早产儿急性肝功能衰竭

doi:10.12372/jcp.2024.22e1420

Abstract

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

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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References 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

Acute Liver Failure of Preterm Infant

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