Effects of diagnosis by newborn screening for cystic fibrosis on weight and length in the first year of life

Baby Observational and Nutrition Study (BONUS) Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network

Research output: Contribution to journalArticle

20 Citations (Scopus)

Abstract

IMPORTANCE: Since the implementation of universal newborn screening (NBS) for cystic fibrosis (CF), the timing and magnitude of growth deficiency or its association with correlates of disease among infants with CF who underwent NBS has not been well described. OBJECTIVE: To examine incremental weight gain, linear growth, and clinical features in the first year of life among infants with CF who underwent NBS. DESIGN, SETTING, AND PARTICIPANTS: The Baby Observational and Nutrition Study (BONUS), a multicenter, longitudinal, observational cohort study, was conducted during regular CF clinic visits in the first 12 months of life at 28 US Cystic Fibrosis Foundation-accredited Care Centers from January 7, 2012, through May 31, 2015. Participants included 231 infants younger than 3.5 months who underwent NBS and had confirmed CF, with a gestational age of at least 35 weeks, birth weight of at least 2.5 kg, and toleration of full oral feeds. Of these, 222 infants (96.1%) had follow-up beyond 6 months of age and 215 (93.1%) completed 12 months of follow-up. EXPOSURE: Cystic fibrosis. MAIN OUTCOME AND MEASURES: Attained weight and length for age and World Health Organization normative z scores at ages 1 to 6 and 8, 10, and 12 months (defined a priori). RESULTS: Of the 231 infants enrolled, 110 infants (47.6%) were female and 121 (52.4%) were male, with a mean (SD) age of 2.58 (0.69) months. BONUS infants had lower than mean birth weights (mean z score, -0.15; 95%CI, -0.27 to -0.04) and higher birth lengths (mean z score, 0.44; 95%CI, 0.26 to 0.62). They achieved normal weight by 12 months, a significant improvement over a prescreening cohort of newborns with CF from 20 years before the contemporary cohort (mean z score increase, 0.57; 95%CI, 0.37-0.77). However, length was lower than the mean at 12 months (mean z score, -0.56; 95%CI, -0.70 to -0.42). Only 30 infants (13.6%) were at less than the 10th percentile of weight for age, whereas 53 (23.9%) were at less than the 10th percentile of length for age at more than half their visits. Male sex, pancreatic insufficiency, meconium ileus, histamine blocker use, and respiratory Pseudomonas aeruginosa infection were associated with lower weight or length during the first year. Insulinlike growth factor 1 levels were significantly lower among low-length infants. Persistently low-weight infants consumed significantly more calories, and weight and length z scores were negatively correlated with caloric intake. CONCLUSIONS AND RELEVANCE: Since initiation of universal NBS for CF, significant improvement has occurred in nutritional status, with normalization of weight in the first year of life. However, length stunting remains common.

Original languageEnglish (US)
Pages (from-to)546-554
Number of pages9
JournalJAMA Pediatrics
Volume171
Issue number6
DOIs
StatePublished - Jun 1 2017

Fingerprint

Cystic Fibrosis
Newborn Infant
Weights and Measures
Observational Studies
Birth Weight
Growth Disorders
Exocrine Pancreatic Insufficiency
Pseudomonas Infections
Meconium
Ileus
Ambulatory Care
Growth
Energy Intake
Nutritional Status
Pseudomonas aeruginosa
Histamine
Gestational Age
Weight Gain
Intercellular Signaling Peptides and Proteins
Cohort Studies

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

Baby Observational and Nutrition Study (BONUS) Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network (2017). Effects of diagnosis by newborn screening for cystic fibrosis on weight and length in the first year of life. JAMA Pediatrics, 171(6), 546-554. https://doi.org/10.1001/jamapediatrics.2017.0206

Effects of diagnosis by newborn screening for cystic fibrosis on weight and length in the first year of life. / Baby Observational and Nutrition Study (BONUS) Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network.

In: JAMA Pediatrics, Vol. 171, No. 6, 01.06.2017, p. 546-554.

Research output: Contribution to journalArticle

Baby Observational and Nutrition Study (BONUS) Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network 2017, 'Effects of diagnosis by newborn screening for cystic fibrosis on weight and length in the first year of life', JAMA Pediatrics, vol. 171, no. 6, pp. 546-554. https://doi.org/10.1001/jamapediatrics.2017.0206
Baby Observational and Nutrition Study (BONUS) Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network. Effects of diagnosis by newborn screening for cystic fibrosis on weight and length in the first year of life. JAMA Pediatrics. 2017 Jun 1;171(6):546-554. https://doi.org/10.1001/jamapediatrics.2017.0206
Baby Observational and Nutrition Study (BONUS) Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network. / Effects of diagnosis by newborn screening for cystic fibrosis on weight and length in the first year of life. In: JAMA Pediatrics. 2017 ; Vol. 171, No. 6. pp. 546-554.
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abstract = "IMPORTANCE: Since the implementation of universal newborn screening (NBS) for cystic fibrosis (CF), the timing and magnitude of growth deficiency or its association with correlates of disease among infants with CF who underwent NBS has not been well described. OBJECTIVE: To examine incremental weight gain, linear growth, and clinical features in the first year of life among infants with CF who underwent NBS. DESIGN, SETTING, AND PARTICIPANTS: The Baby Observational and Nutrition Study (BONUS), a multicenter, longitudinal, observational cohort study, was conducted during regular CF clinic visits in the first 12 months of life at 28 US Cystic Fibrosis Foundation-accredited Care Centers from January 7, 2012, through May 31, 2015. Participants included 231 infants younger than 3.5 months who underwent NBS and had confirmed CF, with a gestational age of at least 35 weeks, birth weight of at least 2.5 kg, and toleration of full oral feeds. Of these, 222 infants (96.1{\%}) had follow-up beyond 6 months of age and 215 (93.1{\%}) completed 12 months of follow-up. EXPOSURE: Cystic fibrosis. MAIN OUTCOME AND MEASURES: Attained weight and length for age and World Health Organization normative z scores at ages 1 to 6 and 8, 10, and 12 months (defined a priori). RESULTS: Of the 231 infants enrolled, 110 infants (47.6{\%}) were female and 121 (52.4{\%}) were male, with a mean (SD) age of 2.58 (0.69) months. BONUS infants had lower than mean birth weights (mean z score, -0.15; 95{\%}CI, -0.27 to -0.04) and higher birth lengths (mean z score, 0.44; 95{\%}CI, 0.26 to 0.62). They achieved normal weight by 12 months, a significant improvement over a prescreening cohort of newborns with CF from 20 years before the contemporary cohort (mean z score increase, 0.57; 95{\%}CI, 0.37-0.77). However, length was lower than the mean at 12 months (mean z score, -0.56; 95{\%}CI, -0.70 to -0.42). Only 30 infants (13.6{\%}) were at less than the 10th percentile of weight for age, whereas 53 (23.9{\%}) were at less than the 10th percentile of length for age at more than half their visits. Male sex, pancreatic insufficiency, meconium ileus, histamine blocker use, and respiratory Pseudomonas aeruginosa infection were associated with lower weight or length during the first year. Insulinlike growth factor 1 levels were significantly lower among low-length infants. Persistently low-weight infants consumed significantly more calories, and weight and length z scores were negatively correlated with caloric intake. CONCLUSIONS AND RELEVANCE: Since initiation of universal NBS for CF, significant improvement has occurred in nutritional status, with normalization of weight in the first year of life. However, length stunting remains common.",
author = "{Baby Observational and Nutrition Study (BONUS) Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network} and Leung, {Daniel H.} and Heltshe, {Sonya L.} and Drucy Borowitz and Daniel Gelfond and Margaret Kloster and Heubi, {James E.} and Michael Stalvey and Ramsey, {Bonnie W.} and A. Stecenko and M. Schechter and E. Hunter and W. Hoover and H. Hathorne and K. Brand and A. Filbrun and M. Linn and D. Borowitz and N. Caci and B. McWilliams and I. Brazil and J. Heubi and M. Bushman and S. McColley and A. Bowen and K. McCoy and K. Sakellaris and P. Sharma and C. Cannon and A. Hebert and S. Sagel and M. Anthony and M. Dyson and H. Urbanek and S. Millard and C. Gile and G. Graff and L. Allwein and P. Hiatt and N. Schaap and N. Krupp and L. Bendy and R. Ahrens and M. Teresi and A. Berlinski and Ramsey, {L. L.} and J. McNamara and M. Johnson and R. Brown and P. Berry and Powers, {Michael (Mike)}",
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T1 - Effects of diagnosis by newborn screening for cystic fibrosis on weight and length in the first year of life

