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320 Changes in anthropometrical parameters in infants with CF diagnosed through newborn screening (NBS)
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Poster Sessions / Journal of Cystic Fibrosis 16S1 (2017) S63–S174
Table 1 Anthropometry, BC and LF outcomes (n = 20) Z-scores Mean (SD)
Weight −0.15 (0.57)
Height −0.07 (0.63)
BMI −0.18 (0.74)
FMI
FFMI
FEV1
−0.49* (0.78)
−0.47** (0.69)
−0.16 (1.05)
* P 0.012, **P 0.007. Table 2 Correlations between variables Z-scores R value P value (95% CI)
BMI and FEV1
FMI and FEV1
FFMI and FEV1
0.32 0.17 (−0.23 to 0.73)
0.03 0.91 (−0.50 to 0.69)
0.40 0.08 (−0.01 to 0.68)
Conclusion: These preliminary findings in a contemporary cohort support the assessment of BC in children with CF, in order to identify changes early. Larger numbers are planned to achieve more definitive results. Future interventions should consider optimising FFM as well as BMI.
Reference [1] Williams JE et al. Am J Clin Nutr. 2010; 92: 1332–1343. 319 Nutritional intervention at the cystic fibrosis reference center of children and adults in Uruguay G. Parallada1, V. Sereno1, G. Caballero1, M.L. Fernández1, M. Castro1, D. Montero1, S. Altez1, A. Rey1, Equipo Pediátrico de Fibrosis Quística y Equipo de Adultos de Fibrosis Quística del CRENADECER. 1Centro de Referencia Nacional de Defectos Congénitos y Enfermedades Raras, Montevideo, Uruguay Introduction: CF in Uruguay incidence is 1/9000. Since 2010 neonatal screening is mandatory and child and adult multidisciplinary teams have more than 20 years working in the same Statal Center. This is National Refence since 2014 (CRENADECER). Objectives: Analyze nutritional status of CRENADECER CF Patients (CCFP). Method: Descriptive, longitudinal, restrospective study of CCFP, classified into 3 group: 1: NBS (newborn screening), 2- no NBS (no newborn screening), 3- TA (teenagers and adults). Nutritional status (NS) is evaluated in each group according WHO directives. Group 3 was evaluated by BMI and body composition, lm (lean mass) and fm (fat mass). Nutritional intervention: oral support (OS), enteral nutrition trough nasogastric NG or gastrostomy (PEG). Results: n = 147, 35 NBS, (range 1–13, mean age 1 month), 44 noNBS (range 5 m – 13 years mean age: 5), 68 TA (range 16–73 mean age: 33). % of PI was 80, 68 and 46 for each group respectively. Malnutrition in NBS was 38% and 49% growth delayed, and 0% malnutrition and 3% delayed growth after nutritional intervention; OS 86% and NG 20%. In noNBS 18% of malnutrition or risk and 66% of growth delayed; after 61% of OS and 9% of PEG, resulted in 0% of malnutrition and 9% of growth delayed. For group 3 at the beginning of the evaluation 30% have any tipe of malnutrition. Nutritional intervention: 4% PEG, 63% OS ( patient in nutritional risk and in normonutrition but with PI or FEV1 very low or severe disease). Now, 10% of TA presents malnutrition. Body composition, lm was normal and high in 68% and low in 13% (these are the ones with severe disease). Conclusion: Team work, timely nutritional intervention, early and adequate, guarantee reaching the nutritional goals, thoughout the incorparation of specialized nutritional outlines disapearing malnutrition NBS. In older children 75% NS is normal. In TA in fact, thoughout specialized nutritional support weight and lean body mass enhance. 320 Changes in anthropometrical parameters in infants with CF diagnosed through newborn screening (NBS) A. Jokic Miletic1, G. Vilotijevic-Dautovic1, S. Cegar1, S. Petrovic1, S. Ilic1, D. Tenjovic1. 1Institute for Children’s and Youth Healthcare of Vojvodina, Pulmonology, Novi Sad, Serbia
