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A Bugalho de Almeida
Revista portuguesa de pneumologia 17(3):107-8. · 0.27 Impact Factor
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ABSTRACT: Despite aggressive treatment, sarcoidosis may be debilitating and progressive. The role of tumor necrosis factor (TNF)-a in the genesis of granulomas is ambiguous. It has proven to be critical in the formation and maintenance of granulomatous inflammation and its antagonist, Infliximab, has therefore been used with success in the treatment of patients with sarcoidosis. There are, however, reports of onset of sarcoidosis in patients in treatment for other conditions and that had no outbursts before submission to this therapy. We used Infliximab in the treatment of patients with sarcoidosis who either didn't respond to corticosteroids and other conventional drugs or developed unacceptable side effects to these drugs. The initial dose was 5mg/Kg body weight and subsequent doses were given at weeks 2, 4 and then every other 8 weeks for a total period of one year. We treated ten patients with biopsy proven sarcoidosis, five men and five women, with a mean age of 47.1 years ranging from 28 to 63 years of age. Three patients had severe neurological symptoms, two had hepatic cirrhosis, one had granulomatous inflammation of the lachrymal gland and had already been submitted to many surgeries, one had extensive pulmonary involvement (stage III), one had disfiguring lupus pernio and two presented disabling cutaneous nodules. In four patients the dosage of corticosteroids or other immunosuppressive drugs was suspended, in three the dosage was reduced and in one, corticosteroids were added to the Infliximab therapy. In five of the patients there was a significant improvement. One of the patients with neurological symptoms displayed a complete recovery, while another had significant improvement of vision deficit enabling her to read again. Two patients withdrew from therapy, one due to lack of improvement of neurological symptoms and the other due to the onset of organizing pneumonia spawned by Infliximab. Two patients developed anti-histone antibodies during treatment. Infliximab seems effective in treating patients who are either refractory or develop side effects to a standard regimen of corticosteroids and immunosuppressive agents. These patients, treated with Infliximab, should be under tight surveillance in order to quickly identify possible secondary effects.
Revista portuguesa de pneumologia 17(2):85-93. · 0.27 Impact Factor
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ABSTRACT: Lung cancer is the leading form of cancer death worldwide. Cancer patients are at a high risk of developing a second cancer. The present study attempts to determine the characteristics of a population with lung cancer diagnosed with another cancer. We analysed records of patients from the Department of Lung Oncology of our hospital from 2000 to 2007 who were identified as having two or more tumours. We found 4.2% (n=44) multiple cancers among the registered cases (n=1046), 88.6% males, (high) mean age 70.1+/-10 years old. About 86% (n=38) of the patients were smokers or ex -smokers. From the patients with record of family history, 65.4% (n=17) had relevant family history of cancer. The majority of the first malignancy diagnosed was prostate, colon, head and neck and bladder. Lung cancer was essentially the second malignancy. The mean time lag between the two diagnoses was 62.9+/-64.9 months (max. 240, min. 0), with the second cancer usually detected at an advanced stage. The mean survival of patients who had a second primary lung cancer was 8.6+/-8.24 months (max. 32, min. 1), with four patients still surviving. Our results suggest that extended follow -up is needed in these patients, using screening strategies which follow international recommendations, and with control of carcinogenic risk factors such as smoking. We suggest a tailored risk algorithm and a further study to assess if there are particular molecular markers in these patients.
Revista portuguesa de pneumologia 16(3):391-405. · 0.27 Impact Factor
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ABSTRACT: Epipericardial fat necrosis is an uncommon benign entity of unknown cause, with only 20 cases reported in the English-language literature. It should be remembered as a possible diagnosis in a person who presents with acute pleuritic chest pain and paracardiac density or pleural effusion on chest radiography (X-ray). The computed tomography (CT) or surgical approach allows the final diagnosis and characterization. The authors describe a case of a 48-year-old woman, admitted to our department because of complaints of fatigue, cough and sudden pleuritic pain on thoracic left side. The chest X-ray showed mild/moderate left pleural effusion and CT scan revealed local slight thickening of pericardium and epipericardial fat, surrounded by thick rim of higher density in the left cardiophrenic space. This made the diagnosis of epipericardial fat necrosis. During hospitalization the patient remained stable and was discharged home with symptomatic relieve therapy. Because of benign, self-limited nature of this entity, only conservative treatment is indicated.
