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Journal articles on the topic 'Cystic fibrosis in children'

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Author: Grafiati

Published: 4 June 2021

Last updated: 30 July 2024

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1

Staab, D. "Cystic fibrosis -- therapeutic challenge in cystic fibrosis children." European Journal of Endocrinology 151, Suppl_1 (August 1, 2004): S77—S80. http://dx.doi.org/10.1530/eje.0.151s077.

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Cystic fibrosis (CF) is the most common autosomal recessive disease with fatal outcome in Caucasians with a frequency of 1 in 2500 life births. It is caused by mutations in a single gene on the long arm of chromosome 7 encoding a protein called the cystic fibrosis transmembrane regulator (CFTR). The defect in CFTR leads to pathological changes in all organs with mucus-secretory glands, e.g. airways, pancreas, gut, biliary tract, vas deferens and sweat glands. Despite impressive advances in understanding the molecular basis of the disease, life expectancy is still limited in CF and chronic infection of the lung resulting in fibrosis and bronchiectasis followed by respiratory insufficiency is still the main factor in morbidity and the leading cause of death. Poor nutritional status is one of the major problems in the vicious cycle of chronic inflammation and lung destruction and its impact on outcome in lung function has been demonstrated. The possible role of growth hormone treatment in this context will be discussed.

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2

Maffessanti, Mario, Manila Candusso, Franca Brizzi, and Francesco Piovesana. "Cystic Fibrosis in Children." Journal of Thoracic Imaging 11, no. 1 (1996): 27–38. http://dx.doi.org/10.1097/00005382-199601110-00002.

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3

Slieker, Martyn G., Anne G. M. Schilder, Cuno S. P. M. Uiterwaal, and Cornelis K. van der Ent. "Children With Cystic Fibrosis." Archives of Otolaryngology–Head & Neck Surgery 128, no. 11 (November 1, 2002): 1245. http://dx.doi.org/10.1001/archotol.128.11.1245.

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4

Littlewood, J. M. "Fibrosing colonopathy in children with cystic fibrosis." Postgraduate Medical Journal 72, no. 845 (March 1, 1996): 129–30. http://dx.doi.org/10.1136/pgmj.72.845.129.

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5

Reichard, Kirk W., Charles D. Vinocur, Maria Franco, Kristin L. Crisci, Jonathan A. Flick, Deborah F. Billmire, Daniel V. Schidlow, and William H. Weintraub. "Fibrosing colonopathy in children with cystic fibrosis." Journal of Pediatric Surgery 32, no. 2 (February 1997): 237–42. http://dx.doi.org/10.1016/s0022-3468(97)90186-x.

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6

Klimov, L. Ya, S. V. Dolbnya, E. I. Kondratyeva, A. A. Dyatlova, E. A. Enina, V. A. Kuryaninova, A. N. Kasyanova, et al. "Vitamin d status during winter months among children and adolescents with cystic fibrosis living in southern Russia." Medical Council, no. 2 (February 16, 2019): 240–49. http://dx.doi.org/10.21518/2079-701x-2019-2-240-249.

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The article presents the results of vitamin D tests in children with cystic fibrosis and in healthy children living in the South of Russia. The study showed the high prevalence of vitamin D deficiency and inadequate levels in patients with cystic fibrosis (86.7%). 25(OH) D level characterizing the vitamin D status decreases progressively in patients and healthy children. With regard to the above mentioned, it is significantly lower in patients with cystic fibrosis than in healthy children in all age periods. The correlation between the serum calcidiol level and the age of patients with cystic fibrosis was r = -0.44 (p = 0.015). Intake of prophylactic (500--1000 IU/day) and therapeutic (1500--3000 IU/day) doses of cholecalciferol results in a less significant increase in calcidiol levels in patients with cystic fibrosis compared with healthy children. A significantly lower 25(OH)D level was detected in patients with cystic fibrosis infected withStaph. aureus.The 25(OH)D levels are significantly lower in children with cystic fibrosis and hypocholesterolemia, than in patients with normal cholesterol levels. Risk factors for the development of severe hypovitaminosis D in children with cystic fibrosis are age, physical developmental delay, exocrine pancreatic insufficiency, presence of chronicStaph. Aureusbronchopulmonary infection. Given the conducted study, the dosage of cholecalciferol in patients with cystic fibrosis should be at least 2 times higher than that in healthy children.

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Amarri, Sergio, Marilyn Harding, W. Andrew Coward, T. John Evans, and Lawrence T. Weaver. "¹³C and H₂ Breath Tests to Study Extent and Site of Starch Digestion in Children with Cystic Fibrosis." Journal of Pediatric Gastroenterology and Nutrition 29, no. 3 (September 1999): 327–31. http://dx.doi.org/10.1002/j.1536-4801.1999.tb02423.x.

