早产儿急性肝功能衰竭

doi:10.12372/jcp.2024.22e1420

临床儿科杂志 ›› 2024, Vol. 42 ›› Issue (2): 151-156.doi: 10.12372/jcp.2024.22e1420

早产儿急性肝功能衰竭

周琳¹, 刘成博¹, 刘明², 朱云开³, 陆永健⁴, 张拥军¹, 夏红萍¹()

1.上海交通大学医学院附属新华医院新生儿科，（上海 200092）
2.上海交通大学医学院附属新华医院医学影像科，（上海 200092）
3.上海交通大学医学院附属新华医院超声诊断科（上海 200092）
4.上海交通大学医学院附属新华医院口腔科（上海 200092）

收稿日期:2022-10-24 出版日期:2024-02-15 发布日期:2024-02-02
通讯作者: 夏红萍电子信箱：xiahongping@xinhuamed.com.cn

Acute Liver Failure of Preterm Infant

ZHOU Lin¹, LIU Chengbo¹, LIU Ming², ZHU Yunkai³, LU Yongjian⁴, ZHANG Yongjun¹, XIA Hongping¹()

1. Department of Neonatology, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China
2. Department of Radiology, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China
3. Department of Ultrasonography, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China
4. Department of Stomatology, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai 200092, China

Received:2022-10-24 Online:2024-02-15 Published:2024-02-02

摘要/Abstract

摘要：

目的探讨妊娠同族免疫性肝病-新生儿血色病（GALD-NH）的临床特点，提高对该疾病的认识，为新生儿肝功能衰竭提供鉴别诊断思路。方法新生儿重症监护室（NICU）收治的1例早产儿，生后持续血小板减少、凝血功能障碍、低蛋白血症，伴胆汁淤积、腹水。新生儿科联合医学影像科、口腔科、超声诊断科多学科讨论，制定进一步诊疗方案。结果患儿生后早期出现肝功能衰竭，经多学科讨论，考虑GALD-NH可能。完善口腔黏膜活检，未发现阳性结果。腹部（肝脾、胰腺）MRI显示肝、脾T2WI弥漫性信号减低。肝穿刺病理显示肝细胞局灶性颗粒样铁沉积，明确诊断为GALD-NH。结论新生儿肝功能衰竭除需考虑感染性肝病、胆道系统阻塞性肝病、遗传性代谢性疾病外，还需考虑妊娠同族免疫性肝病。

关键词: 肝功能衰竭, 妊娠同族免疫性肝病, 新生儿血色病, 早产儿

Abstract:

Objective To investigate the clinical features of gestational alloimmune liver disease-neonatal hemochromatosis (GALD-NH) and to provide differential diagnosis of neonatal liver failure.Method A preterm infant admitted in neonatal intensive care unit shortly after birth. He presented with persistent thrombocytopenia, severe coagulation disorder, hypoproteinemia, cholestasis and ascites. Specialists from Department of Radiology, Ultrasonography, and Stomatology were invited to discuss the case. The diagnosis and treatment were adjusted according to the result of discussion. Results The case was characterized by acute liver failure in the early life. After multi-disciplinary treatment, GALD-NH was considered as the most likely cause of liver failure. Although oral mucosa biopsy was negative, abdominal (liver, spleen, pancreas) MRI showed liver and spleen had hypointense on T2WI. Liver biopsy showed that pathologic siderosis in hepatocytes. The patient finally diagnosed as GALD-NH. Conclusion When neonate with liver failure, neonatal hemochromatosis should be considered in addition to infectious liver disease, obstructive liver disease and genetic metabolic disease.

Key words: liver failure, gestational alloimmune liver disease, neonatal hemochromatosis, preterm

周琳, 刘成博, 刘明, 朱云开, 陆永健, 张拥军, 夏红萍. 早产儿急性肝功能衰竭[J]. 临床儿科杂志, 2024, 42(2): 151-156.

ZHOU Lin, LIU Chengbo, LIU Ming, ZHU Yunkai, LU Yongjian, ZHANG Yongjun, XIA Hongping. Acute Liver Failure of Preterm Infant[J]. Journal of Clinical Pediatrics, 2024, 42(2): 151-156.

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参考文献 20

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指标	D1	D4	D7	D14	D20	D29
血常规
白细胞计数/×10⁹·L^-1	5.39	4.28	6.51	8.24	6.96	4.68
中性粒细胞/%	33.1	43.2	42.7	65.1	47.0	38.7
淋巴细胞/%	50.3	29.9	30.0	22.1	33.9	46.4
血红蛋白/g·L^-1	159	153	163	98	128	134
血小板计数/×10⁹·L^-1	6	76	21	24	69	58
凝血功能
凝血酶原时间/s	>60.0	83.2	64.8	93.5	85.4	37.9
部分凝血活酶时间/s	>180.0	50.6	39.1	49.9	49.3	133.5
纤维蛋白原/g·L^-1	0.50	0.39	1.05	0.45	1.50	0.99
D-D二聚体/mg·L^-1	24.06	20.95	31.32	20.30	70.64	26.27
肝功能
丙氨酸氨基转移酶/U·L^-1	36.3	30.8	32.1	52.0	85.0	74.1
天冬氨酸氨基转移酶/U·L^-1	114.8	168.9	81.4	88.2	140.3	300.5
总胆红素/μmol·L^-1	59.5	188.0	344.8	287.5	358.0	339.8
直接胆红素/μmol·L^-1	9.99	51.44	73.17	91.55	223.11	25.5
白蛋白/g·L^-1	17.7	18.9	32.2	29.7	25.5	31.6

早产儿急性肝功能衰竭

Acute Liver Failure of Preterm Infant

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