Version May 2024
| Recommended frequency | Tests | |||
| At diagnosis | Annually | Other indications | ||
| Beta carotene | At clinician's discretion | Serum levels | ||
| Vitamin A | X* | X | Also check serum retinol-binding protein and retinyl esters in patients with liver disease | Vitamin A (retinol) |
| Vitamin D | X* | X | 25-hydroxyvitamin D | |
| Vitamin E | X* | X | Alpha-tocopherol | |
| Vitamin K | X* | If patient has hemoptysis or hematemesis; in patients with liver disease | PIVKA-II (preferably) or prothrombin time | |
| Essential fatty acids | Consider checking in infants or those with failure to thrive | Biomarkers for essential fatty acid deficiency:
| ||
| Calcium/bone status | X | Begin DXA at age 8 years if risk factors are presentΔ and repeat every 1 to 2 years depending on results (refer to text) | Calcium, phosphorus, intact PTH, DXA scan | |
| Iron | X | X | Consider in-depth evaluation for patients with poor appetite | Hemoglobin, hematocrit Serum transferrin receptor |
| Zinc | Consider 6-month supplementation trial and follow growth | No acceptable measurement | ||
| Serum electrolytes | X | Consider checking if exposed to heat stress and becomes dehydrated | Serum sodium; spot urine sodium if total body sodium depletion suspected | |
| Renal function tests | X | BUN, creatinine | ||
| Liver function tests | X | AST, ALT, bilirubin, alkaline phosphatase | ||
| Protein stores | X | X | Check in patients with nutritional failure or those at risk | Albumin |
| Oral glucose tolerance test◊ | X | HgbA1c ≥6.5% or fasting plasma glucose ≥126 mg/dL supports the diagnosis of CFRD but are not sufficiently sensitive to use for screening | ||
PIVKA: proteins induced by vitamin K absence or antagonism; DXA: dual-energy x-ray absorptiometry; PTH: parathyroid hormone; BUN: blood urea nitrogen; AST: aspartate aminotransferase; ALT: alanine aminotransferase; HgbA1c: hemoglobin A1c; CFRD: cystic fibrosis-related diabetes; FEV1: forced expiratory volume in 1 second.
* For infants diagnosed by neonatal screening, these should be measured approximately 2 months after starting vitamin supplementation and annually thereafter.
¶ Essential fatty acid deficiency is likely when the levels of linoleic, alpha linolenic, eicosapentaenoic, and docosahexaenoic acids are below reference range. This test is not widely available but has the advantage of providing information on omega-3 as well as omega-6 fatty acids.
Δ Risk factors for bone disease include body mass index <10th percentile for age, FEV1 <50% predicted, glucocorticoid use of ≥5 mg daily for ≥90 days/year, delayed puberty, or a history of fractures.
◊ Testing for CFRD using the oral glucose tolerance test should be performed annually in patients 10 years and older and also in patients who develop symptoms of CFRD.Modified with permission from: Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2002; 35:246. Copyright © 2002 Lippincott Williams & Wilkins.
Additional recommendations on bone health from: Aris RM, Merkel PA, Bachrach LK, et al. Guide to bone health and disease in cystic fibrosis. J Clin Endocrinol Metab 2005; 90:1888.آیا می خواهید مدیلیب را به صفحه اصلی خود اضافه کنید؟
