Background: Pectus excavatum is characterized by concave growth of costal cartilage and depression of the lower sternum. Clinical means of classifying these malformations are few and difficult to apply. Objective: To devise clinical tools for quantifying the deformity and comparing preoperative and postoperative findings. Method: A total of 10 pectus excavatum patients who underwent surgery in which the modified Robicsek technique was used by the Thoracic Surgery Group of the Hospital das Clínicas of the University of São Paulo School of Medicine, were clinically and radiologically evaluated in the preoperative and postoperative periods. Ten control individuals, presenting no thoracic or radiological abnormalities, were submitted to identical evaluations. Deformities at the sternum notch level and at the point of maximum deformity were assessed using the anthropometric index and the Haller index. Results: Multivariate analysis of anthropometric index means revealed significant differences between preoperative and control values and between preoperative and postoperative values, as well as a non-significant difference between postoperative and control values. The same results were obtained when Haller index means were analyzed. A paired comparison of preoperative and postoperative means showed two distinct groups. An 86% canonical correlation was found between the anthropometric index and the Haller index. Conclusion: Patients with pectus excavatum can be quantitatively assessed in the preoperative and postoperative periods through the use of the anthropometric index, which allows objective, comparative evaluation of the results and is easily performed.

Keywords: Funnel Chest. Anthropometry/methods.

Pectus excavatum (PEX) is the most frequent congenital deformity of the anterior chest wall and is defined as the dislocation of the medial or inferior portion of the sternal region toward the spinal column. There are various ways to measure the deformity. In this study, we present an objective method of assessing such deformity, the anthropometric index for PEX (AI-PEX). The AI-PEX was developed in the Thoracic Surgery Department of the Heart Institute - University of São Paulo School of Medicine Hospital das Clínicas. The anthropometric measurements are taken during the physical examination. We herein report two cases involving patients with PEX assessed using the AI-PEX and treated with the minimally invasive Nuss technique. The measurements were always taken at the point of greatest deformity. The patients were assessed on the day of the operation and again at 60 days after the surgery. The AI-PEX allowed us to obtain a satisfactory assessment of the defect. In both patients, the post-operative evolution was favorable.

Keywords: Thoracic wall; Thoracic surgery, Video-assisted; Funnel chest.

Objective: To determine the prevalence of congenital anterior chest wall deformities in 11- to 14-year-old students. Methods: Students participating in the study were recruited from public schools in the city of Manaus, Brazil. The statistically significant sample (precision, 1%; 95% CI) comprised 1,332 students. Pectus deformities were identified by physical examination of the chest, and the individuals with one of these deformities completed a questionnaire regarding heredity and symptoms resulting from the chest abnormality. Results: The mean age of the participants was 11.7 years. The prevalence of pectus abnormalities was 1.95% (pectus excavatum: 1.275%; pectus carinatum: 0.675%). Of the 26 students with a pectus deformity, 17 (65.4%) had pectus excavatum, and 18 (69.2%) were male. Concomitant scoliosis was observed in 3 cases (11.5%). A family history of pectus was reported by 17 students (65.4%), and 17 (65.4%) reported chest pain, dyspnea or palpitations. Conclusions: In this study, the prevalence of pectus deformities (1.95%) was lower than that reported in other studies conducted in Brazil (3.6-4.9%) but was higher than that reported in the literature (mean, 1%).

Keywords: Musculoskeletal abnormalities; Prevalence; Funnel chest.

