Objective: To evaluate pulmonary function and functional capacity in children and adolescents with sickle cell disease. Methods: This was a cross-sectional study involving 70 children and adolescents (8-15 years of age) with sickle cell disease who underwent pulmonary function tests (spirometry) and functional capacity testing (six-minute walk test). The results of the pulmonary function tests were compared with variables related to the severity of sickle cell disease and history of asthma and of acute chest syndrome. Results: Of the 64 patients who underwent spirometry, 15 (23.4%) showed abnormal results: restrictive lung disease, in 8 (12.5%); and obstructive lung disease, in 7 (10.9%). Of the 69 patients who underwent the six-minute walk test, 18 (26.1%) showed abnormal results regarding the six-minute walk distance as a percentage of the predicted value for age, and there was a ≥ 3% decrease in SpO2 in 36 patients (52.2%). Abnormal pulmonary function was not significantly associated with any of the other variables studied, except for hypoxemia and restrictive lung disease. Conclusions: In this sample of children and adolescents with sickle cell disease, there was a significant prevalence of abnormal pulmonary function. The high prevalence of respiratory disorders suggests the need for a closer look at the lung function of this population, in childhood and thereafter.

Keywords: Anemia, sickle cell; Respiratory function tests; Exercise test.

Objective: To evaluate quality of sleep and pulmonary function in clinically stable adolescents with sickle cell anemia (SCA). Methods: A cross-sectional descriptive study involving 50 patients with SCA submitted to nocturnal polysomnography and spirometry at the Brasília University Hospital. Anthropometric, polysomnographic and pulmonary function data were analyzed. Patients were divided into two groups according to oxygen saturation by pulse oximetry (SpO2) during rapid eye movement (REM) sleep: SpO2 ≤ 93%; and SpO2 > 93%. Descriptive statistics, Student's t-test, chi-square test and Pearson's correlation coefficient were used. Results: Mean age was 13.9 ± 2.5 years. Total sleep time and REM sleep percentage were lower, whereas REM sleep latency, the number of awakenings, movement during sleep, changes in sleep stage, sleep-disordered breathing index and obstructive apnea index were higher. Two patients (4%) did not present REM. There were statistically significant differences between the groups in most of the polysomnographic variables. The SpO2 in REM sleep presented a strong positive correlation with waking SpO2 and with SpO2 in non-REM sleep, whereas it presented a strong negative correlation with the percentage of total sleep time during which SPO2 was < 90%. Mean spirometric values were within normal ranges. Residual volume and the residual volume/total lung capacity/functional residual capacity ratio were elevated. Conclusion: Sleep impairment in clinically stable patients with SCA is probably due to hemoglobin desaturation and not to individual alterations in pulmonary function.

Keywords: Sleep disorders; Polysomnography; Adolescent; Anemia, sickle cell; Spirometry.

Objective: To estimate the prevalence of obstructive sleep apnea syndrome (OSAS) in children and adolescents with sickle cell anemia (SCA); to investigate the possible correlation between mean annual hemoglobin level and total sleep time with SpO2 < 90%, as well as between mean annual hemoglobin level and total sleep time with SpO2 < 80%; and to investigate the possible correlation between apnea-hypopnea index (AHI) and painful crisis. Methods: The study involved 85 patients with SCA. The patients completed a questionnaire, were submitted to polysomnography and underwent clinical evaluation (by a pediatrician and an otolaryngologist). An AHI > 1 was considered indicative of a diagnosis of OSAS. Results: The prevalence of OSAS was 10.6%. We found a negative correlation between mean annual hemoglobin level and total sleep time with SpO2 < 90% (r = −0.343; p = 0.002), as well as between mean annual hemoglobin level and total sleep time with SpO2 < 80% (r = −0.270; p = 0.016). There was no association between AHI and painful crisis. Conclusions: The prevalence of OSAS in this population was high (10.6%). Therefore, it is important to identify signs of OSAS as soon as possible and to determine the mean annual hemoglobin level because of the inverse correlation between that level and the total sleep time with SpO2 < 90% or < 80%.

Keywords: Prevalence; Sleep apnea, obstructive; Anemia, sickle cell; Polysomnography; Sleep apnea syndromes.

