Andrea Szabó

Similarities and differences between pediatric and adult patients with systemic lupus erythematosus.

Systemic lupus erythematosus (SLE) is a multifactorial autoimmune disease with highest prevalence among women of childbearing age. However, children younger than 16 years also can develop SLE (childhood-onset lupus/juvenile-type SLE). The aim of our study was to compare the clinical course of adult and pediatric-onset SLE. Data from 342 adult patients followed at the University of Debrecen, Hungary, and 79 children documented in the Hungarian National Pediatric SLE registry were analyzed using hospital medical records. Organ manifestations, laboratory parameters, and immunoserological characteristics were reviewed and the results were evaluated using SPSS for Windows software. Gender distribution was not significantly different between groups with disease starting in childhood vs adulthood. The prevalence of the following manifestations was significantly higher for pediatric than for adult-onset disease including: lupus nephritis (43% pediatric vs 26.4% for adult-onset), hematological disorders (57% vs 36.4%), photosensitivity (20% vs 9%), butterfly rash (61% vs 35.5%) and mucosal ulceration (11.4% vs 4%). For adult-onset SLE, neurological symptoms (30% vs 6%) and polyarthritis (86% vs 68%) occurred significantly more frequently than in children. Anti-SSA, anti-SSB and antiphospholipid antibodies were detected at significantly higher levels in adult-onset patients compared to those in pediatrics. Children were more commonly given high-dose intravenous immunoglobulin treatment (6.3% vs 0.6%) and mycophenolate mofetil (15.2% vs 5.3%) than adults. These results suggest that pediatric and adult-onset SLE differ in multiple aspects, and it is important to recognize these differences for optimal treatment and prognosis of these patients.

Efficacy and safety of infliximab induction therapy in Crohn's Disease in Central Europe - A Hungarian nationwide observational study

BackgroundInfliximab (IFX) has proven to be an effective addition to the therapeutic arsenal for refractory, fistulizing, and steroid dependent Crohns disease (CD), with efficacy in the induction and maintenance of clinical remission of CD. Our objective in this study is to report the nationwide, multicenter experience with IFX induction therapy for CD in Hungary.MethodsDuring a 6-year-period, beginning in 2000, a total of 363 CD patients were treated with IFX as induction therapy (5 mg/kg IFX infusions given at week 0, 2 and 6) at eleven centers in Hungary in this observational study. Data analysis included patient demographics, important disease parameters and the outcome of IFX induction therapy.ResultsThree hundred and sixty three patients (183 women and 180 men) were treated with IFX since 2000. Mean age was 33.5 ± 11.2 years and the mean duration of disease was 6.7 ± 6.1 years. The population included 114 patients (31.4%) with therapy-refractory CD, 195 patients (53.7%) with fistulas, 16 patients (4.4%) with both therapy-refractory CD and fistulas, and 26 patients (7.2%) with steroid dependent CD. Overall response rate was 86.2% (313/363). A higher response rate was observed in patients with shorter disease duration (p = 0.05, OR:0.54, 95%CI:0.29-0.99) and concomitant immunosuppressant therapy (p = 0.05, OR: 2.03, 95%CI:0.165-0.596). Concomitant steroid treatment did not enhance the efficacy of IFX induction therapy. Adverse events included 34 allergic reactions (9.4%), 17 delayed type hypersensitivity (4.7%), 16 infections (4.4%), and 3 malignancies (0.8%).ConclusionIFX was safe and effective treatment in this cohort of Hungarian CD patients. Based on our experience co-administration of immunosuppressant therapy is suggested in patients receiving IFX induction therapy. However, concomitant steroid treatment did not enhanced the efficacy of IFX induction therapy.

Efficacy and Safety of Adalimumab in Ulcerative Colitis Refractory to Conventional Therapy in Routine Clinical Practice.

