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©2013 UpToDate ® Print Email Laboratory monitoring of nutritional status for children with cystic fibrosis Recommended frequency Tests At diagnosis Annually Other Beta carotene At physician'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-OH-D Vitamin E X* X α-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 FTT Triene:tetraene ratio or Linoleic acid (as moles percent of total serum phospholipid fatty acids) Calcium/bone status X Begin DXA at age 8 years if risk factors are present • and repeat every 1 to 2 years depending on results (see text) Calcium, phosphorus, intact PTH, DXA scan Iron X X Consider in-depth evaluation for patients with poor appetite Hemoglobin, hematocrit Serum transferrin receptor (sTfR) Zinc Consider 6-month supplementation trial and follow growth No acceptable measurement Sodium Consider checking if exposed to heat stress and becomes dehydrated Serum sodium; spot urine sodium if total body sodium depletion suspected Protein stores X X Check in patients with nutritional failure or those at risk Albumin Oral glucose tolerance test Δ X HgbA1c ≥6.5 percent or fasting plasma glucose ≥126 mg/dL support the diagnosis of CFRD but are not sufficiently sensitive to use for screening 25-OH-D: 25-hydroxyvitamin D; PIVKA: proteins induced by vitamin K absence or antagonism; FTT: failure to thrive; Intact PTH: intact parathyroid hormone; DXA: dual energy x-ray absorptiometry; HgbA1c: hemoglobin A1c. * For infants diagnosed by neonatal screening these should be measured approximately 2 months after starting vitamin supplementation, and annually thereafter. • Risk factors for bone disease include body mass index (BMI) <10th percentile for age, FEV1 <50 percent predicted, glucocorticoid use of ≥5 mg daily for ≥90 days/year, delayed puberty, or a history of fractures. Δ Testing for cystic fibrosis-related diabetes (CFRD) using the oral glucose tolerance test (OGTT) should be performed annually in patients 10 years and older, and also in patients who develop symptoms of CFRD.

25-OH-D: 25-hydroxyvitamin D; PIVKA: proteins induced by vitamin K absence or antagonism; FTT: failure to thrive; Intact PTH: intact parathyroid hormone; DXA: dual energy x-ray absorptiometry; HgbA1c: hemoglobin A1c. * For infants diagnosed by neonatal screening these should be measured approximately 2 months after starting vitamin supplementation, and annually thereafter. • Risk factors for bone disease include body mass index (BMI) <10th percentile for age, FEV1 <50 percent predicted, glucocorticoid use of ≥5 mg daily for ≥90 days/year, delayed puberty, or a history of fractures. Δ Testing for cystic fibrosis-related diabetes (CFRD) using the oral glucose tolerance test (OGTT) 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, et al. Guide to bone health and disease in cystic fibrosis. J Clin Endocrinol Metab 2005; 90:1888.