Demographic, clinical, and laboratory parameters of cystic fibrosis during the last two decades: a comparative analysis.

BACKGROUND: In recent years, patients with cystic fibrosis (CF) have tended to experience a longer life expectancy and higher quality of life. In this context, the aim of the present study was to evaluate and compare the demographic, clinical, and laboratory markers of patients with CF during the last two decades at a CF referral center. METHODS: A retrospective study of the demographic, clinical, and laboratory markers for CF treatment at a CF referral center was performed during two decades: 2000 (DI, 1990-2000, n = 104 patients) and 2010 (DII, 2000-2010, n = 181 patients). RESULTS: The following variables were less common in DI than in DII: (i) pancreatic insufficiency, (ii) meconium ileus, (iii) diabetes mellitus, (iv) Burkholderia cepacia colonization, (v) moderate and severe Shwachman-Kulczycki score (SKS), (vi) F508del mutation screening, (vii) patients without an identified CFTR mutation (class IV, V, or VI mutation), (viii) patients above the 10th percentile for weight and height, (ix) restrictive lung disease, and (x) older patients (p < 0.01). The following variables were more common in DI than in DII: (i) excellent and good SKS, (ii) F508del heterozygous status, (iii) colonization by mucoid and nonmucoid Pseudomonas aeruginosa, (iv) obstructive lung disease, and (v) minimal time for CF diagnosis (p < 0.01). CONCLUSION: Clinical outcomes differed between the two decades. Demographic, clinical, and laboratory markers in patients with CF are useful tools and should be encouraged in CF referral centers to determine the results of CF management and treatment, enabling a better understanding of this disease and its clinical evolution. Early diagnosis and management of CF will improve patients' quality of life and life expectancy until personalized drug therapy is possible for all patients with CF.

Comparing mixed oil to soybean oil lipid emulsion in patients on home parenteral nutrition: a pilot prospective double-blind, crossover, randomized trial.

BACKGROUND: Home parenteral nutrition (HPN) can be associated with increased liver enzymes, catheter-related bloodstream infections (CRBSI), and hospitalizations. Mixed oil (MO) versus soybean oil (SO) lipid emulsion reduces risks in hospitalized patients, but there are no randomized double-blinded controlled trials in HPN. Therefore, the primary objective was to test the study's feasibility such as recruitment and retention in the HPN population and the secondary objective was to assess changes in liver enzymes between MO and SO as well as other clinical and biochemical outcomes. METHODS: This 13-month prospective double-blind crossover randomized pilot trial took place in Toronto, Canada. Participants were HPN patients who were a part of the HPN program at Toronto General Hospital. We recruited patients from the HPN program. HPN patients receiving SO were randomized to either MO or SO, and the study duration was 6 months in each arm (MO or SO) with a 1-month washout period resuming SO. As this is a crossover trial design, the patient is his/her own control. The main outcome measures were descriptions of study feasibility, namely the study recruitment and retention. We also collected biochemical parameters, CRSBI, hospitalization rate, antibiotic use, and mortality. Demographic, nutritional, clinical, and laboratory data were collected at baseline, 3 and 6 months of each arm. The primary analysis population was defined as the per-protocol population who completed the trial including all lipid measurements. RESULTS: A total of 65 HPN patients were assessed, and 60 met the inclusion criteria for the study. Thirty-five percent (21/60) were randomized using a computer-generated random number sequence generator: 10 participants were randomized to receive SO first while 11 were randomized to receive MO first. At 13 months, 3/10 who received SO first completed the study, whereas 9/11 who received MO first completed the study. This did not meet our a priori criteria for success in recruitment and retention. Between types of lipid emulsions, there were no significant differences in changes in liver enzymes or biochemical and clinical outcomes, despite significant changes in plasma free fatty acid composition reflecting MO or SO. CONCLUSIONS: Overall, this pilot trial demonstrated that the use of a prospective double-blind, crossover, randomized trial design was not feasible to conduct in the HPN population because of difficulties in recruiting and retaining patients. In addition, there was no significant impact of MO versus SO lipid emulsion on liver enzymes or most parameters. The lack of significance may be attributed to low sample size from low recruitment and high drop-out rate, short study duration (6 months/arm), and complex care. In a future definitive trial, a multicenter study of longer duration and a larger sample size is recommended, and drop-outs may be reduced by using a parallel study design. TRIAL REGISTRATION: ClinicalTrials.gov, NCT02796833. Registered on 13 June 2016-retrospectively registered.

