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ABSTRACT: OBJECTIVE: Asthma is a common chronic disease complicating pregnancy with a risk for perinatal complications. Control of airway inflammation in asthmatic pregnancy improves pregnancy outcomes. Our aim was to evaluate exhaled breath condensate (EBC) pH, a non-invasive method for the assessment of asthmatic airway inflammation, in healthy and asthmatic pregnancy. DESIGN: Cross-sectional study. SETTING: Hungarian university clinics. POPULATION: 17 healthy pregnant, 21 asthmatic pregnant, 23 healthy non-pregnant and 22 asthmatic non-pregnant women. METHODS: EBC samples were collected using a portable condenser, EBC pH was measured after argon deareation. Main outcome measure. EBC pH. RESULTS: EBC pH (mean±SD) of healthy non-pregnant and asthmatic non-pregnant women was similar (7.75±0.27 vs. 7.54±0.57; p=0.118), probably indicating an optimal control of airway inflammation in asthmatics. On the other hand, EBC pH was higher in healthy pregnant compared to healthy non-pregnant women (8.02±0.43 vs. 7.75±0.27; p=0.017). Higher EBC pH accompanying healthy pregnancy was absent in asthmatic pregnant patients whose EBC pH was lower (7.65±0.38) than that of healthy pregnant women (p=0.006), and it was similar to asthmatic and healthy non-pregnant women's data (p=0.470 and p=0.300, respectively). EBC pH in asthmatic pregnancy correlated positively with birthweight (r=0.49, p=0.047) and negatively with forced vital capacity (r=0.45, p=0.039). EBC pH was not related to blood pH. CONCLUSIONS: EBC pH is higher in healthy but not in asthmatic pregnancy compared to healthy non-pregnant data, indicating that oxidative inflammatory processes induced by asthma may compromise the regulatory mechanisms causing alkaline pH in the airways during pregnancy. © 2013 The Authors Acta Obstetricia et Gynecologica Scandinavica © 2013 Nordic Federation of Societies of Obstetrics and Gynecology.
Acta Obstetricia Et Gynecologica Scandinavica 01/2013; · 1.77 Impact Factor
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ABSTRACT: Asthma has a high burden of morbidity if not controlled and may frequently complicate pregnancy, posing a risk for pregnancy outcomes. Elevated plasma level of the inflammatory biomarker soluble urokinase plasminogen activator receptor (suPAR) is related to a worse prognosis in many conditions such as infectious, autoimmune, or pregnancy-related diseases; however the value of suPAR in asthma and asthmatic pregnancy is unknown. The present study aimed to investigate the suPAR, CRP and IL-6 levels in asthma (asthmatic non-pregnant, ANP; N = 38; female N = 27) and asthmatic pregnancy (AP; N = 15), compared to healthy non-pregnant controls (HNP; N = 29; female N = 19) and to healthy pregnant women (HP; N = 58). The relationship between suPAR levels and asthma control was also evaluated. The diagnostic efficacy of suPAR in asthma control was analyzed using ROC analysis. IL-6 and CRP levels were comparable in all study groups. Circulating suPAR levels were lower in HP and AP than in HNP and ANP subjects, respectively (2.01 [1.81-2.38] and 2.39 [2.07-2.69] vs. 2.60 [1.82-3.49] and 2.84 [2.33-3.72] ng/mL, respectively, p = 0.0001). suPAR and airway resistance correlated in ANP (r = 0.47, p = 0.004). ROC analysis of suPAR values in ANP patients with PEF above and below 80% yielded an AUC of 0.75 (95% CI: 0.57-0.92, p = 0.023) and with ACT total score above and below 20 an AUC of 0.80 (95% CI: 0.64-0.95, p = 0.006). The cut-off value of suPAR to discriminate between controlled and not controlled AP and ANP was 4.04 ng/mL. In conclusion, suPAR may help the objective assessment of asthma control, since it correlates with airway resistance and has good sensitivity in the detection of impaired asthma control. Decrease in circulating suPAR levels detected both in healthy and asthmatic pregnant women presumably represents pregnancy induced immune tolerance.
