Table 3.
Authors [Ref.], year | Age at presentation, days | X chromosome findings | Diazoxide response | Treatment |
---|---|---|---|---|
Glassman et al. [7], 1979 | ||||
Case 1 | 90 | 45,X | − | 80% pancreatectomy |
Case 2 | 120 | 45,X | − | leucine-restricted diet |
Kizaki et al. [8], 2003 | 1 | 45,X/46,X,r(X) | not tested | corn starch |
Alkhayyat et al. [9], 2006 | 1 | 45,X/46,X,r(X) | + | diazoxide |
Pietzner et al. [10], 2014 | 1 | 45,X/46,X,r(X) | + | diazoxide |
Cappella et al. [11], 2015 | 330 | 45,X/46,X,r(X) | + | diazoxide |