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Hypertension and nephrology

SEPTEMBER 10, 2019

[Role of IL-10 family of cytokines in kidney fibrosis]

PAP Domonkos, VERES-SZÉKELY Apor, SZEBENI Beáta, SZIKSZ Erna, KISS József Zoltán, TAKÁCS István Márton, REUSZ György, SZABÓ J. Attila, VANNAY Ádám

[Chronic renal failure is a major health problem, affecting 8 to 16% of the population. Regardless of the etiology the common hallmark of chronic renal failure is inflammation, leading to the activation of renal myofibroblasts. Chronic activation of myofibroblasts lead to abnormal accumulation of extracellular matrix, disruption of the architecture of the kidney and finally to reduced renal function. Although our knowledge is rapidly expanding about the pathomechanism of chronic renal failure, we still have no drug to treat or hinder the progression of the disease. In our present review article, we summarize the role of the cytokines of the IL-10 family in renal scarring.]

Hypertension and nephrology

JUNE 20, 2019

[Experimental models of renal fibrosis]

KÖKÉNY Gábor

[The high incidence of chronic kidney diseases and, regardless of the etiology, their progression to renal fibrosis with end-stage renal failure rise the urgent need to reveal the pathomechanisms. As the disease leads to complex changes in the body, it is essential to use in vivo model systems for these investigations. Animal experiments choosing the appropriate model system helps to develop more sensitive early diagnostic markers and new therapeutic approaches. Several animal experimental model descriptions can be found in the literature, which mimic specific or more general human diseases in order to help the better understanding of the pathomechanisms. Using these model systems, we are able to analyze the detailed pathophysiology of glomerulonephritis, tubulointerstitial fibrosis, glomerular scarring or generalized renal fibrosis. The most commonly used model systems for renal fibrosis are presented and discussed.]

Lege Artis Medicinae

SEPTEMBER 20, 2018

[Multidisciplinarity and pulmonary hypertension in idiopathic pulmonary fibrosis]

BOHÁCS Anikó, KARLÓCAI Kristóf, KERPEL-FRONIUS Anna

[Idiopathic pulmonary fibrosis (IPF) is a subgroup of the fibrotising idiopathic interstitial pneumonias occuring primarily in older adults. It is characterised by progressive decline of lung function and is associated with high mortality. IPF is frequently associated with pulmonary hypertension (PH). PH has unfavourable impact on the prognosis of IPF. PH should be suspected in IPF patients presenting with dyspnoe, desaturation on exertion and disproportionately low diffusion capacity. Transthoracic echocardiography is used to screen for PH in IPF patients. Although right heart catheterization is the gold standard procedure for the diagnosis of PH, this is not regularly performed on IPF patients. Chest high resolution computer tomography (HRCT) is essential for the diagnosis of IPF. IPF is typically characterised by the presence of usual interstitial pneumonia (UIP) pattern on HRCT. Multidisciplinary discussion bet­ween experienced pulmonologists, radiologists, and pathologists is key in the early and accurate diagnosis of IPF. An important role of the interstitial lung disease-multidisciplinary team (ILD-MDT) is to determine whether other diagnostic examinations and surgical lung biopsy is needed, in an attempt to reduce unnecessary risk. ILD-MDT should propose the initiation of antifibrotic therapies that have the potential to reduce disease progression. All patients diagnosed with IPF, with no contraindications for lung transplantation, should be referred early to a transplant committee.]

