Case Reports in Pediatrics (Jan 2017)

Neonatal Liver Failure and Congenital Cirrhosis due to Gestational Alloimmune Liver Disease: A Case Report and Literature Review

  • Carolina Roos Mariano da Rocha,
  • Renata Rostirola Guedes,
  • Carlos Oscar Kieling,
  • Marina Rossato Adami,
  • Carlos Thadeu Schmidt Cerski,
  • Sandra Maria Gonçalves Vieira

DOI
https://doi.org/10.1155/2017/7432859
Journal volume & issue
Vol. 2017

Abstract

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Neonatal liver failure (NLF) is a major cause of neonatal morbidity and mortality, presenting as acute liver failure and/or congenital cirrhosis. Many affected patients show antenatal signs of fetal injury. There are several causes of NLF and early diagnosis is mandatory to elucidate the etiology and determine a specific treatment or the best management strategy. Gestational alloimmune liver disease associated with neonatal hemochromatosis (GALD-NH) is a rare but potentially treatable cause of NLF. It should be considered in any neonate with fetal signs of disease and postnatal signs of liver failure with no other identifiable causes. GALD-NH is often diagnosed late and patients are therefore referred late to specialized centers, delaying treatment. This case highlights the consequences of late diagnosis and treatment of GALD-NH and emphasizes the importance of a high grade of suspicion of this disease in order to refer the patient to a specialized center soon enough to perform the appropriate treatment.