Objective: To quantify elastic fibers (EFs) and smooth muscle (SM) cells, as well as CD4+ and CD8+ T lymphocytes, in stable chronic obstructive pulmonary disease (COPD). Methods: Surgical specimens were obtained from 15 COPD patients, 18 smokers without airflow limitation, and 14 nonsmokers. Histological and immunohistochemical methods were employed in order to quantify EFs, SM cells, CD4+ T cells, and CD8+ T cells. Results: There was no significant difference in EF numbers among the three groups (p > 0.05). The number of EFs per unit area of lung tissue (mm2) and the percentage of EFs in the lung tissue were similar among the three groups. The numbers of SM cells were found to be higher in the COPD patients than in the smokers (p = 0.003) or in the nonsmokers (p = 0.009). There was a tendency toward an increase in CD8+ T-cell counts in the COPD patients. In specimens collected from the COPD patients, CD4+ T-cell counts were lower than in those collected from the smokers (p = 0.015) or from the nonsmokers (p = 0.003). There was a weak correlation between CD4+ T-cell count and the ratio of forced expiratory volume in one second to forced vital capacity (r2 = 0.003). Conclusions: The EF counts were similar among the three groups. Hypertrophy/hyperplasia of airway wall SM cells was found in the COPD patients and in the smokers, indicating that airway remodeling occurs in smokers. The CD4/CD8 ratio was lower in the COPD patients.

Keywords: Pulmonary disease, chronic obstructive; CD4 Lymphocyte Count; Elastic tissue; Muscle, smooth.

Objective: Minimally invasive repair of pectus excavatum (MIRPE) is a surgical treatment for PE. During the procedure, a specialized introducer is used to tunnel across the mediastinum for thoracoscopic insertion of a metal bar. There have been reported cases of cardiac perforation during this risky step. The large introducer can be a dangerous lever in unskilled hands. We set out to determine the safety and feasibility of using regular instruments (i.e., not relying on special devices or tools) to create the retrosternal tunnel during MIRPE. Methods: This was a preliminary study of MIRPE with regular instruments (MIRPERI), involving 28 patients with PE. We recorded basic patient demographics, chest measurements, and surgical details, as well as intraoperative and postoperative complications. Results: Patients undergoing MIRPERI had Haller index values ranging from 2.58 to 5.56. No intraoperative complications occurred. Postoperative complications included nausea/vomiting in 8 patients, pruritus in 2, and dizziness in 2, as well as atelectasis, pneumothorax with thoracic drainage, pleural effusion, and dyspnea in 1 patient each. Conclusions: In this preliminary study, the rate of complications associated with MIRPERI was comparable to that reported in the literature for MIRPE. The MIRPERI approach has the potential to improve the safety of PE repair, particularly for surgeons that do not have access to certain special instruments or have not been trained in their use.

Keywords: Funnel chest; Heart injuries; Thoracic wall; Intraoperative complications; Minimally invasive surgical procedures.

Objective: To compare individuals with and without hyperhidrosis in terms of the intensity of palmar and plantar sweating. Methods: We selected 50 patients clinically diagnosed with palmoplantar hyperhidrosis and 25 normal individuals as controls. We quantified sweating using a portable noninvasive electronic device that has relative humidity and temperature sensors to measure transepidermal water loss. All of the individuals had a body mass index of 20-25 kg/cm2. Subjects remained at rest for 20-30 min before the measurements in order to reduce external interference. The measurements were carried out in a climate-controlled environment (21-24°C). Measurements were carried out on the hypothenar region on both hands and on the medial plantar region on both feet. Results: In the palmoplantar hyperhidrosis group, the mean transepidermal water loss on the hands and feet was 133.6 ± 51.0 g/m2/h and 71.8 ± 40.3 g/m2/h, respectively, compared with 37.9 ± 18.4 g/m2/h and 27.6 ± 14.3 g/m2/h, respectively, in the control group. The differences between the groups were statistically significant (p < 0.001 for hands and feet). Conclusions: This method proved to be an accurate and reliable tool to quantify palmar and plantar sweating when performed by a trained and qualified professional.

Keywords: Hyperhidrosis; Sweat; Dermatology/instrumentation.

We compared bacteremic pneumococcal pneumonia (BPP) and pneumococcal empyema (PE), in terms of clinical, radiological, and laboratory findings, in under-fives. A cross-sectional nested cohort study, involving under-fives (102 with PE and 128 with BPP), was conducted at 12 centers in Argentina, Brazil, and the Dominican Republic. Among those with PE, mean age was higher; disease duration was longer; and tachypnea, dyspnea, and high leukocyte counts were more common. Among those with BPP, fever and lethargy were more common. It seems that children with PE can be distinguished from those with BPP on the basis of clinical and laboratory findings. Because both conditions are associated with high rates of morbidity and mortality, prompt diagnosis is crucial.