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ABSTRACTBackground:Starch is an important source of energy for children with cystic fibrosis, but little is known about their capacity to digest it.Methods:A 13C breath test was used to measure starch digestion and oxidation in 16 children with cystic fibrosis (median [range] age, 7.9 [4‐15] years; 7 girls, 9 boys) and 5 normal healthy control subjects (median age, 8.3 [7‐13] years; 3 girls, 2 boys). A test meal of 13C flour and lactulose was consumed and breath samples were obtained half‐hourly thereafter for 6 hours to measure 13C enrichment by isotope ratio mass spectrometry and H2 by electrochemistry. The test was repeated on 10 children with cystic fibrosis when they were taking pancreatic supplements.Results:The median (range) cumulative percentage 13C dose recovery (cPDR), was 35% (18‐52%) in control subjects, 18% (9‐33%) in children with cystic fibrosis without enzymes, and 29% (22‐51%) in those with pancreatic supplements. cPDR differed significantly between healthy control subjects and children with cystic fibrosis without enzymes (p = 0.01) and between children with cystic fibrosis with and without enzymes (p < 0.0001), but there was no difference between control subjects and children with cystic fibrosis taking enzymes (p = 0.5). Eight children with cystic fibrosis had a cPDR within control range, and in six there was a second peak in 13CO2 enrichment coincident with an increase in H2.Conclusions:Starch digestion and oxidation are diminished in children with cystic fibrosis, but pancreatic enzymes restored them to near normal levels. A second peak in 13CO2 enrichment, suggestive of colonic starch fermentation was absent in healthy children, but present in some children with cystic fibrosis and abolished by pancreatic enzymes.

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8

Burlutskaya, A. V., V. Yu Brisin, A. V. Statova, O. Yu Zenkina, and J. V. Pisotskaya. "Targeted Therapy for Cystic Fibrosis in Children." Innovative Medicine of Kuban, no. 4 (November 17, 2023): 142–47. http://dx.doi.org/10.35401/2541-9897-2023-8-4-142-147.

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A mutation in the CFTR gene causes the malfunctioning of the CFTR protein that is located on the apical membrane of epithelial cells and functions as a chloride channel. The discovery of CFTR modulators is an advance in cystic fibrosis treatment.Objective: To analyze modern Russian and foreign literature on targeted therapy for cystic fibrosis in children. We also describe the history of cystic fibrosis, evolution in its diagnosis and treatment, and mechanisms behind targeted therapy for cystic fibrosis.

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9

Daftari, Tahereh, Naser Havaei, Mandana Rezaei, and Babak Ghalibaf. "Occupational performance status and reliability of the Canadian Occupational Performance Measure in children with cystic fibrosis." International Journal of Therapy and Rehabilitation 27, no. 6 (June 2, 2020): 1–9. http://dx.doi.org/10.12968/ijtr.2019.0088.

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Background/Aims Cystic fibrosis affects the respiratory system and may interfere with an individual's occupational performance. This study intended to evaluate the reliability of the Canadian Occupational Performance Measure in children with cystic fibrosis and to compare the occupational performance of children with cystic fibrosis to that of healthy participants. Methods A total of 21 children with cystic fibrosis were included in the reliability and comparison phases of this study and 30 healthy children were recruited for the comparison phase. The Canadian Occupational Performance Measure was used to collect data on occupational performance and satisfaction. Intraclass correlation coefficients and standard error measurements were obtained to evaluate the reliability of the Canadian Occupational Performance Measure. An independent t-test was used to compare the occupational performance of the two groups. Results The test–retest reliability using a 7-day interval was 0.86–0.87 (standard error: 1.79–1.91). The agreement between therapists' scores for inter-rater reliability ranged from 0.87 to 0.90 (standard error: 1.44–1.68). There was a significant difference in performance subscale scores between children with cystic fibrosis and healthy children (P<0.05). There was no significant between-group difference in satisfaction scores. Conclusions The Canadian Occupational Performance Measure is a reliable tool for evaluating the occupational performance of children with cystic fibrosis. Cystic fibrosis directly and indirectly affects occupational performance. Children with cystic fibrosis and their parents may require help and education in the performance and organisation of activities and occupations.

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10

Stapleton, Denise, Deborah Kerr, Lyle Gurrin, Jill Sherriff, and Peter Sly. "Height and Weight Fail to Detect Early Signs of Malnutrition in Children With Cystic Fibrosis." Journal of Pediatric Gastroenterology and Nutrition 33, no. 3 (September 2001): 319–25. http://dx.doi.org/10.1002/j.1536-4801.2001.tb07464.x.

