[Orosomucoid and sialic acid - structure, functions, clinical relations]. / Orozomukoid és sziálsav ­ szerkezet, funkció, klinikai vonatkozások.

The work focuses on orosomucoid. First, the biological role and the development of this essential molecule is summarized, then the formation of its special molecular structure is introduced. After the summary of the specific characteristics and the physiological, pathophysiological as well as clinical properties of orosomucoid and sialic acid, their most important roles are also presented. Orosomucoid belongs to the family of lipocalins and the subfamily of immunocalins - this determines its immunological, anti-inflammatorical and transporting capacities. Another important quality of the molecule is that it is recognized by lectins. Orosomucoid is highly bioactive. More knowledge is increasing about its function and biological role. The clinical relations are summarised, too. Orv Hetil. 2019; 160(8): 283-290.

[Oligo- and polysacharides, proteoglycans]. / Oligo- és poliszacharidok, proteoglikánok.

After clearing up the confusion in the previously used nomenclature, the paper discusses the family of small-leucin-rich glycoproteins. After mentioning the family and its role in the organisation of the ground substance and in the regulation of inflammation, one unique molecule of proteoglycans is presented. This molecule is the inter-α-trypsin inhibitor. It has turned out that this peculiar molecule originally contains glycosaminoglycan beside the neutral carbohydrates. After summarizing the numerous connections of the proteoglycan molecule, the "self-sacrificing" nature of the molecule is presented. It is noted that the functions of the ovaries are also controlled by this molecule. The unique structure and fundamental function of the CD44 is also analysed. The CD44 is a transmembrane proteoglycan receptor with diverse functions and actions. The organisation has intramolecular, transmembrane sections, a stalk and one globular part and contains glycosaminoglycan and oligosacharids, too. The construction contains sialic acid residues at end positions of the molecular chains, which hinder it from joining other sialic acid structures in terminal positions. Therefore, this molecule has to be separated to open up a way for the sequencing neutral molecule (e.g., hyaluronan) to be attached. CD44 is the most important, but not the only receptor of hyaluronan. It can form connections with a lot of constructions, e.g., blood cells, the human endothelial, adventitial and mesenchymal. Human extracellular matrix proteinases can change the receptor's structures. Both the molecular and the supramolecular complexes boast unique features. The giant hyaluronan chains can connect to hyaluronan-binding proteoglycans - particularly hyaladherins to hyaluronectins - to create supramolecular connections and networks. The molecular mass of the fragments of hyaluronan is in the range of some kDs, while the whole molecule weighs up to several million Ds. The fragments are independent masses and can form attachments by themselves. The fragments have important inflammatory effects. Previously unidentified proteoglycan fragments have also been identified. Five of the ten identified fragments are secreted after synthetizing and stored in the granula of human cells. These strange characteristics are connected to the features of chromogranins/secretogranins. Nevertheless, this is another system of the organism, the functional, molecular structures of which are synthetized in neuroendocrine cells. Orv Hetil. 2018; 159(16): 620-627.

[Physiological, pathophysiological and clinical significance of chromogranins/secretogranins]. / A kromograninok, szekretograninok élettani, kórélettani és klinikai szerepérol.

This paper investigates the fundamental knowledge, build-up, as well as essential structural and important features of the big family of chromogranins/secretogranins. Previously the different properties and the slightly diverging funcional relations of the two family members were in focus. Later on, it has been discovered that they are essentially two similar compounds with identical structures and functions, and they are chemically, biochemically related. From details discovered so far we can tell that they are long polypeptid chains formed from amino acids. Based on insights gained until now we can also state that these compounds are formed in Ca++ containing environments with acidic pH. Among the compounds there are several molecules which have characteristic oligosacharid groups. This is especially interesting because oligosacharid chains with sialic acid in terminal position play an important role in the recognising and connectional processes. The chromogranins/secretogranins are mostly formed in neuroendocrine cells, but are also capable of building up in any cell type in the organism during pathological processes. Intracellular biogenesis takes place in the dense endoplasmatic reticulum across the mitochondrium, developing biogenetic granulums, followed by the stimulus-motivated secretum (exocytosis). The next stage of the molecular development is the specific break-up of the long polypeptid chains into shorter fragments. These fragments have individual effects. Some important clinical (diagnostic, prognostic) significance and connections are also touched upon in this paper, however, the cardiovascular, immunological systems and the tumors are mostly in focus. There are more immunological, cardiovascular and tumoral data. It is stated that as these molecules are in close connection with all of the organisms and systems of the body, a new chief organisator system has been identified. This chief organisator is closely connected with the central nervous system. Orv Hetil. 2017; 158(28): 1092-1099.