Keywords: Empyema, pleural; Pneumonia, pneumococcal; Pneumococcal infections.

Objective: To cross-culturally adapt the Duke Activity Status Index (DASI) for use in Brazil and evaluate the reproducibility of the new (Brazilian Portuguese-language) version. Methods: We selected stable patients with clinical and spirometric diagnosis of COPD. Initially, the DASI was translated into Brazilian Portuguese, and the cross-cultural adaptation was performed by an expert committee. Subsequently, 12 patients completed the questionnaire, so that their questions and difficulties could be identified and adjustments could be made. An independent translator back-translated the final version into English, which was then submitted to and approved by the original author. The final Brazilian Portuguese-language version of the DASI was applied to 50 patients at three distinct times. For the assessment of interobserver reproducibility, it was applied twice within a 30-min interval by two different interviewers. For the assessment of intraobserver reproducibility, it was applied again 15 days later by one of the interviewers. Results: The mean age of the patients was 62.3 ± 10.0 years, the mean FEV1 was 45.2 ± 14.7% of the predicted value, and the mean body mass index was 26.8 ± 5.8 kg/m2. The intraclass correlation coefficients for intraobserver and interobserver reproducibility were 0.95 and 0.90, respectively. The correlations between the DASI and the Saint George's Respiratory Questionnaire (SGRQ) domains were all negative and statistically significant. The DASI correlated best with the SGRQ activity domain (r = −0.70), the total SGRQ score (r = −0.66), and the six-minute walk distance (r = 0.55). Conclusions: The Brazilian Portuguese-language version of the DASI is reproducible, fast, and simple, correlating well with the SGRQ.

Keywords: Activities of daily living; Pulmonary disease, chronic obstructive; Reproducibility of results.

Objective: To translate, adapt and validate the Patient Generated Index (PGI) for Brazilians with chronic obstructive pulmonary disease (COPD). Methods: 50 volunteers with COPD, mostly men (74%), with 73.1 ± 8.9 years of age, FEV1 of 52.3 ± 14.5% of predicted and FEV1 / FVC of 56.2 ± 8.6% of predicted responded to PGI, to the Saint George Respiratory Questionnaire (SGRQ) and to perform Glittre Activities of Daily Living test (Glittre ADL). After 1-2 weeks, PGI was again applied for the analysis of relative and absolute reliability. Results: The translation occurred without changes in the questionnaire. The score obtained in PGI had weak correlation with the SGRQ total score (r = -0.44, p <0.001) and with the impact domain (r = -0.40, p <0.05), presented a moderate correlation with the symptoms domain of the SGRQ (r = -0.55, p <0.001) and weak correlation with the activity domain (r = -0.31, p <0.05). A weak correlation was observed between PGI and Glittre ADL (r = -0.30; p <0.05). It was observed high reliability among the measures of PGI (ICCr = 0.94). Conclusion: This study shows that the Brazilian version of PGI is a reliable and valid instrument to measure health‑related quality of life (HRQL) in patients with COPD. It is a new and individualized form of evaluation of COPD patient-centered quality of life.

Keywords: Quality of life; Chronic obstructive pulmonary disease; Reproducibility of results and translations.

Among the deformities of the thoracic wall,pectus carinatum has not received the same attention as has pectus excavatum. Few pulmonologists, pediatricians, and thoracic surgeons are aware of the approaches to treating this condition. As a consequence, patients with pectus carinatum are not referred for treatment. This deformity, with an incidence of 1:1000 teenagers, is oligosymptomatic. However, for aesthetic and emotional reasons, it accounts for a large number of medical appointments. Such patients are introverted and do not engage in physical activities, since they are unwilling to expose their chest, which also discourages them from going to the beach or to swimming pools. The diagnosis is clinical and visual, and details are obtained through chest X-rays and computed tomography. The treatment is based on a well-known organogram that summarizes orthopedic and surgical procedures. Dynamic compression, combined with physical exercises, is indicated for teenagers with flexible thorax in inferior and lateral pectus carinatum, with limited indication for those with superior pectus carinatum. For individuals of any age with rigid thorax, surgery is indicated for aesthetic reasons. Among the techniques described, the modified sternum chondroplasty stands out due to the excellent aesthetic results achieved.

Keywords: Thoracic wall; Bone Diseases, Developmental/therapeutics; Surgery

Objective: To evaluate whether anthropometric and dietary intake indicators are predictors of pulmonary function in cystic fibrosis (CF) patients. Methods: This was a cross-sectional study involving 69 patients (age range, 5.4-16.5 years) diagnosed with CF under follow-up at the Hospital de Clínicas de Porto Alegre, located in the city of Porto Alegre, Brazil. Anthropometric assessment was based on body mass index (BMI), mid-arm muscle circumference (MAMC), and triceps skinfold thickness (TST). Dietary intake was assessed by using recall data, which were compared with the recommended dietary allowances. Pulmonary function was assessed by ventilatory capacity, expressed as FEV1. Prevalence ratios for the outcome studied (FEV1 < 80% of predicted) were calculated by indicator. Results: In patients with MAMC and TST below the 25th percentile, the prevalence of FEV1 < 80% of predicted was significantly higher than in those with higher MAMC and TST (p < 0.001 and p = 0.011, respectively). In comparison with other patients, those with a BMI below the 50th percentile showed a 4.43 times higher prevalence of FEV1 < 80% of predicted (95% CI: 1.58-12.41), and that prevalence was 2.54 times higher in those colonized with methicillin-resistant Staphylococcus aureus (MRSA) than in those not so colonized (95% CI: 1.43-4.53). The association between dietary intake and the prevalence of FEV1 < 80% of predicted was of only borderline significance (95% CI: 0.95-3.45). Conclusions: Not being colonized with MRSA and having a BMI above the 50th percentile appear to preserve pulmonary function in CF patients.

Keywords: Cystic fibrosis; Respiratory function tests; Nutrition assessment; Energy intake.

Objective: To compare 28-day mortality rates and clinical outcomes in ICU patients with ventilator-associated pneumonia according to the diagnostic strategy used. Methods: This was a prospective randomized clinical trial. Of the 73 patients included in the study, 36 and 37 were randomized to undergo BAL or endotracheal aspiration (EA), respectively. Antibiotic therapy was based on guidelines and was adjusted according to the results of quantitative cultures. Results: The 28-day mortality rate was similar in the BAL and EA groups (25.0% and 37.8%, respectively; p = 0.353). There were no differences between the groups regarding the duration of mechanical ventilation, antibiotic therapy, secondary complications, VAP recurrence, or length of ICU and hospital stay. Initial antibiotic therapy was deemed appropriate in 28 (77.8%) and 30 (83.3%) of the patients in the BAL and EA groups, respectively (p = 0.551). The 28-day mortality rate was not associated with the appropriateness of initial therapy in the BAL and EA groups (appropriate therapy: 35.7% vs. 43.3%; p = 0.553; and inappropriate therapy: 62.5% vs. 50.0%; p = 1.000). Previous use of antibiotics did not affect the culture yield in the EA or BAL group (p = 0.130 and p = 0.484, respectively). Conclusions: In the context of this study, the management of VAP patients, based on the results of quantitative endotracheal aspirate cultures, led to similar clinical outcomes to those obtained with the results of quantitative BAL fluid cultures. (Brazilian Registry of Clinical Trials - ReBEC; identification number RBR-86DCDX [http://www.ensaiosclinicos.gov.br])

Keywords: Bronchoalveolar lavage fluid/diagnosis; Respiratory aspiration; Pneumonia, ventilator-associated.

