José Antônio Baddini Martinez

Lung Function and Airway Hyperresponsiveness in Adult Patients with Sickle Cell Disease

Background:Lung disease is a major cause of morbidity and death in sickle cell disease. Although airway hyperresponsiveness has been noted in children, there are no studies in adult sickle cell patients. The aim of this study was to investigate the prevalence of airway hyperresponsiveness in adult sickle cell patients. Methods:Twenty-six patients with sickle cell disease (10 HbSC, 9 HbSS, and 7 HbSβ) were compared with 28 normal control subjects. Pulmonary function tests, including spirometry, measurements of single-breath diffusing capacity and the methacholine challenge test were performed. Results:There were no significant differences in age, gender, or height between groups. Restrictive ventilatory defect was observed in six patients (24%) in the sickle cell disease group. Obstructive ventilatory defect and reduced diffusing lung DLCO capacity was observed in all sickle cell disease subgroups. A positive methacholine challenge test was obtained in eight (31%) sickle cell patients and in two of the 28 controls (7%). Conclusion:These features suggest that there is a high prevalence of airway hyperresponsiveness in adult patients with sickle cell disease without a history of reactive airway disease.

Diffuse panbronchiolitis in Latin America.

Diffuse panbronchiolitis (DPB) is a unusual form of bronchiolar disease that has been reported almost exclusively in Asians. We describe DPB in a non-Asian Brazilian citizen who has never traveled outside the country. The clinical, radiographic, and histologic features of this case resemble those described in Japanese patients. The present case shows that DPB, although rare in Western countries, is not a disease restricted to Asia. It always should be considered in the differential diagnosis of nodular radiographic opacities associated with airflow limitation, especially in non-smokers with a history of chronic sinusitis.

Protective effect of aminoguanidine in a murine model of pulmonary fibrosis induced by bleomycin

Abstract— Aminoguanidine is a drug known for more than a century, which has been attracting increasing interest in recent years due to the discovery of new pharmacological properties. This study investigated the effects of aminoguanidine on the fibrotic response induced by intratracheal administration of bleomycin to rats. Three groups of animals were studied: Group A (n = 19) corresponded to the control group. Group B (n = 20) received 10 IU/kg bleomycin intratracheal, and Group C (n = 12) received the same amount of bleomycin as Group B followed by 50 mg/kg/day aminoguanidine bicarbonate for 4 weeks. Aminoguanidine led to significant reductions in total hydroxyproline content of the lungs in Group C compared to Group B (Group A: 1.83 ± 0.14 mg ± Group B: 3.46 ± 0.36 mg ± Group C: 2.09 ± 0.22 mg). Morphometric collagen studies carried out on histological sections stained with Sirius red F3BA showed that aminoguanidine promoted a significant reduction of the area occupied by collagen in the axial and septal zones of the lungs (Axial region = Group A: 4.29 ± 1.31% × Group B: 19.30 ± 4.86% × Group C: 8.52 ± 1.96%; Septal region = Group A: 0.15 ± 0.06% × Group B: 0.61 ± 0.21% × Group C: 0.15 ± 0.06%). These results suggest that aminoguanidine is a potential therapeutic agent for the treatment and prevention of pulmonary fibrosis which is associated with different clinical conditions.

Iron stores and coagulation parameters in patients with hypoxemic polycythemia secondary to chronic obstructive pulmonary disease: the effect of phlebotomies

UNLABELLEDnThis study was designed to determine the effects of phlebotomy on iron body contents and coagulation tests of COPD patients with polycythemia secondary to hypoxemia. Seventeen patients with COPD and hematocrits higher than 54 percent (mean Hct: 57 +/- 0.49 percent), who had not received anti-inflammatory or antiplatelet aggregation agents recently. Their mean forced expiratory volume at 1 second (FEV1) was 0.92 +/- 0.11 L.nnnINTERVENTIONnBlood work was collected to evaluate the following: serum iron and ferritin levels, total iron binding capacity, transferrin saturation index, fibrinogen plasma levels, activated partial thromboplastin time, platelet count, platelet aggregation measurements, and thromboelastography coagulation parameters. The blood samples were obtained before and about 7 days after the hematocrit correction by 300-400 ml phlebotomies done every other day. The mean number of phlebotomies done for each patient was 4.4. Postphlebotomy iron serum levels decreased from 90.1 +/- 14.8 to 59.7 +/- 9.9 mg/dl and the ferritin serum levels from 133.8 +/- 37.9 to 70.8 +/- 32.7 ng/ml (p < 0.05). Regarding the coagulation studies, there were significant increases in the platelet count, from 227,300 +/- 13,900 to 312,500 +/- 30,200 per mm3, and in the maximum clot amplitude (a) obtained by thromboelastography (from 53.6 +/- 1.4 percent to 60.4 +/- 1.1 percent). The coagulation time (k) of the thromboelastography also decreased significantly, from 7.5 +/- 0.7mm prephlebotomy to 4.5 +/- 0.3mm postphlebotomy. Although the coagulation changes were small amount, the observed significant decrease in iron contents may have clinical implications.

