystymas sergantiesiems cistine fibroze didina mirties riziką. Šiame straipsnyje aptariamos cistinės fibrozės metu pasireiškiančios artropatijų formos, joms būdingi simptomai ir gydymo galimybės. Taip pat pristatome cistine fibroze (genotipas F508del/F508del) sergančios paauglės, kuriai sąnarių skausmai pirmą kartą pasireiškė 15 metų, klinikinį atvejį.

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Musculoskeletal manifestations in cystic fibrosis

Cited by 83 publications

References 96 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

Alterações posturais e função pulmonar de crianças respiradoras bucais

Cistinės Fibrozės Artropatijos: Klinikinis Atvejis

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