1.
Ultrastructural Pathology of Atherosclerosis, Calcific Aortic Valve Disease, and Bioprosthetic Heart Valve Degeneration: Commonalities and Differences.
Kostyunin, A, Mukhamadiyarov, R, Glushkova, T, Bogdanov, L, Shishkova, D, Osyaev, N, Ovcharenko, E, Kutikhin, A
International journal of molecular sciences. 2020;(20)
Abstract
Atherosclerosis, calcific aortic valve disease (CAVD), and bioprosthetic heart valve degeneration (alternatively termed structural valve deterioration, SVD) represent three diseases affecting distinct components of the circulatory system and their substitutes, yet sharing multiple risk factors and commonly leading to the extraskeletal calcification. Whereas the histopathology of the mentioned disorders is well-described, their ultrastructural pathology is largely obscure due to the lack of appropriate investigation techniques. Employing an original method for sample preparation and the electron microscopy visualisation of calcified cardiovascular tissues, here we revisited the ultrastructural features of lipid retention, macrophage infiltration, intraplaque/intraleaflet haemorrhage, and calcification which are common or unique for the indicated types of cardiovascular disease. Atherosclerotic plaques were notable for the massive accumulation of lipids in the extracellular matrix (ECM), abundant macrophage content, and pronounced neovascularisation associated with blood leakage and calcium deposition. In contrast, CAVD and SVD generally did not require vasculo- or angiogenesis to occur, instead relying on fatigue-induced ECM degradation and the concurrent migration of immune cells. Unlike native tissues, bioprosthetic heart valves contained numerous specialised macrophages and were not capable of the regeneration that underscores ECM integrity as a pivotal factor for SVD prevention. While atherosclerosis, CAVD, and SVD show similar pathogenesis patterns, these disorders demonstrate considerable ultrastructural differences.
2.
[Molecular mechanism of idiopathic basal ganglia calcification].
Wang, C, Xu, X, Li, LL, Wang, T, Zhang, M, Shen, L, Tang, BS, Liu, JY
Yi chuan = Hereditas. 2015;(8):731-40
Abstract
Idiopathic basal ganglia calcification (IBGC), also known as Fahr’s disease, is an inheritable neurodegenerative syndrome characterized by mineral deposits in the basal ganglia and other brain regions. Patients with IBGC are often accompanied with movement disorders, cognitive impairment as well as psychiatric abnormalities. So far, no therapeutic drug has been developed for the treatment of IBGC. Recently, genetic studies have identified several genes associated with IBGC, including SLC20A2, PDGFRB, PDGFB, ISG15 and XPR1. Loss-of-function mutations in these genes have been associated with disturbance in phosphate homeostasis in brain regions, the dysfunction of blood-brain barrier as well as enhanced IFN-α/β immunity. In this review, we summarize the latest research progress in the studies on molecular genetics of IBGC, and discuss the molecular mechanisms underlying the pathophysiology of mutations of different genes.
3.
Pulmonary alveolar microlithiasis and probe-based confocal laser endomicroscopy.
Yserbyt, J, Alamé, T, Dooms, C, Ninane, V
Journal of bronchology & interventional pulmonology. 2013;(2):159-63
Abstract
Pulmonary alveolar microlithiasis (PAM) is a rare disease in which calcium-phosphate microliths accumulate within the alveolar space. We report cases of 2 siblings with PAM, presenting differently as regards the distribution and clinical severity. Immune cytologic analysis of bronchoalveolar lavage showed a CD4 alveolitis in the radiologically most affected patient, whereas the least affected had a normal bronchoalveolar lavage analysis, demonstrating the low specificity of immune cytologic lavage analysis in diagnosing familial PAM. For the first time, we describe the endoscopic findings using a probe-based confocal laser endomicroscopy.