[Biological role of heterogeneous glycoprotein structures]. / A heterogén glikoproteinszerkezetek biológiai szerepe.

Carbohydrate molecules connected mostly with covalent junctions to protein chains are called glycoproteins. These carbohydrate molecules are attached to the protein core in different qualities and order. When the protein core is connected with acidic components such as uronic acid or SO4 radicals, they are called proteoglycans. The currently used name "glycosaminoglycan" in this case is not entirely correct. In the living world polymannane structures occur, too. Glycoproteins do not only exceptionally hold acidic groups but they have neuraminic acid derivatives. Tissue, cellular and matrix structures, and mostly all serum "proteins" are mainly glycoproteins. In the everyday clinical practice glycoproteins are mentioned as proteins. Nevertheless, the inadequate use of the concept may cause errors in the attitudes, too. This paper aims to correct this notion, because the term of "glycobiology" has already been expanded to be an independent scientific field. The practical clinical consequences of recent knowledge in this field are also summarized including novel findings on glycoprotein structures and functions. The importance of the quantity of carbohydrates, and their structural arrangements are also presented. In short, significance of glycoprotein-carbohydrate structures, as well as their physiological and pathological roles are reviewed in order to introduce the field of "glycobiology". Orosomucoid and immunoglobulins are discussed separately. Orv. Hetil., 2016, 157(30), 1185-1192.

[The liver and the immune system]. / A máj és az immunrendszer.

The liver is known to be the metabolic centre of the organism and is under the control of the central nervous system. It has a peculiar tissue structure and its anatomic localisation defines it as part of the immune system having an individual role in the defence of the organism. The determinant of its particular tissue build-up is the sinusoid system. In addition to hepatocytes, one cell row "endothelium", stellate cells close to the external surface, Kupffer cells tightly to its inner surface, as well as dendritic cells and other cell types (T and B lymphocytes, natural killer and natural killer T-cells, mast cells, granulocytes) are present. The multitudes and variety of cells make it possible to carry out the tasks according to the assignment of the organism. The liver is a member of the immune system having immune cells largely in an activated state. Its principal tasks are the assurance of the peripheral immune tolerance of the organism with the help of the haemopoetic cells and transforming growth factor-ß. The liver takes part in the determination of the manner of the non-specific immune response of the organism. In addition to acute phase reaction of the organism, the liver has a role in the adaptive/specific immune response. These functions include retardation of the T and B lymphocytes and the defence against harmful pathogens. With the collaboration of transforming growth factor-ß, immunoglobulins and their subclasses are inhibited just as the response of the T lymphocytes. The only exception is the undisturbed immunoglobulin A production. Particularly important is the intensive participation of the liver in the acute phase reaction of the organism, which is organised and guided by the coordinated functions of the cortico-hypothalamo-hypophysis-adrenal axis. Beside cellular elements, hormones, adhesion molecules, chemokines and cytokines are also involved in the cooperation with the organs. Acute phase reactants play a central role in these processes. Until recently the α2-macroglobulin was not considered as an acute reactant of the organism, but it is now functionally included in the acute phase reaction presumably due to its close connection with the transforming growth factor-ß. Transforming growth factor-ß has extraordinarily important roles in all phases of inflammation and in the specific immune response. The peripheral immune tolerance of the organism involves tightly coupled regulation of proliferation, differentiation and survival of lymphocytes.

[Bone tissue: rebuilding and inflammation]. / Csontszövet: Újdonképzodés és inflammatio.

