High resolution computed tomographic features of pulmonary alveolar microlithiasis

Deniz, Ömer; Örs, Fatih; Tozkoparan, Ergün; Özcan, Ayhan; Gümüş, Seyfettin; Bozlar, Uğur; Bilgiç, Hayati; Ekız, Kudret; Demirci, Necmettin

doi:10.1016/j.ejrad.2005.01.010

Cited by 55 publications

(67 citation statements)

References 18 publications

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“…Each lobe appears surrounded by a fine dense outline like a 'stony lung'. Additionally, findings such as interlobular septal thickening, which can be seen in interstitial lung diseases, ground-glass appearance, peribronchovascular interstitial thickening, and parenchymal bands, may be observed in HRCT examination of the patients [4,6].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Alveolar Microlithiasis and Preterm Delivery: A Case Report

Erdem¹,

Göktan²,

Erbay³

et al. 2014

Tur Toraks Der

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Pulmonary alveolar microlithiasis (PAM) is a rare, chronic lung disease characterised by extensive intra-alveolar calcium and phosphorus deposition throughout the parenchyma of both lungs. The etiology and pathogenesis of the disease is unclear. In our case, PAM with pregnancy was presented; respiratory distress had been observed during the first and second trimesters. In the following weeks, premature activity developed and the foetus was not able to stay alive. This is the third case of PAM in pregnancy described in the literature. A 36 year-old female was referred to our emergency department complaining that she had suffered from dyspnoea on a couple of occasions since the first trimestry of her last pregnancy. On suspecting a pulmonary embolism, dynamic thorax multidetector computed tomography (MDCT) was performed after the delivery. MDCT revealed typical PAM findings. In PAM, radiological signs are not compatible with clinical symptoms; thus, the clinical symptoms are not as dramatic as imaging findings. However, it should be taken into consideration that this disease, with no effective treatment, may rarely progress into end stage pulmonary disease due to conditions which alter pulmonary functions, such as pregnancy.

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Section: Discussionmentioning

confidence: 99%

Pulmonary Alveolar Microlithiasis and Preterm Delivery: A Case Report

Erdem¹,

Göktan²,

Erbay³

et al. 2014

Tur Toraks Der

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“…Radiological alterations varied in relation to extent and to forms of presentation, justifying the inhomogeneous and unpredictable clinical course. (10) Apgar score was 9. The newborn presented normal heart and lung auscultation.…”

Section: Case Reportmentioning

confidence: 99%

Gravidez em paciente com microlitíase alveolar pulmonar grave

et al. 2008

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A microlitíase alveolar pulmonar (MAP) é uma doença rara que atinge ambos os pulmões, caracterizada pela presença de pequenos cálculos (fosfato de cálcio) nos espaços alveolares. Relatamos o caso de uma paciente do sexo feminino, de 26 anos, cujo diagnóstico foi confirmado com base nos achados marcantes na radiografia de tórax e tomografia computadorizada de alta resolução. A paciente, gestante de 28 semanas, retornou ao hospital 10 meses após o diagnóstico apresentando insuficiência respiratória hipoxêmica e com distúrbio ventilatório restritivo grave à espirometria. Após completadas 32 semanas e 4 dias de gestação, foi submetida aparto cesariano, com sucesso para mãe e filha. A MAP tem evolução clínica variável. Tem provável caráter autossômico recessivo e associação com história familiar positiva. A etiologia é incerta, e muitos autores especulam que haja um defeito enzimático local responsável pelo acúmulo intra-alveolar de cálcio. Relatos de pacientes com MAP que engravidaram são excepcionais, sendo o presente caso o primeiro descrito no Brasil. O curso dessa doença costuma ser lentamente progressivo, e os pacientes geralmente falecem devido à insuficiência cardiorrespiratória. O presente caso ilustra a necessidade de se oferecer aconselhamento genético e orientações sobre o risco de gravidez às pacientes, especialmente em casos de doença avançada. Atualmente, a única terapia efetiva é o transplante pulmonar.

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“…Calcified micronodules, ground-glass opacities, interlobular septal thickening, pleural and subpleural calcification and cysts are typical radiological findings seen in HRCT [17,[28][29][30][31][32][33][34][35].…”

Section: Radiological Characteristicsmentioning

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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High resolution computed tomographic features of pulmonary alveolar microlithiasis

Cited by 55 publications

References 18 publications

Pulmonary Alveolar Microlithiasis and Preterm Delivery: A Case Report

Pulmonary Alveolar Microlithiasis and Preterm Delivery: A Case Report

Gravidez em paciente com microlitíase alveolar pulmonar grave

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

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