{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,14]],"date-time":"2026-01-14T16:12:31Z","timestamp":1768407151710,"version":"3.49.0"},"reference-count":43,"publisher":"American Academy of Pediatrics (AAP)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2006,2,1]]},"abstract":"<jats:p>OBJECTIVE. It is unclear why some patients with cystic fibrosis (CF) succeed (\u201cresponders\u201d) in recovering from malnutrition and growth faltering after treatment initiation whereas others fail to do so (\u201cnonresponders\u201d). We conducted a study to test the hypothesis that sustained high energy intake (\u2191EN) and normal plasma essential fatty acid status are critical determinants of treatment responsiveness within 2 years after diagnosis of CF.<\/jats:p><jats:p>METHODS. A total of 71 CF children who had pancreatic insufficiency but not meconium ileus and were enrolled in the Wisconsin CF Neonatal Screening Project were studied. Responders were defined by having achieved adequate weight gain, as indicated by a recovery of weight z score (Wtz) comparable to Wtz at birth (WtzBR) within 2 years of diagnosis. \u2191EN and sustained normal plasma linoleic acid level (\u2191pLA) were defined by achieving energy intake \u2265120% of estimated requirement for \u226575% of the time and maintaining plasma LA \u226526% of total fatty acids for \u226575% of the time, respectively.<\/jats:p><jats:p>RESULTS. Thirty-two (68%) screened patients and 13 (54%) patients whose CF was diagnosed conventionally recovered WtzBR within 2 years of diagnosis. Screened patients responded at significantly younger ages (mean\/median: 6.3\/4.3 months) than patients whose CF was diagnosed conventionally (mean\/median: 15.8\/11.8 months). Proportionately fewer screened patients (33%) achieved \u2191EN compared with patients whose CF was diagnosed conventionally (73%). However, more screened patients responded to \u2191EN and recovered WtzBR (91%) than patients whose CF was diagnosed conventionally (56%), although this difference was of borderline significance. Compared with having neither \u2191EN nor \u2191pLA, the likelihood of being a responder was greatest with combined \u2191EN and \u2191pLA, followed by \u2191EN only. The positive associations between \u2191EN and \u2191pLA to treatment responsiveness remained significant after adjustment for neonatal screening status, baseline height and weight status, and indices of pulmonary disease severity.<\/jats:p><jats:p>CONCLUSION. \u2191EN and \u2191pLA are critical in promoting adequate weight gain in children with newly diagnosed CF.<\/jats:p>","DOI":"10.1542\/peds.2004-2832","type":"journal-article","created":{"date-parts":[[2006,2,1]],"date-time":"2006-02-01T19:21:17Z","timestamp":1138821677000},"page":"391-400","source":"Crossref","is-referenced-by-count":55,"title":["Temporal Associations Among Energy Intake, Plasma Linoleic Acid, and Growth Improvement in Response to Treatment Initiation After Diagnosis of Cystic Fibrosis"],"prefix":"10.1542","volume":"117","author":[{"given":"Suzanne M.","family":"Shoff","sequence":"first","affiliation":[{"name":"Nutritional Sciences"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hong-Yup","family":"Ahn","sequence":"additional","affiliation":[{"name":"Department of Statistics, Dongguk University, Seoul, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lisa","family":"Davis","sequence":"additional","affiliation":[{"name":"General