Hypophosphatemia in critically ill pediatric patients receiving enteral and oral nutrition.

BACKGROUND: Hypophosphatemia(HP) is related to several comorbidities in pediatric intensive care units (PICUs). This study aimed to evaluate the incidence of HP in severely ill pediatric patients receiving oral and/or enteral nutrition. The secondary objectives were to investigate the association between HP and the inflammatory state, PICU length of stay, severity, mortality, nutrition status, and protein, energy, calcium, vitamin D, and phosphate intake. METHODS: A prospective, observational cohort study was conducted in a PICU of a quaternary hospital. Participants aged between 28 days and 14 years were included. Anthropometric and laboratory assessments were performed ≤72 h after PICU admission and repeated after 7 days for three consecutive times. Energy, protein, calcium, phosphate, and vitamin D intake per day of hospitalization were recorded individually. The Pediatric Index of Mortality 2 (PIM2) was used to determine each patient's severity score. RESULTS: A total of 103 participants were included in the study. Hypophosphatemic events ranged from 27.2% to 37.5% among the assessments. HP was associated with high C-reactive protein levels (P = .012) and lower energy adequacy (P = .037). Serum phosphorus was inversely correlated (weak correlation) with PIM2 (P = .017). CONCLUSION: HP is common in critically ill pediatric patients, even when they are not receiving parenteral nutrition. It is necessary to monitor serum phosphorus levels and consider the possibility of early replacement, especially in patients showing high levels of inflammation. In addition to inflammation itself, low energy intake and illness severity were related to HP.

Extubation in the pediatric intensive care unit: predictive methods. An integrative literature review. / Extubação em unidade de terapia intensiva pediátrica: métodos preditores. Uma revisão integrativa da literatura.

For extubation in pediatric patients, the evaluation of readiness is strongly recommended. However, a device or practice that is superior to clinical judgment has not yet been accurately determined. Thus, it is important to conduct a review on the techniques of choice in clinical practice to predict extubation failure in pediatric patients. Based on a search in the PubMed®, Biblioteca Virtual em Saúde, Cochrane Library and Scopus databases, we conducted a survey of the predictive variables of extubation failure most commonly used in clinical practice in pediatric patients. Of the eight predictors described, the three most commonly used were the spontaneous breathing test, the rapid shallow breathing index and maximum inspiratory pressure. Although the disparity of the data presented in the studies prevented statistical treatment, it was still possible to describe and analyze the performance of these tests.

Para a extubação orotraqueal em pacientes pediátricos, é fortemente recomendada a avaliação de sua prontidão. No entanto, a utilização de um dispositivo ou prática que fosse superior ao julgamento clínico ainda não foi determinada com exatidão. Assim, é importante realizar uma revisão sobre as técnicas preditoras de escolha na prática clínica para prever a falha de extubação orotraqueal em pacientes pediátricos. A partir de uma busca nas bases de dados PubMed®, Biblioteca Virtual em Saúde, Cochrane Library e Scopus, realizamos um levantamento das variáveis preditoras de falha de extubação orotraqueal mais comumente utilizadas na prática clínica em pacientes pediátricos. Dos oito preditores descritos, observamos três mais usados: teste de respiração espontânea, índice de respiração rápida e superficial e pressão inspiratória máxima. Embora a disparidade dos dados apresentados nos estudos tenha inviabilizado um tratamento estatístico, foi possível, a partir desse meio, descrever e analisar o desempenho desses testes.

Hypercholesterolemia and hypertriglyceridemia as risk factors of liver dysfunction in children with inflammation receiving total parenteral nutrition.