Revista portuguesa de pneumologia 16(3):507-12. · 0.27 Impact Factor
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ABSTRACT: Malignant peripheral nerve sheath tumors comprehend a rare group of soft tissue sarcomas that tend to occur in patients with neurofibromatosis type 1 or several years after radiotherapy treatments. Its thoracic localization is a very unusual entity. The typical symptoms are due to nerve roots compression which can persist for several months or years before diagnosis. Due to very few patients with this type of tumor its therapeutic approach is still a matter of permanent debate, being surgery the main treatment. This tumor has a bad prognosis because of high local recurrence and metastasis. The clinical case we describe serves as a glimpse for discussion.
Revista portuguesa de pneumologia 16(3):483-92. · 0.27 Impact Factor
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ABSTRACT: to characterise outpatients of a Portuguese central hospital diagnosed with organising pneumonia (OP) and compare results with current literature.
medical processes with diagnosis of OP were retrospectively studied as to demographics, aetiology, clinical and radiological features, average time until and date of diagnosis, laboratory and histological changes, treatment and relapse. Results - thirteen patients with a mean follow -up of 171.6 weeks (max 334 and min 28 weeks) were evaluated. Nine of these patients (70%) had cryptogenic OP (COP) while 30% had secondary OP (SOP), two with rheumatoid arthritis, one with dermatomyositis and another undergoing radiotherapy for breast cancer. Mean age was 55.6 (+ -15.3 years), 92% female, 77% were non -smokers. Average time until diagnosis was 77.2 weeks (min 3 and max 432 weeks). Symptoms at presentation were tiredness (92%), cough (85%), fever (65%), shortness of breath (54%), thoracic pain (23%) and weight loss (23%). At the time of diagnosis, the mean erythrocyte sedimentation rate was 70mm (max 170mm and min 16mm). C -reactive protein level was increased in eight patients. Significant leucocytosis was absent. Chest X -ray and chest CT scan showed bilateral distribution in 12 patients (92%). Consolidation with an air bronchogram was present in 12 patients and in four (31%), consolidation was migratory. Four patients (30%) underwent transbronchial pulmonary biopsy, all uncharacteristic and eight patients surgical pulmonary biopsy, four showed histological confirmation of SOP. Corticosteroids were started in 11 patients and average treatment was 61.6 weeks (16-288 weeks). 15% (2/13) had spontaneous resolution. Four patients (31%) relapsed, one of them five times. Two patients are dependent on a low dose of corticosteroids, one due to underlying disease and another due to multiple relapses. Therapy of relapse was corticosteroids alone in minimum effective dosage or associated to azathioprine or ciclosporin.
such a high incidence in females (92%) may be explained by the limited sample of patients. In 70% of the patients diagnosis were established by clinical and radiology criteria. Mean time to diagnosis was very variable which suggests that in some cases the disease was not diagnosed and treated as another interstitial lung disease or as recurrent pneumonia. Most patients (53.8%) had a favourable clinical course after treatment with corticosteroids with a very low number of relapses (30.8%), much lower than described by other authors (60%). Only in experienced centres should the diagnosis of OP established by clinical and radiological criteria.
Revista portuguesa de pneumologia 16(3):369-89. · 0.27 Impact Factor
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ABSTRACT: Respired particles accumulated in the epithelial regions of trachea and bronchi were identified and characterised using micro-PIXE elemental mapping of thin frozen sections carried out at the Oxford Nuclear Microprobe facility. Isolated particles with diameters of 2–10 μm could be detected, mainly at the trachea epithelial surface. In bronchi respiratory mucosa, granular regions can also be observed that may correspond to particle agglomerations (2–4 μm diameters) and/or inclusions in macrophages. Particles, observed in the upper regions of the respiratory tract consist mainly of earth crust elements such as Al, Si, Ca and Fe. Occasionally, Ti and Zn are also present. Particles observed in the bronchi have a more varied chemical composition. Elements such as V, Cr, Mn, Fe, Cu, Zn were detected, mainly in association with S, K, Ca or Si.