Clinical Research Center"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"HuiChuan","family":"Lai","sequence":"additional","affiliation":[{"name":"Nutritional Sciences"},{"name":"Biostatistics and Medical Informatics"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"the Wisconsin CF Neonatal Screening Group","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"417","published-online":{"date-parts":[[2006,2,1]]},"reference":[{"key":"2021120820063006500_R1","doi-asserted-by":"crossref","unstructured":"Kraemer R, Rudeberg A, Hadorn B, Rossie E. Relative underweight in cystic fibrosis and its prognostic value. Acta Paediatr Scand.\u20081978;67:33\u201337","DOI":"10.1111\/j.1651-2227.1978.tb16273.x"},{"key":"2021120820063006500_R2","unstructured":"Wood RE, Boat TF, Doershuk CF. State of the art: cystic fibrosis. Am Rev Respir Dis.\u20081976;113:833\u2013878"},{"key":"2021120820063006500_R3","doi-asserted-by":"crossref","unstructured":"Lai HC, Kosorok MR, Sondel SA, et al. Growth status in children with cystic fibrosis based on the National Cystic Fibrosis Patient Registry data: evaluation of various criteria used to identify malnutrition. J Pediatr.\u20081998;132:478\u2013485","DOI":"10.1016\/S0022-3476(98)70024-1"},{"key":"2021120820063006500_R4","unstructured":"Cystic Fibrosis Foundation. Cystic Fibrosis Foundation Patient Registry Annual Data Report 2002. Bethesda, MD: Cystic Fibrosis Foundation;2003"},{"key":"2021120820063006500_R5","doi-asserted-by":"crossref","unstructured":"Giglio L, Candusso M, D'Orazio C, Mastella G, Faraguna D. Failure to thrive: the earliest feature of cystic fibrosis in infants diagnosed by neonatal screening. Acta Paediatr.\u20081997;86:1162\u20131165","DOI":"10.1111\/j.1651-2227.1997.tb14836.x"},{"key":"2021120820063006500_R6","doi-asserted-by":"crossref","unstructured":"McNaughton SA, Stormont DA, Shepherd RW, Francis PW, Dean B. Growth failure in cystic fibrosis. J Paediatr Child Health.\u20081999;35:86\u201392","DOI":"10.1046\/j.1440-1754.1999.00329.x"},{"key":"2021120820063006500_R7","doi-asserted-by":"crossref","unstructured":"Beker LT, Russek-Cohen E, Fink RJ. Stature as a prognostic factor in cystic fibrosis survival. J Am Diet Assoc.\u20082001;101:438\u2013442","DOI":"10.1016\/S0002-8223(01)00113-4"},{"key":"2021120820063006500_R8","doi-asserted-by":"crossref","unstructured":"Sahebjami H, Doers JT, Render ML, Bond TL. Anthropometric and pulmonary function test profiles of outpatients with stable chronic obstructive pulmonary disease. Am J Med.\u20081993;94:469\u2013474","DOI":"10.1016\/0002-9343(93)90080-9"},{"key":"2021120820063006500_R9","doi-asserted-by":"crossref","unstructured":"Zemel BS, Jawad AF, FitzSimmons S, Stallings VA. Longitudinal relationship among growth, nutritional status, and pulmonary function in children with cystic fibrosis: analysis of the Cystic Fibrosis Foundation National CF Patient Registry. J Pediatr.\u20082000;137:374\u2013380","DOI":"10.1067\/mpd.2000.107891"},{"key":"2021120820063006500_R10","doi-asserted-by":"crossref","unstructured":"Shwachman H, Redmond A, Khaw KT. Studies in cystic fibrosis. Report of 130 patients diagnosed under 3 months of age over a 20-year period. Pediatrics.\u20081970;46:335\u2013343","DOI":"10.1542\/peds.46.3.335"},{"key":"2021120820063006500_R11","doi-asserted-by":"crossref","unstructured":"Konstan MW, Butler SM, Wohl ME, et al. Growth and nutritional indexes in early life predict pulmonary function in cystic fibrosis. J Pediatr.