1/6. 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) |
2/6. Primary endobronchial actinomycosis associated with broncholithiasis.Primary endobronchial actinomycosis is an extremely rare disease that presents with endobronchial mass. We report 2 cases of primary endobronchial actinomycosis associated with broncholithiasis. There was no foreign body material, suggesting that these broncholiths were formed endogenously. Even though it is very rare, endobronchial actinomycosis should be included in the differential diagnosis of calcified endobronchial mass, especially when there is no clinical or radiological evidence of a granulomatous infection, such as tuberculosis, and the yellowish materials obstructing the bronchi are seen during bronchoscopy.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/6. Absence of pulmonary uptake of Tc-99m methylenediphosphonate in alveolar microlithiasis.Pulmonary alveolar microlithiasis (PAM) is a rare disease of unknown etiology characterized by accumulation of calcific concretions in the alveolar spaces. The paper reports a case of PAM in a 56-year-old male. The patient had persistent dry cough, and gradually progressive dyspnea on exertion. The diagnosis was established on the basis of roentgenography and confirmed by the sputum and transbronchial biopsy findings. Scintigraphy revealed the absence of Tc-99m methylenediphosphonate uptake of lungs. Familial occurrence was not observed. Chest roentgenogram, pulmonary function, and clinical status of the patient have remained stable for 41 months. Radiological and clinical follow-up of the disease continues.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/6. Familial occurrence of pulmonary alveolar microlithiasis in 3 siblings.Pulmonary alveolar microlithiasis (PAM) is a rare disease of unknown etiology characterized by intra-alveolar calcium deposits. More than 500 cases were reported in the literature. The disorder affects people at every age beginning from the early childhood. It occurs probably as a result of autosomal recessive transmission. Familial occurrence is often found with family history of the disease being present in up to 50% of the reported cases. We report PAM in 3 siblings.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/6. Elevated serum surfactant protein A and D in pulmonary alveolar microlithiasis.Pulmonary alveolar microlithiasis (PAM) is a rare disease characterized by widespread localization of calcispherites in the alveolar spaces. The authors report two cases of PAM, with markedly elevated sera concentrations of surfactant protein-A and surfactant protein-D, which showed a tendency to increase as the disease progressed. Therefore, surfactant protein-A and surfactant protein-D may function as serum markers to monitor the disease activity and progression of PAM.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/6. Pulmonary alveolar microlithiasis--a case report.Pulmonary alveolar microlithiasis (PAM) is a rare disease of unknown aetiology. It is characterized by the presence of calcific concretions (calcospherites) inside the alveoli. We present a case of a 22 year old sickle cell anaemia patient with an incidental finding of PAM. This report is to highlight the fact that though rare, PAM is not entirely unheard of in this environment and should be kept in mind as a cause of diffuse opacities of the lungs.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |