Watanabe Toshihiko, Amari Shoichiro, Tsukamoto Keiko, Ito Yushi, Tomizawa Daisuke, Yoshioka Takako, Kanamori Yutaka
Division of Surgery, Department of Surgical Specialties, National Center for Child Health and Development, Tokyo, Japan.
Division of Neonatology, National Center for Child Health and Development, Tokyo, Japan.
Pediatr Int. 2017 Apr;59(4):515-518. doi: 10.1111/ped.13250.
Neonates with Down syndrome are at risk of developing transient abnormal myelopoiesis (TAM), which is characterized by transient clonal myeloproliferation of the blast cells. TAM can resolve spontaneously, but some patients die at an early age due to organ failure. Liver fibrosis in TAM is a life-threatening condition, but treatment options have not yet been established. Here, we report on the case of an infant with TAM complicated by liver disease, whose hyperbilirubinemia was successfully ameliorated with omega-3 fatty acid (ω3FA) lipid emulsion. Timely ω3FA lipid emulsion may be a feasible treatment for liver disease in TAM before serious liver damage develops.
患有唐氏综合征的新生儿有发生短暂异常骨髓造血（TAM）的风险，其特征为原始细胞的短暂克隆性骨髓增殖。TAM可自发缓解，但一些患者会因器官衰竭在幼年死亡。TAM中的肝纤维化是一种危及生命的病症，但尚未确立治疗方案。在此，我们报告一例患有TAM并伴有肝脏疾病的婴儿病例，其高胆红素血症通过ω-3脂肪酸（ω3FA）脂质乳剂成功得到改善。在严重肝损伤发生之前，及时给予ω3FA脂质乳剂可能是治疗TAM中肝脏疾病的一种可行方法。