\u20082003;142:624\u2013630","DOI":"10.1067\/mpd.2003.152"},{"key":"2021120820063006500_R12","unstructured":"National Research Council. Recommended Dietary Allowances. 10th ed. Washington, DC: National Academy Press;1989"},{"key":"2021120820063006500_R13","doi-asserted-by":"crossref","unstructured":"Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr.\u20082002;35:246\u2013259","DOI":"10.1097\/00005176-200209000-00004"},{"key":"2021120820063006500_R14","doi-asserted-by":"crossref","unstructured":"Daniels L, Davidson GP, Martin AJ. Comparison of the macronutrient intake of healthy controls and children with cystic fibrosis on low fat or nonrestricted fat diets. J Pediatr Gastroenterol Nutr.\u20081987;6:381\u2013386","DOI":"10.1097\/00005176-198705000-00014"},{"key":"2021120820063006500_R15","doi-asserted-by":"crossref","unstructured":"Cannella PC, Bowser EK, Guyer LK, Borum PR. Feeding practices and nutrition recommendations for infants with cystic fibrosis. J Am Diet Assoc.\u20081993;93:297\u2013300","DOI":"10.1016\/0002-8223(93)91554-4"},{"key":"2021120820063006500_R16","doi-asserted-by":"crossref","unstructured":"Roy CC, Darling P, Weber AM. A rational approach to meeting macro- and micronutrient needs in cystic fibrosis. J Pediatr Gastroenterol Nutr.\u20081984;3(suppl 1):S154\u2013S162","DOI":"10.1097\/00005176-198400031-00023"},{"key":"2021120820063006500_R17","doi-asserted-by":"crossref","unstructured":"Bell L, Durie P, Forstner GG. What do children with cystic fibrosis eat?J Pediatr Gastroenterol Nutr.\u20081984;3(suppl 1):S137\u2013S146","DOI":"10.1097\/00005176-198400031-00021"},{"key":"2021120820063006500_R18","doi-asserted-by":"crossref","unstructured":"Marcus MS, Sondel SA, Farrell PM, et al. Nutritional status of infants with cystic fibrosis associated with early diagnosis and intervention. Am J Clin Nutr.\u20081991;54:578\u2013585","DOI":"10.1093\/ajcn\/54.3.578"},{"key":"2021120820063006500_R19","doi-asserted-by":"crossref","unstructured":"Lai HC, Kosorok MR, Laxova A, Davis LA, FitzSimmon SC, Farrell PM. Nutritional status of patients with cystic fibrosis with meconium ileus: a comparison with patients without meconium ileus and diagnosed early through neonatal screening. Pediatr.\u20082000;105:53\u201361","DOI":"10.1542\/peds.105.1.53"},{"key":"2021120820063006500_R20","doi-asserted-by":"crossref","unstructured":"Farrell PM, Kosorok MR, Rock MJ, et al. Early diagnosis of cystic fibrosis through neonatal screening prevents severe malnutrition and improves long-term growth. Wisconsin Cystic Fibrosis Neonatal Screening Study Group. Pediatrics.\u20082001;107:1\u201313","DOI":"10.1542\/peds.107.1.1"},{"key":"2021120820063006500_R21","doi-asserted-by":"crossref","unstructured":"Kawchak DA, Zhao H, Scanlin TF, Tomezsko JL, Cnaan A, Stallings VA. Longitudinal, prospective analysis of dietary intake in children with cystic fibrosis. J Pediatr.\u20081996;129:119\u2013129","DOI":"10.1016\/S0022-3476(96)70198-1"},{"key":"2021120820063006500_R22","doi-asserted-by":"crossref","unstructured":"Farrell PM, Lai HC, Li Z, et al. Evidence on improved outcomes with early diagnosis of cystic fibrosis through neonatal screening: enough is enough!J Pediatr.