Navegando por Palavras-chave "anemia, sickle cell"
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- ItemAcesso aberto (Open Access)Fisioterapia respiratória em crianças com doença falciforme e síndrome torácica aguda(Sociedade de Pediatria de São Paulo, 2011-12-01) Hostyn, Sandro Valter [UNIFESP]; Johnston, Cíntia [UNIFESP]; Braga, Josefina Aparecida Pellegrini [UNIFESP]; Carvalho, Werther Brunow de [UNIFESP]; Nogueira, Solange Cristiane; Universidade Federal de São Paulo (UNIFESP); Universidade de São Paulo (USP)OBJECTIVE: To systematically review the medical literature to identify chest physiotherapy techniques applied to children with sickle cell disease and acute chest syndrome, and to report their level of evidence and recommendation. DATA SOURCE: A bibliographic search of published articles found in Medline, Lilacs, SciELO and Cochrane databases, between 1995 and 2009, was carried out using the following keywords: sickle cell disease, acute chest syndrome, physical therapy, child, incentive spirometry, in English and Portuguese; all review studies were excluded. The recovered studies were then classified according to their level of evidence and recommendation. DATA SYNTHESIS: Five papers were retrieved. Among them, three used incentive spirometry that played an important role in the prevention of pulmonary complications associated with acute chest syndrome (evidence levels II, III and IV); one of these studies (evidence II) compared incentive spirometry versus positive expiratory pressure and did not find differences between them. One paper reported a clinical bundle to improve the quality of care, including incentive spirometry (evidence level V). Incentive spirometry was associated with shorter length of stay and less requirement of oral pain medications. Another study evaluated the effect of non-invasive ventilation on respiratory distress in children that could not perform incentive spirometry and reported improvement in the oxygenation and in the respiratory distress (evidence level V). CONCLUSIONS: Physiotherapy techniques with incentive spirometry device, positive expiratory pressure and non-invasive ventilation can be performed in children with sickle cell disease and acute chest syndrome, with a C recommendation level.
- ItemAcesso aberto (Open Access)Pressão arterial em crianças portadoras de doença falciforme(Sociedade de Pediatria de São Paulo, 2012-01-01) Hsien, Ho Chi [UNIFESP]; Carvalhaes, João Tomás de Abreu [UNIFESP]; Braga, Josefina Aparecida Pellegrini [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)OBJECTIVE: To evaluate blood pressure (BP) in children with sickle cell disease (SCD). METHODS: Observational descriptive study of BP in 70 children with SCD. BP values were classified according to the V Brazilian Guidelines in Arterial Hypertension. Patients were divided into groups according to genotype (HbSS, HbSC) and according to age: group I, three to four years and 11 months; group II, five to eight years and 11 months; and group III, nine to 13 years and 11 months. The Student's t test and ANOVA were used for statistical analyses, and the level of significance was set at p<0.05. RESULTS: Mean and standard deviation (SD) of systolic BP (SBP) (mmHg) were 95.9±11.45, and of diastolic BP (DBP), 62.6±7.78. Means according to age group were: group I - SBP 91.2±5.78 and DBP 61.5±7.15; group II - SBP 97.3±10.86 and DBP 64.4±7.89; and group III - SBP 100.0±9.88 and DBP 61.5±4.94. Results showed that 5.7% of the patients had hypertension and 8.6%, pre-hypertension. Mean SBP and DBP of HbSC and HbSS patients did not differ. CONCLUSIONS: Further studies should be conducted to assess BP in patients with SCD and determine possible causes of hypertension in these patients.
- ItemSomente MetadadadosPriapism is Associated with Sleep Hypoxemia in Sickle Cell Disease(Elsevier B.V., 2012-10-01) Roizenblatt, Marina [UNIFESP]; Figueiredo, Maria Stella [UNIFESP]; Cancado, Rodolfo Delfini; Pollack-Filho, Frederico [UNIFESP]; Almeida Santos Arruda, Martha Mariana de [UNIFESP]; Vicari, Perla [UNIFESP]; Sato, Joao Ricardo; Tufik, Sergio [UNIFESP]; Roizenblatt, Suely [UNIFESP]; Universidade Federal de São Paulo (UNIFESP); Universidade Federal do ABC (UFABC)Purpose: We assessed penile rigidity during sleep and the relationship of sleep abnormalities with priapism in adults with sickle cell disease.Materials and Methods: This was a case-control study of 18 patients with sickle cell disease and a history of priapism during the previous year, and 16 controls with sickle cell disease. Participants underwent overnight polysomnography and RigiScan (R) Plus recording to detect penile rigidity oscillations.Results: the priapism group (cases) showed a higher apnea-hypopnea index and oxyhemoglobin desaturation parameters than controls. A lower positive correlation between the apnea-hypopnea index and oxyhemoglobin desaturation time was observed in cases than in controls (Spearman coefficient rho = 0.49, p = 0.05 vs rho = 0.76, p < 0.01), suggesting that desaturation events occurred independently of apnea. Two controls and 14 cases had a total sleep time that was greater than 10% with oxyhemoglobin saturation less than 90% but without CO2 retention. Penile rigidity events were observed during rapid eye movement sleep and during stage 2 of nonrapid eye movement sleep, particularly in cases. the duration of penile rigidity events concomitant to respiratory events was higher in cases than in controls. Regression analysis revealed that the periodic limb movement and desaturation indexes were associated with priapism after adjusting for rapid eye movement sleep and lung involvement. Finally, oxyhemoglobin saturation less than 90% was associated with priapism after adjusting for lung involvement, hyperhemolysis and the apnea-hypopnea index.Conclusions: Oxyhemoglobin desaturation during sleep was associated with priapism history. It may underlie the distribution pattern of penile rigidity events during sleep in these patients.
- ItemAcesso aberto (Open Access)Qualidade de vida em portadores de doença falciforme(Sociedade de Pediatria de São Paulo, 2013-03-01) Menezes, Adeline Soraya de Oliveira da Paz [UNIFESP]; Len, Claudio Arnaldo [UNIFESP]; Hilário, Maria Odete Esteves [UNIFESP]; Terreri, Maria Teresa Ramos Ascensão [UNIFESP]; Braga, Josefina Aparecida Pellegrini [UNIFESP]; Universidade Federal de São Paulo (UNIFESP)OBJECTIVE: To evaluate the quality of life in children and adolescents with sickle cell disease attending a blood reference center, and to assess the quality of life of their relatives. METHODS: Cross-sectional study that included 100 patients with sickle cell disease, which were divided into three subgroups according to age: 5 to 7 (n=18), 8 to 12 (n=32), and 13 to 18 years-old (n=50), and their parents. The Control Group included 50 healthy children and adolescents from a public local school, also divided into the same three age subgroups and their caregivers. The Pediatric Quality of life Inventory (PedsQL), version 4.0, was applied in both groups. The generic questionnaire Medical Outcomes Study 36 - Item Short-Form Health Survey (SF-36) was applied to the relatives. The answers were linearly transformed into a score and compared by non-parametric tests. RESULTS: The PedsQL scores of patients were significantly lower than those obtained in the Control Group (p<0.0001) in all studied areas (physical, emotional, social skills, and school activities). Similarly, SF-36 scores applied to the patients' parents were lower than those obtained in the Control Group in all studied aspects (p<0.0001). CONCLUSIONS: Sickle cell disease affects the quality of life of children, adolescents, and their families. Patients sense restrictions in the emotional, social, family and physical aspects, among others.