In this review the author summarizes the knowledge related to structural elements of bone tissue. The process of bone reorganisation and knowledge about the special feature of bone metabolism in human are also discussed. It is noted that due to the reorganisation, there is a complete renewal of bone tissue in every 10 years, and this renewal lasts throughout the life. However, there are life periods when osteoclast activity is low, e.g. in childhood and the second decade of life when the gain of bone mass may be as much as 40% of the final bone mass. Overactivity of osteoclasts occurs at age 60 years in men and somewhat earlier in women. Reorganization of bone tissue is an elementary requirement for the physiological functions (locomotion, hemopoiesis, immune functions). The RANK-RANKL-osteoprotegerin axis plays an important role in the regulation of bone metabolism. Bone mass is dependent on osteocytes; osteoblasts are building up while osteoclasts are reabsorbing bone tissue. In this process transcription factors, hormone-like substances and a large number of cytokines are involved. In addition, the inflammatory process within the bone tissue as well as the defending, reparative inflammation and specific immune response are of great importance in bone reorganisation. This is particularly valid for α2-macroglobulin and transforming growth factor, although the exact role in bone reorganization has not been fully explored. It can be concluded that the elements, which participate in bone reorganization and in defending inflammatory and specific immunological processes, are essentially identical. Therefore, the existence of an osteo-immunological complex system has emerged.

[Connective tissue and inflammation]. / Kötoszövet és inflammatio.

The author summarizes the structure of the connective tissues, the increasing motion of the constituents, which determine the role in establishing the structure and function of that. The structure and function of the connective tissue are related to each other in the resting as well as inflammatory states. It is emphasized that cellular events in the connective tissue are part of the defence of the organism, the localisation of the damage and, if possible, the maintenance of restitutio ad integrum. The organism responds to damage with inflammation, the non specific immune response, as well as specific, adaptive immunity. These processes are located in the connective tissue. Sterile and pathogenic inflammation are relatively similar processes, but inevitable differences are present, too. Sialic acids and glycoproteins containing sialic acids have important roles, and the role of Siglecs is also highlighted. Also, similarities and differences in damages caused by pathogens and sterile agents are briefly summarized. In addition, the roles of adhesion molecules linked to each other, and the whole event of inflammatory processes are presented. When considering practical consequences it is stressed that the structure (building up) of the organism and the defending function of inflammation both have fundamental importance. Inflammation has a crucial role in maintaining the integrity and the unimpaired somato-psychological state of the organism. Thus, inflammation serves as a tool of organism identical with the natural immune response, inseparably connected with the specific, adaptive immune response. The main events of the inflammatory processes take place in the connective tissue.

[The way of self-defence of the organism: inflammation]. / A szervezeti önvédelem módja: a gyulladás.

The acute and chronic constitutional reactions of the organism elicited by sterile causes and pathogenic structures threatening the soundness of the organism are surveyed by the author. It is emphasized that depending on causes which can be very different, there are various syndromes occurring in the clinical practice. On the basis of multitudiness of pathogenic factors and individual differences, the infammatory reactions are clinically, pathologically and pathobiochemically can be hugely variable. The acute inflammatory response may be sterile. It is often difficult to recognize in these processes whether the inflammation is harmful or beneficial for the organism as a whole. It is possible that the inflammatory response itself is the defending resource of the individual. The non-sterile acute inflammation is evoked by pathogenic microorganisms. The variety of clinical syndromes are explained by the high diversity of pathogenic microbes, the individualities of the defending organisms, and the natural and adaptive immunity of the organism which may be intact or possibly defective. In the latter case the inflammation itself is the disease, as a consequence of a pathological process conducted by the cortico-hypothalamo-adernal axis. The acute inflammation is a defending, preventing and repairing process, constituting an important part of the natural innate immune response. It is inseparable from the natural innate immune response, which is in close cooperation with the adaptive, specific immune response with mutual effects on each of the other. The conductor and the response reactions of the two immune responses are also the same. There are alterations in serum proteins/glycoproteins synthesized mostly by the hepatocytes. Because the concentration of almost all proteins/glycoproteins may change, the use of the discriminative term "acute phase reactant" is hardly relevant. For example, the HDL molecule is a negative "acute phase reactant". On the gound of clinical, pathological and biochemical caracteristics, the chronic sterile inflammation is a very different entity. It has been established that atherosclerosis is one of the ab origine chronic inflammatory syndrome. It is a long-lasting pathological entity progressing, rather than resolving with different celerity, namely a unique vasculitis syndrome. We are speaking about risk factors instead of causes, which constitute larger or smaller groups to elicite the preventing reaction of the host. The propagations and final outcomes are quite different from that of the acute process. The disadvantages or benefits for the organism are scarcely predictable, albeit the chronic process may have roles in its prolonged nature.