Objective: To describe the pulmonary function in children and adolescents with postinfectious bronchiolitis oblite­rans (PIBO), as well as to evaluate potential risk factors for severe impairment of pulmonary function. Methods: The pulmonary function of 77 participants, aged 8-18 years, was assessed by spirometry and plethysmography. The following parameters were analyzed: FVC; FEV1; FEF25-75%; FEV1/FVC; RV; TLC; RV/TLC; intrathoracic gas volume; and specific airway resistance (sRaw). We used Poisson regression to investigate the following potential risk factors for severe impairment of pulmonary function: gender; age at first wheeze; age at diagnosis; family history of asthma; tobacco smoke exposure; length of hospital stay; and duration of mechanical ventilation. Results: The mean age was 13.5 years. There were pronounced decreases in FEV1 and FEF25-75%, as well as increases in RV and sRaw. These alterations are characteristic of obstructive airway disease. For the parameters that were the most affected, the mean values (percentage of predicted) were as follows: FEV1 = 45.9%; FEF25-75% = 21.5%; RV = 281.1%; RV/TLC = 236.2%; and sRaw = 665.3%. None of the potential risk factors studied showed a significant association with severely impaired pulmonary function. Conclusions: The patients with PIBO had a common pattern of severe pulmonary function impairment, characterized by marked airway obstruction and pronounced increases in RV and sRaw. The combination of spirometric and plethysmographic measurements can be more useful for assessing functional damage, as well as in the follow-up of these patients, than are either of these techniques used in isolation. Known risk factors for respiratory diseases do not seem to be associated with severely impaired pulmonary function in PIBO.

Keywords: Respiratory function tests; Airway obstruction; Bronchiolitis obliterans

Objective: Asthma is the most common chronic pulmonary disease, characterized by bronchial inflammation. Some children with asthma have persistent pulmonary function impairment. The prevalence and etiology of this abnormality in children with asthma in developing countries remain unknown. The objective of this study was to estimate the proportion of patients with impaired pulmonary function who were unresponsive to treatment in a group of children and adolescents with asthma, and to describe the phenotypic characteristics of the sample. Methods: Using a standardized questionnaire, we selected outpatients (5-17 years of age) diagnosed with persistent asthma. These patients underwent spirometry and skin prick tests for sensitivity to common aeroallergens. Persistent pulmonary function impairment was defined as an FEV1/FVC ratio < 0.80, even after 10 days of treatment with bronchodilators and oral corticosteroids. We used the atopic index to differentiate between patients with little or no response to the skin prick test and those with a strong response (cut-off point: 4 allergens). Results: We included 96 patients with a mean age of 10.6 years. Of those, 52 (54.1%) were male, and 89 (92.7%) were atopic. Of the 96 patients, 8 (8.3%) had impaired pulmonary function even after the treatment. Among those patients, 8 (100%) were atopic, 7 (87.5%) had moderate or severe asthma, and 7 (87.5%) had a history of hospitalization for acute bronchiolitis. Conclusions: Children and adolescents with moderate or severe asthma can present with impaired pulmonary function and be unresponsive to treatment. This clinical situation has been little studied in developing countries, and its risk factors and etiology will be better understood only through birth cohort studies.

Keywords: Asthma; Respiratory function tests; Allergy and immunology.

The objective of this study was to review the literature on bronchoalveolar lavage fluid cell profiles in healthy children and adolescents, as well as on the use of BAL as a diagnostic and follow-up tool for lung disease patients in this age bracket. To that end, we used the Medline database, compiling studies published between 1989 and 2009 employing the following MeSH descriptors (with Boolean operators) as search terms: bronchoalveolar lavage AND cytology OR cell AND child. In healthy children, the cell profile includes alveolar macrophages (> 80%), lymphocytes (approximately 10%), neutrophils (approximately 2%) and eosinophils (< 1%). The profile varies depending on the disease under study. The number of neutrophils is greater in wheezing children, especially in non-atopic children, as well as in those with pulmonary infectious and inflammatory profiles, including cystic fibrosis and interstitial lung disease. Eosinophil counts are elevated in children/adolescents with asthma and can reach high levels in those with allergic bronchopulmonary aspergillosis or eosinophilic syndromes. In a heterogenous group of diseases, the number of lymphocytes can increase. Evaluation of the BAL fluid cell profile, when used in conjunction with clinical and imaging findings, has proven to be an essential tool in the investigation of various lung diseases. Less invasive than transbronchial and open lung biopsies, BAL has great clinical value. Further studies adopting standard international protocols should be carried out. Such studies should involve various age groups and settings in order to obtain reference values for BAL fluid cell profiles, which are necessary for a more accurate interpretation of findings in children and adolescents with lung diseases.

