Idiopathic pulmonary hemosiderosis with cystic lesions: A rare presentation

This report describes a case of a 49-year-old man with cough, recurrent hemoptysis, and dyspnea during 18 months, presenting with radiological findings of alveolar infiltrate and cystic lesions in left upper lobe. Laboratory studies revealed normocytic hypochromic anemia and normal coagulation tests. C-reactive protein and mucoproteins were negative. Serum protein electrophoresis and complement, urinalysis, serum creatinine, creatinine clearance, and 24-hour urine protein were normal. Tests for antineutrophil cytoplasmic antibodies and anti-glomerular-basement membrane antibodies were negative. Tests for connective tissue diseases were all negative. Histological findings were consistent with those of idiopathic pulmonary hemosiderosis. Radiological findings are discussed.

Use of algorithms for semi-automatic quantification of pulmonary fibrosis and emphysema

Obtaining a semi-automatic quantification of pathologies found in the lung, through images of high resolution computed tomography (HRCT), is of great importance to aid in medical diagnosis. Paraccocidioidomycosis (PCM) is a systemic disease that affects the lung and even after effective treatment leaves sequels such as pulmonary fibrosis and emphysema. It is very important to the area of tropical diseases that the lung injury be quantified more accurately. In this stud, we propose the development of algorithms in computational environment Matlab® able to objectively quantify lung diseases such as fibrosis and emphysema. The program consists in selecting the region of interest (ROI), and through the use of density masks and filters, obtaining the lesion area quantification in relation to the healthy area of the lung. The proposed method was tested on 15 exams of HRCT of patients with confirmed PCM. To prove the validity and effectiveness of the method, we used a virtual phantom, also developed in this research. © 2013 Springer-Verlag.

The Th17 pathway in the peripheral lung microenvironment interacts with expression of collagen V in the late state of experimental pulmonary fibrosis

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)

Usual interstitial pneumonia and smoking-related interstitial fibrosis display epithelial to mesenchymal transition in fibroblastic foci

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Idiopathic pulmonary hemosiderosis: case report

OBJECTIVE: To alert pediatricians about the possibillity of childhood Idiopathic Pulmonary Hemosiderosis, in cases of anemia associated with chronic lung disease. METHODS: This article documents a case of Idiopathic Pulmonary Hemosiderosis in a 6 year-old child, with histopathological documentation, and reviews it against published literature. RESULTS: A 6 year-old child with history of anemia and lung disease characterized by wheezing, recurrent pneumonia and digital clubbing was admitted to the hospital for investigation, where he suffered sudden respiratory failure and hemoptysis.He was submitted to a lung biopsy which showed a histopathological diagnosis compatible with pulmonary hemosiderosis. Therapy with high doses of corticosteroids was initiated with a good early response. After two and a half months of therapy he had a new bleeding episode, culminating in death. CONCLUSIONS: Idiopathic Pulmonary Hemosiderosis should be included as a possible diagnosis of children with anemia and chronic lung disease. This case is a good example.

Avaliação da utilização de biópsia transbrônquica em pacientes com suspeita clínica de doença pulmonar intersticial

OBJETIVO: Estudar os padrões clínicos, radiológicos e histopatológicos da biópsia transbrônquica (BTB) utilizados para a confirmação diagnóstica em pacientes com suspeita clinica de doença pulmonar intersticial (DPI) atendidos em um hospital universitário de nível terciário. MÉTODOS: Os prontuários, laudos radiológicos e de biópsias transbrônquicas de todos os pacientes com suspeita de DPI submetidos a BTB entre janeiro de 1999 e dezembro de 2006 no Hospital das Clínicas de Botucatu, localizado na cidade de Botucatu (SP), foram revisados. RESULTADOS: Foram incluídos no estudo 56 pacientes. Desses, 11 (19,6%) apresentaram o diagnóstico definitivo de fibrose pulmonar idiopática (FPI), que foi significativamente maior nos casos nos quais DPI era uma possibilidade diagnóstica em comparação com aqueles nos quais DPI era a principal suspeita (p = 0,011), demonstrando a contribuição da BTB para a definição diagnóstica dessas doenças. O exame histopatológico dessas biópsias revelou que 27,3% dos pacientes com FPI apresentavam o padrão de pneumonia organizante, o que pode sugerir doença mais avançada. O padrão histológico indeterminado foi o mais frequente, refletindo a característica periférica da FPI. Entretanto, o padrão fibrose apresentou alta especificidade e alto valor preditivo negativo. Para os padrões sugestivos de FPI em TC, a curva ROC indicou que a melhor relação entre sensibilidade e especificidade ocorreu com a presença de cinco alterações radiológicas, sendo o aspecto de favo de mel fortemente sugestivo de FPI (p = 0,01). CONCLUSÕES: Nas DPIs, a TC de tórax deve ser sempre realizada e a BTB usada em situações individualizadas, conforme a suspeita e distribuição das lesões.

Experimental Basis and New Insights for Cell Therapy in Chronic Obstructive Pulmonary Disease

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Phase I clinical trial of cell therapy in patients with advanced chronic obstructive pulmonary disease: follow-up of up to 3 years

BACKGROUND Chronic obstructive pulmonary disease is a major inflammatory disease of the airways and an enormous therapeutic challenge. Within the spectrum of chronic obstructive pulmonary disease, pulmonary emphysema is characterized by the destruction of the alveolar walls with an increase in the air spaces distal to the terminal bronchioles but without significant pulmonary fibrosis. Therapeutic options are limited and palliative since they are unable to promote morphological and functional regeneration of the alveolar tissue. In this context, new therapeutic approaches, such as cell therapy with adult stem cells, are being evaluated.OBJECTIVE This article aims to describe the follow-up of up to 3 years after the beginning of a phase I clinical trial and discuss the spirometry parameters achieved by patients with advanced pulmonary emphysema treated with bone marrow mononuclear cells.METHODS Four patients with advanced pulmonary emphysema were submitted to autologous infusion of bone marrow mononuclear cells. Follow-ups were performed by spirometry up to 3 years after the procedure.RESULTS The results showed that autologous cell therapy in patients having chronic obstructive pulmonary disease is a safe procedure and free of adverse effects. There was an improvement in laboratory parameters (spirometry) and a slowing down in the process of pathological degeneration. Also, patients reported improvements in the clinical condition and quality of life.CONCLUSIONS Despite being in the initial stage and in spite of the small sample, the results of the clinical protocol of cell therapy in advanced pulmonary emphysema as proposed in this study, open new therapeutic perspectives in chronic obstructive pulmonary disease. It is worth emphasizing that this study corresponds to the first study in the literature that reports a change in the natural history of pulmonary emphysema after the use of cell therapy with a pool of bone marrow mononuclear cells.

Quantificação de fibrose e enfisema em pacientes com paracoccidioidomicose (PCM) pulmonar, através de imagens de tomografia computadorizada de alta resolução (TCAR)

Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)

Contribuição da terapia celular com células tronco em fibrose em ratos

Pós-graduação em Pesquisa e Desenvolvimento (Biotecnologia Médica) - FMB

Comparação do cotrimoxazol com o itraconazol no tratamento da paracoccidioidomicose

Pós-graduação em Doenças Tropicais - FMB

Remodelamento parenquimatoso pulmonar em dois modelos experimentais de fibrose

Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)