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These are methionine adenosyltransferase I\/III, glycine N\u2010methyltransferase, S\u2010adenosylhomocysteine hydrolase and adenosine kinase deficiencies. This paper provides the first consensus recommendations for the diagnosis and management of methylation disorders. Following search of the literature and evaluation according to the SIGN\u2010methodology of all reported patients with methylation defects, graded recommendations are provided in a structured way comprising diagnosis (clinical presentation, biochemical abnormalities, differential diagnosis, newborn screening, prenatal diagnosis), therapy and follow\u2010up. Methylation disorders predominantly affect the liver, central nervous system and muscles, but clinical presentation can vary considerably between and within disorders. Although isolated hypermethioninemia is the biochemical hallmark of this group of disorders, it is not always present, especially in early infancy. Plasma S\u2010adenosylmethionine and S\u2010adenosylhomocysteine are key metabolites for the biochemical clarification of isolated hypermethioninemia. Mild hyperhomocysteinemia can be present in all methylation disorders. Methylation disorders do not qualify as primary targets of newborn screening. A low\u2010methionine diet can be beneficial in patients with methionine adenosyltransferase I\/III deficiency if plasma methionine concentrations exceed 800 \u03bcmol\/L. There is some evidence that this diet may also be beneficial in patients with S\u2010adenosylhomocysteine hydrolase and adenosine kinase deficiencies. S\u2010adenosylmethionine supplementation may be useful in patients with methionine adenosyltransferase I\/III deficiency. Recommendations given in this article are based on general principles and in practice should be adjusted individually according to patient's age, severity of the disease, clinical and laboratory findings.<\/jats:p>","DOI":"10.1007\/s10545-016-9972-7","type":"journal-article","created":{"date-parts":[[2016,9,26]],"date-time":"2016-09-26T08:47:19Z","timestamp":1474879639000},"page":"5-20","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":58,"title":["Consensus recommendations for the diagnosis, treatment and follow\u2010up of inherited methylation disorders"],"prefix":"10.1002","volume":"40","author":[{"given":"Ivo","family":"Bari\u0107","sequence":"first","affiliation":[{"name":"Department of Pediatrics University Hospital Center Zagreb Ki\u0161pati\u0107eva 12, Rebro Zagreb 10000 Croatia"},{"name":"University of Zagreb, School of Medicine Zagreb 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