Almost 50% of our sample failed to open with 15 pounds of force, which is the force used in the ASTM standard for children's jewelry. Eighty per cent of our sample failed to open with 1.6 pounds of force, which was the mean force to occlude a young child's airway in a published study. These necklaces pose a strangulation risk to young children if they were to become caught.

Background:We present a rare case of neonatal cholestasis in a female infant with Gaucher Disease (GD), who received liver transplantation. We review the relevant literature on similar disease presentations. Methods:A chart review of the index case was performed. PubMed and Medline databases were searched to identify other cases. Results:A 4-day-old female was referred with conjugated hyperbilirubinemia.Physical examination revealed icterus with hepatosplenomegaly and normal neurologic examination. The diagnosis of GD was confirmed through liver biopsy, low glucocerebrosidase enzyme activity, and two pathogenic mutations in GBA gene.Despite early initiation of ERT, the patient had worsening of her liver failure and underwent a left lateral segment liver transplant from a living donor at 7 months of age. She experienced improvement of her liver enzymes and coagulation, but passed away at 8 months due to the late onset of neurologic involvement. Nine other cases of GD presenting with neonatal cholestasis have been reported. Forty-four percent (4/9) of cases received ERT and none were considered for transplant. Overall, the literature suggests a poor prognosis with death reported in 77% (7/9) cases.

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