Pulmonary alveolar microlithiasis (PAM) is a rare disease characterised by the widespread intra-alveolar accumulation of minute calculi called microliths. It is caused by mutation of the SLC34A2 gene encoding the type IIb sodium phosphate cotransporter in alveolar type II cells. 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 2014 and 544 papers were found, accounting for 1022 cases.PAM is present in all continents and in many nations, in particular in Turkey, China, Japan, India, Italy and the USA. Familiality is frequent. The clinical course is not uniform and the causes of this clinical variability seem to be largely nongenetic. The optimal diagnostic procedure is the association of chest high-resolution computed tomography (HRCT) with bronchoalveolar lavage, but a chest radiograph may suffice in families in which a case has already been diagnosed. Moreover, chest radiography and HRCT allow the classification of the evolutionary phase of the disease and its severity. At present lung transplantation is the only effective therapy. However, better knowledge of the gene responsible offers hope for new therapies. @ERSpublications Familial, genetic, clinical, radiological and therapeutic aspects of pulmonary alveolar microlithiasis epidemiology http://ow.ly/St3Mw

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Pulmonary alveolar microlithiasis (PAM) (MIM 265100) is a rare, autosomal recessive pneumopathy characterized by intra-alveolar formation and accumulation of tiny, roundish corpuscles called "microliths". The name "alveolar microlithiasis" was first used by Puhr in 1933; since then, several reports have appeared, and over 300 individuals with this condition have been reported. We have reviewed the PAM cases in the literature in light of personal experience, focusing on medical implications, disease diagnosis and progression over time, familial predisposition, and geographical and sex distribution. This study confirms autosomal recessive inheritance and does not support the role of other, non-genetic, factors in the pathogenesis of PAM.

Giant splenic artery aneurysms (GSAAs) larger than 8 cm in diameter have rarely been reported, particularly in older people. They are clinically important lesions, often asymptomatic and related to an increased risk of complications such as abrupt rupture, requiring emergency surgical treatment. Comprehensive geriatric assessment (CGA), originally developed for multidimensional clinical evaluation in several geriatric settings, was recently proposed as a fundamental preoperative aid for treatment planning of older patients undergoing elective surgery and preventing adverse post-operative outcomes. We present the first case of an asymptomatic 9-cm partially thrombosed GSAA, accidentally diagnosed during abdominal ultrasound in a 63-year-old woman from the Apulia region in Southern Italy. She successfully underwent aneurysmectomy, highlighting the usefulness of CGA in elective surgical patients.

PurposeTo investigate the correlations between densitometric and CALIPER-derived indices of pulmonary emphysema and their change in the short-term period for groups of patients with different smoking habits.MethodThis retrospective study included 284 subjects from the ITALUNG trial (198 men and 86 women; mean age±sd, 60±4) who underwent low-dose chest CT at baseline and 2-year follow-up. Subjects were divided in four groups (persistent smokers, restarters, quitters, and former smokers) according to their smoking habit at baseline and follow-up. Densitometric and texture analyses were performed, using CALIPER software. A correlation analysis was conducted between CALIPER-derived low-attenuation-areas (LAAs) and densitometric indices, including the 15th percentile of the whole-lung attenuation histogram (Perc15) and the relative areas below −950HU (RA950). Densitometric indices and LAAs were evaluated at baseline and variation assessed longitudinally with comparisons between groups with different smoking habit. Further analysis of parenchymal changes per pulmonary zone was performed.ResultsLAAs were strongly correlated with Perc15 (rs=0.81; p<0.001) and RA950 (rs=0.905; p<0.001). At baseline, the group of smokers showed higher Perc15, lower RA950, lower LAAs (particularly mild sub-class of LAAs) than the group of ex-smokers (p<0.001). At 2-year follow-up, densitometric indices and LAAs increased in persistent smokers, former smokers and quitters (p<0.05). The progression was larger and statistically more significant in quitters (p<0.001).ConclusionCALIPER texture analysis provides an objective measure comparable to traditional density/histogram features to assess the lung parenchymal changes in relation to different smoking habit.