BACKGROUND AND AIM Adalimumab [ADA] was approved for the treatment of ulcerative colitis [UC] refractory to conventional therapy in 2012 in Europe. Due to the observed discrepancies between clinical trials and practice, data on the outcome of ADA therapy are really needed from the real life. The aim of this study was to estimate the short- and long-term efficacy and safety of ADA in UC patients from each Hungarian biological centre. PATIENTS AND METHODS This prospective study consisted of UC patients treated with ADA in 10 Hungarian inflammatory bowel disease centres. The primary endpoints of the study were rates of continuous clinical response, remission, non-response and loss of response at Weeks 12, 30, and 52.The secondary endpoints included mucosal healing at Week 52 and the comparison of the efficacy of ADA between biological naive and infliximab [IFX]-treated groups. Colonoscopy was performed before starting the therapy and at Week 52. RESULTS In all, 73 active UC patients were enrolled in the study: 67.1% of the patients received previous IFX therapy; 75.3% of the patients showed short-term clinical response at Week 12. The probability of maintaining ADA was 48.6% at Week 52 with a continuous clinical response in 92% of these remaining patients. Mucosal healing was achieved in 48.1% of the patients at Week 52. Escalation of ADA was performed in 17.6%, and minor side effects developed in 4% of the patients; 5.4% of the patients underwent colectomy during the 1-year treatment period. CONCLUSION UC is a progressive disease that may need early aggressive therapy to prevent structural and functional complications. The results of our study demonstrated the favourable efficacy of short- and long-term ADA treatment for patients with UC.

A Kupffer-sejt gátlásának hatása az endotoxin indukálta gyulladásos válaszreakciókra és a máj mikrokeringési változásaira kísérletes obstructiós icterusbanr

INTRODUCTION/AIMS In the clinical practice, biliary obstruction often leads to septic complications causing systemic and hepatic inflammatory reactions, which increases mortality and morbidity. Hepatic Kupffer cells (KC) play a pivotal role in this process. Herein we examined the consequences of bile duct ligation during endotoxaemia and the effects of KCs. MATERIAL AND METHODS In the first part of our experiment, the survival rate of male Wistar rats in 48-hr endotoxaemia with or without bile duct ligation was assessed. Time-dependent changes in pro-inflammatory TNF-alpha and IL-6 levels were also monitored. In the second series, hepatic capillary perfusion, neutrophil-endothelial interactions and KC activity were assessed using fluorescence intravital videomicroscopy. KC blockade was induced by gadolinium chloride (GdCl3) pretreatment. RESULTS Serum TNF-alpha and IL-6 levels were significantly increased in early endotoxaemia. Survival rate was deteriorated, while TNF-alpha and IL-6 releases, KC activity and leukocyte activation were increased if obstructive jaundice was also induced. KC blockade improved survival and reduced TNF-alpha and IL-6 productions without ameliorating perfusion failure. CONCLUSIONS In the presence of biliary obstruction, inflammatory and microcirculatory consequences of endotoxaemia are enhanced. The alleviating effect of KC blockade may underline the pathophysiological role of KCs in these conditions.

Screening of trisomy 21 nowadays. Is maternal age so important

INTRODUCTION Trisomy 21 is the most common chromosomal abnormality, therefore, screening and diagnosis of this disorder is in the centre of attention worldwide. An efficient screening method is the combined test based on maternal age, ultrasound signs, biochemical markers, and a risk ratio can be calculated based on these data. AIM The aim of the authors was to determine the causes of missed prenatal diagnosis of Downs syndrome at the 2nd Department of Obstetrics and Gynecology, Semmelweis University. METHOD A retrospective study was carried out by collecting data from medical records of mothers who had delivered a newborn with Downs syndrome in the Department between 2008 and 2012. Each medical record was analyzed individually. RESULTS In most cases the missed diagnosis of Downs syndrome occurred when the expectant mother failed to attend the first trimester screening or did not take the risk of invasive diagnostic procedures needed for fetal kariotyping. CONCLUSIONS Analysis of fetal DNA circulating in maternal plasma can be a solution for those who refuse invasive fetal diagnostics. This test has high sensitivity and very low false positive rate. It has become available since the end of 2011 in the United States and, since the autumn of 2012, in Hungary, too. The test, however, is not reimbursed by national health insurance.

A 21-es triszómia szűrése napjainkban. Az anyai életkor valóban olyan fontos? [Screening of trisomy 21 nowadays. Is maternal age so important?]

