Table 1 Critical Sample Laboratory Data of Case 1 (KCNJ11 gene mutation)

POC Blood glucose

48 mg/dL

70–99 mg/dL

Serum Blood Glucose

58 mg/dL

70–99 mg/dL

Serum Cortisol

23 mcg/dL

7–25 mcg/dL

Serum Growth Hormone

4.6 ng/mL

0–7 ng/mL

Serum Insulin

16.3 mcIU/mL

2–18 mcIU/mL

Beta Hydroxybutyrate

Less than 100

0–269 mcmol/L

Urinary Ketones

N/A

N/A

Serum Lactic Acid

1 mmol/L

0.7–2.1 mmol/L

Serum Pyruvic Acid

0.08 mmol/L

0.08–0.16 mmol/L

Free Fatty Acids

Not collected

N/A

Plasma Acyl carnitine profile^a

Normal

Urine Organic Acids^a

Normal

Serum Ammonia^a

10 mcmol/L

10–62 mcmol/L

Serum Amino Acid Profile^a

Normal

N/A