MARISTELA TREVISAN CUNHA
Índice h a partir de 2011
5
Projetos de Pesquisa
Unidades Organizacionais
Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina
12 resultados
Resultados de Busca
Agora exibindo 1 - 10 de 12
- 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.- Impact of early enzyme-replacement therapy for mucopolysaccharidosis VI: results of a long-term follow-up of Brazilian siblings(2016) FRANCO, J. F.; SOARES, D. C.; TORRES, L. C.; LEAL, G. N.; CUNHA, M. T.; HONJO, R. S.; BERTOLA, D. R.; KIM, C. A.Mucopolysaccharidosis type VI (MPS VI) is an autosomal recessive multisystem lysosomal storage disorder, which is characterized by the deficiency of the enzyme arylsulfatase B encoded by the ARSB gene. Treatment of this disease with enzyme-replacement therapy (ERT) improves the clinical status of and generates hope for MPS VI patients. However, only few reports on patients with MPS VI treated before 5 years of age have been published. Thus, the objective of this study was to compare the clinical parameters of two sisters affected by MPS VI who started ERT at different ages (9 years and 1 year 5 months, respectively) and to determine the most relevant clinical impacts of early treatment after 85 months of evaluation. The treatment was well tolerated by both siblings. ERT in the younger sibling resulted in increased growth, an improved 6-minute walk test, less coarse face, slower progression of cardiac valve disease, and the absence of compressive myelopathy compared to that in her older sister. On the other hand, the older sibling had typical MPS VI phenotypic features before the commencement of ERT. Corneal clouding, clawed hands, and progressive skeletal changes were observed in both siblings despite the treatment. Both siblings displayed reduced frequencies of upper respiratory infections and apnea indices. This study emphasizes that early diagnosis and treatment of MPS VI are critical for a better disease outcome and to enhance the quality of life for these patients.
- 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.
bookPart Histórico da ventilação mecânica em pediatria(2016) LAHOZ, Ana Lúcia Capelari; NICOLAU, Carla Marques; CUNHA, Maristela Trevisan- Motor performance evaluation of newborns with gastroschisis after surgical correction(2021) MOTA, G. A. D. O.; SHIMIZU, G. Y.; LAHOZ, A. L. C.; NICOLAU, C. M.; PAULA, L. C. S. de; CUNHA, M. T.; TANAKA, C.Introduction: Gastroschisis is a congenital malformation that has risk factors for delayed neuropsychomotor development. That is why it is important to recognize early developmental changes in these newborns during hospitalization in the Neonatal Intensive Care Unit. Objective: To evaluate the motor performance of newborns with gastroschisis after surgical correction. Methods: An observational and retrospective study was carried out with newborns with gastroschisis, assessed by the Test of Infant Motor Performance at the Neonatal Intensive Care Center 2 of the Instituto da Criança e do Adolescente. The test allows the classification of the risk for developmental delay in 4 categories: within the average for age, low average, below average, and well below average. Maternal, neonatal, surgical, and motor assessment data were collected through electronic medical records. Results: Motor assessment was performed on 17 newborns, where 88.23% were classified as “below average” for age. The mean maternal age was 20 years, and the average gestational age and birth weight were 36.38 weeks and 2343.9 grams, respectively, with the majority being female. Simple gastroschisis accounted for 64.71%, and primary closure was possible in 82.35%. The average hospital stay of 53.24 days, and sepsis was the most frequent complication (64.71%). Conclusion: Newborns with gastroschisis remain hospitalized for a long time and are susceptible to complications. It was possible to identify the delay in the development of these newborns early during hospitalization, which allows intervention by physiotherapy before the delay worsens. © 2021 The authors. All Rights Reserved.
bookPart Técnicas e aplicações na terapia respiratória(2019) ZUANA, Adriana Della; CUNHA, Maristela Trevisan; PANEGACI, Paula Cristina Harumi Aoki- Six-minute walk test in children and adolescents with renal diseases: tolerance, reproducibility and comparison with healthy subjects(2016) WATANABE, Flavia Tieme; KOCH, Vera Herminia Kalika; JULIANI, Regina Celia Turola Passos; CUNHA, Maristela TrevisanOBJECTIVES: To evaluate exercise tolerance and the reproducibility of the six-minute walk test in Brazilian children and adolescents with chronic kidney disease and to compare their functional exercise capacities with reference values for healthy children. METHODS: This cross-sectional study assessed the use of the six-minute walk test in children and adolescents aged 6-16 with stage V chronic kidney disease. For statistical analysis of exercise tolerance, including examinations of correlations and comparisons with reference values, the longest walked distances were considered. The reproducibility of the six-minute walk test was assessed using intraclass correlation coefficients. RESULTS: A total of 38 patients (14 females and 24 males) were evaluated, including 5 on peritoneal dialysis, 12 on hemodialysis and 21 who had undergone renal transplantation, with a median age of 11.2 years (6.5-16). The median walked distance was 538.5 meters (413-685) and the six-minute walk test was found to be reproducible. The walked distance was significantly correlated with age (r=0.66), weight (r=0.76), height (r=0.82), the height Z score (r=0.41), hemoglobin (r=0.46), hematocrit (r=0.47) and post-test systolic blood pressure (r=0.39). The chronic kidney disease patients predicted walked distance was 84.1% of the reference value according to age, 90.6% according to age-corrected height and 87.4% according to a predictive equation. CONCLUSIONS: The stage V chronic kidney disease patients had a significantly decreased functional exercise capacity, as measured by the six-minute walk test, compared with the healthy pediatric reference values. In addition, the six-minute walk test was shown to be well tolerated, reliable and applicable as a low-cost tool to monitor functional exercise capacity in patients with renal disease.
- 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.
bookPart Reabilitação pulmonar nas pneumopatias crônicas(2019) CUNHA, Maristela Trevisan