title: A fetal case of transient abnormal myelopoiesis with severe liver failure in Down syndrome: prognostic value of serum markers.
abstract: The authors recently encountered a lethal case of Down syndrome with transient abnormal myelopoiesis (TAM). Although the peripheral white blood cell count and blast cells had improved without specific treatment, the patient died of severe coagulopathy due to liver fibrosis when he was 5 years old. The prognosis of TAM with liver fibrosis was poor. The patient had high levels of N-terminal peptide of III procollagen, type IV collagen, and hyaluronic acid. These serum makers are noninvasive indicators of liver fibrosis and may be useful as prognostic indicators of TAM in Down syndrome.
authors.affiliation: Department of Pediatrics, Juntendo University, School of Medicine, 2-1-1 Hongo, Bunkyou-ku, Tokyo # 113-8421, Japan.
authors.forename: Yusuke
authors.initials: Y
authors.lastname: Shiozawa
authors.forename: Hiroo
authors.initials: H
authors.lastname: Fujita
authors.forename: Junya
authors.initials: J
authors.lastname: Fujimura
authors.forename: Kyoko
authors.initials: K
authors.lastname: Suzuki
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Sato
authors.forename: Masahiro
authors.initials: M
authors.lastname: Saito
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Shimizu
authors.forename: Yuichiro
authors.initials: Y
authors.lastname: Yamashiro
mesh_terms: D015415:Biomarkers / Q000097:blood; D024141:Collagen Type IV / Q000097:blood; D004211:Disseminated Intravascular Coagulation; D004314:Down Syndrome / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D017809:Fatal Outcome; D005260:Female; D005315:Fetal Diseases / Q000097:blood / Q000175:diagnosis; D006801:Humans; D006820:Hyaluronic Acid / Q000097:blood; D007231:Infant, Newborn; D008103:Liver Cirrhosis / Q000097:blood / Q000175:diagnosis / Q000209:etiology; D017093:Liver Failure / Q000097:blood / Q000175:diagnosis / Q000209:etiology*; D008297:Male; D038042:Myelopoiesis; D010446:Peptide Fragments / Q000097:blood; D011247:Pregnancy; D011347:Procollagen / Q000097:blood; D011379:Prognosis
chemical_list: D015415:Biomarkers; D024141:Collagen Type IV; D010446:Peptide Fragments; D011347:Procollagen; C041876:procollagen Type III-N-terminal peptide; D006820:Hyaluronic Acid