Lege Artis Medicinae

AUGUST 30, 2018

[How to diagnose idiopathic pulmonary fibrosis. Part 2]

HORVÁTH Ildikó, KERPEL-FRONIUS Anna, HARKÓ Tünde

[Idiopathic pulmonary fibrosis is a severe irreversible lung disease with a progressive course. The disease onset is hard to discover due to the unspecific signs and symp­toms. It occurs mainly in elderly people. In the past decades its prevalence has increased continuously. Physical examination, restrictive pattern on lung function test with decreased diffusion capacity are characteristic features of the disease. Chest X-ray showing fibrotic pattern also points toward the diagnosis of idiopathic pulmonary fibrosis. Differential diagnosis is based on high resolution komputertomográfy. Diag­nosis of IPF is based on the appearance of usual interstitial pneumonia pattern together with the lack of external risk factors and autoimmune or other diseases also known to cause this pattern seen on chest imaging. If no firm diagnosis can be built lung biopsy is required. Multidis­ciplinary teams from clinician, radiologist and pathologist are set in predefined centres that could provide care with novel antifibrotic drugs. These can slow disease progression and are in the frontline in the treatment of the disease. Further research is required to understand the pathomechanism and foster the discovery of further treatment options. ]

Lege Artis Medicinae

JUNE 20, 2018

[Diagnosis of interstitial lung diseases]

MÜLLER Veronika

[Recognition and diagnosis of interstitial lung diseases (ILD) is one of the most challenging tasks of respiratory medicine. In the wide range of different ILD-s idiopathic pulmonary fibrosis (IPF) is one of the most common. Improvements in the diagnosis made the recognition of IPF easier. Typical clinical features and usual interstitial pneumonia pattern on high resolution CT are now enough for the confident diagnosis of IPF in the multidisciplinary ILD-team discussions. Bronchoscopic cryobiopsy is a great achievement of histological sampling as this can give appropriate size tissue for histological analysis making surgical intervention rare. As IPF cannot be cured early diagnosis is crucial. Today available treatments can only slow down progression of the disease, so early stages and better clinical condition of the patients are essential for therapeutic success. Follow-up of IPF patients includes complex lung functional measurements in dedicated centers in Hungary. Former studies confirmed forced vital capacity (FVC) decline as an important measure of disease progression and mortality. Available IPF treatments decrease FVC decline and can prevent the mostly deadly acute exacerbations of the disease. Additionally palliative treatments including supplementary oxygen and in selected patients lung transplantation can be offered. This is the first of a series of three articles about IPF. ]

Hypertension and nephrology

SEPTEMBER 10, 2016

[Hypertension and left ventricular hypertrophy]

MATOLTSY András

[Left ventricular hypertrophy (LVH) is defined as an increase in the mass of the left ventricle. In addition to the absolute increase in mass, the geometric pattern of LVH also may be important. LVH can be secondary to an increase in wall thickness, an increase in cavity size, or both. LVH as a consequence of hypertension usually presents with an increase in wall thickness. This increase in mass predominantly results from a chronic increase in afterload of LV caused by the hypertension, although there is also a genetic component. A significant increase in the number and/or size of sarcomeres is the main pathologic mechanism, but hypertension may also result in interstitial fibrosis. The estimation of mass is commonly derived from measurements obtained by echocardiography. LVH is associated with increased incidence of systolic and/or diastolic dysfunction, heart failure, myocardial infarction, ventricular arrhythmias, sudden cardiac death, aortic root dilatation, and a cerebrovascular event. The cardiovascular risk is directly related to the degree of mass. The regression of LVH is associated with a reduction in cardiovascular risk and improved cardiac function. Regression of LVH is associated with weight loss, dietary sodium restriction, and use of ACE inhibitors, ARBs, some calcium channel blockers, and some sympatholytic agents.]

Lege Artis Medicinae

APRIL 20, 2016

[Extensive retroperitoneal extramedullary hematopoiesis mimicking disseminated abdominal cancer]

