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新生儿急性肝衰竭

Acute liver failure in newborns.

作者信息

Karadağ Nilgün, Okbay Güneş Aslı, Karatekin Güner

机构信息

Department of Neonatal, University of Health Sciences, Zeynep Kamil Women's and Children's Hospital, İstanbul, Turkey.

出版信息

Turk Arch Pediatr. 2021 Feb 3;56(2):108-114. doi: 10.5152/TurkArchPediatr.2021.190205. eCollection 2021 Mar.

Abstract

Acute liver failure is a condition that is defined as a sudden, complete, or nearly complete loss of liver functions without any previous liver disease, usually accompanied by encephalopathy, which can be reversible, but with a mortality rate of 55-70%. Acute liver failure newborns is an acute liver failure in the first 28 days of life. The Pediatric Acute Liver Failure Working Group identified the presence of coagulopathy as the main finding for the identification of acute liver failure in childhood following vitamin K administration. Although the incidence of acute liver failure is reported to be 17/100 000 in all ages, its incidence is not known exactly in newborn and childhood. The most common cause of acute liver failure in the newborn period is the gestational alloimmune liver disease that was previously known as neonatal hemochromatosis. This is followed by viral infections, metabolic diseases, hemophagocytic lymphohistiocytosis, and other rare causes. In the neonatal period, acute liver failure is a rare condition with a high mortality rate. For this reason, the vital signs of the patients should be closely monitored and supportive treatments should be planned according to the follow-up and the etiology of the disease should be clarified urgently. In this process, acyclovir treatment until herpes simplex virus infection is excluded and lactose-free feeding until galactosemia is excluded are recommended as life-saving treatments. In the literature, since there is a limited number of studies related to neonatal acute liver failure, prospective studies investigating the factors affecting treatment and prognosis are needed.

摘要

急性肝衰竭是一种在无既往肝脏疾病的情况下,肝脏功能突然、完全或几乎完全丧失的病症,通常伴有脑病,脑病可能可逆,但死亡率为55% - 70%。新生儿急性肝衰竭是指出生后28天内发生的急性肝衰竭。儿科急性肝衰竭工作组确定,在给予维生素K后,凝血功能障碍是儿童急性肝衰竭诊断的主要依据。虽然据报道急性肝衰竭在各年龄段的发病率为17/10万,但新生儿和儿童的确切发病率尚不清楚。新生儿期急性肝衰竭最常见的病因是既往称为新生儿血色素沉着症的妊娠同种免疫性肝病。其次是病毒感染、代谢性疾病、噬血细胞性淋巴组织细胞增生症和其他罕见病因。在新生儿期,急性肝衰竭是一种罕见但死亡率高的病症。因此,应密切监测患者的生命体征,并根据随访情况规划支持治疗,同时应尽快明确疾病病因。在此过程中,建议在排除单纯疱疹病毒感染之前进行阿昔洛韦治疗,在排除半乳糖血症之前进行无乳糖喂养,作为挽救生命的治疗措施。在文献中,由于与新生儿急性肝衰竭相关的研究数量有限,需要开展前瞻性研究来调查影响治疗和预后的因素。

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