AU - Baby Observational and Nutrition Study (BONUS) Investigators of the Cystic Fibrosis Foundation Therapeutics Development Network

AU - Leung, Daniel H.

AU - Heltshe, Sonya L.

AU - Borowitz, Drucy

AU - Gelfond, Daniel

AU - Kloster, Margaret

AU - Heubi, James E.

AU - Stalvey, Michael

AU - Ramsey, Bonnie W.

AU - Stecenko, A.

AU - Schechter, M.

AU - Hunter, E.

AU - Hoover, W.

AU - Hathorne, H.

AU - Brand, K.

AU - Filbrun, A.

AU - Linn, M.

AU - Borowitz, D.

AU - Caci, N.

AU - McWilliams, B.

AU - Brazil, I.

AU - Heubi, J.

AU - Bushman, M.

AU - McColley, S.

AU - Bowen, A.

AU - McCoy, K.

AU - Sakellaris, K.

AU - Sharma, P.

AU - Cannon, C.

AU - Hebert, A.

AU - Sagel, S.

AU - Anthony, M.

AU - Dyson, M.

AU - Urbanek, H.

AU - Millard, S.

AU - Gile, C.

AU - Graff, G.

AU - Allwein, L.

AU - Hiatt, P.

AU - Schaap, N.

AU - Krupp, N.

AU - Bendy, L.

AU - Ahrens, R.

AU - Teresi, M.

AU - Berlinski, A.

AU - Ramsey, L. L.

AU - McNamara, J.

AU - Johnson, M.

AU - Brown, R.

AU - Berry, P.

AU - Powers, Michael (Mike)