Nuclear Instruments and Methods in Physics Research Section B Beam Interactions with Materials and Atoms 158:499-504. · 1.21 Impact Factor
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ABSTRACT: The authors present the case of a lymphoproliferative disorder (LPD) with pleuro-pulmonary involvement. It was a very aggressive primary gastric lymphoma of the MALT subtype, diagnosed in an 86-year-old woman admitted in Pulmonology ward at our Hospital with pleural effusion. Helicobacter pylori search was negative, what is infrequent. The disease was already disseminated at the time of the diagnosis and did not respond to various treatment modalities, what is also an uncommon finding. Clinical presentation, diagnosis and management of this disease are reviewed, pointing out its unique features and comparing the particular aspects of this case with the published literature. The authors stress that LPD is an important diagnosis to be held in mind in the case of a pleural effusion of unknown aetiology.
Revista portuguesa de pneumologia 15(4):713-20. · 0.27 Impact Factor
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ABSTRACT: The fate of respired particles in the respiratory system is inferred through the chemical characterisation of individual particles at the tracheal and bronchial mucosas, and the accumulation of toxic elements in lung alveoli and lymph nodes. The particles and tissue elemental distributions were identified and characterised using micro-PIXE elemental mapping of thin frozen sections using the ITN Nuclear Microprobe facility. Significant particle deposits are found at the distal respiratory tract. Al, Si, Ti, V, Cr, Fe, Ni, Cu and Zn are elements detected at these accumulation areas. The elemental distributions in the different cellular environments of lymph nodes vary. The major compartments for Al, Si, Ti, Fe and Cr are the phagocytic cells and capsule of lymph nodes, while V and Ni are in the cortex and paracortex medullar areas which retain more than 70% of these two elements, suggesting high solubility of the latter in the cellular milieu. The elemental mobilisation from particles or deposits to surrounding tissues at the respiratory ducts evidences patterns of diffusion and removal that are different than those for elements in the respiratory tract. Mobilisation of elements such as V, Cr and Ni is more relevant at alveoli areas where gaseous exchange takes place. The apparent high solubility of V and Ni in the respiratory tract tissue points towards a deviation of the lymphatic system filtering efficiency for these elements when compared to others.
Nuclear Instruments and Methods in Physics Research Section B Beam Interactions with Materials and Atoms 181:499-505. · 1.21 Impact Factor
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ABSTRACT: Intravascular lymphoma is a very rare form of large B cell non-Hodgkin's lymphoma, characterised by the presence of lymphoma cells in the lumina of small vessels only, particularly in the capillaries. We report a 54 year-old female non-smoker, admitted to hospital for further examination of a four month long clinical condition involving high fever, night sweats, unqualified weight loss and progressive dyspnea. Patient's temperature was 38.5 degrees C, pulse 100/min and respiratory 22 cycles/min. Patient's haemoglobin was 9.4 g/dL, she had leukocytosis, elevated LDH and arterial blood gas analysis with moderate hypoxaemia (FiO2 1l/m: PaO2-63.6 mm Hg). Chest X-ray revealed diffuse interstitial changes. All the possible causes of unknown origin fever were excluded. Diagnosis was made through lung biopsy and treatment with combined chemotherapy and rituximab was prescribed leading to a 48 hours clinical remission. We present this case to show how difficult this diagnosis can be and how a good response to therapy is possible.