PLoS ONE 01/2013; 8(4):e60697. · 4.09 Impact Factor
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Mária Szilasi,
Gabriella Gálffy,
Károly Fónay,
Zsuzsa Márk,
Zoltán Rónai,
Zsuzsa Szalai,
Magdolna E Szilasi,
Marianna Budai, Veronika Müller,
Attila Somfay,
Ildikó Horváth,
Lilla Tamási
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ABSTRACT: BACKGROUND: The casual and severity distribution of allergic rhinitis (AR) in Hungary is unknown.The aim of this survey was to evaluate symptom perception, disease severity, concomitant asthma frequency and the impact of AR on everyday life activities in a cross-sectional, multicenter study in Hungary under the supervision of Hungarian Respiratory Society. METHODS: Data were recorded by 933 AR patients (65.93% women) and their treating specialists. The perceptions of patients regarding the symptoms (nasal, ocular and others) of AR and its severity, together with its impact on everyday life were assessed. Physicians recorded data regarding the diagnosis and severity of AR, and comorbidities. RESULTS: 52.5% of patients suffered from seasonal AR, 35.1% from perennial AR. A large proportion of patients had moderate to severe disease (MS-AR) (57.34%), persistent disease (98.0%) and concomitant asthma (53.32% in the mild, 57.52% in the MS-AR group). MS-AR was more frequent among women. Despite the treatment used, in MS-AR the proportions of patients reporting moderate to severe rhinorrhoea, nasal obstruction, ocular itching/redness, watering, itchy throat and sneezing were as high as 52.0%, 54.0%, 33.8%, 26.5%, 44.0% and 31.2%, respectively. Overall, there was a poor agreement between disease severity reported by patients and specialists. The adherence to oral antihistamines and intranasal corticosteroids was found to be between 50 and 65%; mostly depending on the dosage form. CONCLUSIONS: AR remains a significant health problem in Hungary because of the burden of symptoms, high rate of concomitant asthma and the significant proportion of MS-AR affecting general well being.
Multidisciplinary respiratory medicine. 11/2012; 7(1):49.
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ABSTRACT: Active oncotherapy, combination chemotherapy of lung cancer is accompanied with many side effects which may impair patients' quality of life and compromise the effectiveness of chemotherapy. Most side effects of chemotherapy are preventable or treatable with optimal supportive care which enhances success in patient care and treatment. The aim of this review is to summarize the most important conditions that may be associated with combined chemotherapy of lung cancer from the practical point of view.
Magyar Onkológia 09/2012; 56(3):159-65.
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ABSTRACT: The inducible heat shock protein (HSP)72 plays a central role in antitumor immunomodulation. HSP72 expression was assessed on tumor samples of 43 patients with advanced and metastatic small cell lung cancer (SCLC) by immunohistochemistry and HSP72 [HSPA1B A(1267)G] polymorphism was determined. HSP72 expression of SCLC cells was significantly decreased in GG as compared to cells of AA or AG genotype patients, and was associated with significantly shorter survival in GG patients as compared to carriers of the A allele. Decreased HSP72 expression of SCLC cells associated with HSP72 GG genotype is a negative prognostic factor for survival in SCLC patients.
Cancer Investigation 04/2012; 30(4):317-22. · 1.85 Impact Factor
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Cancer Investigation 03/2012; · 1.85 Impact Factor
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Gergely Toldi,
Attila Molvarec,
Balázs Stenczer, Veronika Müller,
Noémi Eszes,
Anikó Bohács,
András Bikov,
János Rigó,
Barna Vásárhelyi,
György Losonczy,
Lilla Tamási
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ABSTRACT: Asthma is a common chronic disease that may complicate pregnancy and a risk factor for complications; however, immunological mechanisms of the bilateral interactions between asthma and pregnancy are not fully understood. Healthy gestation is characterized by a sensitive balance of T(h)1/T(h)2/T(h)17/regulatory T (Treg) cells that may be altered in asthmatic pregnancy. The aim of this study was to describe the prevalence of these cell subsets in asthmatic compared with healthy pregnancy. The prevalence of T(h)1, T(h)2, T(h)17 and Treg lymphocytes was identified by cell surface and intracellular marker staining in blood samples of 24 healthy non-pregnant (HNP), 23 healthy pregnant (HP), 15 asthmatic non-pregnant (ANP) and 15 asthmatic pregnant (AP) women using flow cytometry. The T(h)1/T(h)2 cell ratio was decreased in both HP and ANP compared with HNP women; however, no further decrease was observed in the AP group. The T(h)17/Treg ratio was decreased in HP, but not in AP women, compared with HNP data. Healthy pregnancy increased Treg cell prevalence compared with HNP data (4.64% versus 2.98%; P < 0.05), and this pregnancy-induced elevation was absent in AP women (2.52% versus 4.64%; P < 0.05). T(h)17 cell prevalence was similar in the HP and HNP groups (2.78% versus 3.17%; P > 0.05). Asthma increased T(h)17 prevalence in non-pregnant patients (3.81% versus 3.17%; P < 0.05), and this asthma-specific increase of T(h)17 cell prevalence was also observed in AP patients (AP versus HP: 3.44% versus 2.78%; P < 0.05). The abnormal asthma-dependent T(h)17 elevation together with blunted Treg increase may play a role in the compromised immune tolerance characterizing asthmatic pregnancy.
