JOAQUIM CARLOS RODRIGUES
Projetos de Pesquisa
Unidades Organizacionais
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina - Médico
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina - Líder
LIM/36 - Laboratório de Pediatria Clínica, Hospital das Clínicas, Faculdade de Medicina - Líder
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- Subclinical pulmonary abnormalities in childhood-onset systemic lupus erythematosus patients(2016) VEIGA, C. S.; COUTINHO, D. S.; NAKAIE, C. M. A.; CAMPOS, L. M. A.; SUZUKI, L.; CUNHA, M. T.; LEONE, C.; SILVA, C. A.; RODRIGUES, J. C.Objective The aims of this study were to analyze the pulmonary function of childhood-onset systemic lupus erythematosus (cSLE) patients and to identify possible correlations between the high-resolution computed chest tomography (HRCT) score, disease activity, disease cumulative damage, and the participants' quality of life. Methods Forty cSLE patients, median age: 14.1 years (range: 7.4-17.9), underwent spirometry and plethysmography. Carbon monoxide diffusing capacity (DLCO), HRCT, disease activity, disease cumulative damage, and quality of life were assessed. Results Pulmonary abnormalities were evident in 19/40 (47.5%) cSLE patients according to spirometry/DLCO. Forced expired volume in one second (FEV1%) was the parameter most affected (30%). The HRCT showed some abnormality in 22/30 patients (73%), which were minimal in 43%. Signs of airway affects were found in 50%. Twelve patients were hospitalized due to cSLE-related pulmonary complications before the study began (median discharge: 2.1 years earlier). Total lung capacity (TLC%), vital capacity (VC%), forced vital capacity (FVC%), and FEV1% were significantly lower in the group with hospitalization compared to the group without hospitalization (p=0.0025, p=0.0022, p=0.0032, and p=0.0004, respectively). Of note, DLCO was positively correlated with disease duration (r=+0.4; p=0.01). The HRCT-score was negatively correlated with FEV1/VC (r=-0.63; p=0.0002), FEV1 (r=-0.54; p=0.018), FEF25%-75% (r=-0.67; p<0.0001), and HRCT-score was positively correlated with resistance (r=+0.49; p=0.0056). Conclusions Almost half of patients with cSLE had subclinical pulmonary abnormalities, especially airway abnormalities. The cSLE-related pulmonary complications seem to determine long-term functional damage.
conferenceObject QUALITY OF LIFE AND LUNG FUNCTION IN CF PATIENTS USING TOBRAMYCIN INHALATION POWDER (TIP)(2016) CUNHA, Maristela Trevisan; JULIANI, Regina C. T. P.; LEONE, Claudio; RODRIGUES, Joaquim C.; ADDE, Fabiola V.- Six minute walk test Z score: Correlations with cystic fibrosis severity markers(2012) STOLLAR, Fabiola; RODRIGUES, Joaquim C.; CUNHA, Maristela T.; LEONE, Claudio; ADDE, Fabiola VillacBackground: The six-minute-walk-test (6MWT) has been increasingly used in cystic fibrosis (CF) patients. However, few studies in children have correlated 6MWT with current parameters used to evaluate CF severity. Moreover, no study transformed the values of distance walked from meters into Z scores to avoid bias like age and gender, which are sources of 6MWT variability. Methods: A cross-sectional descriptive study was performed to analyze the correlations (Spearman) among forced expiratory volume in one second (FEV1), body mass index (BMI), chest radiography (CXR), chest tomography (CT), and 6MWT Z score (Z-6MWT). Clinically stable CF patients, aged 6-21 years, were included. Results: 34 patients, 14F/20M, mean age 12.1 +/- 4.0 years were studied. The mean Z-6MWT was -1.1 +/- 1.106. The following correlations versus Z-6MWT were found: FEV1 (r=0.59, r(2)=0.32, p=0.0002), BMI Z score (r=0.42, r(2)=0.17, p=0.013), CXR (r=0.34, r(2)=0.15, p=0.0472) and CT (r=-0.45, r(2)=0.23, p=0.0073). Conclusions: In conclusion there was a significant, but poor, correlation between the six minute walk test Z score and the cystic fibrosis severity markers currently in use.
