Morphology, pathogenesis and classification of interstitial lung diseases

The current literature data and the authors' material on the interstitial lung diseases (ILD) are presented. The authors' material includes the results of morphological (light and electron microscopy) examination of 36 intrabronchial biopsies in ILD patients, 2 open biopsies, 6 autopsy cases and lungs of laboratory animals with various forms of the bronchoalveolar sclerosis and interstitial pneumonia. ILD is shown to be a chronic inflammatory lung condition with a cooperation between the immunocompetent and inflammatory cells in the alveolar septa and fibrosis of the alveolar walls. The disturbance of the immune homeostasis, particularly that of cell immunity, plays a role in the ILD pathogenesis. Complex intercellular relationships, in which the mediators of cell-to-cell interaction are of importance, are being established in the alveolar interstitium. This makes ILD morphology resembling that of the immune inflammation in other organs. So-called "neutrophilic alveolitis" induced by the immune complexes is of great importance in the pathogenesis of certain diffuse ILD forms, for example, the cryptogenic fibrosing alveolitis. The "neutrophilic alveolitis" is characterized by the presence of few neutrophils among the mononuclear cells infiltrating the alveolar septa. The alterations of the arteries, veins and capillaries in ILD may be subdivided into haemodynamic and inflammatory. ILD can be reproduced experimentally by either intravenous or intratracheal administration of antigen.     

Current understanding of the pathogenesis of infectious diseases

New experimental data in such research fields as molecular biology, biochemistry, genomics and others as well as the changes occurring in the medical-and-epidemiological situation in respect to a number of infectious diseases dictate the necessity to systemize and to revise the appropriate knowledge for the purpose of ensuring a more profound understanding of the processes providing a foundation for the relations within the system of "host--parasite". The article deals with modern aspects of investigating the pathogenesis of infectious diseases, i.e. issues of the genetic and of structural-and-functional organization as well as of regulation of the pathogenetic potential of microorganisms; and mechanism of antimicrobial protection of microorganisms including from the standpoint of evolutionary biology.     

Immune mechanisms in the pathogenesis of demyelinating diseases

The loss of myelin which characterises many human and experimental demyelinating diseases, among them multiple sclerosis, is thought to be immune mediated, but the precise mechanisms responsible remain unknown despite intense research. Normally, myelin in the central nervous system (CNS) is protected from systemic immune responses by the blood brain barrier, which separates nervous tissue from the peripheral circulation. Here we review evidence suggesting that an understanding of the demyelinating disorders may be helped by considering their immune pathogenesis in two stages. The first is damage to the blood brain barrier; this appears to be cell mediated, and allows infiltration into the CNS of other immune effectors. These include complement and also macrophages, which together may mediate the second stage, injury to the myelin/oligodendrocyte complex.     

Developmental mechanisms in the pathogenesis of neurodegenerative diseases

Cellular genes that are mutated in neurodegenerative diseases code for proteins that are expressed throughout neural development. Genetic analysis suggests that these genes are essential for a broad range of normal neurodevelopmental processes. The proteins they code for interact with numerous other cellular proteins that are components of signaling pathways involved in patterning of the neural tube and in regional specification of neuronal subtypes. Further, pathogenetic mutations of these genes can cause progressive, sublethal alterations in the cellular homeostasis of evolving regional neuronal subpopulations, culminating in late-onset cell death. Therefore, as a consequence of the disease mutations, targeted cell populations may retain molecular traces of abnormal interactions with disease-associated proteins by exhibiting changes in a spectrum of normal cellular functions and enhanced vulnerability to a host of environmental stressors. These observations suggest that the normal functions of these disease-associated proteins are to ensure the fidelity and integration of developmental events associated with the progressive elaboration of neuronal subtypes as well as the maintenance of mature neuronal populations during adult life. The ability to identify alterations within vulnerable neuronal precursors present in pre-symptomatic individuals prior to the onset of irrevocable cellular injury may help foster the development of effective therapeutic interventions using evolving pharmacologic, gene and stem cell technologies.     

Lymphomas and lymphoproliferative diseases of the lung

Primary pulmonary lymphoma is a rare disease and the majority of cases represent extranodal marginal zone lymphoma, followed by diffuse large B-cell lymphoma. Other lymphomas that commonly involve the lung include lymphomatoid granulomatosis, a neoplasm of large EBV-positive B cells that are typically outnumbered by non-neoplastic T cells, and classical Hodgkin's lymphoma, which usually reflects systemic dissemination or direct mediastinal extension. The differential diagnosis of marginal zone lymphoma includes secondary involvement by other systemic low-grade B-cell lymphomas and chronic reactive conditions, such as nodular lymphoid hyperplasia, while the other entities elicit a differential diagnosis that includes various high-grade lymphoid neoplasms. A specific diagnosis can usually be achieved on the basis of histological evaluation and immunophenotyping, although molecular genetic studies may be required in certain situations. Such a multiparameter approach may be warranted to accurately diagnose these entities due to differing clinical implications in terms of prognosis and treatment.     

MicroRNA与肺部疾病

MicroRNA(miRNA)在生物的发育、疾病的发生和发展过程中扮演着重要角色,以其在生命活动中起到的调控作用而备受关注.大量研究证实miRNA与包括肺的生长发育、肺内炎性反应、肺癌、肺纤维化等在内的肺部疾病的发生和发展及转归有着密切的关联.MiRNA芯片技术是一种有效地、高通量地获取miRNA表达的手段,已经成功地...     

One view of the pathogenesis of sickle cell diseases

A single amino acid substitution in the beta chain of hemoglobin (beta 6 glutamic acid leads to valine) is responsible for polymerization of deoxyhemoglobin S, and the sickling of red blood cells containing that hemoglobin. Sickled cells are rigid and inflexible, causing obstruction of small blood vessels, which in turn causes obstruction of small blood vessels, which in turn causes ischemic injury. Organs most frequently damaged include the spleen, bone marrow, liver, and kidney. Sickled cells also have a shortened survival; the hemolytic anemia they produce is responsible for aplastic crises, megaloblastic anemia, ankle ulcers, gallstones and gout. "Sickle cell lung disease" is a serious problem, since distinction between infection and infarction is difficult or impossible, and impaired oxygenation of the blood makes further sickling likely. Since the entire patient, not just his blood, is affected by the disease, treatment must go beyond transfusion and drug administration. Each patient presents a new constellation of problems, and therapy must be individualized if it is to be optimal.     

Myeloid diseases in the lung and pleura

Myeloid diseases detected as primary or secondary lesions in the lung and pleura are rare. Clinical presentations and radiographic results may vary significantly depending on the nature of the diseases. The most common diseases associated with lung and pleura involvement are myeloid sarcoma/acute myeloid leukemia (AML) and extramedullary hematopoiesis (EMH). AML typically represents localized involvement by systemic acute leukemia, while EMH is frequently secondary to underlying benign hematolymphoid disorders or myeloproliferative neoplasms. This review provides an overview of the pathogenesis, clinical presentations, radiologic/imaging studies, pathologic and genetic findings, and treatment/outcomes associated with myeloid diseases in the lung and pleura.