International Immunology 09/2011; 23(11):669-77. · 3.41 Impact Factor
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Agnes Prókai,
Andrea Fekete,
Nóra Fanni Bánki, Veronika Müller,
Agota Vér,
Péter Degrell,
Krisztina Rusai,
László Wagner,
Adám Vannay,
Máté Rosta,
Uwe Heemann,
Róbert M Langer,
Tivadar Tulassay,
György Reusz,
Attila J Szabó
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ABSTRACT: Renal ischemia reperfusion injury induces gender-dependent heat-shock protein 72 expression, which maintains membrane localization of renal Na(+)/K(+)ATPase-α1. The erythropoietin has a protecting effect against ischemia reperfusion injury in various organs. In this study, we investigated whether erythropoietin exerts a beneficial effect against post-ischemic renal injury. Furthermore, we studied the erythropoietin signaling on heat-shock protein 72 and Na(+)/K(+)ATPase-α1 expression and localization.
In male and female Wistar rats, rHuEPO (1000 IU/bwkg intraperitoneal) or vehicle was administered 24 hours prior to unilateral left renal ischemia reperfusion (50 minutes). Kidneys were subsequently removed at hours 2 or 24 of the reperfusion; sham-operated rats served as controls (C) (n = 8/group). We measured serum erythropoietin, renal function, evaluated histological injury, and observed heat-shock protein 72 as well as Na(+)/K(+)ATPase-α1 protein level and localization. Additional groups were followed for 7-day survival.
Erythropoietin treatment was associated with better post-ischemic survival and less impaired renal function in males while diminishing the renal structural damage in both sexes. Endogenous erythropoietin was higher in males and increased in both genders after erythropoietin treatment. The erythropoietin treatment elevated protein levels of heat-shock protein 72 and Na(+)/K(+)ATPase-α1 in 24 hours in males, whereas in females, the already higher expression of heat-shock protein 72 and Na(+)/K(+)ATPase-α1 was not increased. Moreover, erythropoietin prevented ischemia reperfusion induced Na(+)/K(+)ATPase-α1 translocation from the basolaterale membrane in males.
Erythropoietin diminishes gender difference in the susceptibility to renal post-ischemic injury and reduces post-ischemic structural damage while preserving kidney function, particularly in males. This additional protection may be associated with a heat-shock protein 72-mediated effect on Na(+)/K(+)ATPase-α1 expression and translocation.
Surgery 07/2011; 150(1):39-47. · 3.10 Impact Factor
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ABSTRACT: Lung cancer carries a relatively high risk of chemotherapy-induced anemia, one of the most frequent hematological complications. Previous data show a lack of optimal anemia correction in patients with chemotherapy-induced anemia. This paper analyzes real-life data considering the prevalence and severity of chemotherapy-induced anemia, together with the frequency and efficacy of erythropoietin treatment of anemia in Hungarian lung cancer patients.
Data of 482 patients with histological or cytological confirmed lung cancer receiving chemotherapy were collected retrospectively between 1 January and 31 December, 2008. In all, 83 (17%) of them developed chemotherapy-induced moderate to severe anemia (44.6% male, 55.4% female; mean age 70 ± 8.6 years; NSCLC 67.5%, small cell lung cancer 32.5%).
More than 50% of the patients suffering from moderate to severe chemotherapy-induced anemia (hemoglobin below 10 g/dl) did not receive erythropoietin treatment. Chemotherapy had to be postponed due to anemia in 32.26% of the patients receiving erythropoietin supplementation, while this was seen in 41.94% of the group without erythropoietin treatment (p < 0.05). In patients not receiving erythropoietin, the severity of anemia increased, while erythropoietin treated patients maintained acceptable hemoglobin levels after the end of the chemotherapy.
The data draws attention to the fact that nowadays chemotherapy-induced anemia is not treated according to current guidelines in many lung cancer cases in Hungary.
