Hematological abnormalities during the first week of life among neonates with Down syndrome: Data from a multihospital healthcare system

被引:69
作者
Henry, E.
Walker, D.
Wiedmeier, S. E.
Christensen, R. D.
机构
[1] Univ Utah, Sch Med, Inst Hlth Care Delivery Res, Intermt Healthcare,Dept Women & Newborns, Salt Lake City, UT 84112 USA
[2] Univ Utah, Sch Med, Dept Pediat, Salt Lake City, UT 84112 USA
关键词
complete blood count; Down syndrome; neutrophilia; polycythemia; thrombocytopenia;
D O I
10.1002/ajmg.a.31442
中图分类号
Q3 [遗传学];
学科分类号
071007 ; 090102 ;
摘要
Various hematological abnormalities have been reported among neonates with Down syndrome. Thrombocytosis, thrombocytopenia, polycythemia, neutrophilia, transient myeloproliferative disorder (TMD), and congenital leukemia have all been reported. The two largest case series previously reported involved 63 and 31 cases. To acquire hematological data from a larger case series, we obtained all CBCs done during the first week after birth on all neonates with Down syndrome cared for in an Intermountain Healthcare QHQ hospital with a date of birth between January 1, 2001 and December 31, 2005. During this period, 145,522 live births were recorded at 18 hospitals. Down syndrome was recognized in 226 (1 in 644). One hundred fifty-eight (70%) of these had one or more CBCs obtained before the seventh clay (144 hr). Neonates who did versus did not have a CBC in the first week had a similar gestational age, birth weight percentage who were LGA and SGA, and length of stay. Neutrophilia was the most common hematological abnormality detected, with 80% of absolute neutrophil counts above the Upper limit of normal for age. Six percent (9/158) had blasts identified on the blood film and three, where this was persistent, were referred to the pediatric hematology service for further evaluation. The next most commonly detected abnormality was thrombocytopenia, with 66% of platelet counts < 150,000/mu l, and with 6% of coLints < 50,000/mu l. The mean platelet volume did not correlate with the platelet count, but tended to run slightly large (9.2 +/- 1.3 fl), with 24% of values above 10 fl. Only one had a platelet transfusion. Polycythemia was the next most common hematological abnomality detected, with 33% of hematocrit values above 65% or hemoglobin concentations ahove 22 g/dl. Six had a reduction transfusion. One patient had significant anemia (hematocrit < 15%) and received an erythrocyte transfusion. One had neutropenia associated with an infection after bowel surgery. Nentrophilia, thrombocytopenia, and polvcythemia were the most common hematological abnormalities observed among neonates with Down syndrome. Anemia, thrombocytosis, and neutropenia were not more common than among, neonates who do not have Down syndrome. Hematological abnormalities were so common in this group that it seems reasonable to recommend that one or more CBCs he obtained on all neonates with Down syndrome. (c) 2006 Wiley-Liss, Inc.
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页码:42 / 50
页数:9
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