KŐVÁRI Bence, DONKÓ Veronika, PIUKOVICS Klára

[INTRODUCTION - Myelofibrosis is a type of chronic myeloproliferative neoplasia frequently associated with extramedullary hematopoiesis. This latter process usually affects the spleen and the liver, and should be designated as nonhepatosplenic extramedullary hematopoiesis if it involves other organs. Nonhepatosplenic extramedullary hematopoiesis is reported to be more common in patients who had splenectomy. CASE REPORT - A 66-year-old woman with 5-year history of myelofibrosis was hospitalized eight month prior to death due to increasing abdominal effusion, abdominal discomfort and dyspnea. Three years before death, splenectomy was performed. The abdominal imaging studies disclosed a circumscribed tumorous mass in the pancreas, with enlargement of the peripancreatic lymph nodes. The lesion interpreted as pancreatic cancer progressed and the patient died. Post mortem histological evaluation confirmed the abdominal mass to represent myeloid metaplasia of the retroperitoneal fat tissue. CONCLUSIONS - Besides the possibility of a secondary primary tumor, the discovery of a novel mass lesion in patients with myelofibrosis should raise the suspicion of extramedullary hematopoiesis, especially when the patient had splenectomy.]

Clinical Neuroscience

MAY 30, 2016

Comparison TachoComb with SurgiWrap, Surgicel and Lyodura in epidural fibrosis: An experimental rat model

HUSEYIN Karasu, IŞIL Güzel

Objectives - We designed this study to evaluate the effect of TachoComb and to compare it with SurgiWrap, Surgicel, and Lyodura in an experimental rat model for epidural fibrosis (EF). Methods - This prospective and experimental rat model study was performed at Dicle University, School of Medicine, Department of Neurosurgery, in Diyarbakır, Turkey, between January 2005 and June 2005, using 50 Sprague Dawley rats (30 female and 20 male) with a mean weight of 290 g. The rats were divided into five groups: TachoComb, Surgicel, SurgiWrap, Lyodura, and control. Results - Eight weeks after laminectomy, the rats were killed and EF was evaluated. EF was statistically different between the groups. The lowest EF score was in the TachoComb group (F=6.915, p<0.001). Conclusions - According to this study, TachoComb was found to be a more effective agent in decreasing EF than Surgicel, SurgiWrap, or Lyodura.

Lege Artis Medicinae

JULY 20, 2015

[Treatment of chronic Pseudomonas aeruginosa lung infections in cystic fibrosis with inhaled tobramycin]

NAGY Béla, HOLICS Klára

[BACKGROUND - Intermittent or chronic pulmonary infections caused by Pseudo­monas aeruginosa (Pa) deteriorate clinical status and worsen lung function in patients with cystic fibrosis (CF). The prognosis of the disease and life expectancy of patients are substantially dependent on lung infections and inflammation; therefore the primary goal of the treatment is the early termination of the infection. PATIENTS AND METHODS - Efficacy of tobramycin (TOBI® 300 mg/5 mL solution for inhalation, henceforth TOBI®) inhalation in Pa pulmonary infections was studied in a non-interventional, observational, open-label, single-arm trial in subjects with CF. Fifty-three patients aged six to 31 years (averaged 15.8 years) were enrolled into the study. Three treatment cycles of TOBI® inhalation (28 days on drug, 28 days off drug) plus 6 months observational period were evaluated. Primary endpoint was changing in the predictive values of forced expiratory volume in 1 sec (FEV1) compared to the initial values after three cycles of the treatment. Secondary endpoints were changing in the FEV1 predictive values at the end of the complete study compared to the initial values; ratios of patients with decreased density of originally Pa-positive result of sputum culture; as well as safety and tolerability of the TOBI® treatment. RESULTS - FEV1 and FEV1% results were unchanged comparing to the initial values at the end of the treatment and after the observational period in the whole study population. However, sputum cultures became negative in 47.2% of all subjects as a result of the treatment, and the FEV1 values were gradually increased in these patients: after the third treatment cycle plus 160 mL, and at the end of the study plus 110 mL comparing to the initial values. Similar considerable increase was detected in the predictive FEV1% in this sub-group: after the first cycle plus 5.2%, at the end of third cycle plus 7%, after the observational period above 3.8% considering the starting results. CONCLUSIONS - TOBI® inhalation proved to be effective in improvement of lung function results and eradication of infection in our patients with Pa positive CF. The treatment was well tolerated and safe.]

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.]