Expert Opinion on Drug Safety 07/2011; 10(4):503-7. · 3.02 Impact Factor
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ABSTRACT: Pulmonary neuroendocrine tumors comprise 20% of all lung cancers. They are separated into 4 subgroups: typical carcinoid tumor, atypical carcinoid tumor, large-cell neuroendocrine carcinoma, and small-cell lung carcinoma. The major symptoms present in 60% of patients are cough, hemoptysis, and obstructive pneumonia. They may also exhibit hormonally related symptoms e.g. carcinoid syndrome. Small cell lung cancer is the most common subgroup, with rapid progression, aggressive metastatic potential and the worst prognosis. Large cell neuroendocrine carcinoma is rare but also has a poor prognosis. Typical carcinoid may be accompanied with hormone related symptoms and has the best prognosis; atypical one on the contrary may cause lymph node and distant metastases in half of the cases. Elevated plasma levels of chromogranin-A are present in majority of pulmonary neuroendocrine tumors and act as tumor marker. The mainstay of treatment is radical surgery if possible. In locally advanced or metastatic disease combination chemotherapy and somatostatin-analogues may have beneficial effect. This review focuses on the general features, and current diagnostic options of pulmonary neuroendocrine tumors.
Orvosi Hetilap 03/2011; 152(10):366-70.
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ABSTRACT: Asthma is one of the most common diseases complicating pregnancy and a risk factor for several maternal and fetal complications, posing a special challenge for physicians treating asthmatic pregnant women. Asthma influences the outcome of pregnancy and - vice versa - pregnancy affects asthma severity with bidirectional immunological interactions that are currently being examined. Supporting pregnancy-induced immunotolerance is the observation that attenuation of allergic responses can be detected in controlled asthmatic pregnant patients. However, uncontrolled asthmatic pregnant women show significant asthma-associated immune reactions, such as diminished pregnancy specific regulatory T cell proliferation, that may - besides other factors - influence fetal growth. Uncontrolled, symptomatic asthma may increase the risk of adverse perinatal outcomes; thus adequate regular anti-asthmatic treatment resulting in optimal asthma control represents a vital need during pregnancy. This review summarizes immunological changes characterizing pregnancy in asthmatic women together with the clinical implications of asthma management during pregnancy.
Respiratory medicine 02/2011; 105(2):159-64. · 2.33 Impact Factor
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ABSTRACT: Although patients have more problems using metered dose inhalers, clinical comparisons suggest they provide similar control to dry powder inhalers. Using real-life situations this study was designed to evaluate asthma control in outpatients with moderate to severe persistent asthma and to compare efficacy of fixed combinations of inhaled corticosteroids (ICS) and long acting beta-agonists (LABA).
This real-life study had a cross-sectional design. Patients using fixed combinations of ICS and LABA had their asthma control and spirometry assessed during regular visits.
111 patients were analyzed: 53 (47.7%) received maintenance therapy of extrafine beclomethasone-formoterol (BDP/F) pressurized metered dose inhaler (pMDI), 25 (22.5%) fluticasone-salmeterol (FP/S) dry powder inhaler (DPI), and 33 (29.7%) budesonide-formoterol (BUD/F) DPI. Severity of asthma at time of diagnosis, assessed by the treating physician, was comparable among groups. Asthma control was achieved by 45.9% of patients; 38.7% were partially controlled and 15.3% were uncontrolled. In the extrafine BDF/F group, asthma control total score, daytime symptom score and rescue medication use score were significantly better than those using fixed DPI combinations (5.8±6.2 vs. 8.5±6.8; 1.4±1.8 vs. 2.3±2.1; 1.8±2.2 vs. 2.6±2.2; p=0.0160; p=0.012 and p=0.025, respectively) and the mean daily ICS dose were significantly lower.
pMDI extrafine BDP/F combination demonstrated better asthma control compared to DPIs formulated with larger particles. This could be due to the improved lung deposition of the dose or less reliance on the optimal inhalation technique or both.
