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

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)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.15Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)FAEPEX- UnicampConselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq)Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)FAPESP [FALM: 2011/12939-4

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

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

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