Boneh A, Allan S, Mendelson D, Spriggs M, Gillam LH, Korman SH. Clinical, ethical and legal considerations in the treatment of newborns with non-ketotic hyperglycinaemia. Mol Genet Metab. 2008;94(2):143-147. doi:10.1016/j.ymgme.2008.02.010
Abstract
Non-ketotic hyperglycinaemia (NKH) is a devastating
neurometabolic disorder leading, in its classical form, to early death or
severe disability and poor quality of life in survivors. Affected neonates may
need ventilatory support during a short period of respiratory depression. The
transient dependence on ventilation dictates urgency in decision-making
regarding withdrawal of therapy. The occurrence of patients with apparent
transient forms of the disease, albeit rare, adds uncertainty to the prediction
of clinical outcome and dictates that the current practice of withholding or
withdrawing therapy in these neonates be reviewed. Both bioethics and law take
the view that treatment decisions should be based on the best interests of the
patient. The medical-ethics approach is based on the principles of
non-maleficence, beneficence, autonomy and justice. The law relating to
withholding or withdrawing life-sustaining treatment is complex and varies
between jurisdictions. Physicians treating newborns with NKH need to provide
families with accurate and complete information regarding the disease and the
relative probability of possible outcomes of the neonatal presentation and to
explore the extent to which family members are willing to take part in the
decision making process. Cultural and religious attitudes, which may
potentially clash with bioethical and juridical principles, need to be
considered.
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