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ABSTRACTBackgroundMany children with cystic fibrosis grow poorly and are malnourished. This study was undertaken to determine whether extensive anthropometry could detect early signs of malnutrition in prepubertal children with cystic fibrosis to prevent deficits in height and weight.MethodsHeight, weight, six skin folds (triceps, subscapular, supraspinale, abdominal, front thigh, and medial calf) and five girths (arm relaxed, forearm, chest, thigh, and calf) were measured in a cross‐sectional study of children aged 6 to 11 years with cystic fibrosis.ResultsThe children with cystic fibrosis were shorter and lighter for their age and gender than those in the reference groups. The mean weight and height z scores for the girls with cystic fibrosis were lower than those for the boys, significantly so for z weight (P < 0.05). Although, the mean percent ideal body weight value of 98.6% suggested that the children with cystic fibrosis were adequately nourished, most of the measures of muscularity and adiposity of the children with cystic fibrosis were significantly lower than those of the reference group (P < 0.05). The z scores of the anthropometric measures revealed that the deficit in muscularity of the children with cystic fibrosis was relatively much greater than the deficit in adiposity.ConclusionsThe percent ideal body weight index does not seem to be an adequate measure of nutritional status in children with cystic fibrosis. Anthropometric assessments should include skin‐fold and circumference measurements of numerous sites on the upper and lower body, the trunk, and the limbs to detect deterioration in nutritional status early. Early detection of deficits in nutritional status may result in the adverse effects of malnutrition on height and weight, and possibly clinical status, being prevented.

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11

Ritivoiu, Mirela-Elena, Cristina Manuela Drăgoi, Dumitru Matei, Iustina Violeta Stan, Alina Crenguţa Nicolae, Mihai Craiu, Ion-Bogdan Dumitrescu, and Alina Angelica Ciolpan. "Current and Future Therapeutic Approaches of Exocrine Pancreatic Insufficiency in Children with Cystic Fibrosis in the Era of Personalized Medicine." Pharmaceutics 15, no. 1 (January 3, 2023): 162. http://dx.doi.org/10.3390/pharmaceutics15010162.

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This review presents current updates of pancreatic enzyme replacement therapy in children with cystic fibrosis based on literature published in the last decade and some special considerations regarding pancreatic enzyme replacement therapy in the era of new therapies, such as cystic fibrosis transmembrane conductance regulator modulator therapies. Few articles evaluate the efficacy of pancreatic enzyme replacement therapy in the pediatric population, and most studies also included children and adults with cystic fibrosis. Approximately 85% of cystic fibrosis patients have exocrine pancreatic insufficiency and need pancreatic enzyme replacement therapy. Fecal elastase is the most commonly used diagnostic test for exocrine pancreatic insufficiency, although this value can fluctuate over time. While it is used as a diagnostic test, it cannot be used for monitoring the effectiveness of pancreatic enzyme replacement therapy and for adjusting doses. Pancreatic enzyme replacement therapy, the actual treatment for exocrine pancreatic insufficiency, is essential in children with cystic fibrosis to prevent malabsorption and malnutrition and needs to be urgently initiated. This therapy presents many considerations for physicians, patients, and their families, including types and timing of administration, dose monitoring, and therapy failures. Based on clinical trials, pancreatic enzyme replacement therapy is considered effective and well-tolerated in children with cystic fibrosis. An important key point in cystic fibrosis treatment is the recent hypothesis that cystic fibrosis transmembrane conductance regulator modulators could improve pancreatic function, further studies being essential. Pancreatic enzyme replacement therapy is addressed a complication of the disease (exocrine pancreatic insufficiency), while modulators target the defective cystic fibrosis transmembrane conductance regulator protein. Exocrine pancreatic insufficiency in cystic fibrosis remains an active area of research in this era of cystic fibrosis transmembrane conductance regulator modulator therapies. This new therapy could represent an example of personalized medicine in cystic fibrosis patients, with each class of modulators being addressed to patients with specific genetic mutations.

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12

Bulatov, V. P., and N. V. Rylova. "COMPLEX ULTRASOUND CHARACTERISTICS OF THE PANCREAS IN CHILDREN WITH CYSTIC FIBROSIS." Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics) 63, no. 5 (November 20, 2018): 155–61. http://dx.doi.org/10.21508/1027-4065-2018-63-5-155-161.