INTRODUCTION Trisomy 21 is the most common chromosomal abnormality, therefore, screening and diagnosis of this disorder is in the centre of attention worldwide. An efficient screening method is the combined test based on maternal age, ultrasound signs, biochemical markers, and a risk ratio can be calculated based on these data. AIM The aim of the authors was to determine the causes of missed prenatal diagnosis of Downs syndrome at the 2nd Department of Obstetrics and Gynecology, Semmelweis University. METHOD A retrospective study was carried out by collecting data from medical records of mothers who had delivered a newborn with Downs syndrome in the Department between 2008 and 2012. Each medical record was analyzed individually. RESULTS In most cases the missed diagnosis of Downs syndrome occurred when the expectant mother failed to attend the first trimester screening or did not take the risk of invasive diagnostic procedures needed for fetal kariotyping. CONCLUSIONS Analysis of fetal DNA circulating in maternal plasma can be a solution for those who refuse invasive fetal diagnostics. This test has high sensitivity and very low false positive rate. It has become available since the end of 2011 in the United States and, since the autumn of 2012, in Hungary, too. The test, however, is not reimbursed by national health insurance.

Eradication of Helicobacter pylori has no effect on gastric acidity in duodenal ulcer patients-evaluation of 24-h pH monitoring

It is accepted that eradication of Helicobacter pylori leads to healing of chronic active gastritis facilitates ulcer healing and prevents ulcer recurrence in duodenal ulcer (DU) patients. However, it is not entirely known whether the eradication of the bacteria normalizes gastric acid secretion and abolishes dyspeptic symptoms after ulcer healing. This study was aimed to evaluate the intragastric acidity and dyspeptic complaints before, and 3 months after, eradication in 18 endoscopically proven H. pylori positive DU patients. Gastric pH was measured by 24-h continuous intraluminal recording, serum gastrin measurements and Congo-red tests were also performed. Dyspeptic complaints and antacid consumptions were recorded in diary cards, antisecretory therapy was not allowed after the cessation of eradication therapy. Endoscopy, H. pylori status and Congo-red tests were controlled at the 6th and 12th week, while pH measurements and serum gastrin tests were performed at inclusion and 3 months later. Three patients dropped out and in 14 out of the remaining subjects healing of DUs and successful eradication was achieved by the 6th and 12th week controls. The 24-h median pH and the percentage of 24-h pH readings under pH 3 were not changing significantly by the 3-month controls (from 1.9+/-0.5 to 1.8+/-0.4 and from 52.6+/-5.5% to 58.6+/-5%, respectively). Similarly, no significant changes were observed in serum gastrin levels and dyspeptic symptom scores (from 72+/-7 pg/ml to 56.7+/-8 pg/ml and from 2.69+/-0.4 to 1.26+/-0.3, respectively). The antacid consumption was almost stable when compared with the pre- and post-eradication periods. It was concluded that despite successful H. pylori eradication and healing of DU, intragastric acidity does not change significantly at least 3 months after the therapy. The persisting dyspeptic symptoms and the need for antacid consumption suggest that some healed ulcer patients require antisecretory therapy in the post-eradication period.

Az adalimumab hatékonysága és biztonságossága hagyományos kezelésre refrakter colitis ulcerosában

Absztrakt Bevezetes: Europaban 2012 ota engedelyezett adalimumab alkalmazasa terapiarefrakter colitis ulcerosa kezelesere. Celkitűzes: A szerzők celja volt, hogy colitis ulcerosas betegek koreben orszagos szinten felmerjek az adalimumab hatasossagat es biztonsagossagat. Modszer: Prospektiv tanulmanyban felmertek a tartos klinikai valasz, a teljes remisszio, illetve elsődleges vagy masodlagos hatasvesztes aranyat a kezeles 12., 30. es 52. heteben. Ertekeltek az 52. heten a nyalkahartya-gyogyulas megoszlasat. Eredmenyek: A vizsgalatba 73 aktiv colitis ulcerosas beteget vontak be. Rovid tavu klinikai valasz a betegek 75,3%-aban alakult ki a 12. hetre. Az adalimumabkezeles fenntarthatosaganak valoszinűsege az 52. hetig 48,6% volt, amelyet tartos klinikai valasz kisert a betegek 92%-anal. Nyalkahartya-gyogyulas a betegek 48,1%-aban volt kimutathato az 52. heten. A betegek 17,6%-anal terapiamodositasra, 5,4%-anal colectomia elvegzesere kerult sor az egyeves időszak alatt. Kovetkeztetesek: A colitis ulcerosa a k...