Familial Pulmonary Alveolar Microlithiasis: Four Cases from Colombia, S. A

Gómez, Gonzalo Esguerra; Lichtemberger, Egon; Santamaría, Armando; Carvajal, Lope; Jiménez-Peñuela, Benigno; Saaibí, Edmond; Barrera, A. Rey; Ordúz, C Elio; Correa-Henao, Alfredo

doi:10.1148/72.4.550

Cited by 26 publications

(9 citation statements)

References 9 publications

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“…Many of the necropsy reports on previous cases do not mention macroscopic or histological examination of the large airways (Caffrey and Altman, 1965;Meyer, Gilbert, and Kent, 1956;Abdel-Hakim, El-Mallah, Hashem, and Abdel-Halim, 1959;Waters, 1960) and in those cases with reports of bronchial examination, the bronchi were normal (Harbitz, 1918;Sharp and Danino, 1953;Sosman et al, 1957;Gomez et al, 1959;Chinachoti and Tangchai, 1957;Hotchi et al, 1970). However, Portnoy et al (1964), in their patient with the milkalkali syndrome, hypercalcaemia, and renal lithiasis as well as pulmonary alveolar microlithiasis, found evidence of slight calcification of the bronchial mucous membrane.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary alveolar microlithiasis

Sears

Chang

Taylor

1971

Thorax

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Section: Discussionmentioning

confidence: 99%

Pulmonary alveolar microlithiasis

Sears

Chang

Taylor

1971

Thorax

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“…Various reports have attempted to link PAM to external irritants. 28 One report of four cases of PAM has indicated that the condition has similarities with desert lung syndrome, a disease caused by the deposition of silica sand in the lungs. The four patients described had heavy exposure to sand particles for long periods.…”

Section: Etiologymentioning

confidence: 99%

“…However, a report from Columbia observed that among seven siblings in a family, PAM developed in four sisters who lived together but not in the three sisters who had left home at an early age. 28 This circumstantial evidence suggests the possibility of environmental factors in the etiology. A report from Thailand described the development of PAM in a patient who was addicted to snuff containing 75% organic matter with 15% silicates, and 8% calcium.…”

Section: Etiologymentioning

confidence: 99%

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Pulmonary Alveolar Microlithiasis

Prakash¹

2002

Seminars in Respiratory and Critical Care Medicine

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Pulmonary alveolar microlithiasis is a rare pulmonary disorder of unknown etiology characterized by intra-alveolar development and deposition of microliths or calcispherytes consisting of calcium phosphate. Approximately 400 cases of this rare disorder have been described in the literature. The age range of patients is from newborn to 80 years, with a mean age at diagnosis of about 35 years. No sexual predominance has been noted. In about half of the reported cases, a familial pattern has been found, with the disease usually affecting the siblings. The progression of the disease is generally very slow, some patients having been followed up for more than 30 years without evidence of change. Pulmonary function studies demonstrate a tendency toward a restrictive pattern. Chest roentgenography, high-resolution computed tomographic chest scan, and technetium-99m lung scan are useful in the diagnosis. A definitive diagnosis can be established by bronchoalveolar lavage or lung biopsy. There is no known therapy for this unusual disorder. In patients who develop progressive respiratory insufficiency and cor pulmonale, lung transplantations have been performed.

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“…Kanra ct al. [ 16] and others [17,18] have hypothesized an autosomal recessive mode of inher itance.…”

Section: Discussionmentioning

confidence: 99%

Composition of Lung Lavage in Pulmonary Alveolar Microlithiasis

et al. 1996

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A case of pulmonary alveolar microlithiasis (PAM) is reported wherein total lung lavage was performed for relief of dyspnea. Characterization of the lavage material and examination of the microliths isolated from the lavage fluid confirmed previous reports of their spherical-ovoid shape and a 2:1 calcium to phosphate composition. The microliths contained considerable amounts of ionizable iron and generated oxidants in an in vitro system. A detailed biochemical analysis of the lavage fluid reflected elevations in total protein, phosphatidylserine, phosphatidylglycerol and the ratio of phosphatidylglycerol to phosphatidylinositol. Surfactant apoprotein-A levels approximated that of normal patients. The potential roles of oxidant generation and alterations in surfactant metabolism are discussed in the context of the pathogenesis of PAM.

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Familial Pulmonary Alveolar Microlithiasis: Four Cases from Colombia, S. A

Cited by 26 publications

References 9 publications

Pulmonary alveolar microlithiasis

Pulmonary alveolar microlithiasis

Pulmonary Alveolar Microlithiasis

Composition of Lung Lavage in Pulmonary Alveolar Microlithiasis

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