Elevation of soluble interleukin-2 receptor levels in the bronchoalveolar lavage from patients with systemic sclerosis

Abstract. This study was designed to investigate the levels of IL-2 and its soluble receptor (sIL-2R) in bronchoalveolar lavage fluid (BALF) from patients with systemic sclerosis (SSc). We studied 18 patients with SSc and 10 healthy volunteers. Based on high-resolution computed tomography lung scans the patients were divided into two groups, those with (SSc-ILD group, n=10) and those without (SSc group, n=8) evidence of interstitial lung disease (ILD). Both groups showed significantly higher total cell and neutrophil counts in the BALF than controls. The SSc group also showed significantly higher levels of lymphocytes than controls. IL-2 was not detectable in BALF. The patients showed significantly higher levels of sIL-2R than controls (77.8% vs 20%, P=0.005). The median sIL-2R levels detected did not differ between the two patient groups (SSc-ILD 270xa0pg/ml, SSc 232xa0pg/ml). This study suggests that SSc patients with or without ILD have elevated levels of sIL-2R in BALF and that in some of these patients this finding could be explained by subclinical pulmonary inflammation.

Effects of ondansetron on respiratory pattern and sensation of experimentally induced dyspnea

CONTEXTnDyspnea remains a therapeutic challenge, especially in chronic respiratory conditions. Recent studies have shown that the induction of unpleasant dyspnea sensations activates areas in the insular cortex.nnnOBJECTIVEnThis study was designed to investigate the potential effects of ondansetron, a potent anti-serotonin agent, on induced dyspnea sensation.nnnTYPE OF STUDYnA randomized double blind study.nnnSETTINGnPulmonary Function Laboratory of Hospital das Clínicas de Ribeirão Preto.nnnPARTICIPANTSnTen healthy male volunteers (mean age +/- standard error = 23.1 +/- 0.41 years) without respiratory diseases and showing normal spirometric tests.nnnINTERVENTIONSnUncomfortable breathing was induced in the volunteers on two different days, via the use of inspiratory resistors (loads of 0, 7, 14 and 21 cm H2O/l/sec) and breathholding, two hours after taking 8 mg of ondansetron (Ond) or placebo (Plac).nnnMAIN MEASUREMENTSnRespiratory discomfort during breathing under loading was evaluated on a 100-mm visual analog scale. The maximum length of time of voluntary apnea was measured in seconds.nnnRESULTSnThe mean maximum voluntary apnea time did not differ between the ondansetron and placebo days (Plac = 96 +/- 6.6 sec vs. Ond = 100 +/- 7.9 sec). Ondansetron did not influence the dyspnea sensation induced by different inspiratory loads (0 cm H2O/l/sec: Ond = 1.4 mm +/- 0.44 vs. Plac = 2.1 +/- 0.85 mm; 7 cm H2O/l/sec: Ond = 16.6 +/- 2.74 mm vs. Plac = 13.7 +/- 2.06 mm; 14 cm H2O/l/sec; Ond = 30.5 +/- 4.50 mm vs. Plac = 27.1 +/- 3.44 mm; 21 cm H2O/l/sec: Ond = 50.3 +/- 6.72 mm vs. Plac = 49.4 +/- 6.72 mm). Ondansetron led to significant decreases in tidal volume under basal conditions and when breathing under the highest inspiratory loading (0 cm H2O/l/sec: Ond = 0.83 +/- 0.26 l vs. Plac = 1.0 +/- 0.28 l; 21 cm H2O/l/sec: Ond = 0.86 +/- 0.23 l vs. Plac = 1.1 +/- 0.22 l)nnnCONCLUSIONnThe present results suggest that 5-HT3 receptors do not play an important role in the mediation of dyspnea sensations.

Atelectasis due to Bronchial Compression by the Vertebral Column

&NA; The authors report the case of an 18‐year‐old woman with a multicore‐minicore syndrome in acute respiratory distress. Initially, she was treated as having pneumonia, without a complete clinical response. Further investigation showed right lower lobe atelectasis associated with an extrinsic compression of the right mainstem bronchus secondary to thoracic lordoscoliosis. Therapeutic interventions involved respiratory therapy, noninvasive ventilation, cephalic traction, and orthopedic surgery, leading to clinical and functional improvement.

Transplante isolado de pulmão: experiência da Escola Paulista de Medicina

Two cases of left single lung transplantation for pulmonary fibrosis are reported, one of a 50 years old white male who is alive and well 8 months after the transplantation and the other of a 36 years old female weighing 45 kg who received an upper lobe transplantation from a 85 kg donor. This patient died of multiple organ failure in the 8th postoperative day due to complications related to an unexplained cardiac tamponade and also possible rejection but was able to maintain good ventilatory and hemodynamic conditions in the first 3 days following transplantation; radiographic studies showed adequate expansion of the transplanted lobe. Autopsy revealed no problems in the anastomotic sites and signs of difuse bronchopneumonia. In the two cases a flap of pericardial fat was utilized instead of omental flap to protect the bronchial anastomosis and in the first case a telescoping anastomotic technique was done. Both patients received corticoids since the begining and the immunossupression protocol consisted of cyclosporin, prednisone and azathioprine and had no airways complications. In spite of being an initial experience, the authors express their hope in single lung and lobe transplantation for selected terminal lung diseases and in the near future also as an alternative in the surgical treatment of complex congenital cardiac anomalies.

Langerhans cell histiocytosis

The authors present a rare case of Langerhans cell histiocytosis in a 31 year old female patient with vulvar, peri-anal and oral lesions, diabetes insipidus, pulmonary skin and bone infiltrations. Skin biopsy immunohistochemistry presented positive S100 protein and vimentin, but the diagnosis was done with the demonstration of Birbeck granules with electronic microscopy. The treatment was based on systematical chemotherapy although vulvar lesion has a bad response to chemotherapy.