BMC Pulmonary Medicine 01/2011; 11:40. · 1.33 Impact Factor
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Krisztina Rusai,
Agnes Prókai,
Beáta Szebeni,
Krisztina Mészáros,
Andrea Fekete,
Balázs Szalay,
Ádám Vannay,
Peter Degrell, Veronika Müller,
Tivadar Tulassay,
Attila J Szabó
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ABSTRACT: Several studies reported sexual dimorphism in the signaling mechanisms of renal ischemia/reperfusion (I/R). The anti-apoptotic serum and glucocorticoid-regulated kinase-1 (SGK-1) is up-regulated and has a significant protective role in renal I/R. SGK-1 has several target molecules, and inhibition of the inducible nitric oxide synthase (iNOS) transcription is one of its effector mechanisms. The objective of the present study was to examine if there is a gender-specific expression and activation of SGK-1 during renal I/R injury. In vitro, treatment of HK-2 kidney proximal tubular cells with different concentrations of 17-beta estradiol had no effect, whereas testosterone increased SGK-1 abundance in a dose-dependent manner. In vivo, in a rat model of unilateral renal I/R injury, there was a higher SGK-1 expression and phosphorylation in males 2 and 24 h after ischemia paralleled by reduction in the mRNA expression of iNOS compared to females. Deprivation of testosterone by castration of males resulted in decreased SGK-1 protein level at all time-points and reduced phosphorylation 2 and 24 h after reperfusion. Our results suggest that testosterone up-regulates SGK-1 in the kidney contributing to sexual dimorphisms in the cell signalling machinery. The significance of the testosterone-regulated SGK-1 level and activity in the kidney needs further investigations.
Cellular Physiology and Biochemistry 01/2011; 27(6):727-38. · 2.86 Impact Factor
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ABSTRACT: Asthma bronchiale and chronic obstructive pulmonary disease are the most prevalent lung diseases characterized by inflammation of the airways. International and Hungarian guidelines provide proper definitions for clinical symptoms, diagnostics and therapy of both diseases. However, in everyday clinical practice, overlap of asthma and chronic obstructive pulmonary disease has become more frequent. As guidelines are mainly based on large, multicenter, randomized, controlled trials that exclude overlap patients, there is a lack of diagnostic and especially therapeutic strategies for these patients. This review summarizes clinical characteristics of asthma and chronic obstructive pulmonary disease overlap, and provides daily practical examples for its management.
Orvosi Hetilap 01/2011; 152(3):114-8.
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ABSTRACT: Asthma influences pregnancy outcome and pregnancy affects asthma severity, but the immunologic mechanisms of these interactions are not fully elucidated.
The prevalence of lymphocyte subsets was identified by cell surface markers and intracellular FoxP3 staining, in healthy non-pregnant (HNP; N = 15), healthy pregnant (HP; N=33), asthmatic non-pregnant (ANP; N=62) and asthmatic pregnant (AP; N=61) women.
Regulatory T cell (Treg) prevalence was higher in HP than in HNP subjects and showed a positive correlation with fetal birth weight, which was blunted in AP group. Treg prevalence was lower and invariable natural killer T cell prevalence was higher in AP patients (compared to HP). Higher naive and lower effector T cell prevalence was observed in AP than in ANP group.
Pregnancy-induced increase in Treg cell prevalence is absent in asthmatic pregnancy that may interfere with physiological intrauterine growth. However, pregnancy-specific inhibition of asthmatic inflammation can be detected in uncomplicated asthmatic pregnancy.
American Journal Of Reproductive Immunology 12/2010; 64(6):393-401. · 2.17 Impact Factor
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Krisztina Gál,
Aron Cseh,
Balázs Szalay,
Krisztina Rusai,
Adám Vannay,
József Lukácsovits,
Uwe Heemann,
Attila J Szabó,
György Losonczy,
Lilla Tamási, Veronika Müller
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ABSTRACT: Smoking is the leading risk factor of chronic obstructive pulmonary disease (COPD) and lung cancer. Corticosteroids are abundantly used in these patients; however, the interaction of smoking and steroid treatment is not fully understood. Heat shock proteins (Hsps) play a central role in the maintenance of cell integrity, apoptosis and cellular steroid action. To better understand cigarette smoke-steroid interaction, we examined the effect of cigarette smoke extract (CSE) and/or dexamethasone (DEX) on changes of intracellular heat shock protein-72 (Hsp72) in lung cells. Alveolar epithelial cells (A549) were exposed to increasing doses (0; 0.1; 1; and 10 μM/μl) of DEX in the medium in the absence(C) and presence of CSE. Apoptosis, necrosis, Hsp72 messenger-ribonucleic acid (mRNA) and protein expression of cells were measured, and the role of Hsp72 on steroid effect examined. CSE reduced the number of viable cells by significantly increasing the number of apoptotic and necrotic cells. DEX dose-dependently decreased the ratio of apoptosis when CSE was administered, without change in necrosis. CSE - DEX co-treatment dose-dependently increased Hsp72 mRNA and protein expression, with the highest level measured in CSE + DEX (10) cells, while significantly lower levels were noted in all respective C groups. Pretreatment with Hsp72 silencing RNA confirmed that increased survival observed following DEX administration in CSE-treated cells was mainly mediated via the Hsp72 system. CSE significantly decreases cell survival by inducing apoptosis and necrosis. DEX significantly increases Hsp72 mRNA and protein expression only in the presence of CSE resulting in increased cellular protection and survival. DEX exerts its cell protective effects by decreasing apoptotic cell death via the Hsp72 system in CSE-treated alveolar epithelial cells.