BACKGROUND AND AIMS: Hepatic dysfunction, due to parenteral nutrition, may become severe and lead to cirrhosis and hepatic failure, especially in newborns and infants. This study aimed to evaluate the association between the exclusive use of total parenteral nutrition (TPN) and changes in the hepatic profile, and to investigate the relationship between age, hypertriglyceridemia, and hypercholesterolemia, and the occurrence of laboratory liver dysfunction. METHODS: A descriptive and historical cohort study was conducted, evaluating 195 pediatric patients (age: 1 month to 19 years) who received TPN. The following hepatic and lipid profiles were assessed: alanine aminotransferase (ALT), aspartate aminotransferase (AST), alkaline phosphatase, gamma-glutamyl transferase (GGT), bilirubin, triglycerides (TGs), cholesterol, high-density lipoprotein (HDL), albumin, and transthyretin. High C-reactive protein and/or low HDL were considered indicative of inflammatory process. RESULTS: The majority of patients presented normal values of AST (79.2%), ALT (74.9%), GGT (56.4%), and alkaline phosphatase (68.1%). Total bilirubin changed in 68.5% of patients, and transthyretin and albumin were low in 87.3% and 65.1% of the patients, respectively. Incidences of high GGT values were related to age (odds ratio [OR], 2.46; confidence interval [CI] 1.28-4.76; p = 0.007), hypercholesterolemia (OR, 3.00; 95% CI, 1.24-7.25; p = 0.015), and hypertriglyceridemia (OR, 2.39; 95% CI, 1.02-5.60; p = 0.046). Incidences of elevated ALT values were associated with hypercholesterolemia (OR, 4.57; 95% CI, 2.03-10.30; p < 0.001). CONCLUSION: Monitoring the hepatic profile from the early stage of TPN is necessary. Changes in the plasma lipid and hepatic profiles were frequently observed during the infusion of TPN, in patients with inflammation. Patients >2 years old and those with high TG and HDL levels were more likely to have elevated GGT levels. Hypercholesterolemia was associated with ALT alterations. Strategies to attenuate these issues should be considered in the early stages, in patients with TPN.

Pancreatic Insufficiency in Cystic Fibrosis: Influence of Inflammatory Response Genes.

OBJECTIVE: Pancreatic insufficiency (PI) in cystic fibrosis (CF) patients is a crucial clinical marker for severity and disease progression. In our study, 125 modifier genes and their SNPs were associated between CF patients with PI or pancreatic sufficiency. METHODS: We prospectively evaluated 214 CF patients admitted at 1 hospital for a 2-year period. The PI status was associated with clinical variables and SNPs related with inflammatory response considering CFTR mutations. Open Array technique was used to perform the SNPs identification. RESULTS: For PI risk, after correction by multiple test, in CF patients and 2 CFTR mutations class I, II, and/or III, there were 6 SNPs with positive association (P < 0.005). The odds ratio amplitude was 0.087 (95% confidence interval [CI], 0.004-0.544) for rs9870255*CG (CTNNB1 gene) to 11.06 (95% CI, 1.746-252.3) for rs729302*AA (IRF5 gene). For all CF patients at the same time, 9 SNPs showed positive association. The odds ratio amplitude was 0.144 (95% CI, 0.028-0.602) for rs2348071*AA (PSMA3 gene) to 5.809 (95% CI, 1.536-37.54) for rs11702779*AA (RUNX1 gene). In our data, we observed the interaction between CFTR mutations, rs9870255*CTNNB1, rs9378805*IRF4, and rs7664617*KCNIP4 to PI status. CONCLUSIONS: Multiple SNPs in inflammatory response genes showed association with PI considering the CFTR mutations screening.

Changes in Home Parenteral Nutrition Practice Based on the Canadian Home Parenteral Nutrition Patient Registry.

