Table 1.
Patient | Age (years) | Gender | Time since diagnosis (years) | Genetic pathology | Medications |
---|---|---|---|---|---|
Patient 1 | 14.5 | Female | 14.5 | Paternally inherited KCNJ11 mutation + interruption of Chromosome 1 | Diazoxide |
Patient 2 | 3.2 | Female | 3.0 | Not identified | Diazoxide |
Patient 3 | 12.3 | Male | 11.9 | Not identified | Diazoxide |
Patient 4 | 5.4 | Male | 5.4 | Maternally inherited dominant ABCC8 mutation | Diazoxide |
Patient 5 | 3.1 | Male | 3.1 | Homozygous ABCC8 mutation | Octreotide |
Patient 6 | 3.4 | Female | 3.4 | Maternally inherited dominant ABCC8 mutation | Diazoxide |
Patient 7 | 17.3 | Male | 17.1 | GLUD1 mutation (de novo) | Diazoxide |
Patient 8 | 13.3 | Female | 13.0 | Homozygous HADH mutation | Diazoxide |
Patient 9 | 17.7 | Male | 7.4 | GCK mutation (inheritance not determined) | Diazoxide |
Patient 10 | 2.1 | Male | 2.1 | Heterozygous HNF4A partial deletion | Diazoxide |
All patients continued on their mediactions for the duration of the study. Patient 6 withdrew from the study at the end of week 8 due to dissatisfaction with the CGM device.