Meningiomas are one of the most frequent primary intracranial tumours, representing one-third of all intracranialneoplasms. The vast majority of meningiomas are histologically benign, but recurrence and progression is quite frequent. They occur usually between the 6th and 7th decade, the female/male ratio is 3:2. Although rare in pregnancy, when occurring, they can cause serious, life-threatening complications due to rapid growth and unfavourable localisation. There are two dominant hypothesis explaining rapid growth in pregnancy: the role of hormonal effects and hemodynamic changes. Several studies tested these theories but none provided unequivocal answer probably because the pathomechanism is complex and multifactorial. We provide an overview of the pathomechanism of meningiomas in pregnancy with emphasis on data obtained by advanced neuropathological, molecular biological, bioinformatic, imaging and epidemiological methods. A better understanding of the processes leading to meningioma development and growth in pregnancy will help us to design personalized therapy and reduce morbidity and mortality.
A Lewy-testes demencia a második leggyakoribb neurodegeneratív demencia. A pontos diagnózisa gyakran csak neuropatológiai vizsgálattal lehetséges. A betegség fő morfológiai jellemzője a kóros α-szinukleinben gazdag Lewy-test és Lewy-neurit -csakúgy, mint a rokon kórkép Parkinson-kór és az ahhoz társuló demencia esetén. A patomechanizmus fontos tényezői a neurotranszmitter rendszerek zavara, a szinaptikus diszfunkció és az ubikvitin-proteaszóma rendszer elégtelen működése. Jellemző a kognitív teljesítmény fluktuációja, parkinsonizmus és vizuális hallucináció. Mivel gyakran nem típusos a klinikai kép és időbeni lefolyás, a képalkotó eljárások és biomarkerek elősegíthetik a korai felismerést. Noha hatékony oki terápia nincs, életminőséget javító kezelések lehetségesek. A klinikopatológiai vizsgálatok kiemelten fontosak a patomechanizmus jobb megértése, a pontos diagnózis és a hatékony terápia érdeké-ben. Orv Hetil. 2017; 158(17): 643-652.Kulcsszavak: Lewy-testes demencia, neuropatológia, biomarkerek, Parkinson-kór, α-szinuklein Clinical and neuropathological characteristics of dementia with Lewy bodiesDementia with Lewy bodies (DLB) is the second most common neurodegenerative dementia. The accurate diagnosis is often possible only by neuropathological examination. The morphologic hallmarks are the presence of α-synuclein-rich Lewy bodies and Lewy neurites, identical to those seen in Parkinson's disease (PD) and Parkinson's disease dementia (PDD). Neurotransmitter deficits, synaptic and ubiquitin-proteasome system (UPS) dysfunction play major role in the pathomechanism. Characteristic symptoms are cognitive fluctuation, parkinsonism and visual hallucinations. Due to the often atypical clinical presentation novel imaging techniques and biomarkers could help the early diagnosis. Although curative treatment is not available, therapies can improve quality of life. Clinicopathological studies are important in exploring pathomechanisms, ensuring accurate diagnosis and identifying therapeutic targets.
Purpose: The aim of this study was to examine the expression pattern of tenascin-C, matrilin-2, and aggrecan in irreversible corneal endothelial pathology such as pseudophakic bullous keratopathy (PBK) and Fuchs’ endothelial corneal dystrophy (FECD), which most frequently require corneal transplantation. Materials and methods: Histological specimens of corneal buttons removed during keratoplasty were investigated in PBK (n = 20) and FECD (n = 9) and compared to healthy control corneas (n = 10). The sections were studied by chromogenic immunohistochemistry (CHR-IHC) and submitted for evaluation by two investigators. Semiquantitative scoring (0 to 3+) was applied according to standardized methods at high magnification (400x). Each layer of the cornea was investigated; in addition, the stroma was subdivided into anterior, middle, and posterior parts for more precise analysis. In case of non-parametric distribution Mann–Whitney test was applied to compare two groups. Kruskal–Wallis and Dunn’s multiple comparisons tests have been applied for comparison of the chromogenic IHC signal intensity among corneal layers within the control and patient groups. Differences of p < 0.05 were considered as significant. Results: Significantly elevated tenascin-C immunopositivity was present in the epithelium and every layer of the stroma in both pathologic conditions as compared to normal controls. In addition, also significantly stronger matrilin-2 positivity was detected in the epithelium; however, weaker reaction was present in the endothelium in PBK cases. Minimal, but significantly elevated immunopositivity could be observed in the anterior and posterior stroma in the FECD group. Additionally, minimally, but significantly higher aggrecan immunoreaction was present in the anterior stroma in PBK and in the posterior stroma in both endothelial disorders. All three antibodies disclosed the strongest reaction in the posterior stroma either in PBK or in FECD cases. Conclusions: These extracellular matrix molecules disclosed up to moderate immunopositivity in the corneal layers in varying extents. Through their networking, bridging, and adhesive abilities these proteins are involved in corneal regeneration and tissue reorganization in endothelial dysfunction.
