Consensus-based recommendations for the management of juvenile localised scleroderma.

In 2012, a European initiative called Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile localised scleroderma (JLS) is a rare disease within the group of paediatric rheumatic diseases (PRD) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. This study aims to provide recommendations for assessment and treatment of JLS. Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was formed, mainly from Europe, and consisted of 15 experienced paediatric rheumatologists and two young fellows. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using a nominal group technique. Recommendations were accepted if ≥80% agreement was reached. In total, 1 overarching principle, 10 recommendations on assessment and 6 recommendations on therapy were accepted with ≥80% agreement among experts. Topics covered include assessment of skin and extracutaneous involvement and suggested treatment pathways. The SHARE initiative aims to identify best practices for treatment of patients suffering from PRDs. Within this remit, recommendations for the assessment and treatment of JLS have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JLS throughout Europe.

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 from 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 from the conception, can be achieved only with the enrichment of cereal grain flours.

[Folic acid supplementation for pregnant women in Hungary]. / A magyarországi várandósok folsavszedési szokásai.

INTRODUCTION: Neural tube defects are common major congenital anomalies. Folic acid supplementation has been shown to reduce the incidence of neural tube defects. In 2003, incidence of neuroblastoma has decreased with 60% in Canada as a result of enriched cereal grain flours with synthetic folic acid. The aim of this study was to investigate the effect of the practice of the folic acid intake by pregnant women (based on the Hungarian recommendation) to the incidence of neural tube defects and neuroblastoma. METHODS: The practice of folic acid supplementation was examined by questionnaires filled according to the documentation of health visitors. The data were worked up by computer. The incidence of neural tube defect was obtained from the data of the Hungarian Congenital Anomalies Registry, however, the data of National Health Insurance Company are also given. The incidence of neuroblastoma was the data of the Hungarian Pediatric Tumor Registry. Regular folic acid intake has been recommended to pregnant women in Hungary, since the eighties of the last century. An official protocol had been released by the Obstetric and Gynecologic Professional Board in 1997. In this paper, the authors report the Hungarian pregnant women's folic acid intake in years of 2001, 2002 and 2003. These years were chosen, because according to the data of the Hungarian Pediatric Cancer Registry 45% of the neuroblastoma cases are less than 1 year old, and 45% of them are 1-5 years old at the time of diagnosis. The authors succeeded to collect the data from 95% of the pregnant women during these years (271,748 women). RESULTS: Based on the statistical analysis of the collected data, 69% of the pregnant women were regularly taking folic acid products in Hungary, however, the dose of the daily intake was below 5 mg. 93% of the pregnant women started the folic acid intake after their 7th weeks of pregnancy The incidence of neural tube defects was constant, and the incidence of neuroblastoma slightly increased during the above mentioned period. CONCLUSIONS: This work highlighted that, the folic acid intake to prevent neural tube defects was started too late, because the formation of neural tube is finished on 28. day of pregnancy. 85% of the pregnant women used less amount of folic acid than 5 mg/day. The increasing number of planned pregnancies would allow to start folic acid intake earlier. However, based on international experience, the enrichment of cereal grain flours with synthetic folic acid could provide optimal results.