\u20082005;147:S30\u2013S36","DOI":"10.1016\/j.jpeds.2005.08.012"},{"key":"2021120820063006500_R23","doi-asserted-by":"crossref","unstructured":"Jelalian E, Stark LJ, Reynolds L, Seifer R. Nutrition intervention for weight gain in cystic fibrosis: a meta analysis. J Pediatr.\u20081998;132:486\u2013492","DOI":"10.1016\/S0022-3476(98)70025-3"},{"key":"2021120820063006500_R24","doi-asserted-by":"crossref","unstructured":"Mischler EH, Parrell SW, Farrell PM, Raynor WJ, Lemen RJ. Correction of linoleic acid deficiency in cystic fibrosis. Pediatr Res.\u20081986;20:36\u201341","DOI":"10.1203\/00006450-198601000-00010"},{"key":"2021120820063006500_R25","doi-asserted-by":"crossref","unstructured":"van Egmond AW, Kosorok MR, Koscik R, Laxova A, Farrell PM. Effect of linoleic acid intake on growth of infants with cystic fibrosis. Am J Clin Nutr.\u20081996;63:746\u2013752","DOI":"10.1093\/ajcn\/63.5.746"},{"key":"2021120820063006500_R26","doi-asserted-by":"crossref","unstructured":"Steinkamp G, Demmelmair G, Ruhl-Bagheri I, von der Hardt H, Koletzko B. Energy supplements rich in linoleic acid improve body weight ad essential fatty acid status of cystic fibrosis patients. J Pedatr Gastroenterol Nutr.\u20082000;31:418\u2013423","DOI":"10.1097\/00005176-200010000-00016"},{"key":"2021120820063006500_R27","unstructured":"Fost N, Farrell PM. A prospective randomized trial of early diagnosis and treatment of cystic fibrosis: a unique ethical dilemma. Clin Res.\u20081989;37:495\u2013500"},{"key":"2021120820063006500_R28","doi-asserted-by":"crossref","unstructured":"Couper RT, Corey M, Moore DJ, Fisher LJ, Forstner GG, Durie PR. Decline of exocrine pancreatic function in cystic fibrosis patients with pancreatic sufficiency. Pediatr Res.\u20081992;32:179\u2013182","DOI":"10.1203\/00006450-199208000-00011"},{"key":"2021120820063006500_R29","doi-asserted-by":"crossref","unstructured":"Lloyd-Still JD, Bibus DM, Powers CA, Johnson SB, Holman RT. Essential fatty acid deficiency and predisposition to lung disease in cystic fibrosis. Acta Paediatr.\u20081996;85:1426\u20131432","DOI":"10.1111\/j.1651-2227.1996.tb13947.x"},{"key":"2021120820063006500_R30","doi-asserted-by":"crossref","unstructured":"Farrell PM, Kosorok MR, Laxova A, et al. Nutritional benefits of neonatal screening for cystic fibrosis. N Engl J Med.\u20081997;337:963\u2013969","DOI":"10.1056\/NEJM199710023371403"},{"key":"2021120820063006500_R31","unstructured":"Kuczmarski RJ, Ogden CL, Guo SS, et al. 2000 CDC growth charts for the United States: methods and development. Vital Health Stat 11.\u20082000;iii\u2013x:1\u2013190"},{"key":"2021120820063006500_R32","doi-asserted-by":"crossref","unstructured":"Farrell PM, Mischler EH, Engle MJ, Brown DJ, Lau SM. Fatty acid abnormalities in cystic fibrosis. Pediatr Res.\u20081985;19:104\u2013109","DOI":"10.1203\/00006450-198501000-00028"},{"key":"2021120820063006500_R33","unstructured":"Pennington JAT, Church HN, eds. Bowes and Church's Food Values of Portions Commonly Used. 14th\u201317th eds. New York, NY: Harper & Row;1985\u20131998"},{"key":"2021120820063006500_R34","unstructured":"National Academy of Sciences. Dietary Reference Intakes for Energy, Carbohydrate, Fiber, Fat, Fatty Acids, Cholesterol, Protein, and Amino Acids (Macronutrients). Washington, DC: National Academies Press;2002"},{"key":"2021120820063006500_R35","doi-asserted-by":"crossref","unstructured":"Greco L, Santamaria F, Salvatore D, de Ritis G. Growth dynamics in cystic fibrosis. Acta Paediatr.