Multicentric plasmocytic Castleman's disease with polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes syndrome and coexistent human herpes virus-6 infection--a possible relationship.

Authors report a case of Castleman's disease (CD) with polyneuropathy, organomegaly, endocrinopathy, M protein, skin change (POEMS) syndrome. According to the present knowledge, these two rare conditions are often induced by Human Herpes Vírus- 8 (HHV-8) or by Human Immunodefeciency Virus, separately or in combination. In this case, however, HHV-6 viral DNA had been detected in the blood and lymph node samples by PCR. The authors conclude that the modulation of immune functions by HHV-6 might be responsible for the development of CD and POEMS syndrome in the referred case.

[Treatment of limb injuries at a regional center]. / Végtagi sérülések ellátása egy regionális centrumban.

INTRODUCTION: The objective of the study was to analyse the causes of injury, surgical approaches, outcome, and complications with vascular trauma in patients operated on over a period between 1986-2006. MATERIAL AND METHODS: In 128 patients with peripheral arterial injuries the mechanism was direct penetration in 90 cases and blunt injury in 30 cases. In 8 cases a chronic damage (false aneurysm, AV fistula) was observed. Isolated vascular trauma was present in 97 patients (75.8%), 31 cases (24.2%) were complicated by concomitant bone fractures,and nerve or soft tissue damage. Most frequently injured vessels were the superficial femoral (22.6%) crural (22.6%),and ulnar and radial (13.2%) arteries. RESULTS: In 132 cases operation had to be performed. Direct suture 26, interposition in arterial injuries 26, interposition in venous injuries 5, end to end anastomosis in 12 cases, venous patches in 7 cases were the operation method. In 28 cases non-reconstructive operation was carried out, and there were 4 endovascular procedures. Five secondary amputations were performed and five patients died. The limb salvage rate was 95%. DISCUSSION: Most vascular injuries of the extremities can be managed successfully unless associated with severe concomitant damage of the bones, nerves and soft tissues.

[Klippel-Trenaunay syndrome]. / Klippel-Trenaunay-szindróma.

The author in connection with three properly observed female patients summarizes the clinical entity of Klippel-Trenaunay syndrome, its partly resembling partly dissociating features facing to other vascular congenital anomalies, and the most important aspects of vasculogenesis. Based on the observation, attention is distinctly called on the first case in whom the Klippel-Trenaunay syndrome presenting with multiple mesenchymal anomalies was associated with selective congenital IgA deficiency. The second was the daughter of this first patient, who also had the syndrome and a selective congenital IgA deficiency. The other daughter of the patient was free from both of the pathological conditions. The third case represented the features of the typical syndrome without verified immunodeficiency. The literary data do not speak about possible correlations between Klippel-Trenaunay syndrome and immunodeficiencies. The pathogenesis of the process and of its possible connections with immunodeficiencies remain to be investigated.

[Glycosaminoglycans, proteoglycans, atherosclerosis]. / Glikózaminoglikánok, proteoglikánok, atherosclerosis.

Glycosaminoglycans, proteoglycans have fundamental roles in the building up of the vascular walls. The research work of the correlations between glycosaminoglycans, proteoglycans and the pathogenesis of atherosclerosis was since more decades proceeding. The prominent experts of the scope are paying more and more attention to the significant influence of the inflammation ongoing in the arterial vessel wall. Glycosaminoglycans, proteoglycans are equally contributing to the development, stopping or remaining, turning into chronic type of the inflammatory process. On the ground of all these this article makes an attempt to outline, using up the newest results of the investigations being in relation with the fundamental structures, the alterations in the composition, the occurrences, the essential functions of the glycosaminoglycans, proteoglycans. Afterwards the diverse important roles of these molecular organizations played in the pathogenesis, in the progression of atherosclerosis, and in the arising of its complications are surveyed. Basing on the considerable data of the observations it can be established that glycosaminoglycans, proteoglycans have determining importance in the individual, peculiar inflammation progressing in the arterial vessel wall, in the surviving of the special vasculitis, in the appearance of atherosclerosis, in the focal nature and progression of that and in the development of its complications. The knowledge becoming continuously more complete affords, a long-range expectation in relation to both the prevention and therapeutic intervention of that widespread disease.

