Table 1. Pancreatic clinical characteristics of patients studied.
Patient | I-1 | I-2 | II-1 | II-2 |
Present age | 12 years | 9 years | Deceased at 12 weeks due to sepsis | 2 years |
Sex | Male | Female | Male | Female |
Country of origin | Saudi Arabia | Saudi Arabia | Kuwait | Kuwait |
Birth weight Percentile |
1980 g 0.4 |
2000 g 2 |
1275 g 0.1 |
1400 g <0.1 |
Gestational age | 38 weeks | 37 weeks | 34 weeks | 36 weeks |
Age at diabetes diagnosis/ Age of permanent Insulin therapy | 1 Day /2 months | 1 day/4 months | 1 day/1 day | 8 days/ 8 days |
Diabetes treatment | Insulin | Insulin | Insulin | Insulin |
Exocrine pancreatic insufficiency requiring replacement? | Yes | No | Yes | Yes |
Clinical basis of exocrine insufficiency | Steatorrhoea, failure to thrive | No symptoms of exocrine insufficiency | Failure to thrive | Steatorrhoea, failure to thrive |
Biochemical basis of exocrine insufficiency | Fecal fat: 3 g/24hr stool Stool chymotrypsin: 1 U/g (4-10U/g) Serum trypsinogen: 4 μg/l (15-25 μg/l) | Fecal fat: 0.5 g/24hr stool Stool chymotrypsin: 5 U/g (4-10U/g) Serum trypsinogen: 18 μg/l (15-25 μg/l) | Fecal elastase: undetectable (>200 μg elastase/g stool) | |
Exocrine pancreatic replacement regimen | 10 000 U/day of lipase | None | 15 000 U/day of lipase | |
Age at initiation of exocrine pancreatic replacement | 12 weeks | N/A | N/A | 10 weeks |
Clinical neurocognitive function | Normal neurocognitive development with some disruption of eye movements on tracking. | Normal neurocognitive development | Normal neurocognitive development | |
Brain imaging | Not performed | Not performed | Not performed | Normal (MRI imaging) |