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Lege Artis Medicinae

FEBRUARY 15, 2015

[Inflammatory myofibroblastic tumor of abdominal wall arising from uterine myoma]

FECSKE Éva, REMETEI Filep Aladár, SALAMON Ferenc, MAGYAR Éva

[INTRODUCTION - The inflammatory pseudotumor appears in various organs. It is well circumscribed, unencapsulated proliferation which simulates malignant tumor clinically as well as radiologically and morphologically. It’s etiology is unknown. The histology prooves inflammatory origin of this process: the nodular structure of the tumor-like mass is composed of fibroblasts, myofibroblasts, plasma cells and lympho­cytes. Recurrence may happen. CASE REPORT - Because of the rapidly expanding uterine myoma of a 40 years old woman laparotomy has been performed. In the lower two-thirds of abdominal wound the myoma penetrated into the abdominal wall. Here the abdominal wall lost it's normal structure, became bacon-like, homogenous in consistency and white-grey in colour. The myoma and the attached part of the abdominal wall was excised together. The part of myoma those in contact with abdominal wall contained proliferating myofibroblasts, fibroblasts and was infiltrated by plasma cells and lymphocytes. These findings could be seen in the abdominal wall too. The rapid proliferation which simulated malignant tumor has been diagnosed as inflammatory myofibroblastic tumor. The patient does well two years after the operation. CONCLUSION - It is important to know about the entity of inflammartory pseudotumor in differential diagnostic point of view, as it mimics malignant tumor. The structure is nodular, the histology is characteristic and the therapy is surgical. The follow up is very important because of possibility of probable recurrence. ]

Hypertension and nephrology

SEPTEMBER 20, 2014

[Signaling pathways in renal fibrosis]

ROKONAY Réka, SZIKSZ Erna, LIPPAI Rita, PAP Domonkos, VERES-SZÉKELY Apor, REUSZ György, SZABÓ Attila, VANNAY Ádám

[Myofibroblasts are the main effector cells of tissue fibrosis in chronic kidney disease. These cells are the main source of collagen rich extracellular matrix in the fibrous tissue. Recent hypotheses suggest that pericytes are the major progenitors of myofibroblasts. Platelet derived growth factor, transforming growth factor β and Wingless/Int signaling pathways play important role in pericyte activation. There are experimental evidences that blocking this pathways inhibits tissue fibrosis, therefore they might be targets for the development of antifibrotic drugs in the future.]

Clinical Neuroscience

MARCH 30, 2014

[HANS SELYE: AN INSPIRING TEACHER]

GIULIO Gabbiani

[The souvenirs of Hans Selye as a teacher of graduate and post graduate students are presented and discussed. The main aim of his teaching was to orient the student toward importance and originality of findings.]

Hypertension and nephrology

DECEMBER 30, 2012

[Molecular mechanisms leading to renal fibrosis: the origin of myofibroblasts]

HIMER Leonóra, SZIKSZ Erna, KOVÁCS S. Krisztián, ÓNODY Anna, Reusz Anna, REUSZ György, FEKETE Andrea, TULASSAY Tivadar, VANNAY Ádám

[There are about a quarter of million patients who need chronic renal replacement therapy in Europe, and the estimated number of patients with chronic kidney disease is about tenfold higher. Interestingly, regardless of the initiating cause the mechanism of fibrosis is similar to each other in the different chronic kidney diseases. In general, the damaged glomerular or tubular cells release danger signals and produce chemotactic stimuli, which trigger the rapid recruitment of leukocytes. The infiltrating immune cells and the damaged renal cells then produce high levels of proinflammatory cytokines, growth factors, chemokines and adhesion molecules which contribute to glomerular/tubular injury, accumulation of further leukocytes and myofibroblasts, which are the effector cells of renal fibrosis. However the origin of myofibroblasts is still controversial. Recent hypotheses suggest that they are originated from different renal cells, such as epithelial and endothelial cells, pericytes or bone marrow derived fibrocytes. The myofibroblasts thus generated serve as key cellular mediators of renal fibrosis. Myofibroblasts have migratory capacity, are resistant to apoptosis, produce several growth factors and cytokines and according to our present knowledge these cells are the main source of collagen-I and -III rich extracellular matrix in the fibrous tissue. Organ fibrosis is characterized with excessive deposition of extracellular matrix leading to glomerular sclerosis and renal tubulointerstitial fibrosis. The excessive deposition of fibrous tissue replaces healthy kidney tissue; nephrons disappear and kidney function declines gradually. In this article the knowledge is summarized on the molecular changes leading to the generation of renal myofibroblasts.]