Objective and method: Neonatal screening for cystic fibrosis started in September 2009 at the Institute for Children’s and Youth Healthcare of Vojvodina as a part of the regional screening program. Of the 85,474 newborns who went through screening before December 2016, 16 were diagnosed with CF, of whom 15 are regularly followed up. A review was conducted to see if early diagnosis improved nutritional status measured by anthropometrical parameters (weight, height, BMI). Results: Growth was normal at birth (3,110 g/50,46 cm median), but declined at the time when diagnosed with CF (median age 45 days, median length 55 cm/7.9th %tile, weight 4.14 kg/6.2th %tile, N = 15). One year later, catch-up growth was observed—median length was 80 cm/64.25th %tile/ 0,4 z-score, weight 10.26 kg/56.5th %tile/0.1 z score, BMI 16.16 kg/m2 (N = 12). Two years after diagnosis established, median height was 89.5 cm/ 61.05th %tile/0,25 z-score, weight 12.5 kg/59,6th %tile/0.86 z-score, BMI 16.05 kg/m2/46.8th %tile/0.1 z-score (N = 12). After three years, median height was 101 cm/86.9th %tile/1.1z-score, weight 14.4 kg/60.6th %tile/0,27 z-score, BMI 14.9 kg/m2/38.2th %tile/−0.3 z/score. Four years after, median length observed was 109.5 cm/88.85th %tile/1.25 z-score, weight 20.35 kg/ 84.25th %tile/1.015 z-score, BMI 17 kg/m2/74.25th %tile/0.65 z-score (N = 4). Five years after diagnosis median length was 118 cm/84th %tile/ 1.2 z-score, weight 22.1 kg/81.59th %tile/0.9 z-score (N = 5), and six years after (2016) median length was 122 cm/90.3th %tile/1.3 z-score, weight 26 kg/92,92th %tile/1.4 z/score/BMI 17.5 kg/m2/88.3th %tile/1.2 z-score (N = 3). In one patient, who underwent ileostomy for meconium ileus, the catch-up growth was delayed. Conclusion: Early diagnosis and care lead to better nutritional status and catch-up growth in children with CF. 321 Paternity’s impact on CF: a retrospective study C. Lehoux-Dubois1,2, M. Mailhot3, A. Coriati1,2, R. Rabasa-Lhoret1,3,4, Y. Berthiaume1,3,4, M. Silviet-Carricart3,4, A. Lavoie3,4, F. Tremblay3,4. 1 Institut de Recherches Cliniques de Montréal, Montréal, Canada; 2Université de Montréal, Nutrition, Montréal, Canada; 3Centre hospitalier de l’Université de Montréal (CHUM), CF Adult Clinic, Montréal, Canada; 4Université de Montréal, Médecine, Montréal, Canada Objectives: While maternity in CF is extensively studied, few reports have addressed paternity and its impact on the disease. Hypothesis: We hypothesized that paternity has a negative impact on lung function and body weight. Methods: We conducted a retrospective analysis of a cohort of CF patients who became fathers between 2002 and 2015. Variation in FEV1% and in BMI were analysed before, at birth and one year following birth of the first child. The data were collected from the electronic medical records of the Centre Hospitalier de l’Université de Montréal (CHUM). Study population: 25 fathers who benefited from in vitro fertilization program were included while 5 fathers with adopted children or unknown child’s birth date in their medical records were excluded. Median age of fathers at time of birth was 31 years: 40% had cystic fibrosis related diabetes, 40% with normal glucose tolerance (20% indeterminate glucose tolerance INDET), and 20% with impaired glucose tolerance. Although five patients INDET had a significant drop in their FEV1% (between −6 and −11%) 1 year after their first child’s birth, overall we did not observed a significant change neither in FEV1% nor BMI 1 year after the birth of the first child. Contrary to our hypothesis, we didn’t observe significant variation in BMI and FEV1% in new CF fathers. Results: See the Table. Table: Caracteristics of CF fathers n = 25] 1 year before FEV1% 2 BMI (kg/m )
76,5 ± 22,0 23,3 ± 3,1
At birth 76,1 ± 23,0 23,7 ± 3,0
1 year after 76,5 ± 24,0 24,0 ± 3,0
Conclusion: Although paternity does not seem to have an impact on CF disease evolution as measured by FEV1% and BMI in this study, it might be interesting to perform a quality of life assessment in order to evaluate the CF fathers ability to adapt to their new family role.