PY - 2017/6/1

Y1 - 2017/6/1

N2 - IMPORTANCE: Since the implementation of universal newborn screening (NBS) for cystic fibrosis (CF), the timing and magnitude of growth deficiency or its association with correlates of disease among infants with CF who underwent NBS has not been well described. OBJECTIVE: To examine incremental weight gain, linear growth, and clinical features in the first year of life among infants with CF who underwent NBS. DESIGN, SETTING, AND PARTICIPANTS: The Baby Observational and Nutrition Study (BONUS), a multicenter, longitudinal, observational cohort study, was conducted during regular CF clinic visits in the first 12 months of life at 28 US Cystic Fibrosis Foundation-accredited Care Centers from January 7, 2012, through May 31, 2015. Participants included 231 infants younger than 3.5 months who underwent NBS and had confirmed CF, with a gestational age of at least 35 weeks, birth weight of at least 2.5 kg, and toleration of full oral feeds. Of these, 222 infants (96.1%) had follow-up beyond 6 months of age and 215 (93.1%) completed 12 months of follow-up. EXPOSURE: Cystic fibrosis. MAIN OUTCOME AND MEASURES: Attained weight and length for age and World Health Organization normative z scores at ages 1 to 6 and 8, 10, and 12 months (defined a priori). RESULTS: Of the 231 infants enrolled, 110 infants (47.6%) were female and 121 (52.4%) were male, with a mean (SD) age of 2.58 (0.69) months. BONUS infants had lower than mean birth weights (mean z score, -0.15; 95%CI, -0.27 to -0.04) and higher birth lengths (mean z score, 0.44; 95%CI, 0.26 to 0.62). They achieved normal weight by 12 months, a significant improvement over a prescreening cohort of newborns with CF from 20 years before the contemporary cohort (mean z score increase, 0.57; 95%CI, 0.37-0.77). However, length was lower than the mean at 12 months (mean z score, -0.56; 95%CI, -0.70 to -0.42). Only 30 infants (13.6%) were at less than the 10th percentile of weight for age, whereas 53 (23.9%) were at less than the 10th percentile of length for age at more than half their visits. Male sex, pancreatic insufficiency, meconium ileus, histamine blocker use, and respiratory Pseudomonas aeruginosa infection were associated with lower weight or length during the first year. Insulinlike growth factor 1 levels were significantly lower among low-length infants. Persistently low-weight infants consumed significantly more calories, and weight and length z scores were negatively correlated with caloric intake. CONCLUSIONS AND RELEVANCE: Since initiation of universal NBS for CF, significant improvement has occurred in nutritional status, with normalization of weight in the first year of life. However, length stunting remains common.

AB - IMPORTANCE: Since the implementation of universal newborn screening (NBS) for cystic fibrosis (CF), the timing and magnitude of growth deficiency or its association with correlates of disease among infants with CF who underwent NBS has not been well described. OBJECTIVE: To examine incremental weight gain, linear growth, and clinical features in the first year of life among infants with CF who underwent NBS. DESIGN, SETTING, AND PARTICIPANTS: The Baby Observational and Nutrition Study (BONUS), a multicenter, longitudinal, observational cohort study, was conducted during regular CF clinic visits in the first 12 months of life at 28 US Cystic Fibrosis Foundation-accredited Care Centers from January 7, 2012, through May 31, 2015. Participants included 231 infants younger than 3.5 months who underwent NBS and had confirmed CF, with a gestational age of at least 35 weeks, birth weight of at least 2.5 kg, and toleration of full oral feeds. Of these, 222 infants (96.1%) had follow-up beyond 6 months of age and 215 (93.1%) completed 12 months of follow-up. EXPOSURE: Cystic fibrosis. MAIN OUTCOME AND MEASURES: Attained weight and length for age and World Health Organization normative z scores at ages 1 to 6 and 8, 10, and 12 months (defined a priori). RESULTS: Of the 231 infants enrolled, 110 infants (47.6%) were female and 121 (52.4%) were male, with a mean (SD) age of 2.58 (0.69) months. BONUS infants had lower than mean birth weights (mean z score, -0.15; 95%CI, -0.27 to -0.04) and higher birth lengths (mean z score, 0.44; 95%CI, 0.26 to 0.62). They achieved normal weight by 12 months, a significant improvement over a prescreening cohort of newborns with CF from 20 years before the contemporary cohort (mean z score increase, 0.57; 95%CI, 0.37-0.77). However, length was lower than the mean at 12 months (mean z score, -0.56; 95%CI, -0.70 to -0.42). Only 30 infants (13.6%) were at less than the 10th percentile of weight for age, whereas 53 (23.9%) were at less than the 10th percentile of length for age at more than half their visits. Male sex, pancreatic insufficiency, meconium ileus, histamine blocker use, and respiratory Pseudomonas aeruginosa infection were associated with lower weight or length during the first year. Insulinlike growth factor 1 levels were significantly lower among low-length infants. Persistently low-weight infants consumed significantly more calories, and weight and length z scores were negatively correlated with caloric intake. CONCLUSIONS AND RELEVANCE: Since initiation of universal NBS for CF, significant improvement has occurred in nutritional status, with normalization of weight in the first year of life. However, length stunting remains common.

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