753 AN INTRAORAL PROSTHESIS FACILITATES CONTINUOUS NOC-TURNAL FEEDINGS (CNF) IN GLYCOGEN STORAGE DISEASES, TYPES I AND III (GSD I/III)

Abstract

CNF is one of the mainstays of treatment of patients with GSD I/III. Previously, this goal has been achieved by infusing glucose-containing fluids intragastically via a nasogastric (NG) tube. However, the NG tube can be both difficult to insert and uncomfortable, is disliked by patients and parents, and occasionally leads to severe epistaxis. To avoid these problems, we have constructed an intraoral prosthesis (IOP) to facilitate CNF in GSD I/III. A piece of thin metal tubing 2mm (D) × 5mm (L) is attached to a circular orthodontic band 3mm in width and affixed to a superior 1st or 2nd molar with the tubing lying parallel to the aveolar ridge in the buccal-gingival sulcus. At night, a NG tube is pressed firmly through the IOP and its orifice positioned at the posterior end of the aveolar ridge. The. CNF is then infused at the normal rate (≤ 0.5 cc/min) and is swallowed readily during sleep. To date, one patient each with GSD I and III have been fitted with the IOP. The IOP has caused no local irritation or hemmorhage, even after nightly infusions of 40% dextrose for 6 months. Blood glucose levels and linear growth rates are equal to or better than those observed previously in the same patients using intragastric CNF. The IOP is well tolerated by both patients and parents because of its simplicity and comfort. The IOP may facilitate CNF in many patients with GSD I/III.

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