Objectives: To evaluate the evolution pattern of dynamic pulmonary flow and volume in cystic fibrosis patients and analyze the relation between the severity of ventilatory attacks and age, gender, genotype and pulmonary colonization. Methods: At the Pediatric Pulmonary Unit and Pulmonary Service of Hospital de Clínicas of Porto Alegre, 243 spirometries performed between 1987 and 1999 in 52 cystic fibrosis patients older than four years of age were reviewed. From the patients' medical records the following informations were extracted: age at diagnosis, genetic data, bronchial colonization and absolute as well as percent values of the flows and volumes of the best annual spirometry. Results: The age of the patients ranged from four to 26 years (mean = 13.04 ± 4.82). Out of the 52 patients, 49 (94%) had at least one sputum culture with P. aeruginosa, 45 (86%) with S. aureus and 13 (25%) with B. cepacia. At initial evaluation, that included patients four to six years old (n = 40), mean values for FVC and FEV1 were 114.24% and 112.25%, respectively. Mean FVC remained above baseline until 18 years of age, when it abruptly dropped to 67.2% (p = 0.0002). Mean FEV1 values dropped to less than 80% at ten years and was 50% at 18 years (p < 0.00001). The FEV1/FVC% relationship, the most obstruction sensitive index, was observed to be reduced as early as initial evaluation. It was 85% between four and six years and gradually decreased to 63% at 18 years. The mean values of FEF50, FEF75 and FMEF were initially normal (94%, 80% and 90%, respectively), showed a greater and earlier statistically significant decline, and reached to 37%, 12.5% and 19%, respectively, at 18 years. Although a tendency to lower terminal flow was seen in the girls, the differences were not statistically significant. Significant correlations between pulmonary function and the airway colonizator or genotype were not detected statistic. Conclusion: The evolution pattern of pulmonary function alterations found in these patients is consistent with those reported in the literature, where obstructive ventilatory disorders with early terminal flow reduction and late FVC onset predominate.

Keywords: Spirometry. Cystic fibrosis. Pulmonary ventilatory.

Objective: To evaluate the anthropometric data obtained for residents of the city of São Paulo, Brazil, in a study of Latin America conducted in two phases (baseline, in 2003, and follow-up, in 2012). Methods: This was an analysis of data obtained for São Paulo residents in a two-phase population-based study evaluating the prevalence of COPD and its relationship with certain risk factors among individuals ≥ 40 years of age. The anthropometric data included values for weight, height, body mass index (BMI), and waist circumference. In the follow-up phase of that study, the same variables were evaluated in the same population sample as that of the baseline phase. Results: Of the 1,000 São Paulo residents enrolled in the baseline phase of that study, 587 participated in the follow-up phase, and 80 (13.6%) of those 587 subjects had COPD. Comparing the baseline and follow-up phases, we found increases in all anthropometric measures in both groups (COPD and non-COPD), although the differences were significant only in the non-COPD group. The subjects with mild COPD showed increases in weight and BMI (Δweight = 1.6 ± 5.7 and ΔBMI = 0.7 ± 2.2), whereas those with moderate or severe COPD showed reductions (Δweight = −1.7 ± 8.1 and ΔBMI = −0.4 ± 3.0), as did those with severe or very severe COPD (Δweight = −0.5 ± 5.4 and ΔBMI = −0.8 ± 3.3). Conclusions: Between the two phases of the study, the subjects with mild COPD showed increases in weight and BMI, whereas those with a more severe form of the disease showed reductions.

Keywords: Pulmonary disease, chronic obstructive; Body mass index; Obesity; Waist circumference.

