Demographic, Clinical, And Laboratory Parameters Of Cystic Fibrosis During The Last Two Decades: A Comparative Analysis.

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. 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). 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). 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.15

Factors impact on growth and nutritional status in cystic fibrosis patients with children under 10 years not subject to newborn screening

Orientador: Antonio Fernando RibeiroDissertação (mestrado) - Universidade Estadual de Campinas, Faculdade de Ciências MédicasResumo: A Fibrose Cística (FC) é uma doença que pode interferir no crescimento na infância por causar desnutrição decorrente da digestão e absorção deficiente de nutrientes, pelo aumento das necessidades calóricas devido ao declínio da função pulmonar. O objetivo deste estudo foi avaliar o impacto da FC, evolutivamente, através de parâmetros clínicos e laboratoriais, sobre o crescimento e o estado nutricional. Realizou-se um estudo retrospectivo, de corte transversal. Foram avaliados 52 pacientes menores de 10 anos, antes do início da triagem neonatal. A coleta dos dados para avaliação antropométrica atual foi realizada em dias de consulta habitual no período de setembro de 2009 a março de 2010. Neste momento também foram coletadas a estatura dos pais. As medidas antropométricas retrospectivas e os marcadores de gravidade clínica foram coletados nos seguintes momentos: ao nascimento, na primeira consulta no serviço, ao diagnóstico e assim anualmente no mês de aniversário do paciente. Quanto ao estado nutricional, 4 pacientes apresentaram escore Z ? -2 para os índice estatura/idade e IMC/idade. As variáveis, menor número de internações, maior tempo entre a primeira consulta ao diagnóstico, maior tempo entre nascimento e o diagnóstico e início tardio de manifestações respiratórias apresentaram associação estatisticamente significativa com melhor relação estatura/idade. As variáveis CVF(%), VEF(%), FEF 25/75%, tempo de gestação, peso ao nascimento e início de manifestações respiratórias apresentaram associação estatisticamente significativa com o índice IMC/I. Pode-se concluir que, para os principais parâmetros estudados, houve impacto negativo da FC sobre o crescimento e o estado nutricional dos pacientes avaliadosAbstract: Cystic Fibrosis (CF) is a disease that can interfere at growth in childhood to cause malnutrition due to poor digestion and absorption of nutrients, the increased energy requirements due to the decline in lung function. The aim of this study was to evaluate the impact of CF evolution through clinical and laboratory parameters on nutritional status during treatment of these patients. We conducted a retrospective cross sectional. Were evaluated 52 patients less than 10 years, before the start of neonatal screening. Data collection for the current anthropometric assessment was performed on days of normal consultation between September 2009 and March 2010. At this time were also collected parents' statures. The retrospective anthropometric and clinical risk markers were collected at the following times: at birth, the first appointment in the service, diagnosis, and thus every year in the patient's birthday. Regarding nutritional status, 4 patients had Z score ? -2 score height/age and BMI/age. The variables, fewer number of hospitalizations, greater time from first consultation to diagnosis and longer time between birth and late early diagnosis of respiratory disease were significantly associated with the best height/age. The FVC(%), FEV1(%), FEF 25/75%, gestational age, birth weight and early respiratory symptoms were significantly associated with BMI/I. It can be concluded that for the main parameters studied, there was impact of FC on nutritional status of patientsMestradoSaude da Criança e do AdolescenteMestre em Saude da Criança e do Adolescent

Parenteral nutrition : metabolic complication in pediatric patients hospitalized patients and changes in clinical practice in home patients in Canada

