Presentation
Sporadic cases are more common, but the disease also presents in an inherited familial form. The greatest number of reported cases is from Europe and especially Turkey. We present a 43-year-old female with complaints of dyspnea for many years. [ncbi.nlm.nih.gov]
Respiratoric
-
Cough
Although most children with this disease are asymptomatic, our patient had persistent cough for more than 3 years. It is likely that his chronic cough was a direct consequence of the disease. [ncbi.nlm.nih.gov]
-
Dry Cough
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. [ncbi.nlm.nih.gov]
-
Hemoptysis
There was no history of expectoration, hemoptysis, chest pain, or tuberculosis. General physical examination showed no evidence of clubbing of the nails or lymphadenopathy. [ncbi.nlm.nih.gov]
There was no history of fever, hemoptysis or chest pain. Patient was a non-smoker. There was no history of tuberculosis within the family members or any known history of pulmonary disease. [ijcasereportsandimages.com]
The most common symptoms include the following: dyspnea dry cough chest pain sporadic hemoptysis asthenia pneumothoraces PAM is hereditary and another involved family member can be identified in 36% to 61% of cases. [en.wikipedia.org]
However, a certain degree of dyspnea with a productive cough may occur together with a sporadic hemoptysis and thoracic pains. [scienceopen.com]
-
Pulmonary Disorder
disorder of unknown etiology characterized by intra-alveolar development and deposition of microliths or calcispherytes consisting of calcium phosphate. [thieme-connect.com]
Gastrointestinal
-
Failure to Thrive
She was asymptomatic except for failure to thrive. Plain chest radiographs on admission showed sand-like opacities with calcific densities throughout both lung fields, predominantly in the lower zones. [ncbi.nlm.nih.gov]
CASE REPORT A fifteen-year-old boy was investigated for failure to thrive. He was totally asymptomatic and no respiratory complaint at the time of evaluation. [scolaris.beta.semantico.com]
Workup
X-Ray
-
X-Ray Abnormal
A 12-year-old girl of Turkish descent was referred 6 weeks after an influenza A infection because of persistent chest X-ray abnormalities compatible with interstitial lung disease. [ncbi.nlm.nih.gov]
-
X-Ray Abnormal
A 12-year-old girl of Turkish descent was referred 6 weeks after an influenza A infection because of persistent chest X-ray abnormalities compatible with interstitial lung disease. [ncbi.nlm.nih.gov]
-
Chest X-Ray Abnormal
A 12-year-old girl of Turkish descent was referred 6 weeks after an influenza A infection because of persistent chest X-ray abnormalities compatible with interstitial lung disease. [ncbi.nlm.nih.gov]
-
Chest X-Ray Abnormal
A 12-year-old girl of Turkish descent was referred 6 weeks after an influenza A infection because of persistent chest X-ray abnormalities compatible with interstitial lung disease. [ncbi.nlm.nih.gov]
Pulmonary Function Test
-
Restrictive Pulmonary Function
Many patients are asymptomatic and the majority of patients either have normal or restrictive pulmonary function. The clinical course of the disease varies. [ncbi.nlm.nih.gov]
Treatment
Hui-Ying Wang, Update on diagnosis and treatment of pulmonary alveolar microlithiasis, World Journal of Respirology, 4, 3, (26), (2014). [doi.org]
The pathogenetic mechanism of this treatment on the genetic basis of disease is discussed. [ncbi.nlm.nih.gov]
Prognosis
The long-term prognosis is poor and no treatment has been discovered to date. The aim of this review is to describe the main pathological, clinical, and imaging aspects of PAM, ranging from its genetic basis to treatment. [ncbi.nlm.nih.gov]
Scrutiny of the long-term follow-up data of PAM patients reveals that the prognosis for PAM is poor. The establishment of an effective treatment, which is not yet available, is mandatory. [doi.org]
Treatment and prognosis No known treatment is available. Overall prognosis is good but occasionally slow progression can result in end-stage lung fibrosis requiring lung transplantation. [radiopaedia.org]
Differential Diagnosis Same "crazy-paving" described with alveolar proteinosis may be seen in microlithiasis Silicosis Metastatic calcification in renal disease Treatment None Complications Respiratory insufficiency Cyanosis Clubbing Pulmonary hypertension Prognosis [learningradiology.com]
Etiology
The etiology of the disease is still unknown and many authors have supposed that an inherited local enzymatic defect is responsible for calcium deposition. [ncbi.nlm.nih.gov]
Epidemiology
@article{Mariotta1997PulmonaryAM, title {Pulmonary alveolar microlithiasis: Review of Italian reports}, author {Salvatore Mariotta and Laura Guidi and Maria Rosaria Papale and Alessandro Ricci and Alberto Bisetti}, journal {European Journal of Epidemiology [semanticscholar.org]
The present study explores the epidemiological, familial, genetic, clinical, diagnostic, radiological and therapeutic aspects with the aim of contributing to a better understanding of this uncommon disease.We searched articles on PAM published up to December [ncbi.nlm.nih.gov]
Each chapter focuses on the pathogenic mechanisms and therapeutic targets suggested by basic research and follows an easy to read format: brief introduction followed by discussion of epidemiology, genetic basis and molecular pathogenesis, animal models [books.google.com]
Pathophysiology
We suspect that the markedly enhanced pulmonary FDG uptake may have some relation to the pathophysiology of PAM. [ncbi.nlm.nih.gov]
Pathophysiology The alveoli in pulmonary alveolar proteinosis (PAP) are filled with proteinaceous material, which has been analyzed extensively and determined to be normal surfactant composed of lipids and surfactant-associated proteins A, B, C, and D [emedicine.medscape.com]
Prevention
There are still no proven treatments, but earlier diagnosis may allow for evaluation of preventative strategies that could improve outcome. [ncbi.nlm.nih.gov]
A low-phosphate diet prevents microlith formation in young animals and reduces lung injury on the basis of reduction in serum SP-D. [doi.org]
To date, no treatment has been proven to effectively reverse or prevent the progression of PAM. [en.wikipedia.org]
Transplantation Substance Use and Addiction Surgery Surgical Innovation Surgical Pearls Teachable Moment Technology and Finance The Rational Clinical Examination Tobacco and e-Cigarettes Toxicology Trauma and Injury Treatment Adherence United States Preventive [jamanetwork.com]