Nationwide retrospective observational study of idiopathic dendriform pulmonary ossification : clinical features with a progressive phenotype

Background: Diffuse pulmonary ossification is a specific lung condition that is accompanied by underlying diseases. However, idiopathic dendriform pulmonary ossification (IDPO) is extremely rare, and the clinical features remain unclear. In this study, we aimed to report the clinical characteristics of IDPO. Methods: We conducted a nationwide survey of patients with IDPO from 2017 to 2019 in Japan and evaluated the clinical, radiological, and histopathological findings of patients diagnosed with IDPO. Results: Twenty-two cases of IDPO were identified. Most subjects (82%) were male, aged 22-56 years (mean (SD), 37.9 (9.1)) at diagnosis. Nearly 80% of the subjects were asymptomatic, and the condition was discovered during a medical check-up. However, 36% of the subjects showed a decline in forced vital capacity (%FVC) predicted <80% at diagnosis. The typical radiological features of high-resolution CT (HRCT) are calcified branching structures that are predominantly distributed in the lower lung fields without any other conspicuous finding. Histopathological analysis also showed dendriform ossified lesions from the intraluminal areas to interstitial areas. Notably, during the follow-up period of 20 years, disease progression was found in 88% on HRCT and more than 50% on pulmonary function tests (FVC and/or forced expiratory volume in 1s). Two cases with rapid decline of 10% /year in %FVC predicted were observed.)) at diagnosis. Nearly 80% of the subjects were asymptomatic, and the condition was discovered during a medical check-up. However, 36% of the subjects showed a decline in forced vital capacity (%FVC) predicted <80% at diagnosis. The typical radiological features of high-resolution CT (HRCT) are calcified branching structures that are predominantly distributed in the lower lung fields without any other conspicuous finding. Histopathological analysis also showed dendriform ossified lesions from the intraluminal areas to interstitial areas. Notably, during the follow-up period of 20 years, disease progression was found in 88% on HRCT and more than 50% on pulmonary function tests (FVC and/or forced expiratory volume in 1s). Two cases with rapid decline of 10% /year in %FVC predicted were observed. )) at diagnosis. Nearly 80% of the subjects wereasymptomatic, and the condition was discovered during a medical check-up. However, 36% of the subjects showed a decline in forced vital capacity (%FVC) predicted <80% at diagnosis. The typical radiological features of high-resolution CT (HRCT) are calcified branching structures that are predominantly distributed in the lower lung fields without any other conspicuous finding. Histopathological analysis also showed dendriform ossified lesions from the intraluminal areas to interstitial areas. Notably, during the follow-up period of 20 years, disease progression was found in 88% on HRCT and more than 50% on pulmonary function tests (FVC and/or forced expiratory volume in 1s). Two cases with rapid decline of 10% /year in %FVC predicted were observed. Conclusions: IDPO develops at a young age with gradually progressive phenotype. Further research and long-term (>20 years) follow-up are required to clarify the pathogenesis and clinical findings in IDPO

A Method of Topographical Correction upon the Intensity of Gravity and its Application to the Intensity of Vertical Gradient of Gravity

In this paper a new method of computing topographic effect upon the intensity of gravity determined at the earth's surface is described. The computation of topographic effect is done by tables of which the tabular values of the effect are obtained in microgal in order to meet the sensitivity of the modern gravimeter, 0.01 milligal(mgal) or 10 microgal(μgal). In this method the area around a gravity station is divided into 300 sectorial compartments by 26 concentric circles with the center at the station and 12 equally spaced radial lines passing through it. Radius of the circle increases with the common ratio β which is 1.5849, the smallest and the largest radii being respectively 1 m and 100 km. A transparent graticule, on which the above concentric circles and radial lines are drawn, is placed on a topographic map with a center at the gravity station represented on the map. The mean height of land surface within each compartment is estimated. The vertical attraction of each sectorial compartment block with its height equal to the above obtained mean height is computed in microgal by the tables and by assuming an appropriate value to the density of the block. The accuracy of the value of the attraction thus obtained by the tables is examined by employing a model topography of which the vertical attraction at the station can be exactly calculated other than by the tables. The result is that the tabular value differs from the exact value by a little less than 0.1 mgal. The computation tables for gravity is applicable to the topographical correction upon the intensity of vertical gradient of gravity which is obtained by (g1-g2)/δz, where g1 is the gravity value at a ground station and g2 at a station at a height δz vertically above the former. Convinient tables for obtaining the effect of near topography which may be treated as twodimensional are obtained. At four selected stations in the mountainous area of the central Honshû of Japan, the topographic corrections have been done by the tables mentioned above upon the intensities of gravity and its vertical gradient, and the values of the Modified Bouguer anomaly at sea-level at these stations have been obtained to prove that the Simple Bouguer anomalies at stations in a lofty mountainous area are dangerous as data to find the state of isostatic equilibrium or, generally speaking, the mass distribution below such an area

イギリス テッコウ キギョウ ボルコゥ ヴォーン シャ ノ トウシ カツドウ 1865 1913ネン

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