Case Of Pulmonary Alveolar Microlithiasis Research Articles

Pulmonary alveolar microlithiasis with calcified pleural plaques

Pulmonary alveolar microlithiasis (PAM) is a rare disease. Herein we report a case of pulmonary alveolar microlithiasis who was suspected to have the disease on chest X-ray and was confirmed on high resolution CT and transbronchial lung biopsy. These investigations showed characteristic features of pulmonary alveolar microlithiasis with diffuse interstitial pulmonary fibrosis.

Pulmoner Alveolar Mikrolitiazis

Pulmonary alveolar microlithiasis (PAM) is a rare disease of unknown etiology characterized by extensive intraalveolar calcium and phosphate deposition. Here, we present a case of PAM in a 26-year-old female. The patient had persistent dry cough. Clinical presentation, radiological findings and transbronchial biopsy results were consistent with PAM. Scintigraphy revealed the absence of Tc-99m methylenediphosphonate uptake of lungs. Familial occurrence was observed. Radiological findings, pulmonary functions, and clinical status of the patient have remained stable for 24 months. Tc-99m uptake may not be observed at the early stages of this disease. Our case is at the early stage of the disease with regard to clinic, radiographic and scintigraphic findings. Family screening of PAM index cases to detect the disease in early asymptomatic stage is important.

Pulmonary alveolar microlithiasis with cor pulmonale: An autopsy case demonstrating a marked decrease in pulmonary vascular beds

Pulmonary alveolar microlithiasis (PAM) is a very rare autosomal recessive disorder in which microliths are formed in the alveolar space. PAM is infrequently complicated by pulmonary hypertension, the cause of which is unclear. The author in this paper found that the pulmonary hypertension was caused by a marked decrease in pulmonary vascular beds. Here, an autopsy case of PAM with a marked cor pulmonale is reported. A 14-year-old woman was found to have an abnormal pulmonary shadow, but the cause was unclear. At 24 years, she was diagnosed with a diffuse pulmonary abnormal shadow. At 42 years, she was diagnosed with PAM by imaging techniques. Her condition gradually worsened and she had to be treated with oxygen. She died of respiratory failure at 54 years. An autopsy revealed severe PAM and marked cor pulmonale. The heart weighed 360 g and right ventricular thickness was 10 mm (normal, 2-3 mm). Microscopically, the alveolar space was diffusely filled with microliths, and heart failure cells were recognized. Bone formations were scattered. The alveolar walls showed fibrous thickening, and pulmonary arteries showed atherosclerosis. The right ventricle showed marked cardiac hypertrophy. Chronic severe liver congestion was noted. A morphometric analysis using CD34-stained specimens showed a marked decrease (one tenth) in pulmonary capillary beds (capillary number: 8.6 +/- 3.1 per image), compared with normal lungs obtained from two other autopsies (85.3 +/- 9.4 and 96.2 +/- 10,3). It was concluded that the cor pulmonale and pulmonary hypertension in the present case were caused by the marked decrease of the pulmonary arterial vascular beds. More research is required regarding the etiology and treatment of PAM.

A case of pulmonary alveolar microlithiasis with an intragenetic deletion in SLC34A2 detected by a genome-wide SNP study

A case of pulmonary alveolar microlithiasis occurring in an inbred family is presented. A genome-wide analysis of the patient’s genomic DNA using a high-density single nucleotide polymorphism (SNP) array revealed...

Lung transplantation for pulmonary alveolar microlithiasis

Since the first description of pulmonary alveolar microlithiasis (PAM) by Puhr in 1933, nearly 500 related cases have been reported worldwide. No effective treatment for PAM currently exists, with the exception of lung transplantation. Herein, we present the case of a 63-year-old woman with PAM who, to the best of our knowledge, is the oldest successful lung transplant recipient with end-stage PAM reported. In addition, the present work reviews the outcomes of other cases of PAM after lung transplantation.

La microlithiase alvéolaire pulmonaire : à propos de quatre cas

Pulmonary alveolar microlithiasis is a rare disease characterised by the formation and deposition of calcium phosphate microliths in the lung. It is an autosomal recessive disorder, for which mutation in the SLC34A2 gene was recently found to be responsible for the disease. We report on four cases of pulmonary alveolar microlithiasis. Three patients were asymptomatic. The diagnosis was made after histological confirmation in three patients. The outcome was marked by the death of one patient. Pulmonary alveolar microlithiasis is a rare disease. Diagnosis is made with high-resolution computed tomography, which exhibits the calcic character and distribution of the lesions, thus avoiding the need to perform lung biopsy. We suggest that a literature review be performed.

