title: Life-threatening complications of transient abnormal myelopoiesis in neonates with Down syndrome.
abstract: Neonates with Down syndrome can present with a haematological disorder called transient abnormal myelopoiesis (TAM). While TAM is usually a self-limiting disease, patients with severe complications such as hydrops fetalis, cardiorespiratory failure and liver fibrosis have been described. Here, we present five consecutive neonates with trisomy 21 and TAM, four of whom were critically ill and were therefore treated with cytosine-arabinoside. All five patients survived.
  
  
  CONCLUSIONS
  severely affected neonates with Down syndrome and transient abnormal myelopoiesis might benefit from early cytostatic treatment with cytosine-arabinoside.
authors.affiliation: Department of Paediatrics and Adolescent Medicine, University of Freiburg, Mathildenstrasse 1, 79106 Freiburg, Germany. dormann@kikli.ukl.uni-freiburg.de
authors.forename: Sabine
authors.initials: S
authors.lastname: Dormann
authors.forename: Marcus
authors.initials: M
authors.lastname: Krüger
authors.forename: Roland
authors.initials: R
authors.lastname: Hentschel
authors.forename: Regina
authors.initials: R
authors.lastname: Rasenack
authors.forename: Brigitte
authors.initials: B
authors.lastname: Strahm
authors.forename: Udo
authors.initials: U
authors.lastname: Kontny
authors.forename: Charlotte
authors.initials: C
authors.lastname: Niemeyer
mesh_terms: D001772:Blood Cell Count; D001773:Blood Cells / Q000187:drug effects / Q000378:metabolism; D003561:Cytarabine / Q000627:therapeutic use; D004314:Down Syndrome / Q000150:complications*; D005260:Female; D006402:Hematologic Diseases / Q000209:etiology*; D006801:Humans; D015160:Hydrops Fetalis / Q000209:etiology; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007231:Infant, Newborn; D008103:Liver Cirrhosis / Q000209:etiology; D008297:Male; D038042:Myelopoiesis; D012131:Respiratory Insufficiency / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D003561:Cytarabine