A 5 year clinical evaluation of a therapeutic program for patients with cystic fibrosis

Doershuk, Carl F.; Matthews, Larry S.; Tucker, Arthur S.; Nudelman, Hermann; Eddy, George; Wise, Melvin; Spector, Samuel

doi:10.1016/s0022-3476(64)80152-9

Cited by 150 publications

(79 citation statements)

References 9 publications

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“…However, the measure of disease severity in this study was limited to only one measure of lung function, FEV1%pred., which measures the individual's expiratory volume as a percentage of the expected volume for their age and height. By contrast, Chace et al (1983) used a more comprehensive measure, scoring patients on a clinical scale of 0-100 that covered many aspects of health (Doershuk et al, 1964).…”

Section: Discussionmentioning

confidence: 99%

Desulfurization of mucin by Pseudomonas aeruginosa: influence of sulfate in the lungs of cystic fibrosis patients

Robinson

Elkins

Bialkowski

et al. 2012

Journal of Medical Microbiology

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Pseudomonas aeruginosa is a common cause of chronic respiratory infection in cystic fibrosis (CF) patients. Infection is established within the lung epithelial mucus layer through adhesion to mucins. Terminal residues on mucin oligosaccharide chains are highly sulfated and sialylated, which increases their resistance to degradation by bacterial enzymes. However, a number of microbes, including P. aeruginosa, display mucin sulfatase activity. Using ion chromatography, the levels of sulfation on different respiratory mucins and the availability of inorganic sulfate to pathogens in sputum from CF patients were quantified. The ability of clinical isolates of P. aeruginosa to desulfate mucin was tested by providing mucin as a sole sulfur source for growth. All tested P. aeruginosa strains isolated from the lungs of CF patients were able to use human respiratory mucin as a source of sulfur for growth, whereas other non-clinical species of the genus Pseudomonas were not. However, measured levels of inorganic sulfate in sputum from CF patients suggested that bacteria resident in the lung have sufficient inorganic sulfate for growth and are unlikely to require access to mucin sulfur as a sulfur source during chronic infection. This was confirmed when expression of sulfate-repressed P. aeruginosa genes atsK and msuE was found to be repressed in the sputum of CF patients, which was detected by using quantitative RT-PCR. These results indicate that sulfate starvation is unlikely to occur in pathogens residing in the sputum of CF patients and, therefore, mucin desulfation may have an alternative purpose in the association between P. aeruginosa and the airways of CF patients.

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Section: Discussionmentioning

confidence: 99%

Desulfurization of mucin by Pseudomonas aeruginosa: influence of sulfate in the lungs of cystic fibrosis patients

Robinson

Elkins

Bialkowski

et al. 2012

Journal of Medical Microbiology

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“…A gravidade da doença fibrocística também foi considerada utilizando-se o escore de Shwachman (ES) modificado por Doershuk 15 . O ES aborda itens relacionados à atividade geral, exame físico, nutrição e achados radiológicos, apresentando uma escala de 20 a 100 pontos.…”

Section: Métodounclassified

“…Os pacientes foram classificados como: graves (pontuação menor que 40), moderados (entre 40 e 55), médios (de 56 a 70), bons (entre 71 e 85) e excelentes (de 86 a 100 pontos). Para análise dos dados, a amostra foi dividida em dois grupos: o grupo de menor gravidade (pacientes classificados como bons e excelentes pelo ES) e o grupo de maior gravidade (classificados como graves, moderados e médios) 15 , sendo considerada dicotomizada para a análise dos dados.…”

Section: Métodounclassified

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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“…The Shwachman-Kulczycki system was modified by Doershuk et al (36) to enhance its detail. This system awards 25 points in each of four categories: interval symptomatic history, pulmonary physical findings and the nature of the CF patient's cough, growth pattern, and the chest roentgenogram appearance.…”

Section: Patients' Age In Yearsmentioning

confidence: 99%

“…A physician unaware of the CF patients' serum mucin-associated antigen measurements retrospectivly reviewed the charts and chest roentgenograms of the 20 patients in the CF serial sample group. For each outpatient visit when a serum specimen was obtained, the physician assigned each patient a Shwachman-Kulczycki clinical score (as modified by Doershuk, et al (36) and a Brasfield chest roentgenogram score (37). Mucin assay.…”

mentioning

confidence: 99%

Serum Mucin-Associated Antigen Levels of Cystic Fibrosis Patients Are Related to Their Ages and Clinical Statuses

et al. 1989

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A 5 year clinical evaluation of a therapeutic program for patients with cystic fibrosis

Cited by 150 publications

References 9 publications

Desulfurization of mucin by Pseudomonas aeruginosa: influence of sulfate in the lungs of cystic fibrosis patients

Desulfurization of mucin by Pseudomonas aeruginosa: influence of sulfate in the lungs of cystic fibrosis patients

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

Serum Mucin-Associated Antigen Levels of Cystic Fibrosis Patients Are Related to Their Ages and Clinical Statuses

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