Objective: To determine whether there are significant differences between rapid-eye-movement (REM)-related obstructive sleep apnea (OSA) and non-REM (NREM)-related OSA, in terms of the demographic, anthropometric, and polysomnographic characteristics of the subjects. Methods: This was a retrospective study of 110 patients (75 males) with either REM-related OSA (n = 58) or NREM-related OSA (n = 52). To define REM-related and NREM-related OSA, we used a previously established criterion, based on the apnea-hypopnea index (AHI): AHI-REM/AHI-NREM ratio > 2 and ≤ 2, respectively. Results: The mean age of the patients with REM-related OSA was 49.5 ± 11.9 years, whereas that of the patients with NREM-related OSA was 49.2 ± 12.6 years. The overall mean AHI (all sleep stages combined) was significantly higher in the NREM-related OSA group than in the REM-related OSA group (38.6 ± 28.2 vs. 14.8 ± 9.2; p < 0.05). The mean AHI in the supine position (s-AHI) was also significantly higher in the NREM-related OSA group than in the REM-related OSA group (49.0 ± 34.3 vs. 18.8 ± 14.9; p < 0.0001). In the NREM-related OSA group, the s-AHI was higher among the men. In both groups, oxygen desaturation was more severe among the women. We found that REM-related OSA was more common among the patients with mild-to-moderate OSA, whereas NREM-related OSA was more common among those with severe OSA. Conclusions: We found that the severity of NREM-related OSA was associated mainly with s-AHI. Our findings suggest that the s-AHI has a more significant effect on the severity of OSA than does the AHI-REM. When interpreting OSA severity and choosing among treatment modalities, physicians should take into consideration the sleep stage and the sleep posture.

Keywords: Sleep, REM; Sleep stages; Sleep apnea, obstructive; Apnea; Sleep apnea syndromes.

Objective: The aim of this study was to identify the best experimental model in which to observe the pulmonary alterations characterizing hepatopulmonary syndrome (HPS). Methods: Male Wistar rats, with mean weight of 250 g, were used in four experimental models: inhaled carbon tetrachloride; intraperitoneal carbon tetrachloride; partial portal vein ligation; and bile duct ligation (BDL). The animals in all groups were divided into control and experimental subgroups. The following variables were measured: transaminase levels; blood gases; lipoperoxidation, using thiobarbituric acid reactive substances (TBARS) and chemiluminescence; and levels of superoxide dismutase (SOD) anti-oxidant activity. Anatomopathological examination of the lung was also performed. Results: There were statistically significant differences between the BDL control and BDL experimental groups: aspartate aminotransferase (105.3 ± 43 vs. 500.5 ± 90.3 IU/L); alanine aminotransferase (78.75 ± 37.7 vs. 162.75 ± 35.4 IU/L); alkaline phosphatase (160 ± 20.45 vs. 373.25 ± 45.44 IU/L); arterial oxygen tension (85.25 ± 8.1 vs. 49.9 ± 22.5 mmHg); and oxygen saturation (95 ± 0.7 vs. 73.3 ± 12.07%). Lipoperoxidation and antioxidant activity also differed significantly between the two BDL groups (control vs. experimental): TBARS (0.87 ± 0.3 vs. 2.01 ± 0.9 nmol/mg protein); chemiluminescence (16008.41 ± 1171.45 vs. 20250.36 ± 827.82 cps/mg protein); and SOD (6.66 ± 1.34 vs. 16.06 ± 2.67 IU/mg protein). The anatomopathological examination confirmed pulmonary vasodilatation in the BDL model. In the other models, there were no alterations that were characteristic of HPS. Conclusions: The data obtained suggest that the BDL model can be used in future studies involving hepatic alterations related to oxidative stress and HPS.

Keywords: Hepatopulmonary syndrome; Lung; Oxidative stress; Rats.