Keywords: Bronchoalveolar lavage; Bronchoalveolar lavage fluid/cytology; Child; Adolescent.

Objective: To evaluate the association between nutritional status measurements and pulmonary function in children and adolescents with cystic fibrosis. Methods: We evaluated the nutritional status of 48 children and adolescents (aged 6-18 years) with cystic fibrosis based on body mass index (BMI) and body composition measurements-mid-arm muscle circumference (MAMC) and triceps skinfold thickness (TST)-at a referral center in the city of Rio de Janeiro, Brazil. Pulmonary function was assessed by means of spirometry, using FEV1 to classify the severity of airway obstruction. We used Student's t-tests for comparisons between proportions and linear regression analysis for associations between continuous variables. The level of significance was set at p < 0.05. Results: The evaluation of nutritional status based on BMI identified a smaller number of malnourished patients than did that based on MAMC (14 vs. 25 patients, respectively). Most of the patients presented mild pulmonary disease. Mean FEV1 was 82.5% of predicted. Pulmonary function was found to correlate significantly with BMI, MAMC and TST (p = 0.001, p = 0.001 and p = 0.03, respectively). All subjects with moderate or severe pulmonary involvement were considered malnourished based on BMI and body composition parameters. Of the 25 patients considered malnourished based on body composition (MAMC), 19 were considered well-nourished based on their BMI. Conclusions: In the present study, all nutritional status measurements correlated directly with the pulmonary function of children and adolescents with cystic fibrosis. However, body composition measurements allowed earlier detection of nutritional deficiencies.

Keywords: Cystic fibrosis; Body composition; Spirometry; Nutrition assessment.

Objectives: To perform a review of the mechanisms involved in the origin of malnutrition in patients with chronic obstructive pulmonary disease (COPD) and a systematic review of randomized controlled studies to clarify the contribution of nutritional supplementation in patients with stable COPD. Methods: Systematic review of article published in this subject, in all languages from several sources, including Medline, Embase, Cinahl and Cochrane Registry in COPD, as well as studies presented in American and European. Results: Studies with nutritional supplementation longer than two weeks showed that the effect was not big and did not reach statistical significance. A study with linear regression showed that old age, relative anorexia, and high inflammatory response are associated with non-response to nutritional therapy. Conclusion: Currently, there is no evidence of the efficacy of nutritional supplementation in patients with COPD. Factors related to lack of response suggest a relationship with the degree of inflammation, including high levels of TNF alpha. Measuring inflammation markers may be useful to determine prognostic and adequate therapy. Treatment with anti-inflammatory cytokines or cytokine inhibitors are promising for the future.

Objective: To evaluate the level of self-reported adherence to physical therapy recommendations in pediatric patients (6-17 years) with cystic fibrosis (CF) and to ascertain whether the different levels of adherence correlate with pulmonary function, clinical aspects, and quality of life. Methods: This was a cross-sectional study. The patients and their legal guardians completed a questionnaire regarding adherence to physical therapy recommendations and a CF quality of life questionnaire. We collected demographic, spirometric, and bacteriological data, as well as recording the frequency of hospitalizations and Shwachman-Kulczycki (S-K) clinical scores. Results: We included 66 patients in the study. Mean age, FEV1 (% of predicted), and BMI were 12.2 ± 3.2 years, 90 ± 24%, and 18.3 ± 2.5 kg/m2, respectively. The patients were divided into two groups: high-adherence (n = 39) and moderate/poor-adherence (n = 27). No statistically significant differences were found between the groups regarding age, gender, family income, and total S-K clinical scores. There were statistically significant differences between the high-adherence group and the moderate/poor-adherence group, the latter showing lower scores for the "radiological findings" domain of the S-K clinical score (p = 0.030), a greater number of hospitalizations (p = 0.004), and more days of hospitalization in the last year (p = 0.012), as well as lower scores for the quality of life questionnaire domains emotion (p = 0.002), physical (p = 0.019), treatment burden (p < 0.001), health perceptions (p = 0.036), social (p = 0.039), and respiratory (p = 0.048). Conclusions: Low self-reported adherence to physical therapy recommendations was associated with worse radiological findings, a greater number of hospitalizations, and decreased quality of life in pediatric CF patients.

Keywords: Cystic fibrosis; Patient compliance; Physical therapy modalities; Quality of life.