Poster Sessions / Journal of Cystic Fibrosis 16S1 (2017) S63–S174
Table 1 Anthropometry, BC and LF outcomes (n = 20) Z-scores Mean (SD)
Weight −0.15 (0.57)
Height −0.07 (0.63)
BMI −0.18 (0.74)
FMI
FFMI
FEV1
−0.49* (0.78)
−0.47** (0.69)
−0.16 (1.05)
* P 0.012, **P 0.007. Table 2 Correlations between variables Z-scores R value P value (95% CI)
BMI and FEV1
FMI and FEV1
FFMI and FEV1
0.32 0.17 (−0.23 to 0.73)
0.03 0.91 (−0.50 to 0.69)
0.40 0.08 (−0.01 to 0.68)
Conclusion: These preliminary findings in a contemporary cohort support the assessment of BC in children with CF, in order to identify changes early. Larger numbers are planned to achieve more definitive results. Future interventions should consider optimising FFM as well as BMI.
Reference [1] Williams JE et al. Am J Clin Nutr. 2010; 92: 1332–1343. 319 Nutritional intervention at the cystic fibrosis reference center of children and adults in Uruguay G. Parallada1, V. Sereno1, G. Caballero1, M.L. Fernández1, M. Castro1, D. Montero1, S. Altez1, A. Rey1, Equipo Pediátrico de Fibrosis Quística y Equipo de Adultos de Fibrosis Quística del CRENADECER. 1Centro de Referencia Nacional de Defectos Congénitos y Enfermedades Raras, Montevideo, Uruguay Introduction: CF in Uruguay incidence is 1/9000. Since 2010 neonatal screening is mandatory and child and adult multidisciplinary teams have more than 20 years working in the same Statal Center. This is National Refence since 2014 (CRENADECER). Objectives: Analyze nutritional status of CRENADECER CF Patients (CCFP). Method: Descriptive, longitudinal, restrospective study of CCFP, classified into 3 group: 1: NBS (newborn screening), 2- no NBS (no newborn screening), 3- TA (teenagers and adults). Nutritional status (NS) is evaluated in each group according WHO directives. Group 3 was evaluated by BMI and body composition, lm (lean mass) and fm (fat mass). Nutritional intervention: oral support (OS), enteral nutrition trough nasogastric NG or gastrostomy (PEG). Results: n = 147, 35 NBS, (range 1–13, mean age 1 month), 44 noNBS (range 5 m – 13 years mean age: 5), 68 TA (range 16–73 mean age: 33). % of PI was 80, 68 and 46 for each group respectively. Malnutrition in NBS was 38% and 49% growth delayed, and 0% malnutrition and 3% delayed growth after nutritional intervention; OS 86% and NG 20%. In noNBS 18% of malnutrition or risk and 66% of growth delayed; after 61% of OS and 9% of PEG, resulted in 0% of malnutrition and 9% of growth delayed. For group 3 at the beginning of the evaluation 30% have any tipe of malnutrition. Nutritional intervention: 4% PEG, 63% OS ( patient in nutritional risk and in normonutrition but with PI or FEV1 very low or severe disease). Now, 10% of TA presents malnutrition. Body composition, lm was normal and high in 68% and low in 13% (these are the ones with severe disease). Conclusion: Team work, timely nutritional intervention, early and adequate, guarantee reaching the nutritional goals, thoughout the incorparation of specialized nutritional outlines disapearing malnutrition NBS. In older children 75% NS is normal. In TA in fact, thoughout specialized nutritional support weight and lean body mass enhance. 320 Changes in anthropometrical parameters in infants with CF diagnosed through newborn screening (NBS) A. Jokic Miletic1, G. Vilotijevic-Dautovic1, S. Cegar1, S. Petrovic1, S. Ilic1, D. Tenjovic1. 1Institute for Children’s and Youth Healthcare of Vojvodina, Pulmonology, Novi Sad, Serbia