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Objective:to evaluate the condition of the pancreas in children with cystic fibrosis using complex ultrasound.We examined 80 children from 3 to 18 years: 50 children with mixed cystic fibrosis and 30 conditionally healthy children. The complex ultrasound consisted of gray scale US, color dopplerography, energy dopplerography and pulse-wave dopplerography.Results.A complex study of such parameters as splenic vein diameter, volume and linear blood flow with postprandial response help to obtain more complete picture of pancreatic damage in children with cystic fibrosis. This highly sensitive and specific method with nutritional load has no contraindications, it is non-invasive, readily available, and it does not require large costs and opens up wide prospects in diagnosing the structural condition of the pancreas in children with cystic fibrosis.

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13

Bellini, Sarah Gunnell, Perry Chapman, Kayla Szendre, Catherine McDonald, Nicole Williams, Lauren Hopkin, Dennis Eggett, and April Dean. "Changes in handgrip strength in children with cystic fibrosis compared to children without cystic fibrosis." Clinical Nutrition ESPEN 42 (April 2021): 206–11. http://dx.doi.org/10.1016/j.clnesp.2021.01.038.

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14

Kirk, J. M., and A. Westwood. "Interpretation of Sweat Sodium Results—The Effect of Patient Age." Annals of Clinical Biochemistry: International Journal of Laboratory Medicine 26, no. 1 (January 1989): 38–43. http://dx.doi.org/10.1177/000456328902600105.

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In a retrospective study of sweat tests performed on 650 patients, sweat sodium was shown to increase with age in children without cystic fibrosis. The clearest separation of children with and without cystic fibrosis was in the youngest infants, while the highest proportion of repeatedly equivocal results was in the older children. Applying age-related reference ranges did not resolve the equivocal cases without also missing some cases of cystic fibrosis.

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15

Walker, Lynn S., Melissa B. Ford, and William D. Donald. "Cystic Fibrosis and Family Stress: Effects of Age and Severity of Illness." Pediatrics 79, no. 2 (February 1, 1987): 239–46. http://dx.doi.org/10.1542/peds.79.2.239.

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Stress and adjustment in mothers of children with cystic fibrosis was compared with that in a control group of mothers of healthy children. Mothers of children in four age groups were included: preschool, middle childhood, early adolescence, and late adolescence. Mothers of children with cystic fibrosis did not report significantly higher levels of stress than did the control group mothers; nor did they report greater feelings of inadequacy as parents. However, mothers of children with cystic fibrosis in two age groups, preschool and early adolescence, scored higher on a measure of depression than did mothers of healthy children in the same age groups. The relationship of illness severity to maternal stress and adjustment was examined in the cystic fibrosis group. The mother's subjective rating of the child's illness severity was a better indicator of her reported stress than was the Shwachman clinical rating. It appears that many mothers are able to adapt to the presence of cystic fibrosis in the family, although certain periods in the child's life and perceived increases in illness severity are associated with increased maternal distress.

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16

Panova, L. V., E. S. Ovsyankina, E. I. Kondratyeva, and E. D. Nikolaeva. "Tailored approach to the treatment of pulmonary mycobacteriosis in patients with cystic fibrosis." Voprosy praktičeskoj pediatrii 17, no. 3 (2022): 141–46. http://dx.doi.org/10.20953/1817-7646-2022-3-141-146.

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Diagnosis, treatment, and follow-up of children with cystic fibrosis and pulmonary mycobacteriosis pose a challenge due to lack of scientifically justified clinical recommendations on patient management. The article provides two clinical observations, illustrating a personalized approach to administration of antibacterial therapy to children with cystic fibrosis and bronchopulmonary comorbidity caused by nontuberculous mycobacteria. Clinical, microbiological, and radiological study results were interpreted to define treatment tactics. Key words: children, cystic fibrosis, pulmonary mycobacteriosis, nontuberculous mycobacteria

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17

Dolbnya, S. V., A. A. Dyatlova, L. Ya Klimov, E. I. Kondratyeva, I. N. Zakharova, and E. A. Enina. "Relationship between vitamin D sufficiency and interferon-γ levels in children with chronic lung diseases." Meditsinskiy sovet = Medical Council, no. 12 (July 27, 2023): 231–39. http://dx.doi.org/10.21518/ms2023-221.

