Use of N ‐Acetyl Cysteine for the Treatment of Parenteral Nutrition–induced Liver Disease in Children Receiving Home Parenteral Nutrition

The use of long-term parenteral nutrition (PN) in children with significant gut failure has been associated with progressive cholestatic liver disease (1–4). Progression to liver cirrhosis and end-stage liver disease are well-known consequences of long-term dependence on PN. The etiology of liver disease related to PN administration seems to be multifactorial (1–3). There is evidence linking the macronutrient and micronutrient content of PN solutions to the onset and severity of PN-induced liver cholestasis (4). Recent studies in animal models, in particular, have demonstrated an association between the onset of cholestatic liver disease and intravenous (IV) methionine (MET) intake (4–6). Most commercial PN solutions have relatively higher amounts of MET and little or no cysteine (CYS) because of the instability of CYS in solution (7). Inasmuch as CYS is an important precursor of glutathione (GSH), it was hypothesized that provision of parenteral CYS could potentially counteract the oxidative stress that occurs in liver cholestasis (8). Recently, our group showed that Nacetyl cysteine (NAC) can be used as a source of CYS in PN solutions in piglets (9). We supplemented 2 infants and 1 child with PN-induced liver disease who were receiving PN at home with IV NAC as an adjunctive therapy to minimize further liver damage induced by PN.