Improvements of lung function in cystic fibrosis

Tauber, Erich; Eichler, Irmgard; Gärtner, Ch.; Halmerbauer, G.; Götz, M.; Rath, R; Wojnarowski, Claudia; Frischer, Thomas

doi:10.1002/ppul.10076

Cited by 12 publications

(10 citation statements)

References 16 publications

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“…23 Recent research has shown that early diagnosis reduces morbidity 24 and highlights the importance of starting treatment for pancreatic insufficiency and malnutrition early and also respiratory physiotherapy to maintain airways unobstructed. 25 One study demonstrated that infection by P. aeruginosa was the main risk factor for morbidity and mortality, pointing out that early diagnosis would aid intervention at the onset of colonization by this agent and contribute to better prognosis.…”

Section: Discussionmentioning

confidence: 99%

Fibrose cística em um centro de referência no Brasil: características clínicas e laboratoriais de 104 pacientes e sua associação com o genótipo e a gravidade da doença

Alvarez¹,

Ribeiro²,

Hessel³

et al. 2004

J. Pediatr. (Rio J.)

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Objective: To identify the clinical, laboratory and radiographic characteristics of the cystic fibrosis patients under care at Universidade Estadual de Campinas (UNICAMP) in the last decade of the twentieth century, and to investigate the association of these characteristics with genotype and severity of the disease as measured by the Shwachman score.Methods: Descriptive, retrospective and cross-sectional study of the patients assisted at UNICAMP hospitals Cystic Fibrosis Clinic from July 1990 to July 2000. Results:One hundred and four patients were studied; 53.8% male; 93.3% Caucasian; 89.4% presented with respiratory symptoms; 59.6% presented with digestive symptoms; 5.8% had meconium ileus; 4.8% had diabetes. The mean age at onset of symptoms was 3 months, and the mean age at diagnosis was 2 years and 4 months. At diagnosis, 69.9 and 56.6% of the patients had weight and height below 10 th percentile, respectively; in 10.6%, sweat chloride was < 60 mEq/l. Staphylococcus aureus was found in 80.2%, Pseudomonas aeruginosa in 76.0%, and Burkholderia cepacia in 5.2%. ∆F508 homozygosis was observed in 18.75%, whereas 62.50% of the patients were ∆F508 heterozygous. A moderate/severe Shwachman score was found in 15.7%. Eighteen patients died in that period (17.3%). The mean age at death was 7 years and 8 months; median survival after diagnosis was 18 years and 4 months. Patients who have at least one ∆F508 mutation have more frequent alterations in fecal fat levels when compared to patients who do not have this mutation (p < 0.05). There were no differences in any parameter between ∆F508 homozygous and heterozygous patients. Conclusions:The clinical and laboratory characteristics of the 104 patients studied were similar to the characteristics described for patients in other countries. Exceptions are the higher age at diagnosis and lower survival. Our results support the recommendation for early diagnosis and the need for more treatment opportunities in the population of cystic fibrosis patients.J Pediatr (Rio J). 2004;80(5):371-9: Cystic fibrosis, ∆F508, genotype/phenotype relation, Pseudomonas aeruginosa, Burkholderia cepacia, Shwachman score, pediatric lung disease.

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Section: Discussionmentioning

confidence: 99%

Fibrose cística em um centro de referência no Brasil: características clínicas e laboratoriais de 104 pacientes e sua associação com o genótipo e a gravidade da doença

Alvarez¹,

Ribeiro²,

Hessel³

et al. 2004

J. Pediatr. (Rio J.)

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“…Foi demonstrado que o volume expiratório forçado no primeiro segundo (VEF 1 ) reflete com precisão a progressão da doença pulmonar em CF e é preditor de mortalidade 8,9 . Os motivos para as melhoras observadas na função pulmonar da população não podem ser determinados com base neste estudo, mas é provável que sejam multifatoriais e podem estar relacionadas a uma série e mudanças no tratamento de pacientes com FC por uma equipe multidisciplinar 7,18,19 , conforme descrito na introdução. Embora tenha havido um número muito semelhante de pacientes recebendo terapia supressiva crônica para P. aeruginosa, o aumento do uso de azitromicina oral para pacientes colonizados por P. aeruginosa pode ter contribuído para a melhora de função pulmonar, conforme relatado anteriormente 20 .…”

Section: Introductionunclassified

Improvements in lung function of a pediatric cystic fibrosis population in a developing country

Morrow¹,

Argent²,

Zar³

et al. 2008

J Pediatr (Rio J)

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“…The majority (82%) of children aged 6-18 yrs attending the CF centre in Gothenburg, Sweden, have FEV1 results that are within the normal range. A recent report from the University Children9s Hospital in Vienna, Austria, describes how the predicted FEV1 of a 6-yr-old child attending their CF clinic has risen from 75 to 108% predicted over the last decade [5]. There is clearly a need for alternative, more sensitive measures of lung function that can be used to monitor paediatric CF subjects.…”

mentioning

confidence: 99%

Evaluation of ventilation maldistribution as an early indicator of lung disease in children with cystic fibrosis

Gustafsson¹,

Aurora²,

Lindblad³

2003

Eur Respir J

298

295

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Many children with cystic fibrosis (CF), receiving modern, aggressive CF care, have normal spirometry results. This study aimed to see if homogeneity of ventilation distribution is impaired early in the course of CF lung disease, and if ventilation inhomogeneity is a more frequent finding than abnormal spirometry in children benefiting from modern CF care.The study compared spirometry findings to two indices of ventilation inhomogeneity (mixing ratio (MR) and lung clearance index (LCI)) from multiple-breath inert gas washout in 43 children with CF, aged 3–18 yrs, and 28 healthy children.In total, 10/43 CF subjects (23%) had reduced forced expiratory volume in one second (FEV1) and 14/34 (41%) showed abnormal maximum expiratory flow at 25% of forced vital capacity (MEF25). In contrast, MR was abnormal in 31/43 (72%) and LCI in 27/43 (63%). MR was abnormal in 22/33 CF subjects with normal FEV1,versus0/28 controls (p<0.001), and abnormal MR was found in 10/20 CF subjects with normal MEF25,versus0/22 controls (p<0.001). Nine of the 10 CF subjects with reduced FEV1and 12/14 with abnormal MEF25showed abnormal MR.Inert gas washout discloses airway dysfunction in the majority of children with cystic fibrosis with normal lung function judged by spirometry. These findings suggest that multiple-breath inert gas washout is of greater value than spirometry in detecting early cystic fibrosis lung disease.

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Improvements of lung function in cystic fibrosis

Cited by 12 publications

References 16 publications

Fibrose cística em um centro de referência no Brasil: características clínicas e laboratoriais de 104 pacientes e sua associação com o genótipo e a gravidade da doença

Fibrose cística em um centro de referência no Brasil: características clínicas e laboratoriais de 104 pacientes e sua associação com o genótipo e a gravidade da doença

Improvements in lung function of a pediatric cystic fibrosis population in a developing country

Evaluation of ventilation maldistribution as an early indicator of lung disease in children with cystic fibrosis

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