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dc.contributor.authorDereksson, Kristjan
dc.contributor.authorKjartansson, Sveinn
dc.contributor.authorHjartardóttir, Hulda
dc.contributor.authorArngrimsson, Reynir
dc.date.accessioned2013-08-13T13:40:42Z
dc.date.available2013-08-13T13:40:42Z
dc.date.issued2012
dc.date.submitted2013-08-13
dc.identifier.citationBMJ Case Rep 2012, 2012:en_GB
dc.identifier.issn1757-790X
dc.identifier.pmid22927265
dc.identifier.doi10.1136/bcr.02.2012.5823
dc.identifier.urihttp://hdl.handle.net/2336/298076
dc.description.abstractIchthyosis prematurity syndrome (IPS) is a rare inherited skin disorder. Children are born prematurely with thick skin and have been found to develop neonatal asphyxia due to occlusions in the bronchial tree from debris in the amniotic fluid. At 31 weeks of gestation, separation of amniotic and chorionic membranes was identified as well as polyhydramnion. The child was born 2 weeks later, with thickened skin with a granular appearance and required immediate ventilation and intensive care. At 2 years of age, the patient has developed an atopic skin condition with severe itching, recurrent skin infections, food intolerance and periods of wheezing. Prenatal observation of separation of foetal membranes or dense amniotic fluid may be signs of IPS and severe complication immediately after birth.
dc.language.isoenen
dc.relation.urlhttp://dx.doi.org/10.1136/bcr.02.2012.5823en_GB
dc.relation.urlhttp://casereports.bmj.com/content/2012/bcr.02.2012.5823.abstracten_GB
dc.rightsArchived with thanks to BMJ case reportsen_GB
dc.subject.meshAdulten_GB
dc.subject.meshAmnionen_GB
dc.subject.meshAsphyxia Neonatorumen_GB
dc.subject.meshBreech Presentationen_GB
dc.subject.meshCesarean Sectionen_GB
dc.subject.meshChild, Preschoolen_GB
dc.subject.meshChorionen_GB
dc.subject.meshCodon, Nonsenseen_GB
dc.subject.meshDiagnosis, Differentialen_GB
dc.subject.meshExonsen_GB
dc.subject.meshFatty Acid Transport Proteinsen_GB
dc.subject.meshFemaleen_GB
dc.subject.meshFetal Membranes, Premature Ruptureen_GB
dc.subject.meshFollow-Up Studiesen_GB
dc.subject.meshHomozygoteen_GB
dc.subject.meshHumansen_GB
dc.subject.meshIchthyosisen_GB
dc.subject.meshImage Interpretation, Computer-Assisteden_GB
dc.subject.meshImaging, Three-Dimensionalen_GB
dc.subject.meshInfanten_GB
dc.subject.meshInfant, Newbornen_GB
dc.subject.meshInfant, Premature, Diseasesen_GB
dc.subject.meshPolyhydramniosen_GB
dc.subject.meshPregnancyen_GB
dc.subject.meshSwedenen_GB
dc.subject.meshUltrasonography, Prenatalen_GB
dc.titleIchthyosis prematurity syndrome with separation of fetal membranes and neonatal asphyxia.en
dc.typeArticleen
dc.contributor.departmentPediatrics Department, Skåne University Hospital, Malmö, Sweden.en_GB
dc.identifier.journalBMJ case reportsen_GB
dc.rights.accessClosed - Lokaðen
html.description.abstractIchthyosis prematurity syndrome (IPS) is a rare inherited skin disorder. Children are born prematurely with thick skin and have been found to develop neonatal asphyxia due to occlusions in the bronchial tree from debris in the amniotic fluid. At 31 weeks of gestation, separation of amniotic and chorionic membranes was identified as well as polyhydramnion. The child was born 2 weeks later, with thickened skin with a granular appearance and required immediate ventilation and intensive care. At 2 years of age, the patient has developed an atopic skin condition with severe itching, recurrent skin infections, food intolerance and periods of wheezing. Prenatal observation of separation of foetal membranes or dense amniotic fluid may be signs of IPS and severe complication immediately after birth.


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