Ultrasonography and computed tomography findings in pulmonary alveolar microlithiasis

Reşorlu, Mustafa; Toprak, Canan Akgün; Aylanç, Nilüfer; Karatağ, Ozan

doi:10.1055/a-0612-7974

Cited by 5 publications

(4 citation statements)

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“…Chest ultrasonography may reveal pleural thickening and irregularities as well as echogenic foci on the order of millimeters without acoustic shadowing in the subpleural area [47,55]. The absence of expected acoustic shadow artefact (also described as the "comet tail" phenomenon) is attributed to the complex pleural interface with thickened pleura, subpleural microcysts and thickened interstitium that may reduce deep penetration of ultrasound waves.…”

Section: Ultrasonographymentioning

confidence: 99%

Pulmonary alveolar microlithiasis

et al. 2020

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Pulmonary alveolar microlithiasis (PAM) is a fascinating rare lung disease that is associated with the accumulation of hydroxyapatite microliths within the lumen of the alveolar spaces. In most patients, PAM is discovered incidentally on radiographs performed for other purposes, and the typical disease course is characterised by slowly progressive respiratory insufficiency over decades. Recent genetic analyses that have revealed that the deficiency of the sodium-phosphate cotransporter NPT2B is the cause of PAM have enabled the development of powerful animal models that inform our approach to disease management and treatment. Here we review the epidemiology and molecular pathophysiology of PAM, as well as the diagnostic approach, clinical manifestations, radiographic and pathologic features, and clinical management of the disease. Although there are no proven treatments for PAM, progress in our understanding of disease pathogenesis is providing insights that suggest strategies for trials.

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Section: Ultrasonographymentioning

confidence: 99%

Pulmonary alveolar microlithiasis

et al. 2020

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“…Thoracic ultrasonography has gained increasing interest in recent years and increased thickening and irregularities of a hyper-echoic pleural line and millimetric echogenic foci with no marked acoustic shadowing in the subpleural and basal fields area has been described [41]. However, discrepancy of thoracic ultrasonography findings compared with conventional CT imaging limits its use so far [41,56].…”

Section: (Figure 3)mentioning

confidence: 99%

Pulmonary alveolar microlithiasis: no longer in the stone age

Bendstrup

Jönsson

2020

ERJ Open Res

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Pulmonary alveolar microlithiasis (PAM) is a rare parenchymal lung disease caused by variants in the SCL34A2 gene and characterised by the accumulation of intra-alveolar microliths. PAM has been reported in fewer than 1100 cases throughout the world. It is an autosomal recessive hereditary disease and often associated with consanguinity. Progress with respect to the genetic background and pathophysiology has resulted in an increased understanding of the disease in recent years. Until now, 30 genetic different SLC34A2 variants have been reported, which all are considered significant for disease development. There is no sex difference and the majority of cases are diagnosed at the age of 30–40 years. Many patients are asymptomatic and the diagnosis is made at random. When symptomatic, dyspnoea, cough, chest pain and fatigue are common complaints. The diagnosis of PAM can confidently be based on typical radiographic findings and genetic testing proving rare biallelic SCL34A2 gene variants. Bronchoalveolar lavage and histopathology may show microliths. There is no disease-specific treatment and management is supportive. Lung transplantation should be considered in advanced cases.

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“…However, they did not demonstrate acoustic shadows or comet tail artifacts of microliths. 8 It is remarkable that, despite significant pulmonary involvement in PAM, no substantial reverberation or abnormal artifacts on LUS are detected. Porzeziska et al described a case of pulmonary alveolar proteinosis in which repeated LUS findings were consistent with CT features, and speculated on the use of LUS for monitoring pulmonary alveolar proteinosis.…”

Section: Discussionmentioning

confidence: 95%

“…LUS was performed using a Philips Affinity 70 ultrasound device (Philips Healthcare, Best) using both a curvilinear probe (3)(4)(5) and linear probe (8)(9)(10)(11)(12) in the supine and sitting posture. equipped with a high-performance gradient system using a body coil.…”

Section: Equipment and Proceduresmentioning

confidence: 99%

Comparison of lung ultrasonography and magnetic resonance imaging versus computed tomography in pulmonary alveolar microlithiasis

Hizal,

Gunel,

Ozcan

et al. 2024

Pediatric Pulmonology

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Ultrasonography and computed tomography findings in pulmonary alveolar microlithiasis

Cited by 5 publications

References 0 publications

Pulmonary alveolar microlithiasis

Pulmonary alveolar microlithiasis

Pulmonary alveolar microlithiasis: no longer in the stone age

Comparison of lung ultrasonography and magnetic resonance imaging versus computed tomography in pulmonary alveolar microlithiasis

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