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Journal of Pediatric Genetics Jun 2016Von Hippel-Lindau disease is an autosomal dominant syndrome which occurs secondary to germline mutations in the VHL tumor suppressor gene, located on chromosome 3.... (Review)
Review
Von Hippel-Lindau disease is an autosomal dominant syndrome which occurs secondary to germline mutations in the VHL tumor suppressor gene, located on chromosome 3. Clinically von Hippel-Lindau disease is characterized by an increased risk of developing simple visceral cysts, most commonly in the pancreas and kidneys, in addition to an increased risk of developing neoplasms, often with clear cell features, in a multitude of organ systems. The most common neoplasms are cerebellar and retinal hemangioblastomas, adrenal pheochromocytomas, clear cell renal cell carcinomas, pancreatic neuroendocrine tumors, pancreatic serous cystadenomas, and endolymphatic sac tumors. These lesions most commonly present during adulthood; however, screening and surveillance for the development of these lesions should begin in the pediatric years for patients with von Hippel-Lindau disease. In this review article, the genetics and most common neoplasms of von Hippel-Lindau disease are reviewed, with an eye towards implications for the pediatric patient.
PubMed: 27617152
DOI: 10.1055/s-0036-1579757 -
Folia Histochemica Et Cytobiologica 2016Normal pericardium consists of an outer sac called fibrous pericardium and an inner one called serous pericardium. The two layers of serous pericardium: visceral and... (Review)
Review
Normal pericardium consists of an outer sac called fibrous pericardium and an inner one called serous pericardium. The two layers of serous pericardium: visceral and parietal are separated by the pericardial cavity, which contains 20 to 60 mL of the plasma ultrafiltrate. The pericardium acts as mechanical protection for the heart and big vessels, and a lubrication to reduce friction between the heart and the surrounding structures. A very important role in all aspects of pericardial functions is played by mesothelial cells. The mesothelial cells form a monolayer lining the serosal cavity and play an important role in antigen presentation, inflammation and tissue repair, coagulation and fibrinolysis. The two major types of mesothelial cells, flat or cuboid, differ substantially in their ultrastructure and, probably, functions. The latter display abundant microvilli, RER, Golgi dense bodies, membrane-bound vesicles and intracellular vacuoles containing electron-dense material described as dense bodies. The normal structure and functions of the pericardium determine correct healing after its injury as a result of surgery or microbial infection. The unfavorable resolution of acute or chronic pericarditis leads to the formation of adhesions between pericardial leaflets which may lead to serious complications.
Topics: Animals; Heart; Humans; Pericardium
PubMed: 27654013
DOI: 10.5603/FHC.a2016.0014 -
Frontiers in Cell and Developmental... 2021Tissue-resident innate immune cells exert a wide range of functions in both adult homeostasis and pathology. Our understanding of when and how these cellular networks... (Review)
Review
Tissue-resident innate immune cells exert a wide range of functions in both adult homeostasis and pathology. Our understanding of when and how these cellular networks are established has dramatically changed with the recognition that many lineages originate at least in part from fetal sources and self-maintain independently from hematopoietic stem cells. Indeed, fetal-derived immune cells are found in most organs and serous cavities of our body, where they reside throughout the entire lifespan. At the same time, there is a growing appreciation that pathologies manifesting in adulthood may be caused by adverse early life events, a concept known as "developmental origins of health and disease" (DOHaD). Yet, whether fetal-derived immune cells are mechanistically involved in DOHaD remains elusive. In this review, we summarize our knowledge of fetal hematopoiesis and its contribution to adult immune compartments, which results in a "layered immune system." Based on their ontogeny, we argue that fetal-derived immune cells are prime transmitters of long-term consequences of prenatal adversities. In addition to increasing disease susceptibility, these may also directly cause inflammatory, degenerative, and metabolic disorders. We explore this notion for cells generated from erythro-myeloid progenitors (EMP) produced in the extra-embryonic yolk sac. Focusing on macrophages and mast cells, we present emerging evidence implicating them in lifelong disease by either somatic mutations or developmental programming events resulting from maternal and early environmental perturbations.
PubMed: 33708774
DOI: 10.3389/fcell.2021.648313 -
Frontiers in Physiology 2015The pericardium is one of the serosal cavities of the mammals. It consists of two anatomical structures closely connected, an external sac of fibrous connective tissue,... (Review)
Review
The pericardium is one of the serosal cavities of the mammals. It consists of two anatomical structures closely connected, an external sac of fibrous connective tissue, that is called fibrous pericardium and an internal that is called serous pericardium coating the internal surface of the fibrous pericardium (parietal layer) and the heart (visceral layer) forming the pericardial space. Between these two layers a small amount of fluid exists that is called pericardial fluid. The pericardial fluid is a product of ultrafiltration and is considered to be drained by lymphatic capillary bed mainly. Under normal conditions it provides lubrication during heart beating while the mesothelial cells that line the membrane may also have a role in the absorption of the pericardial fluid along with the pericardial lymphatics. Here, we provide a review of the the current literature regarding the physiology of the pericardial space and the regulation of pericardial fluid turnover and highlight the areas that need to be further investigated.
PubMed: 25852564
DOI: 10.3389/fphys.2015.00062 -
Acta Medica Portuguesa Jan 1998Spinal arachnoiditis, an inflammatory process involving all three meningeal layers as well as the nerve roots, is a cause of persistent symptoms in 6% to 16% of... (Review)
Review
Spinal arachnoiditis, an inflammatory process involving all three meningeal layers as well as the nerve roots, is a cause of persistent symptoms in 6% to 16% of postoperative patients. Although spinal surgery is the most common antecedent associated with arachnoiditis, multiple causes have been reported, including infection, intrathecal steroids or anesthetic agents, trauma, subarachnoid hemorrhage and ionic myelographic contrast material--both oil soluble and water soluble. In the past, oil-based intrathecal contrast agents (Pantopaque) were associated with arachnoiditis especially when this material was introduced into the thecal sac and mixed with blood. Arachnoiditis is apparently rarely idiopathic. The pathogenesis of spinal arachnoiditis is similar to the repair process of serous membranes, such as the peritoneum, with a negligible inflammatory cellular exudate and a prominent fibrinous exudate. Chronic adhesive arachnoiditis of the lower spine is a myelographic diagnosis. The myelographic findings of arachnoiditis were divided into two types by Jorgensen et al. In type 1, "the empty thecal sac" appearance, there is homogeneous filling of the thecal sac with either absence of or defects involving nerve root sleeve filling. In type 2 arachnoiditis, there are localized or diffuse filling defects within the contrast column. MRI has demonstrated a sensitivity of 92% and a specificity of 100% in the diagnosis of arachnoiditis. The appearance of arachnoiditis on MRI can be assigned to three main groups. The MRI findings in group I are a conglomeration of adherent roots positioned centrally in the thecal sac. Patients in group II show roots peripherally adherent to the meninges--the so called empty sac. MRI findings in group III are a soft tissue mass within the subarachnoid space. It corresponds to the type 2 categorization defined by Jorgensen et al, where as the MRI imaging types I and II correspond to the myelographic type 1.
Topics: Arachnoiditis; Contrast Media; Humans; Lumbar Vertebrae; Myelography
PubMed: 9542180
DOI: No ID Found