Background: Spirometry is widely used in the diagnosis and quantification of respiratory disorders such as asthma. A microspirometer is a portable and easily used apparatus that can substitute for a regular spirometer, although there is little evidence of its accuracy. Objective: To compare the microspirometer to a regular spirometer for use in asthmatic children and adolescents. Methods: The instruments used were a Micro Spirometer, manufactured by Micro Medical, and a conventional Cosmed Pony Graphic 3.5 spirometer, both with turbinometers (flow sensors). The study sample consisted of 62 children and adolescents, of both genders, clinically diagnosed with asthma and under treatment at a pulmonology clinic. Ages ranged from 5 and 16 years. All spirometric tests were carried out according to the guidelines established by the American Thoracic Society and by the Associacão Brasileira de Normas Técnicas (Brazilian Technical Standards Association). For each patient, microspirometry was performed first, followed by conventional spirometry. The parameters analyzed in both devices were forced vital capacity, forced expiratory volume in one second and peak expiratory flow. Data were analyzed using the Student's t-test and Pearson's correlation test. Results: Strong correlations were found between the two devices in the parameters analyzed: forced expiratory volume in one second: r = 0.97; forced vital capacity: r = 0.97; and peak expiratory flow: r = 0.91. Conclusion: These results demonstrate that the microspirometer is a useful diagnostic tool that can be used when a conventional spirometer is unavailable.

Keywords: Key words: Asthma. Spirometry. Micro spirometer. Puomonary ventilation.

Objective: Respiratory muscle weakness is a functional repercussion of chronic lung disease (CLD). The objective of this study was to assess the effects of home-based respiratory muscle training (RMT) in children and adolescents with CLD or neuromuscular disease (NMD). Methods: This was a quasi-experimental study involving children and adolescents with CLD or NMD. Before and after 6 months of home-based RMT, we measured respiratory muscle strength (MIP and MEP), PEF, and peak cough flow (PCF). We made statistical comparisons between the pre-RMT and post-RMT values, as well as evaluating the correlation between the duration and effect of RMT. Results: The study included 29 patients, with a mean age of 12 years (range, 5-17 years), of whom 18 (62.1%) were male. The CLD group comprised 11 patients (37.9%), and the NMD group comprised 18 (62.1%). The mean duration of the RMT was 60 weeks (range, 46-90 weeks) in the CLD group and 39 weeks (range, 24-89 weeks) in the NMD group. In comparison with the pre-RMT values, the post-RMT values for MIP and MEP were significantly higher in both groups, whereas those for PEF and PCF were significantly higher only in the NMD group. We found no correlation between the duration and the effect of RMT. Conclusions: Home-based RMT appears to be an effective strategy for increasing respiratory muscle strength in children and adolescents with CLD or NMD, although it increased the ability to cough effectively only in those with NMD.

Keywords: Breathing exercises; Cystic fibrosis; Bronchiolitis obliterans; Neuromuscular diseases; Cough.

Objective: To assess respiratory system impedance (Zrs) and spirometric parameters in children and adolescents with and without a history of preterm birth. Methods: We evaluated a sample of 51 subjects between 11 and 14 years of age: 35 who had a history of preterm birth (preterm group) and 16 who had been born at term (full-term group). Lung function was measured by spirometry, spectral oscillometry, and intra-breath oscillometry. Results: Neither spirometry nor spectral oscillometry revealed any statistically significant differences between the preterm and full-term groups. However, intra-breath oscillometry demonstrated significant differences between the two groups in terms of the change in resistance, reactance at end-inspiration, and the change in reactance (p < 0.05 for all). Conclusions: Our findings suggest that abnormalities in Zrs persist in children and adolescents with a history of preterm birth and that intra-breath oscillometry is more sensitive than is spectral oscillometry. Larger studies are needed in order to validate these findings and to explore the impact that birth weight and gestational age at birth have on Zrs later in life.

Keywords: Oscillometry; Premature birth; Respiratory function tests; Respiratory mechanics; Spirometry.