BACKGROUND: Since 2005, the Canadian home parenteral nutrition (HPN) registry has collected data on patients' demography, outcomes, and HPN clinical practice. At annual meetings, Canadian HPN programs review and discuss results. AIM: To evaluate changes over time in patient demography, outcomes, and HPN clinical practice using the registry data. METHODS: This retrospective study evaluated 369 patients who were prospectively entered in the registry. Two periods were compared for the first data entry: 2005-2008 (n = 182) and 2011-2014 (n = 187). Patient demography, indications for HPN, HPN regimen, nutrition assessment, vascular access, and number of line sepsis per 1000 catheter days were evaluated. RESULTS: For 2011-2014 compared with 2005-2008, indications for HPN changed significantly, with an increased proportion of patients with cancer (37.9% vs 16.7%) and with fewer cases of short bowel syndrome (32% vs 65.5%); line sepsis rate decreased from 1.58 to 0.97 per 1000 catheter days; and the use of tunneled catheters decreased from 64.3% to 38.0% and was no longer the most frequently chosen vascular access method. In contrast, the proportion of peripherally inserted central catheters increased from 21.6% to 52.9%. In addition, there was a reduction in number and days of hospitalizations related to HPN, and favorable changes were noted in the prescription of energy, proteins, and trace elements. CONCLUSION: The Canadian HPN registry is useful in tracking trends in demography, outcomes, and clinical practice. Results suggest a shift in patient demography and line access with improvement in line sepsis, hospitalizations, and HPN prescriptions.

Hypophosphatemia, Hypomagnesemia, and Hypokalemia in Pediatric Patients Before and During Exclusive Individualized Parenteral Nutrition.

BACKGROUND: Hypophosphatemia, hypomagnesemia, and hypokalemia occur in patients receiving parenteral nutrition (PN), mainly when the body's stores are depleted due to fasting or inflammation. Although these disorders are potentially fatal, few studies have reported the incidence in the pediatric population. METHODS: This study evaluated, in a historical cohort of pediatric patients, the prevalence of hypophosphatemia, hypokalemia, and hypomagnesaemia until 48 hours before initiation of PN infusion (P1) and from days 1-4 (P2) and days 5-7 (P3) of PN infusion and investigated if malnutrition, calories, and protein infusion were correlated to these disorders. RESULTS: Malnutrition was present in 32.8% (n = 119) of the subjects; 66.4% of the patients were in the pediatric intensive care unit. Survival rate was 86.6%. P1 had the highest prevalence of mineral disorders, with 54 events (58.1%; P2, n = 35, 37.6%; P3, n = 4, 4.3%). Hypokalemia events were related to malnutrition (odds ratio, 2.79; 95% confidence interval, 1.09-7.14; P = .045). In the first 7 days, infused calories were below the amount recommended by current guidelines in up to 84.9% of patients, and protein infused was adequate in up to 75.7%. Protein infused above the recommendation in the first 4 days was related to hypomagnesaemia (odds ratio, 5.66; 95% confidence interval, 1.24-25.79; P = .033). CONCLUSION: Hypophosphatemia, hypokalemia, and hypomagnesemia were frequent in hospitalized pediatric patients before and during the first 4 days of PN infusion. Patients with malnutrition had more chances of having hypokalemia, and those who received high protein infusion had an increased chance of developing hypomagnesemia.

Extubação em unidade de terapia intensiva pediátrica: métodos preditores. Uma revisão integrativa da literatura / Extubation in the pediatric intensive care unit: predictive methods. An integrative literature review

RESUMO Para a extubação orotraqueal em pacientes pediátricos, é fortemente recomendada a avaliação de sua prontidão. No entanto, a utilização de um dispositivo ou prática que fosse superior ao julgamento clínico ainda não foi determinada com exatidão. Assim, é importante realizar uma revisão sobre as técnicas preditoras de escolha na prática clínica para prever a falha de extubação orotraqueal em pacientes pediátricos. A partir de uma busca nas bases de dados PubMed®, Biblioteca Virtual em Saúde, Cochrane Library e Scopus, realizamos um levantamento das variáveis preditoras de falha de extubação orotraqueal mais comumente utilizadas na prática clínica em pacientes pediátricos. Dos oito preditores descritos, observamos três mais usados: teste de respiração espontânea, índice de respiração rápida e superficial e pressão inspiratória máxima. Embora a disparidade dos dados apresentados nos estudos tenha inviabilizado um tratamento estatístico, foi possível, a partir desse meio, descrever e analisar o desempenho desses testes.

