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 (g) |
1,980 |
2,000 |
1,275 |
1,400 |
Percentile |
0.4 |
2 |
0.1 |
<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 |
Steatorrhea, failure to thrive |
No symptoms of exocrine insufficiency |
Failure to thrive |
Steatorrhea, failure to thrive |
Biochemical basis of exocrine insufficiency |
Fecal fat: 3 g/24 h stool
Stool chymotrypsin: 1 U/g (normal range 4–10 U/g)
Serum trypsinogen: 4 μg/L (normal range 15–25 μg/L) |
Fecal fat: 0.5 g/24 h Stool chymotrypsin: 5 U/g (4–10 U/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 |
Not applicable |
Not applicable |
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) |