Orientadores: Antonio Fernando Ribeiro, José Roberto Negrão NogueiraTese (doutorado) - Universidade Estadual de Campinas, Faculdade de Ciências MédicasResumo: Introdução O desenvolvimento da nutrição parenteral (NP) na década de 1960 e sua subsequente utilização na prática clínica tem tido um enorme impacto sobre os pacientes com numerosas doenças para as quais a nutrição foi impossível por outra via. Mas, esta nova tecnologia tem riscos associados, incluindo o desenvolvimento de distúrbios metabólicos, superalimentação e complicações infecciosas. Método Foram realizados dois estudos. O primeiro estudo teve como objetivo avaliar, em uma coorte histórica de pacientes pediátricos no Brasil que receberam NP individualizada e exclusiva, a prevalência de hipofosfatemia, hipocalemia e hipomagnesemia em até 48 horas antes do início da infusão de NP (P1), do 1° ao 4° dia (P2); 5° ao 7° dia (P3) e, investigar se a infusão de caloria e proteína e também a desnutrição foram relacionadas com esses distúrbios. O segundo estudo foi feito no Canadá. Trata-se de um estudo retrospectivo e multicêntrico, avaliando pacientes sob nutrição parenteral domiciliar (NPD), prospectivamente inseridos no Home Parenteral Nutrition Registry (HPN Registry) nos períodos: 2005-2008 ou 2011-2014. Mudanças na demografia, indicações para NPD, prescrição, avaliação nutricional, acesso vascular e número de infecção de cateter por 1000 dias de cateter foram avaliados. Resultados A desnutrição esteve presente em 32,8% dos 119 pacientes avaliados no primeiro estudo, 66,4% estavam em unidade de terapia intensiva pediátrica (UTI Ped), 13,5% morreram. O período de maior prevalência de distúrbios minerais foi o P1 54 (45,3%), no P2 = 35 (31,8%), no P3 = 4 (3,57%). Hipocalemia esteve relacionada à desnutrição OR 2,79 (95% CI 1,09-7,14) p = 0,045. Nos primeiros sete dias, foram infundidas calorias inferior à quantidade recomendada pelas recomendações atuais em até 84,9% dos pacientes e proteína adequada em até 75,7%. Proteína infundida acima da recomendação nos primeiros quatro dias foi relacionada com hypomagnesaemia OR: 5,66 (IC 95% 1,24 - 25,79) p = 0,033. No estudo canadense, comparando os períodos 2011-2014 com 2005-2008, as indicações para a NPD mudaram significativamente com o aumento da proporção de pacientes com câncer (37,9% versus 16,7%) e diminuição da síndrome do intestino curto (32% versus 65,5%). A taxa de infecção de cateter diminuiu de 1,58 para 0,97 por 1.000 dias de cateter; o uso de cateter tunelizado diminuiu de 64,3% para 38,0% e a proporção de cateteres centrais de inserção periférica (PICC) aumentou de 21,6% para 52,9%. Além disso, houve uma redução no número e dias de internações relacionadas à NPD, e mudanças na prescrição de energia, proteína e oligoelementos. Conclusão Hipofosfatemia, hipocalemia e hipomagnesemia foram eventos frequentes, sendo a individualização ferramenta primordial para gerenciá-los. Pacientes desnutridos tiveram maior chance de desenvolver hipocalemia e os que receberam proteína acima da recomendação tiveram mais chances de desenvolver hipomagnesemia. Os resultados sugerem uma mudança no perfil demográfico e acesso venoso no Canadá, com melhora na infecção de cateter, hospitalizações relacionadas à NPD, e prescriçõesAbstract: Introduction: The development of parenteral nutrition (PN) in the 1960s and its subsequent use in clinical practice has had a huge impact on patients with numerous diseases for which nutrition was impossible by other route. But, this new technology has associated risks, including the development of metabolic disorders, overfeeding, and infectious complications. Methods We conducted two studies The first study aims to evaluate in a historical cohort of pediatric patients, the prevalence of hypophosphatemia, hypokalemia and hypomagnesaemia until 48th hours before beginning PN infusion (P1), from 1st¿4th day (P2); 5th¿7th day (P3) of PN infusion and, investigate if malnutrition, calories, and proteins infusion were correlated to these disorders. In Canada, a retrospective study evaluating patients who were prospectively entered in the registry either in 2005¿2008 or in 2011¿2014 was done. Changes in patient demography, indications for Home Parenteral Nutricion (HPN), regimen, nutritional assessment, vascular access, and number of line sepsis per 1000 catheter days were evaluated. Results Malnutrition was present 32.8% of 119 patients participants from the first study, 66.4% were in pediatric intensive care unit (PICU), 13.5% died. The P1 was the period of highest prevalence mineral disorders 54 (45.3%), P2 had 35 (31.8%) and, P3=4 (3.57%). Hypokalemia events were related to malnutrition OR 2.79 (95% CI 1.09-7.14) p = 0.045. In the first seven 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 recommendation was related to hypomagnesaemia OR: 5,66 (95% CI 1,24 ¿ 25,79) p=0,033. In 2011¿2014 compared with 2005¿2008, indications for HPN changed significantly with an increased proportion of patients with cancer (37.9% versus 16.7%) and decreased short bowel syndrome (32% versus 65.5%). The line sepsis rate decreased from 1.58 to 0.97 per 1,000 catheter days; tunnelled catheters decreased as the most frequently chosen vascular access method from 64.3% to 38.0% and the proportion of peripherally inserted central catheters (PICC) increased from 21.6% to 52.9%. In addition, there was a reduction in number, and days of hospitalizations related to HPN, and changes in the prescription of energy, proteins, and trace elements were noted. Conclusion Hypophosphatemia, hypokalemia and hypomagnesemia were frequent events, being individualization primary tool to manage them. Malnourished patients were more likely to develop hypokalemia, and patients receiving protein above the recommendation were more likely to develop hypomagnesemia. Results suggest a shift in patient demography and line access in Canada, with improvement in line sepsis, hospitalizations and HPN prescriptionsDoutoradoSaude da Criança e do AdolescenteDoutora em Ciência

