Cases reported "Lung Diseases"

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1/74. Early assessment of pulmonary involvement in limited scleroderma. A case report.

    Limited scleroderma is typified by insidious progression of skin involvement. The onset of internal organ involvement is delayed until the second decade, the lungs being the most important from the prognostic point of view. Early detection of pulmonary lesions is of paramount importance. This paper presents a 16-year-old male patient with a history of Raynaud's phenomenon followed by progressive tightening of skin over the fingers, hands and face. He had early pulmonary involvement detected by high resolution computed tomography (HRCT) and proven by histopathologic examination as usual interstitial pneumonia; even the chest x-ray and pulmonary function tests were normal. A combination of prednisolone and D-penicillamine was planned for treatment because of his having both pulmonary and gastrointestinal system involvement. 99 m technetium diethylenetriamine pentaacetate (99 m Tc-DTPA) test is very sensitive for pulmonary lesions and it has shown a rapid clearance in the early stage. This method is also useful for following up the therapeutic trial.
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2/74. munchausen syndrome presenting as pulmonary talcosis.

    We describe a patient with self-induced inhalational pulmonary talcosis originally diagnosed as asthma. A 35-year-old female respiratory technologist developed severe asthma that was refractory to steroids and methotrexate. An open lung biopsy specimen showed scattered aggregates of refractile golden crystals within membranous and respiratory bronchioles. The particles ranged in size from 30 to 100 microm and were birefringent when viewed with polarized light. Following review of the lung biopsy specimen, the patient admitted to regularly inhaling large amounts of hospital baby powder. Analysis of the lung biopsy specimen and a sample of the hospital baby powder by x-ray energy dispersion showed identical spectroscopic peaks, including elemental peaks for magnesium silicate. Many patients with self-induced illness lack the picturesque symptomatology classically attributed to munchausen syndrome. awareness of these more subtle and varied patterns of presentation may aid in earlier recognition.
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3/74. Intrapulmonary ectopic liver.

    A case of intrapulmonary ectopic liver in a patient who had undergone right-sided diaphragmatic hernia is described. The intrapulmonary ectopic liver was found incidentally in chest x-ray at the age of 6 years. The final diagnosis was established at thoracotomy. Fewer than 10 cases of intrathoracic ectopic liver have been reported previously, but an intrapulmonary ectopic liver like the present case has not been described previously.
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4/74. gastric outlet obstruction and pulmonary infiltrate in a patient with Crohn's disease: successful treatment by Billroth-II-resection.

    We present a 28-year-old women with a 3 yr history of duodenal ulcers. Following four treatment attempts to eradicate helicobacter pylori she was admitted because of gastric outlet obstruction and a weight loss of 20 kg within the last two years. endoscopy and x-ray showed a circular inflammatory stenosis of the proximal duodenum extending over 8 cm. Additionally, chest x-ray showed a circumscript infiltrate in the third segment of the right lung. Mycobacterial infection could be excluded. Ileocolonoscopy and small intestinal follow-through beyond the duodenum were unremarkable, and Zollinger-Ellison-syndrome was ruled out. Bronchopulmonary histology showed intramucosal epithelioid-cell granulomas and bronchiolitis obliterans. Because the patient did not improve under conservative therapy a Billroth-II-resection was carried out. Histologically the resected specimen showed Crohn-like lesions. Postoperatively, severe peripheral arthritis was treated by steroids over 6 weeks. At follow-up the patient regained 20 kg and was free of symptoms without any medication. The pulmonary infiltrate had subsided almost completely. In summary, this extremely rare coincidence of isolated stenosing duodenal Crohn's disease and pulmonary involvement was successfully treated by Billroth-II-resection. This course of disease is compatible with the hypothesis that Crohn's disease may be maintained by antigens derived from ingested food.
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5/74. Presentation and prognosis of the shrinking lung syndrome in systemic lupus erythematosus.

    BACKGROUND AND OBJECTIVES: Systemic lupus erythematosus (SLE) may affect all the components of the respiratory system, including upper airways, lung parenchyma, pulmonary vasculature, pleura, and respiratory muscles. The shrinking lung syndrome (SLS) is a rare complication of SLE. This study describes the presenting features, investigation findings, treatment measures, and outcome of 7 patients with SLE and SLS. methods: Five patients with SLE/SLE were chosen retrospectively by examination of patient records, and 2 patients were chosen prospectively. All patients attended St. Thomas' Hospital or the Royal london Hospital between 1984 and 2001, with a total population of 2650 patients with SLE. RESULTS: Clinical features included dyspnea and pleuritic chest pain. Chest x-ray films showed small but clear lung fields, or basal atelectasis, with diaphragmatic elevation. No evidence of major parenchymal lung or pleural disease was found on the computerized tomography scan. Lung volumes were reduced on pulmonary function testing (PFT) in a restrictive pattern. Treatment of SLS included theophylline, increase in corticosteroid dosage, and intensification of immunosuppressive medication to include methotrexate or cyclophosphamide. During follow-up, 5 of 7 patients showed objective evidence on PFT of stabilization or improvement. CONCLUSIONS: The long-term prognosis of our SLS patients was reasonable, highlighting the importance of establishing a correct diagnosis and in particular differentiating it from fibrosing lung disease. Immunosuppressive therapy was helpful in stabilizing SLS and improving respiratory symptoms and PFT in some cases. RELEVANCE: SLS represents a rare complication of SLE, and it is important to be aware of its presenting features and prognosis.
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6/74. mitral valve prolapse with pulmonary haemosiderosis and severe anaemia: cause or association?