Hypertension and nephrology

DECEMBER 08, 2012

[Terciary hyperparathyreosis or not? ? You cannot solve it alone: combined treatment in severe osteitis fibrosa cystica]

HERSZÉNYI Eszter, PATÓ Éva, SZALAY László, BÍRÓ Zsolt, György Andrea, DEÁK György

[Phosphate retention, consequential rise of the phosphaturic fibroblast growth factor-23 that decreases the level of calcitriol resulting in hypocalcemia facilitates the development of secondary hyperparathyroidism (sHPT) in chronic kidney disease (CKD). Hyperphosphatemia, hypocalcemia and low calcitriol level result in increasing secretion of parathormone (PTH). While sHPT occures frequently in CKD, the development of therapy-resistant and hypercalcemic tertiary hyperparathyroidism is rare due to current therapeutic approaches. We present the case of a 41 year old, treated schizophrenic, hemodialized male patient with severe osteitis fibrosa cystica, severe hyperparathyroidism (PTH 2500 pg/ml) - considered to be tertiary - and with repeated pathologic fractures. While hospitalized, the patient was under supervised, combined therapy with the vitamin D receptor activator paricalcitol and the calcimimetic cinacalcet that resulted in marked decrease of PTH level to 1589 pg/ml. However, after discharge from the hospital due to the lack of compliance he failed to take his medications and PTH had risen to the initial level. This case demonstrates that severe hyperparathyroidism thought to be therapy resistant responds well to a combination of paricalcitol and cinacalcet however, patient compliance is essential to therapeutic success.]

Lege Artis Medicinae

OCTOBER 20, 2011

[Pulmonary arterial hypertension in systemic autoimmune diseases]

VÉGH Judit, ZEHER Margit

[Pulmonary arterial hypertension is a rare disease, but it occurs more often in systemic autoimmune diseases, where it represents one of the most severe, life-threatening complications. Its development is due to an immunoregulatory disorder characteristic to systemic diseases, persistent inflammation and the subsequent endothelial dysfunction, the presence of pathogenic autoantibodies, smooth muscle cell dysfunction and complex angiogenetic disorder. As a consequence of endothelial cell dysfunction, the balance between regulatory factors of vasoconstriction and vasodilation is disrupted. Intimal hyperplasia, endothelial cell proliferation, media hypertrophy and local thrombus formation can be observed and one of the main pathomorphological characteristic features, plexiform lesion develops, leading to obliterative vasculopathy. A more severe form of the disease develops in systemic sclerosis, which is explained by the main pathophysiological elements of scleroderma, namely immunoregulatory disorder, vasculopathy and fibroblast dysfunction. It is not easy to monitor the disease in these cases, because the deterioration can be caused by many other factors as well. Therefore, beseides the usual examinations, biomarkers and screening methods have a significant role. Treatment is not simple either, since no wellapplicable algorithms are available. In many disorders (systemic lupus erythematosus, mixed connective tissue disease, rheumatoid arthritis), effective immunosuppressive therapy started in time is crucial, whereas in case of systemic sclerosis, the principles of therapy applied for the idiopathic form should be followed.]

