Table 4.
Overview of recommendations for monitoring. (a) Recommendations on baseline checks for newly starting KDT as parenteral nutrition. (b) Recommendations on monitoring during KD‐PN
| (a) |
|---|
| Exclude contraindicated metabolic disorders (i.e. fatty acid oxidation disorders, carnitine deficiency [primary], carnitine palmitoyltransferase I or II deficiency, carnitine translocase deficiency, pyruvate carboxylase deficiency) |
| Anthropometrics (weight, height, head circumference at <1y) |
| Dietary intake and output |
| Complete blood count with platelets |
| Electrolytes to include serum bicarbonate, total protein, calcium, zinc, selenium, magnesium, phosphate |
| Trace elements; zinc, selenium, copper, CRP |
| Serum liver and kidney tests (including albumin, AST, ALT, blood urea, nitrogen, creatinine) |
| Fasting lipid profile |
| Serum acylcarnitine profile |
| Urine analysis |
| Urine calcium and creatinine |
| Antiepileptic drug levels (if applicable) |
| During initiation |
| Glucose daily/every other day |
| β‐hydroxyburyrate daily/every other day |
| (b) | ||||
|---|---|---|---|---|
| Item |
Age <1y Unstable |
Age <1y Stable |
Age >1y Unstable |
Age >1y Stable |
| Weight | Daily | Weekly | 2×wk | Weekly |
| Height | Weekly | Monthly | 1×2wks | 1×3mo |
| Head circumference | 1×2wks | Monthly | — | — |
| Intake/output | Daily | Daily | Daily | Daily |
| Seizures | Daily | Daily | Daily | Daily |
| Blood | ||||
| Blood gas and osmolarity | Daily | 2–3×wk | Daily | 2×wk |
| Sodium, potassium, chloride | Daily | 2–3×wk | Daily | 2×wk |
| Calcium, magnesium, phosphorus | Daily | 2–3×wk | Daily | 2×wk |
| Copper | Monthly | Monthly | Monthly | Monthly |
| Complete blood count | Unknown | Weekly | Unknown | Weekly |
| Albumin | Daily | 2×wk | Daily | Weekly |
| Glucosea | 1×4h | 2–4×d | 1×4–6h | 1–3×d |
| Billirubin total/direct | Daily | 2×wk | Daily | Weekly |
| ASAT, ALAT, ALP | 2×wk | 2×wk | Weekly | Weekly |
| Amylase/lipase | Unknown | Weekly | Unknown | Weekly |
| Urea | Daily | Daily | Daily | 2×wk |
| Creatinine | Daily | Daily | Daily | 2×wk |
| Triglyceridesb | Daily | Weekly | Daily | Weekly |
| β‐hydroxybutyratec | Daily | Daily | Daily | Daily |
| Carnitine free/total | Unknown | 1×6mo | Unknown | 1×6mo |
| Lipid profile | Daily when lipids off for 4h | Monthly | Unknown | Monthly |
| Hemoglobin | Weekly | Weekly | Weekly | Weekly |
| Serum ferritin | Monthly | Monthly | Unknown | Monthly |
| Vitamins A, D 25‐OH | Monthly | Monthly | Monthly | Monthly |
| Vitamins B1, B12 | Monthly | Monthly | Monthly | Monthly |
| Folic acid, Zn, Al | 1×6–12mo | 1×6–12mo | 1×6–12mo | 1×6–12mo |
| Copper, chromium, manganese | At indication | At indication | At indication | At indication |
| Selenium | Monthly | 1×6mo | Unknown | 1×6mo |
| Urine | ||||
| Sodium | Weekly | Monthly | Monthly | Monthly |
| Potassium | Weekly | Monthly | Monthly | Monthly |
| Calcium/creatinine ratio | Weekly | Monthly | Weekly | Monthly |
| Urine ketonesd (at indication) | Daily | 2×d | Daily | 2×d |
| Osmolarity | Weekly | Monthly | Monthly | Monthly |
aGlucose levels of 2–2.5mmol/L (approximately 40–45mg/dL) should be treated immediately with 2–4g of carbohydrates (20–40mL glucose 10%). Blood glucose should be rechecked 15–20min after treatment and if not improved a further dose is given until the glucose level is above 3mmol/L (approximately 55mg/dL).39 bThe upper limit for triglycerides is 10mmol/L (approximately 387mg/dL). At a level of 5mmol/L (approximately 200mg/dL), start carnitine 50mg/kg with a maximum of 1g/d.40 cThe goal is within the range 2–5mmol/L β‐hydroxybutyrate (approximately 20–52mg/dL). dIdeal range is 8–16mmol/L or 3–4+; the exact level depends on the type of urine dipstick. KDT, ketogenic diet therapy; KD‐PN, ketogenic parenteral nutrition; CRP, C‐reactive protein; AST, aspartate transaminase; ALT, alanine transaminase; ASAT, aspartate aminotransferase; ALAT, alanine aminotransferase; ALP, alkaline phosphatase.