FDG-PET/CT finding of high uptake in pulmonary alveolar microlithiasis

Pulmonary alveolar microlithiasis (PAM) is a rare lung disease characterized by progressive intra-alveolar calcification. We present a case of PAM with abnormal accumulation of 18F-fluorodeoxyglucose (FDG) in both lungs. A 55-year-old man was referred to our hospital for progressive dyspnea. He had been diagnosed with PAM 25 years earlier by transbronchial lung biopsy. High-resolution computed tomography revealed multiple dense calcifications with little aerated lung. Combined positron emission tomography and computed tomography using 18F-FDG (FDG-PET/CT) showed the abnormal accumulation of FDG in both lungs with a maximal standardized uptake value of 7.3. High FDG uptake was observed mainly in the lung regions showing sparing calcification. The patient died of respiratory failure a month later and an autopsy revealed no significant inflammatory changes in either lung. We suspect that the markedly enhanced pulmonary FDG uptake may have some relation to the pathophysiology of PAM.

Elevated serum surfactant protein A and D in pulmonary alveolar microlithiasis

Pulmonary alveolar microlithiasis (PAM) is a rare disease characterized by widespread localization of calcispherites in the alveolar spaces. The authors report two cases of PAM, with markedly elevated sera concentrations of surfactant protein-A and surfactant protein-D, which showed a tendency to increase as the disease progressed. Therefore, surfactant protein-A and surfactant protein-D may function as serum markers to monitor the disease activity and progression of PAM.

Absence of pulmonary uptake of tc-99m methylenediphosphonate in alveolar microlithiasis

Pulmonary alveolar microlithiasis (PAM) is a rare disease of unknown etiology characterized by accumulation of calcific concretions in the alveolar spaces. The paper reports a case of PAM in a 56-year-old male. The patient had persistent dry cough, and gradually progressive dyspnea on exertion. The diagnosis was established on the basis of roentgenography and confirmed by the sputum and transbronchial biopsy findings. Scintigraphy revealed the absence of Tc-99m methylenediphosphonate uptake of lungs. Familial occurrence was not observed. Chest roentgenogram, pulmonary function, and clinical status of the patient have remained stable for 41 months. Radiological and clinical follow-up of the disease continues.

Pulmonary alveolar microlithiasis in childhood: Clinical and radiological follow‐up

This report describes a case of pulmonary alveolar microlithiasis that was diagnosed in an 8.5-year-old girl by high-resolution computed tomography (CT) and open lung biopsy. Presence of symptoms (productive cough, fever), their periodic occurrence (lasting up to 1 week), and comparatively long asymptomatic periods should be emphasized. Despite extensive X-ray abnormalities, tests of pulmonary interstitium involvement and exercise tests revealed normal results. A therapeutic regimen, including disodium etidronate, was administered for 18 months with no significant clinical or radiological improvement.

Pulmonary microlithiasis. Report of two cases.

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.

Pulmonary alveolar microlithiasis: review of Italian reports.

Pulmonary alveolar microlithiasis (PAM) is a rare disease of unknown etiology, characterized by the presence of calcific concretions in the alveolar spaces. A familial occurrence is frequently found so that an inherited trait is thought to be involved. The chest X-ray is characterized by a 'sandstrom' picture while the clinical state undergoes to a slow and progressive impairment resulting in respiratory failure at the end stage. We have reviewed the Italian literature of the past 50 years detecting 48 case-reports of PAM (19 males and 29 females). Only 20 out of them were documented in international journals. A familial occurrence of 43.7% was found and 18 patients were under age fifteen. There was a prevalence in the female sex (60.4%) and in the second decade of life. Chest X-ray was the most important tool to diagnose PAM revealing the characteristic picture in all patient. Bronchoalveolar lavage (BAL) and open lung biopsy respectively show the characteristic calcospherites in the recovered fluid (BALF) and in the alveolar spaces. About 300 cases of PAM are reported in the international literature. We believe these data are probably underestimated because many case-reports are not published in international literature.

Interstitial Thickening in Pulmonary Alveolar Microlithiasis

We report a case of pulmonary alveolar microlithiasis in which interstitial thickening is a prominent radiographic feature. We wish to emphasize this finding.

