Focus: Rare Disease: Pulmonary Alveolar Microlithiasis – A Review

Pulmonary Alveolar Microlithiasis (PAM) is a rare genetic disorder causing widespread deposition of calcium-phosphate crystals in the alveolar space. A hallmark of the disease is the discrepancy between perceived symptoms upon diagnosis compared with the extensive, sandstorm-like appearance of the microliths on chest X-ray or HRCT. Caused by a defective sodium-dependent phosphate transport protein due to loss-of-function variants of the SLC34A2 gene, PAM is an autosomal recessive transmitted disorder, and as such has a high correlation to consanguinity. The most common variants of the SLC34A2 gene are single nucleotide biallelic changes, but larger deletions are described. Initial suspicion of PAM on radiological examination should be followed by genetic testing to verify the diagnosis and identify the disease-causing variant. When not available, the diagnosis can be made by means of invasive techniques, such as transbronchial forceps or cryobiopsy, or a surgical lung biopsy. In families with a history of PAM, genetic counseling should be offered, as well as preimplantation/prenatal testing if necessary. As of writing this review, no definitive treatment exists, and PAM may in some cases progress to severe pulmonary disease with respiratory failure and potential death. Patients with PAM should be offered preventative and symptomatic treatments such as vaccinations and oxygen therapy when needed. In some cases, lung transplantation may be required.     

Armes de chasse dans l’Épipaléolithique du Caucase du Nord

The article is devoted to the traceological study of points and geometric microliths that are characteristic of the Epipaleolithic sites of the northwestern Caucasus and the Elbrus region. The regularities between the type of stone tools and their functional use are analyzed. Composite hunting weapons were effectively used in hunting a variety of ungulate herd animals. The composition of faunal assemblages largely depended of climatic and landscape conditions of the sites in different periods of the Late Glacial period: from the Oldest Dryas to the Pleistocene–Holocene transition. The proximity of lithic industries within a vast region in the North Caucasus, similar shapes of projectiles and lithic inserts into composite hunting weapons could be associated with active human movements, which are confirmed by the transportation of obsidian from the Elbrus region to the northwestern Caucasus and the transportation of high-quality flint from the west to the Elbrus region in the north-central Caucasus.     

Předmostí III : un site pavlovien de la Porte de Moravie (République tchèque,Europe centrale)

The complex of sites of Předmostí is one of the most famous in the Central Europe. The site Předmostí III has been excavated several times during the last thirty years. This article focuses on the materials discovered in 1982 and 1983. The lithic industry has been knapped in situ on radiolarite river pebbles. The association of a Kostenki’ knife with geometric microlith indicates a cultural attribution to the Pavlovian and more particularly to the kind described as the “group with geometric microliths”. Blades and bladelets are mainly produced with an organic hammer. This fact could fit with the discover of a piece of mammoth tusk with percussion marks on its the distal end that could have been used as a hammer. In spite of the small number of artefacts preserved, the reassessment of that collection contributes to a better understanding of the Pavlovian industries.