1/34. High-resolution computed tomography of pulmonary alveolar microlithiasis.Pulmonary alveolar microlithiasis (PAM) is a rare disease. It has been reported predominantly in turkey. We report a case of PAM with characteristic high-resolution computed tomography (CT) findings. A 45-year-old Taiwanese woman had progressive difficulty in breathing for 7 years. Her chest radiographs showed diffuse high-density micronodules and reticular lines that obliterated the bronchovascular bundles and the margin of the heart and diaphragm. The micronodules were scattered throughout both lung fields with basal predominance. Examination of a transbronchial lung biopsy specimen showed PAM. High-resolution CT showed a unique and characteristic calcified reticular pattern and thickening of the interlobular septa of the lung parenchyma, with predominant basal and peripheral lung distribution. Reticulonodular changes of the interlobular septa and intralobular interstitial lines associated with subpleural air cysts and paraseptal emphysema were evident. These high-resolution CT findings are pathognomonic for PAM. Thus, lung biopsy may be avoided in the presence of this characteristic finding.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/34. A case of parenchymal pulmonary endometriosis.Pulmonary endometriosis is a rare disease entity and we report a 23-year-old single woman with a history of hemoptysis in association with menstruation. She was previously treated effectively with hormone therapy for 3 months, but decided to undergo surgical resection because of the high cost of hormone therapy. Radiographic finding of the chest showed haziness in the right lower lung field, and chest CT showed a ground-glass appearance in the posterobasal and laterobasal segment. The patient underwent basal segmentectomy of the right lower lobe. There was no incidence of hemoptysis during her menstruation following the operation.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/34. Pulmonary manifestation of systemic mast cell disease.Systemic mast cell disease is a rare disease of unknown aetiology. Systemic infiltration and proliferation of mast cells in skin, bone marrow, gastrointestinum and lymph nodes is the central pathological feature. This study reports a patient with mastocytosis of the skin (urticaria pigmentosa) for 10 yrs. The patient was referred to hospital for dyspnoea. Chest radiograph showed moderate reticular infiltration of both lungs, computerized tomography revealed multiple lymph nodes of the mediastinum and faint nodular lesions of middle and upper areas of lungs. Transbronchial biopsy demonstrated mast cell infiltration of the lung with formation of mast cell granuloma. According to the current literature, systemic mast cell disease with pulmonary involvement is a very rare entity. After a treatment with interferon alpha-2a over 6 months, the patient's condition and particularly dyspnoea showed improvement in parallel with an amelioration of the lesions as demonstrated by thorax computed tomography.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/34. Pulmonary alveolar lithiasis in two siblings.Pulmonary alveolar microlithiasis (PAM) is a rare disease of unknown etiology and is characterized by the deposition of calcium phosphate microliths within the alveolar airspaces. We report 2 asymptomatic siblings, a 7-year-old girl and her 13-year-old brother, with PAM. In the girl, chest X-ray and computed tomography revealed diffuse interstitial changes but no uptake of technetium 99m (99mTc) on bone scan was noted in the lung. Microliths stained pink with Papanicolaou dye in bronchoalveolar lavage fluid (BALF) but did not stain with von Kossa. In the brother, characteristic radiological findings and 99mTc uptake in the lung were detected. The microliths stained pink with Papanicolaou in BALF and black with von Kossa as well. We hypothesize that the first case is in the early phase of PAM because of lack of 99mTc uptake.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/34. 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.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/34. Primary pulmonary lymphangiectasis in a premature infant: resolution following intensive care.Primary pulmonary lymphangiectasis is a rare disease, often described as fatal within the neonatal period. We report on an infant who developed severe bilateral chylous pleural effusions with computed tomography (CT) features of pulmonary lymphangiectasis. She recovered spontaneously with supportive treatment only. We discuss aspects of her intensive care which might have contributed to the good outcome, and conclude that modern intensive care may allow spontaneous recovery in conditions described in the literature as having a very poor outcome.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/34. Familial clustering of dendriform pulmonary ossification.Dendriform pulmonary ossification is an extremely rare disease, which is usually found and analysed postmortem. Pathogenesis of pulmonary ossification is therefore still unknown. We describe two males in the same family (a 29 year-old patients and his 58 year-old father) with pulmonary ossification. The young male was symptomatic pneumothorax but his father had been asymptomatic since youth. Familial clustering of pulmonary ossifications strongly suggests a genetic predisposition for the pathogenesis. This is the first report of two cases of dendriform pulmonary ossification in the same family.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/34. Pulmonary alveolar microlithiasis--report of three cases with pulmonary function and exercise studies.Pulmonary alveolar microlithiasis is a rare disease involving accumulation of calcific concretions in the alveoli of the lungs. Three cases of this disease are reported with their varying and unusual clinical features. Radiologic studies demonstrated presence of diffuse calcific densities in the lungs with varying degrees of emphysematous changes. Bone scans showed diffuse uptake of the tracer in the lungs. Pulmonary functions showed hyperinflation and small airways dysfunction in one case besides the presence of restrictive ventilatory defect. exercise studies demonstrated arterial oxygen desaturation on exercise.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/34. Cardiopulmonary complications in multicentric reticulohistiocytosis. Report of a case.Multicentric reticulohistiocytosis (MR) is a rare disease. Only recently was its systemic nature appreciated. It affects the skin, mucous membranes, joints, muscles, tendon sheaths, synovial membranes, bones, liver, kidney, lymph nodes, heart, and lungs. Our patient, a 50-year-old woman, had life-threatening cardiopulmonary complications of MR. The connection between the skin lesions, the arthritis, and the pathologic changes in the heart and lungs is still obscure.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/34. Congenital pulmonary lymphangiectasia: CT and pathologic findings.Congenital pulmonary lymphangiectasia is a rare disease characterized by dilation of lymphatic channels without lymphatic proliferation. The disease is seen almost exclusively in infancy and early childhood. The authors report 2 cases of pulmonary lymphangiectasia. The patients were a 12- and a 25-year-old male who presented with progressive dyspnea and hemoptysis. The diagnosis was confirmed by open lung biopsy. The radiographic findings consisted of bilateral reticular changes, peribronchial cuffing, and bilateral pleural effusions. High-resolution CT demonstrated extensive bilateral septal and peribronchovascular interstitial thickening, areas of ground-glass attenuation, and bilateral pleural effusions. The histologic findings consisted of ectatic and tortuous lymphatic channels in the interlobular septa, bronchovascular sheaths, and pleura.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
| | Next -> |