Pulmonary Alveolar Microlithiasis in Children: Case Series and Review of Literature

2011 ◽  
Vol 24 (4) ◽  
pp. 221-224
Author(s):  
Meenu Singh ◽  
Arushi G. Saini ◽  
Joseph Mathew ◽  
Uma Nahar ◽  
Kim Vaiphei
2019 ◽  
Vol 12 (3) ◽  
pp. e227406 ◽  
Author(s):  
Manu Chopra ◽  
Manjit Sharad Tendolkar ◽  
Vasu Vardhan

Pulmonary alveolar microlithiasis (PAM) is a rare disease characterised by calcific deposits in lung parenchyma. PAM being a progressive disease with dissociation between severity of clinical symptoms and radiological picture, it is often detected incidentally. Mutations in the SLC34A2 gene encoding the type IIb sodium phosphate cotransporter in alveolar type II cells are considered to be involved in the pathogenesis of PAM. The majority of the patients are diagnosed usually between the ages of 20 and 40 years, although paediatric PAM has also been reported. Diagnosis is confirmed by combination of radiological features, bronchial lavage and histopathological testing. At present, lung transplant is the only definitive treatment available. Though rare, the prevalence of PAM is worldwide. Till June 2018, 86 cases have been reported from India and 1042 cases have been reported worldwide. We report three cases from India, including a student, cement factory worker and a tailor, which will highlight the varied clinical and radiological presentations of this rare disease along with the response to treatment.


Author(s):  
Richa Mittal ◽  
Rohit Kumar ◽  
Juvva Kishan Srikanth ◽  
Siddharth Raj Yadav ◽  
Ravi Chandak ◽  
...  

Pulmonary alveolar microlithiasis (PAM) is a rare inherited disorder in which patients present with either nonspecific signs and symptoms or are asymptomatic. Clinical-radiological dissociation is a commonly seen feature of the disease. Majority of cases are diagnosed in second and third decade of life, although presentation in paediatric and elderly population is also reported. Pathognomonic radiological findings, with normal calcium and phosphate levels can confirm the diagnosis without the need of further investigations in large number of cases. A high index of suspicion and knowledge of the disease is of paramount importance as it has a wide range of presentation and variable course thus posing a diagnostic challenge. We present two cases of this rare disease in elderly females with distinct presentations although with similar and characteristic radiological findings along with a brief review of literature of the disease.


2014 ◽  
Vol 2014 (mar31 1) ◽  
pp. bcr2013201300-bcr2013201300
Author(s):  
H. Al-Sardar ◽  
D. J. S. Al-Habbo ◽  
R. M. A. Al-Hayali

2019 ◽  
Vol 24 (38) ◽  
pp. 4534-4539 ◽  
Author(s):  
Eric Zimmermann ◽  
Fawzi Ameer ◽  
Berhane Worku ◽  
Dimitrios Avgerinos

Introduction: Proximal aorta interventions impose significant bleeding risk. Patients on concomitant anticoagulation regimens compound the risk of bleeding in any surgery, but especially cardiothoracic interventions. The employment of direct-acting oral anticoagulants (DOAC), namely those that target clotting factors II or X, has expanded at a precipitous rate over the last decade. The emergence of their reversal agents has followed slowly, leaving clinicians with management dilemmas in urgent surgery. We discuss current reversal strategies based on the available published data and our experience with proximal aortic surgery in patients taking DOACs. Literature Search: We performed a review of literature and present three cases from our experience to offer insight into management strategies that have been historically successful. A review of literature was conducted via PubMed with the following search string: (NOAC or DOAC or TSOAC) and (aorta or aortic or (Stanford and type and a)). Case Presentation: We present three case presentations that illustrate the importance of DOAC identification and offer management strategies in mitigating associated bleeding risks in urgent or emergent surgeries. Conclusion: Treatment teams should be aware of the technical limitations of identifying and reversing DOACs. In view of the tendency toward publishing positive outcomes, more scientific rigor is required in the area of emergency DOAC reversal strategies.


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