Hungarian Immunology

DECEMBER 20, 2002

[Molecular and cellular basis of fibrosis: recent insights to the pathomechanism of scleroderma from animal models and fibroblast studies]

LAKOS Gabriella, SHINSUKE Takagawa, JOHN Varga

[Scleroderma is a chronic, progressive connective tissue disorder featuring inflammation, fibrosis, vascular injury, and immunologic abnormalities. Fibrosis, a hallmark of the disease, is characterized by excessive synthesis and deposition of extracellular matrix components, mainly type I collagen in affected tissue. The key target organs are the skin, lungs, kidneys, gastrointestinal tract and heart. The pathogenesis of fibrosis remains poorly understood, and effective treatments are lacking. While unifying concept to explain the pathogenesis of fibrosis has not yet emerged, multiple alterations result in the development of pathological tissue fibrosis have been recently identified. Transforming growth factor-β, a potent profibrotic cytokine plays a key role in the process. There is growing knowledge on identifying the cytokine and growth factor mediators of fibrosis, characterizing their interactions, and in delineating the cellular and molecular signaling pathways that are activated by these mediators. This review summarizes recent results obtained from fibroblast studies, animal models, and gene expression experiments. A major goal of investigations into the pathomechanism of fibrosis is identifying new therapeutic targets for scleroderma.]

Clinical Neuroscience

JUNE 20, 2002

[β-amyloid peptide-induced intracellular calcium level changes in Alzheimer fibroblasts]

PALOTÁS András, KÁLMÁN János, LASKAY Gábor, JUHÁSZ Anna, JANKA Zoltán, PENKE Botond

[Rationale - β-amyloid peptides, comprising the major neuropathological lesions of Alzheimer's disease, have been found to form depositions in various peripheral tissues, including the skin. Neurons in the disorder succumb to the altered ionic homeostasis and some other factors caused by this toxic peptide. In line with these findings, our study aimed to find differences in biochemical processes of cultured cutaneous fibroblasts derived from sporadic Alzheimer patients and from agematched control individuals that may mirror changes in the central nervous system. Methods - Intracellular ionic homeostasis of Alzheimer and control fibroblasts was measured in Fura-2AMloaded human fibroblasts by dual wavelength spectrofluorimetry. Results - Cells derived from Alzheimer patients exhibited lower intracellular free calcium levels as compared to the control cultures. Exposure of fibroblasts to β-amyloid resulted in increased calcium concentrations of the control cells, but not of Alzheimer ones. Conclusion - Our findings indicate that Alzheimer’s disease is a systemic disorder that, among others, affects the calcium homeostasis of fibroblasts. Even though it is unknown whether the diminished ionic response of Alzheimer fibroblasts is a disease or actual status marker, it could prove to be a useful model for the analysis of Alzheimer specific changes.]

Hungarian Radiology

DECEMBER 27, 2010

[Inflammatory myofibroblastic tumor in rare abdominal localisation]

NAGY Tamás, GÁBOR Valéria, NAGY Csaba Balázs, PUSKÁS Tamás

[INTRODUCTION - Inflammatory myofibroblastic tumor is a rare entity. The etiology and pathomechanism of this tumor is still unknown. In most of the cases it behaves as a benign or locally recurrent tumor and does not metastasize, but because of its aggressive local growth it can be judged malignant. For an accurate diagnosis adequate imaging (CT, MR), invasive intervention which is usually surgical excision and pathologic or histological examination must be performed. CASE REPORT - A case of a 19 year old woman is presented who was diagnosed by a CT scan with a 4 cm tumor in the midline of the upper third of the abdominal cavity. After surgical excision the inflammatory myofibroblastic tumor was confirmed after the pathological inspection. CONCLUSION - Despite that inflammatory myofibroblastic tumor is infrequent in virtue of the clinical picture and imaging exams we have to take this disease into account. The misdiagnosis can lead to unnecessary invasive interventions and psychological effort for the patient which could be avoided by the cooperation of the different subspecialties and thoroughgoing medical examination.]