Thirty-five males with severe (Class 1) G6PD deficiency (c.637G>T) in a North American family of European ancestry.

In North America, jaundiced neonates are not usually tested for G6PD deficiency if the family is of European ancestry. However, we describe such a family where ≥35 males have had severe (Class I) G6PD deficiency. Many of the jaundiced neonates did not have this diagnosis considered, at least three of whom developed bilirubin neurotoxicity. Over seven generations 35 affected males were identified. Three developed signs of kernicterus spectrum disorder; three had exchange transfusions for hyperbilirubinemia; and nine received one or more blood transfusions during childhood.

Treatment of neonatal pain without a gold standard: the case for caregiving interventions and sucrose administration.

Although many different neonatal pain assessment scales are used in clinical research, no gold-standard instrument exists. The multitude of pain assessment scales used has, in fact, threatened the validity of many studies and confused the construct of pain. This integrative review of the neonatal pain literature provides the recommendations from practice guidelines and the current evidence for modifying the pain response prior to and during common painful procedures.