Revista portuguesa de pneumologia 14(6):857-68. · 0.27 Impact Factor
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ABSTRACT: We conducted a review of asthma hospital admissions in mainland Portugal 2000-2007 to evaluate if one of the aims of the National Asthma Control Programme, a 20% reduction in hospital admissions in children and adolescents in 2007, had been achieved. Using the Health Services Central Administration data base we reviewed 24271 admissions and saw that 48.7% of patients admitted were aged under 19 years old and that 61.8% were aged 40 years old or under. Mean global annual asthma hospital admission rate was 30.79 per 100,000 inhabitants. Mean global hospital stay was 5.94 days, range 2.93 - 9.73 days, depending on the age group. We saw a drop in admission in both the northern and the Lisbon and Tagus Valley regions over the 8 year period. 189 patients died, 25 of who were aged 40 years old or under. The mortality rate ranged from 0.162 in 2002 to 0.324 per 100,000 inhabitants in 2005, with a mean patient death of 0.77 patients per 100 admissions. Mean annual costs were in excess of euro3.3 million, meaning an estimated annual financial asthma burden of around euro117.5 million in Portugal. We concluded that many asthma sufferers in Portugal do have sufficient disease controlled. Improved management would equal better quality of life and reduced disease costs.
Revista portuguesa de pneumologia 15(3):367-83. · 0.27 Impact Factor
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ABSTRACT: Several studies have demonstrated that obstructive sleep apnoea syndrome (OSAS) patients have a higher rate of road traffic accidents. Our study aimed to analyse any differences in OSAS patients between those who reported having had road traffic accidents and/or near misses and those who did not.
We studied 163 patients with OSAS (apnoea- hypopnoea index (AHI)>10/h) diagnosed using nocturnal polysomnography (NPSG), all drivers, 18.4% of whom drove for a living. Patients were asked at their first clinical interview to self-report road traffic accidents and/or near misses over the past 3 years which had been caused by abnormal daytime drowsiness. This allowed patients to be divided into two groups, those who had had road traffic accidents and/or near misses and those who had not. Both were compared as to age, body mass index (BMI), Epworth Sleepiness Scale (ESS), daytime PaO2 and PaCO2, Functional Outcomes of Sleep Questionnaire (FOSQ) test and NPSG data. This latter was total sleep time (TTS), sleep efficiency, sleep stages, arousal index (ARI), AHI, minimal and average SaO2, % of time with SaO2 < 90% (T90), desaturation index (ODI), total duration of apnoea-hypopnoea (TDAH) (T test).
Group I (no road traffic accidents) No=89 patients; group II (road traffic accidents) No=74 patients. Age (years) was 57.6+/-11.8 vs. 54.7+/-10.9 (ns); male gender, 75% vs. 78.4%; ESS, 12.3+/-5.4 vs. 17.6+/-4.3 (p<0.001); BMI, (Kg/m2) 36.2+/-8.1 vs. 35.6+/-6.3 (ns); PaO2 (mmHg), 76.1+/-11.4 vs. 78.5+/-12.6 (ns); PaCO2 (mmHg), 42.6+/-5.1 vs. 42.2+/-4.7 (ns); FOSQ, 15.1+/-3.1 vs. 12.9+/-3.4 (p<0.001). NPSG data revealed differences only in AHI: 45.0+/-21.6 vs. 56.2+/-29.7 (p=0.01) and in TDAH (minutes), 98.5+/-63.7 vs. 133.3+/-83.2 (p=0,005).
In our experience patients who had road traffic accidents and/or near misses had a more severe OSAS, with higher AHI, excessive daytime sleepiness and lower quality of life.
Revista portuguesa de pneumologia 15(3):419-31. · 0.27 Impact Factor
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ABSTRACT: Smoking is an important cause of pulmonary pathology and this addiction can be regarded as a chronic, recurrent disease. The benefits of smoking cessation are unquestionable and all physicians should become more active and assertive in recommending it.
To characterise the population seeking medical support for smoking cessation and understand why some successfully stop smoking and others do not.
Retrospective analysis of medical records of outpatients in follow-up between January 2003 and June 2006. Age, gender, age at smoking initiation, smoking burden (number of pack-years), associated diseases, degree of dependence (Fagerström test for nicotine addiction), prior attempts at and motivation for smoking cessation, need for cognitive/behavioural support and success and abandonment rates were evaluated.
