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It is characterised by deficiency of MPI caused by pathogenic variants in\n                    <jats:italic>MPI<\/jats:italic>\n                    gene. The manifestation of MPI\u2010CDG is different from other CDGs as the patients suffer dominantly from gastrointestinal and hepatic involvement whereas they usually do not present intellectual disability or neurological impairment. It is also one of the few treatable subtypes of CDGs with proven effect of oral mannose. This article covers a complex review of the literature and recommendations for the management of MPI\u2010CDG with an emphasis on the clinical aspect of the disease. A team of international experts elaborated summaries and recommendations for diagnostics, differential diagnosis, management, and treatment of each system\/organ involvement based on evidence\u2010based data and experts' opinions. Those guidelines also reveal more questions about MPI\u2010CDG which need to be further studied.\n                  <\/jats:p>","DOI":"10.1002\/jimd.12241","type":"journal-article","created":{"date-parts":[[2020,4,8]],"date-time":"2020-04-08T05:32:29Z","timestamp":1586323949000},"page":"671-693","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":62,"title":["Consensus guideline for the diagnosis and management of mannose phosphate isomerase\u2010congenital disorder of glycosylation"],"prefix":"10.1002","volume":"43","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5802-6006","authenticated-orcid":false,"given":"Anna","family":"\u010cechov\u00e1","sequence":"first","affiliation":[{"name":"Department of Pediatrics and Adolescent Medicine, First Faculty of Medicine Charles University and General University Hospital in Prague  Prague Czech 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