Improper Supplementation Habits of Folic Acid Intake by Hungarian Pregnant Women: Improper Recommendations

Background: Neural tube defects (NTDs) are some of the most common congenital anomalies. Proper folic acid supplementation is a dominant risk factor, which has been shown to decrease the incidence of NTDs. In Canada, the incidence of neuroblastoma has presented a considerable decrease of 60% as a result of enrichment cereal grain flours with synthetic folic acid. The aim of this study was to investigate the effect of folic acid intake by pregnant women on the incidence of NTDs and neuroblastoma. Methods: Regular folic acid intake has been recommended to pregnant women in Hungary since the eighties of the last century by health visitors eventually raking effect as an official protocol which had been released in 1997. During 2001, 2002 and 2003. folic acid intake habits of pregnant women were evaluated by health visitors, proving to be successful in collecting data front 95.06% of the pregnant women. The incidence of NTDs has been registered by the Hungarian National Centre of Epidemiology, Department of Human Genetics and Teratology. The Pediatric Cancer Registry provided the incidence of neuroblastoma in children. Results: Consistent findings revealed a regular intake of supplementary folic acid products by 68.71% of the pregnant women. Out of these. 93.13% of pregnant women who were taking folic acid, started the supplementation after their 7 weeks of pregnancies, a time designated as the completion period of the development of the neural tube. The dose of folic acid supplementation was evaluated as less than 5 mg/day in 84.75% of the pregnant women. In Hungary, the incidence of NTDs has remained constant, while the incidence of neuroblastoma has shown constant slight increase in spite of the introduction of folic acid supplementation in 1997. Conclusions: Based on our experience, folic acid supplementation was initiated after the recognition of pregnancy and its application in a dose of lower than 5 mg/day neither decreased the incidence of NTDs nor did it have an effect on the neuroblastoma incidence. It is implicated that proper folic acid supplementation, which is started front the conception. can be achieved only with the enrichment of cereal grain flours

Recent Advances in Systemic Scleroderma in Childhood

Ann Paediatr Rheumatol Annals of paediatric rheumatolog http://www.aprjournal.org/ 2146-2909 (Print

Disease course, frequency of relapses and survival of 73 patients with juvenile or adult dermatomyositis

Objective Our aim is to present the disease course, frequency of relapses and survival of juvenile and adult dermatomyositis (JDM/DM) patients. Methods Analysis was performed using data on 73 patients. The median follow-up for 38 JDM patients was 32 months and 78 months for 35 adult DM patients. Results 23/38 JDM patients (60%) had monophasic, 12/38 (31.6%) had polycyclic and 3138 (7.9%) had chronic disease. Among children treated only with glucocorticoids, 12/20 (60%) had monophasic and 8/20 (40%) had polycyclic disease. 10/17 (58.8%) children, who required second-line immunosuppressive agents, had monophasic and 4/17 (23.5%) had polycyclic disease. 18/35 DM (51.4%) patients had monophasic, 13/35 (37.1%) had polycyclic, 1/35 (2.9%) had chronic disease and 3135 (8.6%) had fulminant myositis. Among DM patients requiring only glucocorticoids, 12/20 (60%) were monophasic and 8/20 (40%) were polycyclic. In patients requiring second-line immunosuppressive agents, 6/15 patients (40%) had monophasic and 5/15 (33.3%) had polycyclic disease. Among patients with polycyclic disease, the risk of relapse was higher during first year than later in the disease course. None of the JDM patients have died, while 4 disease-specific deaths occurred in adult patients. There was no significant difference between the survival of JDM and DM patients. Discussion There was no correlation between relapse-free survival and the initial therapeutic regimen. Many of our patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed for at least 2 years. Although we found a favourable survival rate, further investigations are needed to assess functional outcome

A polymyositis/dermatomyositisben alkalmazott terápia hatásának tanulmányozása a patomechanizmusban szerepet játszó kóros immunválaszok egyes elemeire. Sejtes és humorális elemek, valamint az NF- B szignalizációs útvonal vizsgálata = Studying the effect of the therapy of polymyositis/dermatomyositis on the pathological immunological responses

Az idiopathiás inflammatorikus myopathiák (IIM) szisztémás autoimmun betegségek. Bohan és Peter kritériumok alapján diagnosztizált, 145 IIM-es betegben vizsgáltuk bizonyos MHC-II allélek jelenlétét. Elemeztük ezen allélek összefüggését a különböző myositis alcsoportokkal, a klinikai tünetekkel, az előforduló antitestekkel, a betegség lefolyásával és az alkalmazott terápiával. A HLA-DRB1, DQA1, DQB1 antigének különböző alléljeit szekvenciaspecifikus primerek segítségével polimeráz láncreakcióval határoztuk meg.Mindegyik myositis alcsoportban gyakoribb volt a DRB1*03 és a kapcsolt DQA1*05 valamint a DQB1*02 allélek jelenléte. Ezen allélek előfordulása magasabb volt a glükokortikoidon kívül egyéb immunszuppresszióra szoruló betegek körében. Az egyes autoantitestekkel való asszociációt tekintve Scl-70 pozitív mindkét beteg DRB1*03, DQA1*05, DQB1*02 genotípusú volt.A HLA DQA1 genotípusnál látható a különbség: a kontrollcsoport 11%-a *01 és/vagy *05 genotípusú, a betegcsoportban ugyanezek az allélok 60%-os gyakoriságot mutattak. A HLA DQB1*05 genotípust emelhető ki rizikó szempontjából, elsősorban ebben az esetben találtunk kiemelkedő asszociációt a betegcsoportban. Gyakori volt a DQB1*02 (40%) és a *03 allél (60%). . A JDM-es alcsoportban magas előfordulási arányt mutatott a DRB1*03, DQA1*01 és *05, a DQB1*02 és *05 allélok jelenléte. Szívizomérintettség esetén a betegek nem hordozták a DRB1*03 allélt, mint már korábban igazolt myositis rizikófaktorszerepű gént. | Idiopathic inflammatory myopathies (IIM) are a group of systemic autoimme diseases, which characteristically effect striated muscle tissues, resulting in immunemediated inflammation that leads to progressive muscle degeneration.In our current examinations were conducted using the Bohan and Peter critera in 145 patients, in whom we examined the presence of various MHC-II alles. Based on these alleles, we summarized the correlations of the various myositis subgroups, clinical symptoms, present antibodies, with the disease cource and applied therapies. With the help of the polymerase chain reaction, we determined the sequence specific primes of the HLA-DRBI, DQA1, and DQB1 antigens.In all myositis subgroups, the presence of DRB1*03 and associated DQA1*05, and DQB1*02 alleles was common.The prognosis may be worse in those cases where patients reacted well only to second and third line therapies. With respect to the disease course, in the more severe and/or complicated cases, those genes which are associated with higher riskfactors (DRB1*03, DQA1*01 and *05, DQB1*02) where more frequent than in cases of monophasic disease course cases. We found a much higher incidence of the DQA1*05 allele compared to the control group: 66% were polyphasic, 67% in the chronic course. The associations of various haplotypes with certain phenotype characteristics may have diagnostic value