Five hundred and twenty six patients were studied, 50% male with an average age of 45.5+/-11.4 years. Almost half (43.1 %; n=227) of the patients started smoking before the age of 15. Average smoking burden was 35.8+/-20 pack-years although 21.4% (n=113) smoked more than 50 pack-years. Respiratory disease was present in 52.1% (COPD, 39.9% and others, 12.2%) and cardiovascular disease in 14.6% of the patients. In 46% of patients (n=242) a relevant psychiatric disorder was identified; depression (21.4%), anxiety disorder (19.4%), other dependencies (2.1%) bipolar disorder (1.5%) and schizophrenia (0.6%). The evaluation of degree of addiction revealed maximum level in 69.7% of the patients (n=380). Many patients (72.2%; n=380) reported prior attempts to quit smoking. The strongest reasons for giving up smoking were concern over health (83.5%), financial issues (8.2%) and search for better quality of life (5.7%). Most patients (81.7%; n=430) had undergone nicotine replacement therapy; skin patches (53.3%), chewing gum (1.1%) or both (45.6%). Psychopharmacological treatment included administration of sedative-hypnotics (86.5%), bupropion hydrochloride (2.3%) and antidepressants (0.6%). Seventy six patients (14%) benefited from cognitive/ behavioural support. Two hundred and twenty three patients (42.4%) were successful in giving up smoking while 219 (41.6%) abandoned follow up, the majority after the first appointment. Most patients that abandoned follow up reported lack of motivation and the price of therapy.
The population under study had a high rate of psychiatric disorders and a high level of dependence and lack of motivation that might justify the drop-out rate. Successful treatment was associated with close follow up, behavioural support and pharmacological therapy.
Revista portuguesa de pneumologia 15(2):179-97. · 0.27 Impact Factor
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ABSTRACT: Ehlers-Danlos syndrome (cutis hyperelastica), is a group of connective tissue disorders characterized by abnormalities of the skin, ligaments and internal organs. It is a hereditary syndrome, usually with autossomal dominant inheritance; that primarily affects the collagen synthesis. The skin and blood vessels are extremely fragile and elastic. The skin is soft with rubber consistency and easily bruising. There are hypermobile joints with increased extensibility. We summarize the case of a sixteen year old boy with a history of joint hypermobility since childhood and splenic fracture that was diagnosed with Ehlers-Danlos syndrome after the occurrence of recidivant spontaneous pneumothorax. We present the most common pulmonary complications of Ehlers-Danlos syndrome and discuss the importance of not forgetting the least commons etiologies of pneumothorax, in cases of spontaneous pneumothorax.
Revista portuguesa de pneumologia 12(4):471-80. · 0.27 Impact Factor
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ABSTRACT: Asthma control is a key point in patient management. GINA's most recent report emphasises the need to investigate uncontrolled asthma, of which non-compliance with treatment, COPD, smoking, chronic sinusitis, gastroesophageal reflux disease and obesity are the usual causes. The aim of this work is to evaluate the role of pulmonary thromboembolism (PTE) in cases of difficult- -to-treat asthma. We reviewed the case reports of patients with severe persistent asthma followed in our Asthma Outpatients Clinic between 2004 and 2006. We selected the ones that maintained uncontrolled disease despite an optimal therapeutical approach and investigated the causes. In this group (n=254), 28 (11%) had severe persistent asthma and their mean age was 44 +/- SD18 years old. 86% were females. Of these, 57% (n=16) had uncontrolled disease: 35% (n=6) due to non-compliance with treatment; 29% (n=5) pulmonary thrombombolism (scintigraphic confirmation); 12% (n=2) severe rhinosinusitis; 6% (n=1) hypereosinophilic syndrome; 6% (n=1) persistent allergen exposure and 6% (n=1) are still being investigated. Patients with TPE (mean age 56 +/- SD9 years old; 80% females; 80% Caucasians) were diagnosed with asthma as adults (mean age 37 +/- SD14 years old). The mean time until the diagnosis of TPE was 18 +/- SD12 years. Predisposing factors for TPE were venous insufficiency (40%), hypertension (40%) and deficit of functional protein C and S (20%). All these patients received anticoagulant therapy (80% are still medicated). It should be noted that after the beginning of anticoagulants, 40% of the patients achieved control of their asthma and 40% have partially controlled disease. There were no hospital admissions for asthma exacerbations after the beginning of anticoagulation in this group. This study supports the inclusion of TPE in the group of comorbidities to consider while investigating uncontrolled asthma.
Revista portuguesa de pneumologia 13(6):775-87. · 0.27 Impact Factor
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ABSTRACT: Transbronchial needle aspiration was initially invented in 1949 by Schieppati. After its adaptation to the flexible bronchoscope in 1983 by Wang this technique has gain firm indications in the diagnosis and staging of lung cancer, in peripheral pulmonary nodules and masses; in the evaluation of endobronchial masses; in the disease of submucosal, in benign diseases, i.e. sarcoidoses and mediastinal cysts and abscesses. The yield of this technique published in the literature makes it more than useful. The material available has different indications and usefulness in different clinical settings. Despite the almost absence of complications this procedure is yet underutilized, in spite of its twenty years of results which may be due to the established routines and the lack of training.
Revista portuguesa de pneumologia 11(3):307-19. · 0.27 Impact Factor
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ABSTRACT: We herein report a case of a male patient, who presented a mediastinal mass that had a slow growth over the years. After surgery, that made the diagnosis of idiopathic mediastinal fibrosis, the growth stabilized. Four years latter it was observed a substantial increase which lead to the inevitable compression of vital structures which, in turn, led to pulmonary hypertension. Idiopathic mediastinal fibrosis is an extremely rare pathology. There are same cases in which it is associated with other pathologies but has always a fatal prognosis when surgery is not an option.
Revista portuguesa de pneumologia 11(2):155-63. · 0.27 Impact Factor
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ABSTRACT: Whole lung lavage is a technique that was developed in the 1960s with the purpose of removing lipoproteinaceous material that accumulates in the bronchi of patients with alveolar proteinosis, leading to clinical and functional improvement. There has been an evolution in the technique; initially it was performed under local anesthesia to each segment of the lung and currently it is performed under general anesthesia sequentially to both lungs. This review describes the whole lung lavage, its major indication, alveolar proteinosis and some data on the experience of our hospital.
Revista portuguesa de pneumologia 15(1):77-88. · 0.27 Impact Factor
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ABSTRACT: Chronic obstructive pulmonary disease (COPD) is highly prevalent and its pathogenesis is still not completely clarified. Clinically stable patients (n=21) and healthy subjects (n=24) were studied for blood markers of oxidative injury and antioxidant status. The plasma concentration of protein carbonyls was significantly increased in COPD patients, both ex-smokers (0.76 +/- 0.28 nmol mg(-1)) and smokers (0.99 +/- 020 nmol mg(-1)) versus controls (0.49 +/- 0.14 nmol mg(-1)) . The concentration of total thiols was slightly enhanced in plasma of the COPD patients (ex-smokers 492 +/- 23 micromol 1(-1) and smokers 505 +/- 36 micromol 1(-1) versus controls 450 +/- 67 micromol 1(-1); p < 0.05). The activity of the antioxidant enzyme superoxide dismutase was increased in erythrocytes (activity in U g(-1) haemoglobin; ex-smokers 4460 +/- 763 and smokers 4114+/- 1060 versus 3015 +/- 851 in controls; p > 0.01), while glutathione peroxidase activity was decreased in total blood (activity in U g(-1) haemoglobin: ex-smokers 27 +/- 9 and smokers 23 +/- 9 versus 47 +/- 25; p < 0.01). Lower levels of selenium in plasma were also found for COPD patients (concentration in mg 1(-1): ex-smokers 0.030 +/- 0.019 and smokers 0.032 +/- 0.024 versus 0.058 +/- 0.023 in controls; p < 0.01), being more evident in those with very low levels of arterial oxygen pressure. In addition, the levels of potassium and rubidium were increased in blood cells of the patient group. All these changes might reflect oxidant damage and an altered electrolytic homeostasis, and can be interpreted as markers of COPD rather than as indicators of smoking habits.
Biomarkers 9(6):461-9. · 2.21 Impact Factor
