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伴有极端实验室检查异常的同种免疫性溶血和胆汁淤积的保守治疗

Conservative Management of Alloimmune Hemolysis and Cholestasis With Extreme Laboratory Abnormalities.

作者信息

Kotch Chelsea, Friedman David F, Wilkins Benjamin J, Samelson-Jones Benjamin J

机构信息

Division of Hematology and.

Department of Pathology and Laboratory Medicine, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; and.

出版信息

Pediatrics. 2021 Feb;147(2). doi: 10.1542/peds.2019-3367.

Abstract

Alloimmune hemolytic disease of the fetus or newborn (HDFN) is a rare cause of neonatal cholestasis. HDFN-associated cholestasis has most often been reported secondary to anti-D alloimmunization. In utero transfusions are also an identified risk factor. A variety of diagnostic and therapeutic strategies have been described, mostly in case reports. Here, we report 2 cases of HDFN-associated cholestasis that were notable for extreme laboratory abnormalities including a peak ferritin of 24 700 ng/mL and a peak alanine aminotransferase of 1406 U/L (33.5-fold upper limit of normal). One case was due to alloimmunization other than anti-D. These cases help define the range of laboratory derangements that are consistent with HDFN-associated cholestasis, including extreme hyperferritinemia. Although in a number of cases, researchers have reported the use of iron chelation in these infants, herein, we describe successful management without iron chelation.

摘要

胎儿或新生儿同种免疫性溶血性疾病(HDFN)是新生儿胆汁淤积的罕见病因。HDFN相关的胆汁淤积最常报道为抗-D同种免疫继发。宫内输血也是一个已确定的危险因素。已经描述了多种诊断和治疗策略,大多是病例报告。在此,我们报告2例HDFN相关的胆汁淤积病例,其显著特点是实验室检查异常极端,包括铁蛋白峰值达24700 ng/mL,丙氨酸氨基转移酶峰值达1406 U/L(正常上限的33.5倍)。1例是由抗-D以外的同种免疫引起。这些病例有助于明确与HDFN相关胆汁淤积相符的实验室紊乱范围,包括极端高铁蛋白血症。尽管在许多病例中,研究人员报告了在这些婴儿中使用铁螯合治疗,但在此我们描述了未使用铁螯合治疗的成功管理方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2736/10170749/97d5b6d130e5/nihms-1896831-f0001.jpg

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