Hypophosphatemia, Hypomagnesemia, And Hypokalemia In Pediatric Patients Before And During Exclusive Individualized Parenteral Nutrition

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.31222322

Dual x-ray absorptiometry monitoring in pediatric short bowel syndrome: an integrative review

ABSTRACT Objective: To analyze the bone health of pediatric patients with short bowel syndrome intestinal failure (SBS-IF). Data source: An integrative literature review was performed using the data published in the MEDLINE-PubMed and Scientific Electronic Library Online (SciELO) databases between January 2010 and April 2021, and through a manual search of the reference lists of relevant studies. Studies were included if they assessed bone mineral density by the Dual X-Ray Absorptiometry (DXA) technique, incorporated pediatric patients (up to 20 years of age) with SBS under parenteral nutrition (PN) and were written in English. Eleven primary sources met the inclusion criteria for this study. Data synthesis: Pediatric patients with SBS-IF under long-term parenteral nutrition experienced frequent changes in bone metabolism, leading to osteoporotic fractures and growth failure. These patients have deficiencies in multiple nutrients, such as calcium, magnesium, phosphorus, and vitamin D. Consequently, there are variations in the secretion and regulation of the parathyroid hormone. In addition, the pharmacotechnical limitations related to calcium and phosphorus in the PN solution, use of glucocorticoids, and difficulty performing physical activity are risk factors for the development of metabolic bone disease in pediatric patients with SBS-IF. Conclusions: Low bone mineral density was associated with a high risk of developing osteoporosis, fractures, and growth deficiency in pediatric patients with SBS-IF on PN therapy in the long term

Série histórica de mortalidade por suicídio em município paulista segundo dados epidemiológicos

Objetivo: Analisar a tendência e o perfil epidemiológico do suicídio, em um município de grande porte do interior do estado de São Paulo, no período de 2000 a 2018. Métodos: Estudo ecológico de série temporal realizado em 2019 que analisou a mortalidade por suicídio na cidade de Campinas, São Paulo, Brasil, no período de 2000 a 2018. A coleta ocorreu por meio de dados do Sistema de Informação sobre Mortalidade, sendo as variáveis: sexo, idade, raça, categoria do óbito, mês e local de ocorrência, com dados analisados pelo número/taxa de óbitos e taxas de óbitos padronizadas por faixa etária e sexo. Estimaramse modelos de regressão linear simples para número de óbitos e ano. Na análise da proporção de óbitos por suicídio e demais variáveis estudadas utilizou-se teste de qui-quadrado com significância de 5%. Resultados: Ocorreram 904 óbitos por suicídio, com aumento significativo ao longo dos anos e taxa geral de 3,20/100.000 hab. (2000) e 5,42/100.000 hab. (2018). Observou-se aumento no sexo masculino 5,30/100.000 hab. (2000) e 8,45/100.000 hab. (2018), faixas etárias de 20 a 40 incompletos com 3,53/100.000 hab. (2000) e 6,84/100.000 hab. (2018) e de 40 a 60 incompletos com 4,69/100.000 hab. (2000) e 7,61/100.000 hab. (2018). A maioria dos óbitos ocorreu com pessoas brancas (673; 74,9%), por enforcamento (503; 55,6%), em domicílio (524;58,0%), em setembro (93; 10,3%) e dezembro (92; 10,2%). Conclusão: Houve crescente aumento das taxas de suicídios em Campinas no período analisado, sendo as vítimas predominantemente homens, adultos, brancos, por enforcamento e realizado em domicílios

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 hypomagnesemia312223228sem informaçã

Hypophosphatemia, Hypomagnesemia, And Hypokalemia In Pediatric Patients Before And During Exclusive Individualized Parenteral Nutrition.

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. 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. 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). 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.3

[factors Impacting The Growth And Nutritional Status Of Cystic Fibrosis Patients Younger Than 10 Years Of Age Who Did Not Undergo Neonatal Screening.]

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. 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. 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. 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