\u20081993;82:254\u2013260","DOI":"10.1111\/j.1651-2227.1993.tb12654.x"},{"key":"2021120820063006500_R36","doi-asserted-by":"crossref","unstructured":"Pederzini F, D'Orazio C, Tamiazzo G, Faraguna D, Giglio L, Mastella G. Growth evaluation at one year of life in infants with cystic fibrosis diagnosed by neonatal screening. Pediatr Pulmonol.\u20081991;7(suppl):64\u201368","DOI":"10.1002\/ppul.1950110713"},{"key":"2021120820063006500_R37","doi-asserted-by":"crossref","unstructured":"Bronstein MN, Sokol RJ, Abman SH, et al. Pancreatic insufficiency, growth, and nutrition in infants identified by newborn screening as having cystic fibrosis. J Pediatr.\u20081992;120:533\u2013540","DOI":"10.1016\/S0022-3476(05)82478-3"},{"key":"2021120820063006500_R38","doi-asserted-by":"crossref","unstructured":"Ihaka R, Gentleman RR: A language for data analysis and graphics. J Computat Graphic Stat.\u20081996;5:299\u2013314","DOI":"10.1080\/10618600.1996.10474713"},{"key":"2021120820063006500_R39","doi-asserted-by":"crossref","unstructured":"Kosorok MR, Jalaluddin M, Farrell PM, et al. Comprehensive analysis of risk factors for acquisition of Pseudomonas aeruginosa in young children with cystic fibrosis. Pediatr Pulmonol.\u20081998;26:81\u201388","DOI":"10.1002\/(SICI)1099-0496(199808)26:2<81::AID-PPUL2>3.0.CO;2-K"},{"key":"2021120820063006500_R40","doi-asserted-by":"crossref","unstructured":"Lai HC, Kosorok MR, Laxova A, Makholm LM, Farrell PM. Delayed diagnosis of US females with cystic fibrosis. Am J Epidemiol.\u20082002;156:165\u2013173","DOI":"10.1093\/aje\/kwf014"},{"key":"2021120820063006500_R41","doi-asserted-by":"crossref","unstructured":"Farrell PM, Li Z, Kosorok MR, et al. Bronchopulmonary disease in children with cystic fibrosis after early or delayed diagnosis. Am J Respir Crit Care Med.\u20082003;168:1100\u20131108","DOI":"10.1164\/rccm.200303-434OC"},{"key":"2021120820063006500_R42","doi-asserted-by":"crossref","unstructured":"Stark LJ, Mulvihill MM, Jelalian E, et al. Descriptive analysis of eating behavior in school-age children with cystic fibrosis and healthy control children. Pediatrics.\u20081997;99:665\u2013671","DOI":"10.1542\/peds.99.5.665"},{"key":"2021120820063006500_R43","doi-asserted-by":"crossref","unstructured":"Tomezsko JL, Stallings VA, Scanlin TF. Dietary intake of healthy children with cystic fibrosis compared with normal control children. Pediatrics.\u20081992;90:547\u2013553","DOI":"10.1542\/peds.90.4.547"}],"container-title":["Pediatrics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/117\/2\/391\/1012385\/zpe00206000391.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/publications.aap.org\/pediatrics\/article-pdf\/117\/2\/391\/1012385\/zpe00206000391.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,6]],"date-time":"2023-05-06T10:43:51Z","timestamp":1683369831000},"score":1,"resource":{"primary":{"URL":"https:\/\/publications.aap.org\/pediatrics\/article\/117\/2\/391\/68466\/Temporal-Associations-Among-Energy-Intake-Plasma"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,2,1]]},"references-count":43,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2006,2,1]]},"published-print":{"date-parts":[[2006,2,1]]}},"URL":"https:\/\/doi.org\/10.1542\/peds.2004-2832","relation":{},"ISSN":["0031-4005","1098-4275"],"issn-type":[{"value":"0031-4005","type":"print"},{"value":"1098-4275","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,2,1]]}}}