Neonatal Lupus Erythematosus Presenting as Neonatal Acute Liver Failure
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SCIENTIFIC LETTER
Neonatal Lupus Erythematosus Presenting as Neonatal Acute Liver Failure M. G. Kartheeka 1 & Uthaya Kumaran 1 & Nilesh Nikhilesh Rao 1 & Malathi Raja 1 & Praveena Shenoi 2 & Arvind Shenoi 1 Received: 5 August 2020 / Accepted: 16 October 2020 # Dr. K C Chaudhuri Foundation 2020
To the Editor: Neonatal lupus erythematosus (NLE) is a multisystem disease and is diagnosed if – either the mother or the infant has anti-Ro/SSA or anti-La/SSB or anti-ribonucleo protein antibodies and the neonate has features of either cardiac, cutaneous, hepatic or hematological manifestations [1, 2]. Hepatobiliary involvement in NLE usually presents as conjugated hyperbilirubinemia or mild elevation of hepatic enzymes. We report a neonate with NLE presented as acute liver failure (ALF) mimicking gestational alloimmune liver disease– neonatal hemochromatosis (GALD-NH) [3]. A 26-y-old primigravida with systemic lupus erythematosus (anti-Ro/SSA antibodies +) on hydroxychloroquine delivered a 2100 g female growth restricted neonate at 36 wk gestation. The neonate was admitted to neonatal unit with recurrent hypoglycemia and found to have conjugated hyperbilirubinemia with raised transaminases (SGOT 194 U/L, SGPT 40 U/L, GGT 29 U/L), deranged coagulation (INR 3.39, PTT 65.8 s/ 40 s), hyperferritinemia (2000 ng/ml) and raised serum alphafetoprotein (50,695 ng/ml) suggesting GALD-NH [4]. A complete evaluation for sepsis, galactosemia, tyrosinemia, hemophagocytic lymphohistiocytosis, mitochondrial disorders and herpes simplex hepatitis, TORCH were all normal. MRI T2 imaging showed reduced signal intensity in the liver, with normal signal intensity elsewhere suggesting GALD-NH. The neonate received IVIG (3 doses) and 2 double volume exchange transfusions during the course. As the ALF was persisting, the parents were counseled and referred to a liver transplant centre.
GALD is the process of causing injury to fetal liver and NH is the phenotypic expression of the liver injury [4, 5]. In our neonate, the insult was mostly due to maternal anti-Ro/SSA antibody causing damage to the fetal liver. Biopsy of oral mucosa and liver were planned for the infant but could not be done due to coagulopathy. There are three similar reports of NLE presenting with only hepatobiliary manifestations as GALD -NH, all of which succumbed to severe ALF in the neonatal period [3]. Neonates with GALD-NH tend to respond to IVIG and exchange transfusion, while this neonate did not. This suggests that liver injury occurs antenatally and becomes irreversible at birth. Our case is a rare form of NLE presenting as ALF mimicking GALD-NH, refractory to IVIG and exchange transfusions, without any associated manifestations.
Acknowledgements We acknowledge the help of Prof. SK Yaccha in the clinical management of this case.
Compliance with Ethical Standards Conflict of Interest None.
References 1. 2.
* Arvind Shenoi [email protected] 1
Department of Pediatrics, Cloudnine Hospital, Old Airport Road, Bangalore, Karnataka 560017, India
2
Departmen
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