ABSTRACT For extubation in pediatric patients, the evaluation of readiness is strongly recommended. However, a device or practice that is superior to clinical judgment has not yet been accurately determined. Thus, it is important to conduct a review on the techniques of choice in clinical practice to predict extubation failure in pediatric patients. Based on a search in the PubMed®, Biblioteca Virtual em Saúde, Cochrane Library and Scopus databases, we conducted a survey of the predictive variables of extubation failure most commonly used in clinical practice in pediatric patients. Of the eight predictors described, the three most commonly used were the spontaneous breathing test, the rapid shallow breathing index and maximum inspiratory pressure. Although the disparity of the data presented in the studies prevented statistical treatment, it was still possible to describe and analyze the performance of these tests.

[Factors impacting the growth and nutritional status of cystic fibrosis patients younger than 10 years of age who did not undergo neonatal screening]. / Fatores que afetam o crescimento e estado nutricional de pacientes com fibrose cística com idade inferior a 10 anos e que não foram submetidos à triagem neonatal.

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 [FEF25-75(%)], forced expiratory volume in the first second [FEV1(%)], gestational age, birth weight and early respiratory symptoms were associated with IMC/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.

Associação entre estado nutricional e Escore Prognóstico de Glasgow modificado em pacientes com câncer / Association between nutritional status and Score Glasgow Prognostic Score patients with the cancer

Introdução: A desnutrição é um achado comum em pacientes oncológicos e sua etiologia é multicausal, sendo influenciada significativamente pela resposta inflamatória sistêmica. Os indicadores de inflamação podem ser ferramentas úteis na avaliação nutricional dos pacientes com câncer. Objetivo: Avaliar a associação entre o estado nutricional e o Escore Prognóstico de Glasgow modificado (EPGm) em pacientes com câncer. Métodos: Pacientes admitidos em um hospital universitário, com diagnóstico confirmado de câncer, participaram deste estudo. Exames laboratoriais de albumina e proteína C-reativa (PCR) foram realizados para obtenção do EPGm. A avaliação nutricional foi realizada por meio da Avaliação Subjetiva Global (ASG), índice de massa corporal (IMC) e variáveis antropométricas. Resultados: Foram avaliados 70 pacientes com idade média de 58,51±14,85 anos, dos quais 50 (71,5%) apresentaram algum grau de desnutrição pela ASG, destes 27 (38,6%) apresentaram desnutrição grave. As categorias de risco do EPGm estiveram presentes em 41 (58,6%). O Escore Prognóstico de Glasgow modificado associou-se à presença da desnutrição (p<0,05). Conclusão: O Escore Prognóstico de Glasgow se associou ao estado nutricional definido pela Avaliação Subjetiva Global (AU)

Introduction: Malnutrition is a common finding in patients with cancer; its etiology is multifactorial and significantly influenced by the systemic inflammatory response. Inflammation indicators can be useful tools in the nutritional assessment of patients. Objective: To evaluate the association of the nutritional status with modified Glasgow Prognostic Score (mGPS) in cancer patients. Methods: We evaluated patients who were admitted to university hospital with a confirmed diagnosis of cancer participated in this study. Laboratory tests for albumin and C-reactive protein (CRP) were conducted to obtain mGPS scores. Nutritional assessment was carried out by subjective global assessment (SGA), body index massa (BMI) and anthropometric variables. Results: A total of 70 patients with a mean age of 58.51 ± 14.85 years were evaluated, of which 50 (71.5%) presented some degree of malnutrition, of these 27 (38.6%) presented severe malnutrition. The risk categories of mGPS was present in 41 (58.6%) patients. Conclusion: The modified Glasgow Prognostic Score was associated with the nutritional status defined by the Global Subjective Assessment (AU)

Associação entre desnutrição, escore prognóstico de Glasgow modificado e índice inflamatório nutricional em pacientes com câncer / Association between malnutrition, modified Glasgow prognostic score and nutritional inflammatory index in cancer patients

