Evans N, Gaskin K
Department of Perinatal Medicine, King George V Hospital, Camperdown, New South Wales, Australia.
J Paediatr Child Health. 1993 Dec;29(6):478-80. doi: 10.1111/j.1440-1754.1993.tb03026.x.
This report describes a patient with neonatal lupus erythematosus (NLE) in whom there was multisystem involvement including neonatal hepatitis. The hepatitis, defined pathologically as a giant cell hepatitis, presented with severe cholestasis, was unrelated to other known causes of neonatal hepatitis and resolved spontaneously by 6 months of age. Both mother and infant were positive for Sjögren syndrome A+B antibodies (SS-A[Ro] and SS-B[La]) as well as having high titres of antinuclear antibody (ANA). Three of the four cases described in the one previous report of this association were also ANA positive. This antibody may be a marker for the development of the hepatitis. Maternal and infant ANA status should be determined in cases labelled as idiopathic neonatal hepatitis to exclude undiagnosed maternal SLE as a cause of neonatal cholestasis.
本报告描述了一名患有新生儿红斑狼疮（NLE）的患儿，该患儿存在多系统受累，包括新生儿肝炎。这种肝炎在病理上被定义为巨细胞肝炎，表现为严重胆汁淤积，与其他已知的新生儿肝炎病因无关，并在6个月龄时自发消退。母亲和婴儿的干燥综合征A+B抗体（SS-A[Ro]和SS-B[La]）均为阳性，且抗核抗体（ANA）滴度很高。此前关于这种关联的一份报告中所描述的4例病例中有3例ANA也呈阳性。这种抗体可能是肝炎发生的一个标志物。对于被标记为特发性新生儿肝炎的病例，应确定母婴的ANA状态，以排除未被诊断出的母亲系统性红斑狼疮作为新生儿胆汁淤积的病因。