Objective: To compare children/adolescents with mild or moderate asthma and healthy subjects in terms of respiratory muscle strength, correlating it with spirometric variables in the former group. Methods: This was a cross-sectional study involving individuals 6-16 years of age and clinically diagnosed with mild/moderate asthma, together with a group of healthy, age- and gender-matched subjects. We determined spirometric values, as well as MIP and MEP, and we selected three reproducible measurements (variation < 10%). Results: We evaluated 75 patients with asthma and 90 controls. The mean age was 10.0 ± 2.6 years. There were no statistically significant differences between the controls and the asthma group regarding MIP (−89.7 ± 26.7 cmH2O vs. −92.2 ± 26.3 cmH2O; p = 0.541) or MEP (79.2 ± 22.9 cmH2O vs. 86.4 ± 24.0 cmH2O; p = 0.256). The groups were subdivided by age (children and adolescents: 6-12 and 13-16 years of age, respectively). Within the asthma group, there was a significant difference between the child and adolescent subgroups in terms of MEP (74.1 ± 24.1 cmH2O vs. 92.1 ± 21.9 cmH2O; p < 0.001) but not MIP (p = 0.285). Within the control group, there were significant differences between the child and adolescent subgroups in terms of MIP (−79.1 ± 17.7 cmH2O vs. −100.9 ± 28.1 cmH2O; p < 0.001) and MEP (73.9 ± 18.7 cmH2O vs. 90.9 ± 28.1cmH2O; p < 0.001). In the asthma group, spirometric variables did not correlate with MIP or MEP. Conclusions: In our sample, asthma was found to have no significant effect on respiratory muscle strength.

Objective: Barriers to physical activity can affect the functional capacity and quality of life of patients with pulmonary hypertension (PH). This study aimed to identify the main barriers to physical activity in patients with PH and to examine whether those barriers are related to functional capacity, echocardiographic variables, or quality of life. Methods: This was a cross-sectional observational study involving 70 patients. Participants scored seven potential barriers to their activities, with a score = 5 indicating a significant barrier. Participants completed the Medical Outcomes Study 36-item Short-Form Health Survey (SF-36) and Manchester Respiratory Activity of Daily Living questionnaire, as well as the six-minute walk test. Correlation analysis, univariate analysis and multiple logistic regression were performed. Results: As a perceived barrier to physical activity, 'lack of will' or 'lack of energy' was cited by 67% of the patients. The 'lack of will' barrier was found to correlate with all SF-36 domains except bodily pain. We also identified a correlation between the SF-36 vitality domain and the barriers 'lack of energy', 'lack of will' and 'lack of structure'. The logistic regression analysis indicated that the vitality domain correlated significantly with the barriers 'social influence', 'lack of energy', 'lack of will', and 'lack of structure'. For each unit decrease in the vitality score, there was a 10% increase in the probability of citing the barrier 'lack of will'. No significant correlations were identified between any of the perceived barriers and echocardiographic parameters. Conclusions: The perceived barrier most commonly reported was 'lack of will/energy', which correlated with almost all SF-36 domains, especially vitality. The 'lack of will' barrier also correlated with functional capacity.

Keywords: Pulmonary arterial hypertension; Exercise; Motivation; Quality of life; Surveys and questionnaires; Walk test.

Objective: To determine whether air trapping (expressed as the percentage of air trapping relative to total lung volume [AT%]) correlates with clinical and functional parameters in children with obliterative bronchiolitis (OB). Methods: CT scans of 19 children with OB were post-processed for AT% quantification with the use of a fixed threshold of −950 HU (AT%950) and of thresholds selected with the aid of density masks (AT%DM). Patients were divided into three groups by AT% severity. We examined AT% correlations with oxygen saturation (SO2) at rest, six-minute walk distance (6MWD), minimum SO2 during the six-minute walk test (6MWT_SO2), FVC, FEV1, FEV1/FVC, and clinical parameters. Results: The 6MWD was longer in the patients with larger normal lung volumes (r = 0.53). We found that AT%950 showed significant correlations (before and after the exclusion of outliers, respectively) with the clinical score (r = 0.72; 0.80), FVC (r = 0.24; 0.59), FEV1 (r = −0.58; −0.67), and FEV1/FVC (r = −0.53; r = −0.62), as did AT%DM with the clinical score (r = 0.58; r = 0.63), SO2 at rest (r = −0.40; r = −0.61), 6MWT_SO2 (r = −0.24; r = −0.55), FVC (r = −0.44; r = −0.80), FEV1 (r = −0.65; r = −0.71), and FEV1/FVC (r = −0.41; r = −0.52). Conclusions: Our results show that AT% correlates significantly with clinical scores and pulmonary function test results in children with OB.

Keywords: Multidetector computed tomography; Respiratory function tests; Bronchiolitis obliterans.

