Table 1.

Clinical characteristics of patients studied

	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 (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)