Electron Microscopic Studies on Pulmonary Alveolar Microlithiasis

Kurabayashi, Masahiko; Satō, Shigeru; Takishima, Tamotsu

doi:10.1620/tjem.126.343

Cited by 16 publications

(7 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…37 Results of one study imply that the microliths grow by surface deposition and coalesce during early stages of formation. 38 The alveolar reaction consists of deposition of microscopic layer upon layer of salts made of calcium phosphate salts. Whether all the calcium and phosphate salts are exuded into the alveoli from pulmonary capillaries or develop from other mechanisms is unknown.…”

Section: Pathogenesis and Pathologymentioning

confidence: 99%

See 1 more Smart Citation

Pulmonary Alveolar Microlithiasis

Prakash¹

2002

Seminars in Respiratory and Critical Care Medicine

View full text Add to dashboard Cite

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.

show abstract

Section: Pathogenesis and Pathologymentioning

confidence: 99%

“…3, 4). 38 The cross-section of a microlith shows a laminated appearance with the core of the microlith staining pink with PAS (pe-riodic acid-Schiff ) stain (see Fig. 1).…”

Section: Pathogenesis and Pathologymentioning

confidence: 99%

Pulmonary Alveolar Microlithiasis

Prakash¹

2002

Seminars in Respiratory and Critical Care Medicine

View full text Add to dashboard Cite

show abstract

“…In the subpleural region, diffuse interstitial fi brosis was most extensive, with emphysema-tous cysts up to 5 mm in diameter. Electron microscopy [17,28] revealed elastosis in the alveolar interstitial tissue and collagen depo sition in the bronchiolar epithelial cells and apart from the microlithiasis, there were no specific changes on electron microscopy. The characteristic microliths (calcosphèrites) are formed of apatite (calcium fluorophosphate).…”

Section: Casementioning

confidence: 95%

Pulmonary Alveolar Microlithiasisc

Lulu¹,

Humad²,

Anzi³

1996

Med Principles Pract

View full text Add to dashboard Cite

show abstract

“…Typical findings within the alveolar spaces are numerous calcified bodies, concentrically laminated with an onion skin-like appearance. The microliths usually range from 50 to 1,000 mm in diameter [22,25,[35][36][37][38][39], although microliths up to 5,000 mm in size have been reported [40]. The microliths are mainly composed of calcium and phosphorus and, to a lesser degree, of varying amounts of iron, magnesium, potassium and copper.…”

Section: Pathological Characteristicsmentioning

confidence: 99%

“…Some authors have reported survival of .20 yrs [3,6,10,18,39,52,58,62,64,67,71], and only a few cases with a follow-up period of .40 yrs after the diagnosis have been reported [10,12,22]. However, most cases only have up to 10 yrs of followup time after the initial diagnosis [3, 6, 9, 23, 25, 31, 36, 38, 47, 48, 54, 56, 58, 59, 61, 66, 68-70, 72, 73].…”

Section: Long-term Coursementioning

confidence: 99%

Pulmonary alveolar microlithiasis: two case reports and review of the literature

Jönsson

Simonsen

Hilberg

et al. 2012

European Respiratory Review

View full text Add to dashboard Cite

Pulmonary alveolar microlithiasis is a rare diffuse lung disease characterised by deposition of calcium phosphate within the alveolar airspaces. The disease is usually discovered from birth up to 40 yrs of age and is often diagnosed incidentally during radiography of the chest for other reasons. Many patients are asymptomatic and the majority of patients either have normal or restrictive pulmonary function. The clinical course of the disease varies. While it remains static in some patients, it progresses into pulmonary fibrosis, respiratory failure and cor pulmonale in others. With the exception of lung transplantation, there is no known effective treatment for the disease. Although the aetiology remains unclear, mutations of the solute carrier family 34 (sodium phosphate), member 2 gene (the SLC34A2 gene), which encodes a sodium/phosphate cotransporter, are considered to be the cause of the disease.We present two cases of pulmonary alveolar microlithiasis with different mutations in the SLC34A2 gene that have not been previously described, and a review of the literature.KEYWORDS: Alveolar microlithiasis, genetic polymorphisms, type IIb sodium-dependent phosphate co-transporter P ulmonary alveolar microlithiasis (PAM) is a rare diffuse lung disease. It was first named in 1933 by PUHR [1], and is characterised by deposition of spherical calcium phosphate concretions (microliths) in the alveoli [2][3][4]. Although the aetiology remains unclear, PAM is thought to be an autosomal recessive disease caused by mutations of the solute carrier family 34 (sodium phosphate), member 2 gene (the SLC34A2 gene), which encodes a sodium phosphate cotransporter [5].Herein, we present two cases of PAM with different mutations in the SLC34A2 gene that have not been previously described and, in addition, we review the literature. CASE ONEA 49-yr old male from Denmark was referred to the Dept of Respiratory Medicine, Aarhus University Hospital (Aarhus, Denmark) for diagnostic investigation in April 2005. The patient was adopted, and therefore the family history was unknown. He was complaining of fatigue and progressively worsening dyspnoea over the last 4 yrs. He had no cough or sputum. He was a heavy smoker (30 pack-yrs).Upon first admission, the ECG showed signs of serious ischaemia and he was transferred to the

show abstract

Electron Microscopic Studies on Pulmonary Alveolar Microlithiasis

Cited by 16 publications

References 4 publications

Pulmonary Alveolar Microlithiasis

Pulmonary Alveolar Microlithiasis

Pulmonary Alveolar Microlithiasisc

Pulmonary alveolar microlithiasis: two case reports and review of the literature

Contact Info

Product

Resources

About