    A 12 years boy presented with the history of pallor for one month. Two days before hospitalisation he developed fever, cough, shortness of breath. He had past history of such episode.On examination, his heart rate was found to be 120/minute, respiratory rate 40/minute and moderate anaemia was detected. Scattered creptus was audible over mid and lower lung fields and a soft systolic murmur was auscultated at apex. On investigations, Hb was found as 4.6 g/dl and HbF was less than 2%. plasma Hb was 5 g/dl. Straight x-ray chest showed bilateral patchy opacities over mid and lower zones. His sputum was found to be blood stained and prompted the possibility of blood loss occurring in the lungs, which was confirmed by demonstrating haemosiderin laden macrophages on three consecutive sputum specimens. echocardiography revealed a systolic displacement of mitral valve leaflets into the left atrium with co-optation superior to the plane of mitral annulus. Doppler study showed a minimal late systolic regurgitation. It was decided to treat the case as idiopathic pulmonary haemosiderosis. oxygen inhalation, hypertonic saline nebulisation, i.v. hydrocortisone, packed cell transfusion followed by oral prednisolone improved the patient's condition. After 3 months of discontinuing prednisolone, he remained asymptomatic. Here one case of pulmonary haemosiderosis characterised by abnormal accumulation of haemosiderin in the lungs following repeated alveolar haemorrhages with the presence of mitral valve prolapse is reported.
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7/74. recurrence of idiopathic pulmonary hemosiderosis in a young adult patient after bilateral single-lung transplantation.

    BACKGROUND: Idiopathic pulmonary hemosiderosis (IPH) is a rare disease of unknown etiology characterized by hemoptysis, diffuse pulmonary infiltration, and anemia. diagnosis requires a detailed clinical history and transbronchial lung biopsy (TLB). methods AND RESULTS: A 19-year-old man developed progressive dyspnea, hemoptysis, and anemia. The chest x-rays showed bilateral opacities. IPH was diagnosed on the basis of clinical findings and TLB. The patient was treated with corticosteroidal therapy. His respiratory function worsened, and he underwent lung transplantation in 1997. The pathological examination on native lungs confirmed the previous histologic diagnosis. In 2000, the patient again developed hemoptysis, fever, and hypoxemia. A recurrence of the disease was established by TLB. CONCLUSIONS: This is the first report of recurring IPH. The possibility of recurrent IPH raises the question whether these patients should be disqualified from lung transplantation. This question is unanswerable because incidence of recurrence, time course, and impact on the graft function are presently unknown and unpredictable.
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8/74. Radiological presentation of pulmonary calcinosis: case report.

    A 64-year old man presented to the Department of radiology for evaluation. He had a seven year history of chronic cough. The chest x-ray (CXR) done showed high density diffuse cotton wool parenchymal opacities in both lungs sparing the pleura and broncho vascular bundles. These were also demonstrated on CT. This type of pulmonary calcinosis did not fit in with the classical appearances.
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9/74. Giant cell granuloma of the lung.

    In a 50-year-old man without bronchopulmonary symptoms a round mass lesion close to the hilum of the right lung was detected in a routine chest x-ray and confirmed by computed tomography. Histological examination of two biopsy specimens did not result in a definitive diagnosis. Therefore thoracotomy with enucleation of the focus was performed. The histological picture of the lesion is characterized by connective tissue proliferation, multinucleated giant cells, ossification, localised hemorrhage, deposits of hemosiderin and foci of foam cells. The findings are interpreted as a giant cell granuloma of the lung.
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10/74. Traumatic pneumatocele.

    Traumatic pneumatocele is a recognized but uncommon complication of blunt thoracic trauma. Its clinical relevance lies in the difficulty the attending surgeon encounters in differentiating the x-ray appearances from more serious pathology requiring urgent surgical intervention. We document this case because it is important that surgeons be aware of this condition and of its benign course so as to avoid unnecessary diagnostic or operative procedures.
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