Role of Bone Scanning in Pulmonary Alveolar Microlithiasis Report of Two Cases

Two cases of pulmonary alveolar microlithiasis showing intense pulmonary uptake of Tc-99m MDP are reported. Pulmonary alveolar microlithiasis, which is a relatively rare disorder of unknown etiology, is characterized by the presence of innumerable tiny calcispherytes within the alveoli of the lungs. A local defect of the calcium metabolism has been implicated as the possible etiologic factor of this disorder. The calcispherytes, ranging between 0.01 to 0.3 mm in diameter, are composed chiefly of calcium and phosphorus, with apatite as the major chemical state. This chemical composition of the microliths is most probably responsible for the uptake of the bone seeking radiopharmaceutical in the lungs.

Pulmonary Alveolar Microlithiasis: A Morphologic Study of Six Cases Including TEM and SEM with EDX Analysis and Immunocytochemistry

Pulmonary alveolar microlithiasis (PAM) is a rare lung disease of unknown etiology characterized by calcified spherules which fill alveolar spaces. Although there are approximately 110 cases of PAM previously reported, only 26 have been confirmed by histologic examination. Increased interstitial fibrous tissue is seen in some cases, and some patients develop shortness of breath. However, many patients remain assymptomatic for years following the diagnosis of PAM.Six cases from the files of the Armed Forces Institute of Pathology and Hartford Hospital were studied. Light, scanning and transmission electron microscopic studies were performed as were chemical, immunocytochemical and energy dispersive x-ray analysis. Analyses suggest that they are composed in part of mucopolysaccharides and Ca3(PO4)2. Our findings indicate that microliths form initially around a cellular nidus by the deposition of calcium and phosphate salts from the extracellular matrix vesicles of surrounding cells similar to the method previously demonstrated 1n the formation of bone and psammoma bodies. PAM is not usually associated with Identifiable systemic disturbances in calcium and phosphorus metabolism.

Pulmonary alveolar microlithiasis in Afghan pika (Ochotona rufescens rufescens).

Lung lesions of 60 Afghan pikas (Ochotona rufescens rufescens) were examined histologically; 38 cases of pulmonary alveolar microlithiasis were found. Neither sex was predominantly affected, and there was no predilection for age.

Is the Desert Lung Syndrome (Nonoccupational Dust Pneumoconiosis) a Variant of Pulmonary Alveolar Microlithiasis?

Four cases of pulmonary alveolar microlithiasis are presented. These patients had heavy exposure to sand particles for long periods. This is to hypothesize that those sand particles are responsible for triggering a hyperimmune response resulting in the formation of microlithiasis. Previously expressed theories had linked microlithiasis to external irritants and the condition has similarities with desert lung syndrome, a disease definitely caused by the deposition of sand silica in the lungs.

Bone Scintigraphy in Pulmonary Alveolar Microlithiasis: A Comparative Study of Radioactivity and Density Distribution

A histologically proven case of pulmonary alveolar microlithiasis that demonstrated intense lung uptake of Tc-99m-MDP on a bone scan is presented. The spatial distribution within the lungs of the radioactivity and density was assessed either by single photon emission computed tomography (SPECT), or x-ray computed tomography (CT) respectively, and the regional magnitude of the two parameters were compared. The quantitative study of regional radioactivity and density revealed that both the parameters distributed symmetrically, showing gradual increase in magnitude from the upper to the lower lung regions. The radioactivity-density association observed in our patient may indicate on-going formation of microcalculi throughout the lungs, the degree of which is greater at the sites of more severe involvement.

Pulmonary alveolar microlithiasis with an unusual radiological pattern

We present a case of pulmonary alveolar microlithiasis with an unusual radiological pattern in an anotherwise asymptomatic young woman. It was characterized by a reticulonodular pattern predominantly in the lower lung fields with Kerley B lines. The diagnosis was made by transbronchial lung biopsy. We found an otherwise asymptomatic brother with the same radiological pattern.

Pulmonary alveolar microlithiasis with an unusual radiological pattern

We present a case of pulmonary alveolar microlithiasis with an unusual radiological pattern in an anotherwise asymptomatic young woman. It was characterized by a reticulonodular pattern predominantly in the lower lung fields with Kerley B lines. The diagnosis was made by transbronchial lung biopsy. We found an otherwise asymptomatic brother with the same radiological pattern.