Objective and method: Neonatal screening for cystic fibrosis started in September 2009 at the Institute for Children’s and Youth Healthcare of Vojvodina as a part of the regional screening program. Of the 85,474 newborns who went through screening before December 2016, 16 were diagnosed with CF, of whom 15 are regularly followed up. A review was conducted to see if early diagnosis improved nutritional status measured by anthropometrical parameters (weight, height, BMI). Results: Growth was normal at birth (3,110 g/50,46 cm median), but declined at the time when diagnosed with CF (median age 45 days, median length 55 cm/7.9th %tile, weight 4.14 kg/6.2th %tile, N = 15). One year later, catch-up growth was observed—median length was 80 cm/64.25th %tile/ 0,4 z-score, weight 10.26 kg/56.5th %tile/0.1 z score, BMI 16.16 kg/m2 (N = 12). Two years after diagnosis established, median height was 89.5 cm/ 61.05th %tile/0,25 z-score, weight 12.5 kg/59,6th %tile/0.86 z-score, BMI 16.05 kg/m2/46.8th %tile/0.1 z-score (N = 12). After three years, median height was 101 cm/86.9th %tile/1.1z-score, weight 14.4 kg/60.6th %tile/0,27 z-score, BMI 14.9 kg/m2/38.2th %tile/−0.3 z/score. Four years after, median length observed was 109.5 cm/88.85th %tile/1.25 z-score, weight 20.35 kg/ 84.25th %tile/1.015 z-score, BMI 17 kg/m2/74.25th %tile/0.65 z-score (N = 4). Five years after diagnosis median length was 118 cm/84th %tile/ 1.2 z-score, weight 22.1 kg/81.59th %tile/0.9 z-score (N = 5), and six years after (2016) median length was 122 cm/90.3th %tile/1.3 z-score, weight 26 kg/92,92th %tile/1.4 z/score/BMI 17.5 kg/m2/88.3th %tile/1.2 z-score (N = 3). In one patient, who underwent ileostomy for meconium ileus, the catch-up growth was delayed. Conclusion: Early diagnosis and care lead to better nutritional status and catch-up growth in children with CF. 321 Paternity’s impact on CF: a retrospective study C. Lehoux-Dubois1,2, M. Mailhot3, A. Coriati1,2, R. Rabasa-Lhoret1,3,4, Y. Berthiaume1,3,4, M. Silviet-Carricart3,4, A. Lavoie3,4, F. Tremblay3,4. 1 Institut de Recherches Cliniques de Montréal, Montréal, Canada; 2Université de Montréal, Nutrition, Montréal, Canada; 3Centre hospitalier de l’Université de Montréal (CHUM), CF Adult Clinic, Montréal, Canada; 4Université de Montréal, Médecine, Montréal, Canada Objectives: While maternity in CF is extensively studied, few reports have addressed paternity and its impact on the disease. Hypothesis: We hypothesized that paternity has a negative impact on lung function and body weight. Methods: We conducted a retrospective analysis of a cohort of CF patients who became fathers between 2002 and 2015. Variation in FEV1% and in BMI were analysed before, at birth and one year following birth of the first child. The data were collected from the electronic medical records of the Centre Hospitalier de l’Université de Montréal (CHUM). Study population: 25 fathers who benefited from in vitro fertilization program were included while 5 fathers with adopted children or unknown child’s birth date in their medical records were excluded. Median age of fathers at time of birth was 31 years: 40% had cystic fibrosis related diabetes, 40% with normal glucose tolerance (20% indeterminate glucose tolerance INDET), and 20% with impaired glucose tolerance. Although five patients INDET had a significant drop in their FEV1% (between −6 and −11%) 1 year after their first child’s birth, overall we did not observed a significant change neither in FEV1% nor BMI 1 year after the birth of the first child. Contrary to our hypothesis, we didn’t observe significant variation in BMI and FEV1% in new CF fathers. Results: See the Table. Table: Caracteristics of CF fathers n = 25] 1 year before FEV1% 2 BMI (kg/m )
76,5 ± 22,0 23,3 ± 3,1
At birth 76,1 ± 23,0 23,7 ± 3,0
1 year after 76,5 ± 24,0 24,0 ± 3,0
Conclusion: Although paternity does not seem to have an impact on CF disease evolution as measured by FEV1% and BMI in this study, it might be interesting to perform a quality of life assessment in order to evaluate the CF fathers ability to adapt to their new family role.