Objective: To compare religious coping (RC) in patients with COPD and healthy individuals, as well as to determine whether RC is associated with demographic characteristics, quality of life, depression, and disease severity in the patients with COPD. Methods: This was a cross-sectional study conducted between 2014 and 2016, involving outpatients with moderate to severe COPD seen at one of two hospitals in Fortaleza, Brazil, as well as gender- and age-matched healthy controls. The Brief RCOPE scale assessed RC in all of the participants. We also evaluated the COPD group patients regarding symptoms, quality of life, and depression, as well as submitting them to spirometry and a six-minute walk test. Results: A total of 100 patients were evaluated. The mean age was 67.3 ± 6.8 years, and 54% were men. In the COPD group, the mean positive RC score was significantly higher than was the mean negative RC score (27.17 ± 1.60 vs. 8.21 ± 2.12; p = 0.001). The mean positive RC score was significantly higher in women than in men (27.5 ± 1.1 vs. 26.8 ± 2.8; p = 0.02). Negative RC scores were significantly higher in the COPD group than in the control group (p = 0.01). Negative RC showed an inverse association with six-minute walk distance (6MWD; r = −0.3; p < 0.05) and a direct association with depressive symptoms (r = 0.2; p < 0.03). Positive RC correlated with none of the variables studied. Multiple regression analysis showed that negative RC was associated with 6MWD (coefficient = −0.009; 95% CI: −0.01 to −0.003). 6MWD explained the variance in negative RC in a linear fashion. Conclusions: Patients with COPD employ negative RC more often than do healthy individuals. Exercise capacity and depressive symptoms are associated with negative RC.

Keywords: Religion; Spirituality; Adaptation, psychological; Quality of life; Pulmonary disease, chronic obstructive.

Objective: To analyze HRCT findings in patients with Niemann-Pick disease (NPD) type B, in order to determine the frequency of HRCT patterns and their distribution in the lung parenchyma, as well as the most common clinical characteristics. Methods: We studied 13 patients (3 males and 10 females) aged 5 to 56 years. HRCT images were independently evaluated by two observers, and disagreements were resolved by consensus. The inclusion criteria were presence of abnormal HRCT findings and diagnosis of NPD type B confirmed by histopathological examination of a bone marrow, lung, or liver biopsy specimen. Results: The most common clinical findings were hepatosplenomegaly and mild to moderate dyspnea. The most common HRCT patterns were smooth interlobular septal thickening and ground-glass opacities, which were both present in all patients. Intralobular lines were present in 12 patients (92.3%). A crazy-paving pattern was observed in 5 patients (38.4%), and areas of air trapping were identified in only 1 case (7.6%). Pulmonary involvement was bilateral in all cases, with the most affected area being the lower lung zone. Conclusions: Smooth interlobular septal thickening, with or without associated ground-glass opacities, in patients with hepatosplenomegaly is the most common finding in NPD type B.

Keywords: Niemann-Pick diseases; Tomography, X-ray computed; Lung diseases.

Objective: To investigate the effects of age on pulmonary emphysema, based on the values of the emphysema index (EI) in a cohort of patients who had never smoked and who had no recognizable lung disease. Methods: We reviewed the CT scans, reported as normal, of 315 patients. Exclusion criteria were a history of smoking, cardiorespiratory disease, and exposure to drugs that could cause lung disease. From this cohort, we selected 32 patients (16 men and 16 women), matched for gender and body mass index, who were divided equally into two groups by age (< 50 years and  50 years). We quantified emphysema using a computer program specific to that task. The EI was calculated with a threshold of −950 HU. We also evaluated total lung volume (TLV) and mean lung density (MLD). Results: The overall means for TLV, MLD, and EI were 5,027 mL, −827 HU, and 2.54%, respectively. Mean values in the older and younger groups, respectively, were as follows: for TLV, 5,229 mL vs. 4,824 mL (p > 0.05); for MLD, −846 HU vs. −813 HU (p < 0.04); and for EI, 3.30% vs. 1.28% (p < 0.001). Significant correlations were found between EI and age (r = 0.66; p = 0.001), EI and TLV (r = 0.58; p = 0.001), and EI and MLD (r = −0.67; p < 0.001). The predicted EI per age was defined by the regression equation (r2 = 0.43): p50(EI) = 0.049 × age − 0.5353. Conclusions: It is important to consider the influence of age when quantifying emphysema in patients over 50 years of age. Based on the regression analysis, EI values of 2.6%, 3.5%, and 4.5% can be considered normal for patients 30, 50, and 70 years of age, respectively.

Keywords: Pulmonary emphysema; Tomography, spiral computed; Aging; Pulmonary disease, chronic obstructive.