A case of alveolar microlithiasis: observation over 22 years and recovery of material by lavage.

Mascie‐Taylor, B H; Wardman, A.G.; Madden, C A; Page, R L

doi:10.1136/thx.40.12.952

Cited by 29 publications

(23 citation statements)

References 3 publications

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“…Cardiopulmonary exercise testing before and after lavage may have been better able to quantify the degree of response but we were not able to perform this during our patient's visit. Though total lung lavage is a recognized palliative therapy for pulmonary' alveolar proteinosis, several reports have suggested that it offers no advantage for PAM because removal of the cal cifications does not produce a coordinate clinical im provement [3][4][5]. In contrast, our patient improved clini cally after total lung lavage, although the mechanism of improvement is uncertain.…”

Section: Discussioncontrasting

confidence: 43%

“…We performed whole lung lavage incorporating vigor ous chest percussion and multiple exchanges with large tidal volumes in a symptomatic patient with PAM. Our patient demonstrated symptomatic but no functional im provement similar to results of more limited lavage proce dures [3][4][5]. We also examined the lavage fluid and obtained microliths for characterization.…”

Section: Introductionmentioning

confidence: 99%

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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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Section: Discussioncontrasting

confidence: 43%

Section: Introductionmentioning

confidence: 99%

Composition of Lung Lavage in Pulmonary Alveolar Microlithiasis

et al. 1996

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“…Nevertheless, this disease slowly develops into pulmonary fibrosis and the end stage is characterized by chronic respiratory failure and cor pulmonale [9], Radiological features reveal a widespread involvement of the pulmo nary parenchyma with microspherules of mineral density. Sometimes chest X-rays also highlight visible subpleural blebs, which may burst and worsen the clinical picture by inducing pneumothorax [13]. The densimetric study of PAM by HRCT helps to establish the various degrees of calcification.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Microlithiasis

Mariotta

Guidi²,

Mattia³

et al. 1997

Respiration

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Pulmonary alveolar microlithiasis (PAM) is a rare disease characterized by widespread localization of calcispherites in the alveolar spaces. The patients are symptomless for a long time. Nevertheless, this disease slowly develops into pulmonary fibrosis and cardiac failure. The chest X-rays and high-resolution computed tomography strongly point towards a diagnosis of PAM. As for therapeutic approaches, repeated broncho-alveolar lavages (BAL) have been performed with improvement of symptoms but without recovery, and a new oral drug treatment is still under way. We report 2 familial cases of PAM. Both patients underwent chest X-ray examination showing diffuse bilateral micronodular opacities of calcific density. After 5 years, in May 1993, one of them developed exertional dyspnoea, cyanosis, dry cough and was admitted to our Division. Cardiokinetic and diuretic drugs as well as oxygen were administered with satisfactory results. Then repeated BAL were performed. The chest X-ray after 6 months of sodium etidronate (300 mg t.i.d.) administration was unchanged.

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“…Observational studies have found therapeutic BAL to be ineffective [62][63][64], except for symptomatic improvement in one case, without improvement in conventional pulmonary radiography and HRCT [36].…”

Section: Treatmentmentioning

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

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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

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A case of alveolar microlithiasis: observation over 22 years and recovery of material by lavage.

Cited by 29 publications

References 3 publications

Composition of Lung Lavage in Pulmonary Alveolar Microlithiasis

Composition of Lung Lavage in Pulmonary Alveolar Microlithiasis

Pulmonary Microlithiasis

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

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