A bulbusra óraüvegszerűen ráfekvő cornea a szem első fénytörő közege. A szaruhártya átlátszóságát az azt felépítő rendezett, lamellaris elhelyezkedésű kollagénrostoknak, valamint a rostok között kapcsolatot teremtő, leucinban gazdag proteoglikánoknak köszönheti. A felnőttcorneát főként fibrilláris kollagénrostok építik fel. A fibrillumokhoz kapcsolódó kollagének és a nonfibrilláris kollagének kisebb mennyiségben vannak jelen a szaruhártyában. A cornea fő proteoglikánjai keratán-szulfát-tartalmú proteoglikánok, amelyek mellett dermatán-szulfát-tartalmú proteoglikánok is megtalálhatók. A proteoglikánszintézis defektusai az egyedi szerkezet felbomlásához, a normálisnál vastagabb át-mérőjű kollagénrostok kialakulásához vezet. Az abnormális extracellulárismátrix-szerkezet cornealis betegségeket, ezáltal a szaruhártya transzparenciájának elvesztését eredményezi. A proteoglikánok és kollagénrostok a sebgyógyu-lásban is kiemelt szerepet töltenek be. A corneát ért sérülés következtében az extracelluláris mátrixot termelő keratocyták növekedési faktorok hatására fokozott kollagén-és proteoglikánszintézisbe kezdenek. A különböző növekedé-si faktorok a sérült corneában több helyről származhatnak. A sebgyógyulásban részt vevő sejtek és növekedési faktorok arányától függően rendezett extracelluláris mátrix vagy hegszövet alakul ki. Orv. Hetil., 2016, 157(25), 995-999. Kulcsszavak: cornealis kollagének, cornealis proteoglikánok, cornealis extracelluláris mátrix, cornealis sebgyógyulás The corneal wound healing and the extracellular matrixThe cornea is the first refractive element of the eye. The transparency of the cornea results from the regularly arranged collagen fibrils, forming lamellar structure and the leucin rich proteoglycans, which make interactions between the fibrils. The adult cornea consists mainly of fibril-forming collagens. The cornea has less amount of fibril associated and non-fibrillar collagens. The main proteoglycans of the cornea are keratan-sulfate proteoglycans and it also contains dermatan-sulfate proteoglycans. Disorders of the proteoglycan synthesis lead to the disruption of the unique pattern and result in thicker collagen fibrils. The abnormal structure of the extracellular matrix can generate corneal disorders and the loss of corneal transparency. Furthermore, proteoglycans and collagens have an important role in wound healing. In injury the keratocytes produce higher amounts of collagens and proteoglycans mediated by growth factors. Depending on the ratio of the cells and growth factors the extracellular matrix returns to normal or corneal scar tissue develops.Keywords: corneal collagens, corneal proteoglycans, corneal extracellular matrix, corneal wound healing Varkoly, G., Bencze, J., Hortobágyi, T., Módis, L. [The corneal wound healing and the extracellular matrix]. Orv. Hetil., 2016, 157(25), 995-999. (Beérkezett: 2016 elfogadva: 2016. április Rövidítések CTGF = (connective tissue growth factor) kötőszöveti növeke-dési faktor; ECM = extracelluláris mátrix; EGF = (epidermal growth facto...
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