[The pathogenesis of atherosclerosis]. / Az atherosclerosis patogenezise.

The article dealing with the pathogenesis of atherosclerosis, summarizes the knowledge, which relies on the latest investigative results, and which is in connection with the starting of atherosclerosis and with the chronic type inflammation ongoing in the arterial vessel wall, having individual characteristics. Then the general features of the inflammatory response of the organism, the emerging infections, just as that of the autoimmune phenomena are touched upon. The importance of the contribution to the pathogenetic chain of the lipoproteins, the connective tissue matrix proteoglycans and glycosaminoglycans is summed up in distinct chapters. The distinguished significance of the attachment between the proteoglycans and lipoproteins, oxydized lipopoteins is particularly underlined. It is established that the chronic nature inflammatory process, possessing individual characteristics, has essential role in the starting, development and in the entire course of the pathological process. The impacts of infections, and that of the possible immunodeficiencies are uncertain. In the author's opinion the definition of atherosclerosis syndrome might be as follows. The point is a non specific chronic inflammatory response of the organism in the form of a specific vasculitis, which constitutes ground to the development of the atherosclerotic process.

Pearson-type I distribution function for polydisperse polymer systems. Molar mass distribution.

This paper presents an attractive feature of the distribution function, which uses a relatively simple expression for approximating probability density. The Pearson-type I distribution function is used to represent the molar mass distribution (MMD) function for polymers for which the number average (M(n)), mass average (M(w)), z-average (M(z)), and (z+1)-average (M(z)(+1)) values are available. In continuation, the Pearson-type I distribution is applied as the model MMD function in which model parameters (M(n), M(w), M(z), and M(z)(+1)) are fitted from experimentally determined MMD. As the result, different molar mass averages are estimated with satisfactory agreement with experimental data.

Human fetuin/alpha2HS-glycoprotein level as a novel indicator of liver cell function and short-term mortality in patients with liver cirrhosis and liver cancer.

OBJECTIVE: Human fetuin/alpha2HS-glycoprotein (AHSG) is synthesized by hepatocytes. We intended to determine whether liver dysfunction or acute phase reaction is dominant in the regulation of its serum concentrations and to see if decreased AHGS levels are associated with short-term mortality. DESIGN: We determined the serum AHSG levels in patients with acute alcoholic, acute A, B, and Epstein-Barr virus hepatitis, alcoholic cirrhosis, and hepatocellular cancer and correlated them to conventional laboratory parameters of inflammation and liver function. Patients were followed for 1 month. METHODS: Serum AHSG was determined by radial immunodiffusion. RESULTS: Compared to controls, significantly lower AHSG levels were found in patients with liver cirrhosis and hepatocellular cancer but not the acute viral hepatitides. Strong positive correlation with serum transferrin, albumin and prothrombin was found. Febrile episodes were not associated with significantly decreased AHSG levels. Concentrations below 300 microg/ml were associated with high mortality rate (52.0%; relative risk, 5.497; 95% confidence interval, 2.472-12.23; P < 0.0001). Of all laboratory parameters studied serum AHSG levels showed the greatest difference between deceased and survived patients with cirrhosis and cancer. Moreover, other acute phase reactants did not differ significantly. The multiple logistic regression analysis indicated that the decrease of serum AHSG is independent of all other variables that were found decreased in deceased patients. CONCLUSIONS: Decreased serum AHSG concentration is due rather to hepatocellular dysfunction than the acute phase reaction and is an outstanding predictor of short-term mortality in patients with liver cirrhosis and liver cancer.