A Case of Pulmonary Alveolar Microlithiasis Diagnosed by Transbronchial Biopsy

Arpağ, Hüseyin; Sayan, Muhammet; Atilla, Nurhan; Bozkuş, Fulsen; Bahar, Abdülkadir Yasir; Kahraman, Hasan; Tokur, Mahmut

doi:10.5152/turkthoracj.2017.17015

Cited by 9 publications

(6 citation statements)

References 8 publications

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“…Diagnosis of PAM most commonly occurs in third or fourth decade, however, the disease may manifest at any age. [6] Initially the disease is asymptomatic and often the diagnosis of PAM is made incidentally on chest radiography done for other reasons. [3] Serum calcium and phosphate concentrations and hepatic, renal, and parathyroid values, are normal.…”

Section: Discussionmentioning

confidence: 99%

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An Unusual Case of Pulmonary Alveolar Microlithiasis Masquerading as Pulmonary Tuberculosis

Ilyas¹,

Poflee²,

Bobde³

et al. 2019

AJMR

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Pulmonary Alveolar Microlithiasis (PAM) is a rare chronic lung disease characterized by bilateral intra-alveolar calcium and phosphate deposition throughout the lung parenchyma. PAM is known to occur over wide age range. Exact etiopathogenesis of PAM is not known and long term survival is uncertain. Patient can be asymptomatic or present with progressive pulmonary symptoms. Radiological features of PAM are pathognomic for radiologist who is aware of this rare pulmonary disease. However the changes of PAM on imaging can be misdiagnosed as those due to pulmonary tuberculosis which is a relatively common diagnosis in the Indian setting. Definitive diagnosis is based on typical microscopic features seen on transbronchial or open lung biopsy. We report a case of 41 years old female who was treated as a case of pulmonary tuberculosis for two years without relief of symptoms at a peripheral medical centre. Final diagnosis of PAM was made on the basis of characteristic radiological findings and microscopic features at our Institute.

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Section: Discussionmentioning

confidence: 99%

“…[5] Lung transplantation is the only definite treatment to be considered in PAM. [6] Supplemental oxygen therapy and pneumococcal vaccinations are also recommended for these patients. [5]…”

Section: Ilyas Et Al; An Unusual Case Of Pulmonary Alveolar Microlithmentioning

confidence: 99%

An Unusual Case of Pulmonary Alveolar Microlithiasis Masquerading as Pulmonary Tuberculosis

Ilyas¹,

Poflee²,

Bobde³

et al. 2019

AJMR

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“…Although the disease is rare in Africa, Zaghba et al [13] reported a 42-year-old African lady with PAM by chest X-ray, chest CT scan, and transbronchial biopsy. These tools were also used by researchers of Turkey for confirmation of their case of PAM [14].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary alveolar microlithiasis and interstitial pneumonitis: a case report of the west of Iran

Ramezani¹,

Aminparast²,

Sadeghi³

2019

BioMedicine

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Pulmonary alveolar microlithiasis (PAM) is a rare disease with autosomal recessive inheritance. Herein, a 20-year-old lady referred to the hospital with a dry cough for two years. The chest X-ray findings were bilateral reticulonodular opacities in both lungs and honeycomb appearance suspicious for miliary tuberculosis and idiopathic pulmonary fibrosis. A wedge biopsy of lung showed that there were several intraalveolar laminated concretions in the pathology report compatible with pulmonary alveolar microlithiasis and interstitial infiltration of lymphocytes and neutrophils compatible with interstitial pneumonitis. PAM is a rare progressive disease with the production of microliths in pulmonary alveoli. The pathologist, radiologist, and clinician should be familiar with this entity for diagnosis and appropriate management. The family of the patient especially siblings must be evaluated for earlier diagnosis.

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“…The presence of calcified lamellar bodies within the lumen of the alveolar space is often definitive. 42 Surgical biopsy by the video-assisted thoracoscopic approach should be reserved for the rare cases in which the diagnosis remains elusive after noninvasive methods are exhausted. Innumerable microliths within the intraalveolar space and interstitial fibrosis with thickening of the alveolar walls are typical findings on pathological analysis.…”

Section: Diagnosismentioning

confidence: 99%

Pulmonary Alveolar Microlithiasis

Shaw

McCormack

2020

Semin Respir Crit Care Med

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Pulmonary alveolar microlithiasis (PAM) is a rare, autosomal recessive disorder that is caused by mutations in SCL34A2 that encodes for the type IIb sodium-dependent phosphate cotransporter (Npt2b). The loss of Npt2b transporter function from alveolar epithelial cells results in failure to export inorganic phosphate from the alveolar lining fluid, which then accumulates, binds to calcium, and forms hydroxyapatite microliths. Radiographs and computed tomography of the chest demonstrate hyperdense infiltrates that are often quite dramatic and distinctive, and in many cases, the diagnosis can be made without invasive measures. The most common presenting symptom of PAM is dyspnea on exertion, but the disease is frequently first noted as an incidental finding in asymptomatic patients who have chest films performed for unrelated reasons. Pulmonary fibrosis, pulmonary hypertension, and respiratory failure can develop as the disease progresses, and treatment remains supportive. Lung transplantation is an option for those with end stage disease.

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A Case of Pulmonary Alveolar Microlithiasis Diagnosed by Transbronchial Biopsy

Cited by 9 publications

References 8 publications

An Unusual Case of Pulmonary Alveolar Microlithiasis Masquerading as Pulmonary Tuberculosis

An Unusual Case of Pulmonary Alveolar Microlithiasis Masquerading as Pulmonary Tuberculosis

Pulmonary alveolar microlithiasis and interstitial pneumonitis: a case report of the west of Iran

Pulmonary Alveolar Microlithiasis

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