Neonatal Transient Diabetes Mellitus

Neonatal transient diabetes mellitus (TNDM), with an incidence in newborns of 1/400,000 to 1/500,000 (Fosel, 1995; Shield et al., 1997), is characterized by intrauterine growth retardation, failure to thrive, hyperglycemia, glucosuria, dehydration, polyuria, occasional ketonemia and ketonuria, lethargy, and fever. Occasional features include anemia, macroglossia (Figure 1), and umbilical hernia. The diabetic condition may be permanent, subside to recur later as type 2 diabetes (Shield et al., 1997), or vanish completely (Christian et al., 1999).

TNDM may arise from paternal UPD6 (Temple et al., 1995; Hermann and Soltesz, 1997), from duplication of paternal chromosome 6q22-23 (Gardner et al., 1999; Temple et al., 1996; Arthur et al., 1997), or from other, as yet undefined, causes. Paternal UPD6 may account for approximately 20% of patients with TNDM

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