Dear Editor,

I have read with interest a systematic and well-referenced review of causes of neonatal coagulation disorders in recent Archives [1]. However, amongst non-haematological causes of the coagulopathies the author has failed to mention a small, but significant subgroup of patients with neonatal liver failure (NLF).

This condition could often be difficult to diagnose due to presence of jaundice (easily confused with physiological jaundice), sepsis and subtle, non-specific symptoms of encephalopathy, such as poor feeding or drowsiness. The commonest conditions causing NLF are vertically acquired herpes infection, perinatal haemochromatosis, haemophagocytic lymphohistiocytosis and mitochondrial cytopathies [2]. Furthermore, some liver-based metabolic conditions such as PiZZ alpha-1-antitrypsin deficiency or galactosaemia could cause a life-threatening late haemorrhagic disease of the newborn [5]. Need for a better awareness of these conditions has also been recently recognised by British Paediatric Surveillance Unit, which has conducted a prospective epidemiological study on incidence of vitamin K non-responsive haemorrhagic disease of the newborn.

Management of NLF has significantly improved in the recent years, not only following a success of liver transplantation [3], but also by early introduction of antioxidant medications for perinatal haemochromatosis [4], prompt use of intravenous acyclovir [3], even in the absence of obvious maternal symptoms, and, to some extent, immunosuppressive treatment for haemophagocytic lymphohistiocytosis. The outcome of these rare, but previously uniformly fatal conditions may not need to be that bleak provided they are suspected early and referred to specialist centres.

References

(1). Chalmers EA. Neonatal coagulation problems. Arch Dis Child 2004;89:F475-F478.

(2). Durand P, Debray D, Mandel R et al. Acute liver failure in infancy: a 14-year experience of a paediatric liver transplantation center. J Pediatr 2001;130;871-6.

(3). Twagira M, Hadzic N, Smith M et al. Disseminated neonatal herpes simplex virus (HSV) type 2 infection diagnosed by HSV DNA detection in blood and successfully managed by liver transplantation. Eur J Pediatr. 2004 Mar;163:166-9.

(4). Israels SJ, Gilfix BM. Alpha-1-antitrypsin deficiency with fatal intracranial haemorrhage in a newborn. J Pediatr Hematol Oncol 1999;21:447 -50.

(5). Flynn DM, Mohan N, McKiernan P, et al. Progress in treatment and and outcome for children with neonatal haemochromatosis. Arch Dis Child 2003;88:F124-7.