Cell Stress and Chaperones 12/2010; 16(4):369-78. · 3.01 Impact Factor
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ABSTRACT: High-dose (75 mg/m2) cisplatin is baseline chemotherapy in lung cancer. To prevent nephrotoxicity, patients generally receive saline infusion on the day of chemotherapy prior to and following cisplatin (total of 3.5-4.0 liters during 3-4 hours). Despite these measures nephrotoxicity has remained frequent, especially among patients also suffering from cardiovascular disease or diabetes mellitus. Since 2005 several international recommendations have been formed about prevention of cisplatin nephrotoxicity. According to these recommendations: 1) renal function should not be evaluated by serum creatinine concentration; 2) evaluation of renal function should be based on calculated creatinine clearance (e.g. by the Cockcroft-Gault equation); 3) patients to be treated by high-dose cisplatin should be euvolemic and should have saline diuresis (urine NaCl concentration ~1%) of at least 100 ml/hour prior to, during and several days following the administration of cisplatin. Keeping these recommendations ensures prolonged cisplatin treatability of lung cancer patients. Moreover, decreased renal function will not limit the full dose administration of several other cytotoxic agents. Losonczy G, Máthé C, Müller V, Szondy K, Moldvay J. Incidence, risk factors and prevention of cisplatin-induced nephrotoxicity in patients with lung cancer.
Magyar Onkológia 12/2010; 54(4):289-96.
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Tamás Constantin,
Annamária Székely,
Andrea Ponyi,
Vera Gulácsy,
Csaba Ambrus,
Krisztina Kádár,
Ildikó Vastagh,
Angéla Dajnoki,
Beáta Tóth,
Gergely Bokrétás, [......],
Viktor Nagy,
Pál Magyar,
Mohamed Mahdi,
Eva Rákóczi,
Krisztina Németh,
Dániel Bereczki,
Miklós Garami,
Melinda Erdos,
László Maródi,
György Fekete
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ABSTRACT: Fabry disease is a rare, X-linked lysosomal storage disorder that leads to accumulation of globotriaosylceramide in different tissues of the body. The disease is progressive and the first symptoms usually present in childhood. Consequences of the disease are disability and premature death. The disease in females could be as severe as in males although women may be asymptomatic. The possibility of enzyme replacement therapy has made it necessary to elaborate a comprehensive guideline for the diagnosis and treatment follow-up. The guideline has been summarized by a Hungarian multi-disciplinary working group consisting of physicians who are involved in diagnosis and care of Fabry patients. Previous clinical studies, published articles, and recently established international treatment guidelines were reviewed by the group.
Orvosi Hetilap 08/2010; 151(31):1243-51.
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Tamás Constantin,
Eva Rákóczi,
Andrea Ponyi,
Csaba Ambrus,
Krisztina Kádár,
Ildikó Vastagh,
Angéla Dajnoki,
Beáta Tóth,
Gergely Bokrétás, Veronika Müller, [......],
Sarolta Kárpáti,
Viktor Nagy,
Pál Magyar,
Mohamed Mahdi,
Krisztina Németh,
Dániel Bereczki,
Miklós Garami,
Melinda Erdos,
László Maródi,
György Fekete
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ABSTRACT: Fabry disease is a rare, X-linked lysosomal storage disorder that leads to accumulation of globotriaosylceramide in different tissues of the body. The disease is progressive, first symptoms usually present in childhood. Consequencies of the diseases are disability and premature death. The disease in females could be as severe as in males although women may also be asymptomatic. The possibility of enzyme replacement therapy has made it necessary to elaborate a comprehensive guideline for the diagnosis and treatment follow-up. The guideline was established by a Hungarian multi-disciplinary working group, consisting of physicians who are involved in health care of Fabry patients. Previous clinical studies, published materials, and recently established international treatment guidelines were reviewed by the group.
Orvosi Hetilap 02/2010; 151(7):243-9.