Introdução: A desnutrição é um achado comum em pacientes oncológicos e sua etiologia é multicausal, sendo influenciada significativamente pela resposta inflamatória sistêmica. Os indicadores de inflamação podem ser ferramentas úteis na avaliação nutricional dos pacientes com câncer. Objetivo: Avaliar a associação entre o Escore Prognóstico de Glasgow modificado e o Índice Inflamatório Nutricional (IIN) com a presença e o grau de desnutrição em pacientes com câncer. Método: Pacientes admitidos em um hospital universitário, com diagnóstico confirmado de câncer, participaram deste estudo. Exames laboratoriais de albumina e proteína C-reativa foram realizados para obtenção do EPGm e do IIN. A avaliação nutricional foi realizada por meio da Avaliação Subjetiva Global. Resultados: Foram avaliados 70 pacientes com idade média de 58,51±14,85 anos, dos quais 50 (71,5%) apresentaram algum grau de desnutrição, enquanto 27 (38,6%) apresentaram desnutrição grave. As categorias de risco do EPGm e do IIN estiveram presentes em 41 (58,6%) e 39 (55,7%) pacientes, respectivamente. O EPGm e o IIN associaram-se à presença da desnutrição (p<0,05). Conclusão: O EPGme o IIN mostraram-se ferramentas úteis, de fácil interpretação, com possibilidade de complementar a avaliação nutricional do paciente oncológico, uma vez que se associaram com a desnutrição.(AU)

Introduction: Malnutrition is a common finding in patients with cancer; its etiology is multifactorial and significantly influenced by the systemic inflammatory response. Inflammation indicators can be useful tools in the nutritional assessment of patients with cancer. Objective: We intended to evaluate the association of the modified Glasgow Prognostic Score (mGPS) and the InflammatoryNutritional Index (INI) with the presence and degree of malnutrition in cancer patients. Methods: We evaluated patients who were admitted to university hospital with a confirmed diagnosis of cancer participated in this study. Laboratory tests for albumin and C-reactive protein were conducted to obtain mGPS and INI scores. Nutritional assessment was carried out by subjective global assessment. Results: A total of 70 patients with a mean age of 58.51±14.85 years were evaluated, of which 50 (71.5%) presented some degree of malnutrition, while 27 (38.6%) presented severe malnutrition. The risk categories of mGPS and INI were present in 41 (58.6%) and 39 (55.7%) patients, respectively. Conclusion: Them GPS Score and the INI are simple tools, easy to interpret, with the possibility of complementing the nutritional evaluation of cancer patients, since they were associated with malnutrition.(AU)

Factors impacting the growth and nutritional status of cystic fibrosis patients younger than 10 years of age who did not undergo neonatal screening / Fatores que afetam o crescimento e estado nutricional de pacientes com fibrose cística com idade inferior a 10 anos e que não foram submetidos à triagem neonatal

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 [FEF25-75(%)], 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. .

OBJETIVO: Avaliar por meio de parâmetros clínicos e laboratoriais como a fibrose cística (FC) afeta o crescimento e estado nutricional de crianças submetidas ao tratamento de FC que não foram submetidas à triagem neonatal. MÉTODOS: Uma coorte histórica com 52 pacientes com FC menores de 10 anos foi acompanhada em um centro de referência em Campinas, Sudeste do Brasil. Peso e altura foram coletados de prontuários médicos até março de 2010, quando a triagem neonatal foi implementada. Entre setembro de 2009 a março de 2010 a altura dos pais foi medida. RESULTADOS: Quatro pacientes tiveram escores Z ≤ -2 para altura/idade (A/I) e índice de massa corporal/idade (IMC/A). As seguintes variáveis foram associadas com melhor razão A/I: menor número de hospitalizações, maior tempo entre a primeira consulta e o diagnóstico, maior tempo entre o nascimento e o diagnóstico e início tardio da doença respiratória. Capacidade vital forçada [CVF(%)], fluxo expiratório forçado entre 25-75% da CVF [FEF25-75(%)], volume expiratório forçado no primeiro segundo [VEF1(%)], idade gestacional, peso ao nascer e início dos sintomas respiratórios foram associados com melhor IMC/I. CONCLUSÕES: Maior número de hospitalizações, retardo no diagnóstico e início precoce da doença respiratória tiveram impacto negativo no crescimento. Menores valores espirométricos, menor idade gestacional, menor peso ao nascer e o início precoce dos sintomas respiratórios tiveram impacto negativo no estado nutricional. A desnutrição foi observada em 7,7% dos casos, mas 23% das crianças apresentaram risco nutricional. .