Factors impacting the growth and nutritional status of cystic fibrosis
               patients younger than 10 years of age who did not undergo neonatal
               screening

Hortencio, Taís Daiene Russo; Nogueira, Roberto José Negrão; Marson, Fernando Augusto de Lima; Hessel, Gabriel; Ribeiro, José Dirceu; Ribeiro, Antônio Fernando

doi:10.1016/j.rpped.2014.11.004

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Revista Paulista de Pediatria

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Original Articles • Rev. paul. pediatr. 33 (1) • Jan-Mar 2015 • https://doi.org/10.1016/j.rpped.2014.11.004 copy

Factors impacting the growth and nutritional status of cystic fibrosis patients younger than 10 years of age who did not undergo neonatal screening

Authorship SCIMAGO INSTITUTIONS RANKINGS

OBJECTIVE:

The aim of this study was to evaluate by clinical and laboratory parameters how cystic fibrosis (CF) affects growth and nutritional status of children who were undergoing CF treatment but did not receive newborn screening.

METHODS:

A historical cohort study of 52 CF patients younger than 10 years of age were followed in a reference center in Campinas, Southeast Brazil. Anthropometric measurements were abstracted from medical records until March/2010, when neonatal screening program was implemented. Between September/2009 and March/2010, parental height of the 52 CF patients were also measured.

RESULTS:

Regarding nutritional status, four patients had Z-scores ≤-2 for height/age (H/A) and body mass index/age (BMI/A). The following variables were associated with improved H/A ratio: fewer hospitalizations, longer time from first appointment to diagnosis, longer time from birth to diagnosis and later onset of respiratory disease. Forced vital capacity [FVC(%)], forced expiratory flow between 25-75% of FVC [FEF_25-75(%)], forced expiratory volume in the first second [FEV₁(%)], gestational age, birth weight and early respiratory symptoms were associated with improved BMI/A.

CONCLUSIONS:

Greater number of hospitalizations, diagnosis delay and early onset of respiratory disease had a negative impact on growth. Lower spirometric values, lower gestational age, lower birth weight, and early onset of respiratory symptoms had negative impact on nutritional status. Malnutrition was observed in 7.7% of cases, but 23% of children had nutritional risk.

Cystic fibrosis; Child; Neonatal screening; Anthropometry; Nutritional status

H/A Z-score	Number of patients	%	nM/F	%M/F
<–3 Z-Score	2	3.8	1/1	3.7/4.0
≥–3 Z-Score and <–2 Z-core	2	3.8	1/1	3.7/4.0
≥–2 Z-Score and <–1 Z-Score	16	30.8	9/7	33.3/28.0
≥–1 Z-Score and ≤+1 Z-Score	26	50.0.	12/14	44.4/56.0
>+1 Z-Score and ≤+2 Z-Score	5	9.6	4/1	14.8/4.0
>+2 Z-Score and ≤+3 Z-Score	1	1.9	0/1	0/4.0
>+3 Z-Score	0	0.	0/0	0/0

BMI/A Z-score	Number of patients	%	nM/F	%M/F
<–3 Z-Score	2	3.8	0/2	0/8
≥–3 Z-Score and <–2 Z-core	2	3.8	2/0	8/0
≥–2 Z-Score and <–1 Z-Score	12	23.1	6/6	22/24
≥–1 Z-Score and ≤+1 Z-Score	30	57.7	14/16	52/64
>+1 Z-Score and ≤+2 Z-Score	5	9.6	5/0	18.5/0
>+2 Z-Score and ≤+3 Z-Score	1	1.9	0/1	0/4
>+3 Z-Score	0	0.	0/0	0/0

Variables	Estimate	Standard error	95%CI		p value
Pseudomonas aeruginosa
No	0.203	0.123	—0.038	0.444	0.099
Yes	0	0	0	0	—
Pancreatic Insufficiency
No	0.169	0.137	—0.099	—0.437	0.217
Yes	0	0	0	0	—
Hepatopathy
No	0.066	—0.123	—0.176	—0.308	0.593
Yes	0	0	0	0	—
Spirometry
FVC (%)	—0.001	0.003	—0.007	0.005	0.788
FEV¹ (%)	—0.001	0.004	—0.009	0.006	0.749
FVC/FEV¹ (%)	—0.007	—0.007	—0.017	0.004	0.204
FEF^25-75 (%)	—0.002	—0.001	—0.004	—0.004	0.938
Gestational age
Term	0.177	0.128	—0.073	—0.428	0.166
Preterm	0	0	0	0	—
Birth weight
≥2.5kg	0.704	1.054	1.367	2.769	0.504
<2.5kg	0	0	0	0	—
Meconium ileus
Yes	—0.091	0.122	—0.329	0.147	0.452
No	0	0	0	0	—
Period of exclusive breastfeeding
—	0.028	0.017	0.005	0.061	0.094
Number of hospitalizations
—	—0.101	0.022	—0.144	–0.058	<0.001
Time from 1 ^st appointment to diagnosis
—	0.004	0.001	0.001	0.007	0.004
Time between birth and diagnosis
—	0.004	0.001	0.002	0.006	<0.001
Known alleles
1 known allele	—0.023	0.131	—0.280	0.234	0.861
2 known alleles	—0.044	0.146	—0.330	0.242	0.764
No known alleles	0	0	0	0	—
Onset of gastrointestinal symptoms
—	0.002	0.001	—0.001	0.004	0.161
Onset of respiratory symptoms
—	0.004	0.002	0.001	0.007	0.010

Variables	Estimate	Standard error	95%CI		p value
Pseudomonas aeruginosa
No	—0.021	0.082	—0.182	0.140	0.799
Yes	0	0	0	0	—
Pancreatic Insufficiency
No	—0.056	0.100	—0.251	0.139	0.573
Yes	0	0	0	0	—
Hepatopathy
No	—0.071	—0.071	—0.091	—0.240	0.436
Yes	0	0	0	0	—
Spirometry
FVC (%)	0.008	0.003	0.003	0.013	0.002
FEV¹ (%)	0.009	0.003	0.004	0.014	0.001
FVC/FEV¹ (%)	—0.001	0.005	—0.010	0.008	0.824
FEF^25-75 (%)	0.004	0.002	0.001	0.007	0.008
Gestational age
Term	0.378	0.123	0.136	0.619	0.002
Preterm	0	0	0	0	—
Birth weight
≥2.5kg	0.479	0.115	0.255	0.703	<0.001
<2.5kg	0	0	0	0	—
Meconium ileus
Yes	—0.118	0.111	—0.335	0.098	0.285
No	0	0	0	0	—
Period of exclusive breastfeeding
—	0.017	0.016	—0.014	0.047	0.281
Number of hospitalizations
—	—0.022	0.027	—0.076	0.032	0.422
Time from 1 ^st appointment to diagnosis
—	0.002	0.002	—0.002	0.005	0.294
Time between birth and diagnosis
—	0.002	0.001	—0.001	0.004	0.065
Known alleles
1 known allele	0.065	0.145	—0.224	0.355	0.657
2 known alleles	0.004	0.153	—0.254	0.346	0.763
No known allele	0	0	0	0	—
Onset of gastrointestinal symptoms
—	0.002	0.001	0	0.003	0.054
Onset of respiratory symptoms
—	0.002	0.001	0.001	0.004	0.014

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[1] * Corresponding author. E-mail: ghessel@fcm.unicamp.br (G. Hessel)