- Shwachman-Kulczycki score still useful to monitor cystic fibrosis severity(2011) STOLLAR, Fabiola; ADDE, Fabiola Villac; CUNHA, Maristela T.; LEONE, Claudio; RODRIGUES, Joaquim C.INTRODUCTION: The Shwachman-Kulczycki score was the first scoring system used in cystic fibrosis to assess disease severity. Despite its subjectivity, it is still widely used. OBJECTIVE: To study correlations among forced expiratory volume in one second (FEV(1)), chest radiography, chest computed tomography, 6-minute walk test, and Shwachman-Kulczycki score in patients with cystic fibrosis and to test whether the Shwachman-Kulczycki score is still useful in monitoring the severity of the disease. METHODS: A cross-sectional prospective study was performed to analyze the correlations (Spearman). Patients with clinically stable cystic fibrosis, aged 3-21 years, were included. RESULTS: 43 patients, 19F/24M, mean age 10.5 +/- 4.7 years, with a median Shwachman-Kulczycki score of 70 were studied. The median Brasfield and Bhalla scores were 17 and 10, respectively. The mean Z score for the 6-minute walk test was -1.1 +/- 1.106 and the mean FEV(1) was 59 +/- 26 (as percentage of predicted values). The following significant correlations versus the Shwachman-Kulczycki score were found: FEV(1) (r = 0.76), 6-minute walk test (r = 0.71), chest radiography (r = 0.71) and chest computed tomography (r = -0.78). When patients were divided according to FEV(1), a statistically significantly correlation with the Shwachman-Kulczycki score was found only in patients with FEV(1) <70% (r = 0.67). CONCLUSIONS: The Shwachman-Kulczycki score remains an useful tool for monitoring the severity of cystic fibrosis, adequately reflecting the functional impairment and chest radiography and tomography changes, especially in patients with greater impairment of lung function. When assessing patients with mild lung disease its limitations should be considered and its usefulness in such patients should be evaluated in larger populations.
- Quality of life amongst adolescents and young adults with cystic fibrosis: correlations with clinical outcomes(2018) GANCZ, Daniela W.; CUNHA, Maristela T.; LEONE, Claudio; RODRIGUES, Joaquim C.; V, Fabiola AddeOBJECTIVES: The current study sought to evaluate the quality of life of young patients with cystic fibrosis and correlate these results with the clinical parameters indicative of disease severity. METHODS: This cross-sectional study applied the validated Portuguese version of a cystic fibrosis specific quality of life questionnaire to clinically stable patients aged 14 to 21 years old. The correlations between the questionnaire domain scores and forced expiratory volume in one second (FEV1) values, the Shwachman-Kulczycki score, and body mass index were assessed, and correlations were considered as significant when p<0.05. RESULTS: A total of 31 patients (11 females; 16.4 +/- 2.3 years old) were evaluated, and the median scores on the questionnaire domains ranged from 66.7 to 100. A significant correlation was found between body mass index and the weight (r=0.43, p=0.016) and the eating questionnaire domains (r=0.44, p=0.013); between FEV1 and the physical (r=0.53, p=0.002) and treatment burden (r=0.41, p=0.023) domains; and between the Shwachman-Kulczycki score and the physical (r=0.39, p=0.03), health (r=0.41, p=0.023), and role (r=0.37, p=0.041) domains. A significant difference was found amongst patients with FEV1 values above or below 60% of the predicted value with regard to the role and health domains. No differences in the scores were found according to gender. CONCLUSIONS: The current cystic fibrosis specific quality of life questionnaire scores exhibited wide variability across all domains; however, they indicated a relatively satisfactory quality of life amongst the patients studied. Certain domains exhibited significant correlations with clinical parameters; thus, this instrument has consistent associations with clinical outcomes.