Objective: To describe and analyze clinical symptoms and spirometric alterations of patients with chronic obstructive pulmonary disease (COPD) and history of exposure to wood and tobacco smoke. Methods: We retrospectively evaluated data related to 170 patients distributed into 3 groups: 34 exposed only to wood smoke, 59 patients exposed only to tobacco smoke and 77 patients exposed to both. Results: The groups did not differ significantly in terms of age (p = 0.225) or degree of exposure, considering each type of exposure in isolation or in combination (p = 0.164 and p = 0.220, respectively). Females predominated in the group exposed to wood smoke. There were no differences among the groups regarding respiratory symptoms (p > 0.05), and moderate dyspnea predominated in the three groups (p = 0.141). The group exposed to wood smoke presented higher percentages of forced expiratory volume in one second/forced vital capacity ratio and of forced expiratory volume in one second (p < 0.05). Positive results on bronchodilator testing occurred more frequently in the group exposed to tobacco smoke. The percentage of severe and extremely severe obstruction was significantly higher in the group exposed to tobacco smoke (44.1%) than in that exposed to wood smoke (11.8%; p = 0.006). Conclusions: Respiratory symptoms and pulmonary function alterations consistent with COPD were observed in the groups of patients exposed to wood smoke. However, those alterations were not as significant as the alterations observed in the groups exposed to tobacco smoke. This study emphasizes the importance of prospective studies in evaluating the risk of wood-smoke-related COPD in Brazil, as well as the need for preventive measures in this area.

Keywords: Pulmonary disease, chronic obstructive; Smoking; Air pollution, indoor; Smoke.

Objective: To assess airway hyperresponsiveness to 4.5% hypertonic saline solution in comparison to that obtained through challenge with other bronchoconstriction agents and in relation to patient allergic sensitization. Methods: A cross-sectional, experimental study was conducted, initially involving 85 subjects. After exclusions, the final sample consisted of 62 patients, divided into two groups: a study group of those with asthma (n = 45) and a control group of those with no asthma or allergies (n = 17). Hypertonic saline was nebulized using an ultrasonic nebulizer and administered successively for 0.5, 1, 2, 4 and 8 minutes until a drop in forced expiratory volume in one second of = 15% was achieved in relation to the baseline value. The level of specific immunoglobulin E to Dermatophagoides pteronyssinus level was determined by ImmunoCAP assay and was considered positive when > 0.35 kU/L. Results: In the 36 asthma group subjects presenting a response, the mean drop in forced expiratory volume in one second after hypertonic saline nebulization was 27.4%. None of control group subjects (immunoglobulin E < 0.35 kU/L) presented a positive response to hypertonic saline. The mean forced expiratory volume in one second for control group subjects was 9%. The results of a bronchial provocation test were negative in 9 of the asthma group subjects. The frequency of bronchial provocation test positivity was higher in the subjects presenting elevated levels of specific immunoglobulin E, indicating that there is a relationship between bronchial hyperresponsiveness and the level of specific immunoglobulin E. The sensitivity and specificity of the test were 80% and 92%, respectively. Conclusion: Bronchial provocation with hypertonic saline presents satisfactory sensitivity and specificity. Therefore, in addition to being a low cost procedure that requires very little equipment, it is a useful means of assessing hyperresponsiveness in children and adolescents.

Keywords: Bronchial provocation tests; Bronchial hyperreactivity; Saline solution, hypertonic; Administration, inhalation;

Pulmonary hypertension is a common complication of sickle cell anemia. Despite the fact that the elevations in pulmonary artery pressures are slight, morbidity and mortality are high. In adult sickle cell anemia patients, pulmonary hypertension is emerging as a major risk factor for death. The pathogenesis of sickle cell anemia-related pulmonary hypertension is multifactorial, including hemolysis, impaired nitric oxide bioavailability, chronic hypoxemia, thromboembolism, chronic liver disease and asplenia. In the majority of patients, pulmonary arterial hypertension is the main cause of elevated pulmonary artery pressures. However, pulmonary venous hypertension also plays a role in a subgroup of patients. Specific data on the effects of treatment modalities for pulmonary hypertension in patients with sickle cell anemia are scarce. It is likely that all patients would benefit from maximization of sickle cell anemia therapy, and that patients with the severe form of the disease would benefit from treatment with selective pulmonary vasodilators and antiproliferative agents. Large trials evaluating the effects of treatment for pulmonary hypertension in the sickle cell anemia population are underway.

Keywords: Anemia, Sickle cell; Hemolysis; Hypertension, pulmonary; Nitric oxide.