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Introduction. The role and mechanism of the effect of vitamin D on the course of chronic lung diseases in children are not yet fully understood. In particular, there are not enough studies on the effect of vitamin D status on the production of interferon-γ (IFN-γ) in children with cystic fibrosis and bronchial asthma.Aim. To analyse the changes in IFN-γ levels in children with cystic fibrosis and bronchial asthma according to the serum 25(OH)D level before and during cholecalciferol supplementation.Materials and methods. A total of 114 children (aged from 3 months to 18 years old) were examined, including 51 (44.7%) children with bronchial asthma, 34 (29.8%) children with cystic fibrosis, and 29 (25.5%) children of the control group. If hypovitaminosis D was diagnosed, the children received a three-month cholecalciferol course at doses in accordance with the recommendations of the national consensus - Cystic Fibrosis: Definition, Diagnostic Criteria, Therapy and the national program - Vitamin D Deficiency in Children and Adolescents of the Russian Federation: Current Approaches to Management. The 25(OH)D and IFN-γ levels were twice determined in all children.Results. Median 25(OH)D levels in patients with cystic fibrosis and bronchial asthma did not reach the level of healthy children either before or after supplementation with cholecalciferol drugs at the recommended doses. Vitamin D intake resulted in a decrease in IFN-γ levels in healthy children from 3.07 [2.29; 4.81] pg/ml to 2.18 [1.74; 3.45] pg/ml (p < 0.05), whereas such changes were not detected in the paediatric patients with cystic fibrosis and bronchial asthma in the general population. However, after supplementation with cholecalciferol, the IFN-γ level was significantly higher in patients with cystic fibrosis and bronchial asthma - 2.86 [2.13; 3.86] pg/ml and 3.11 [0.89; 5.0] pg/ml, respectively, than in healthy children - 2.18 [1.74; 3.45] pg/ml (p < 0.05). A statistically significant decrease in IFN-γ level was observed in girls with cystic fibrosis and in healthy girls after supplementation with cholecalciferol, whereas no significant changes in IFN-γ levels were detected in the groups of boys.Conclusion. Modulation of IFN-γ levels appears to be one of the mechanisms of immunotropic effect of vitamin D on the course of chronic inflammation of infectious (cystic fibrosis) and allergic (bronchial asthma) etiology in children.

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18

Tsyunchyk, Y. G. "The early diagnosis of hepatobiliary lesions in children with cystic fibrosis." CHILD`S HEALTH 18, no. 4 (July 14, 2023): 277–82. http://dx.doi.org/10.22141/2224-0551.18.4.2023.1600.

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Background. An increase in life expectancy of patients with cystic fibrosis contributes to the formation of severe pathology of the hepatobiliary system, leading to the development of fatal biliary cirrhosis. The purpose was to prospectively assess the predictive value of a combination of serum liver enzymes, ultrasound liver parameters and transient elastography for diagnosis of clinically significant liver fibrosis. Materials and methods. We enrolled 108 children aged 0–17 years with cystic fibrosis. The fibrosis stage was determined using transient elastography on FibroScan® 502 (Echosens, France). The activity of enzymes (alanine transaminase, aspartate transaminase, alkaline phosphatase, gamma-glutamyl transferase, lactate dehydrogenase-5), ultrasound parameters of the liver at different stages of liver fibrosis have been investigated. Results. Liver fibrosis of varying severity was detected in 29.6 % of patients with cystic fibrosis (liver elasticity ranged from 5.9 to 49.0 kPa). Liver cirrhosis was observed in 14.8 % of children with cystic fibrosis. The dependence of an increase in the activity of alkaline phosphatase, gamma-glutamyl transpeptidase, lactate dehydrogenase-5 and an enlargement of the left lobe of the liver, a reduction in the k ratio of the sizes of the right and left lobes of the liver on the degree of fibrosis F1-F4 (р < 0.05) was found. Conclusions. The combined use of transient elastography FibroScan with increased activity of the alkaline phosphatase, gamma-glutamyl transpeptidase, lactatе dehydrogenase-5 and changing of ultrasound liver parameters could be used for early diagnosis of hepatobiliary lesions in cystic fibrosis. The age of a patient with cystic fibrosis over 6 years old, male gender and the presence of ΔF508 deletion in the genotype have a high positive predictive value for liver fibrosis and cirrhosis.

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19

Gardner, Lisa. "Teaching young children about cystic fibrosis." Paediatric Nursing 16, no. 4 (May 2004): 34–36. http://dx.doi.org/10.7748/paed.16.4.34.s26.

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Gardner, Lisa. "Teaching young children about cystic fibrosis." Paediatric Care 16, no. 4 (May 2004): 34–36. http://dx.doi.org/10.7748/paed2004.05.16.4.34.c917.

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21

David, Tj. "Cystic fibrosis in children and adults." Journal of the Royal Society of Medicine 104, no. 1_suppl (July 2011): 1. http://dx.doi.org/10.1258/jrsm.2011.s11110.

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David, T. J. "Cystic fibrosis in children and adults." Journal of the Royal Society of Medicine 105, no. 2_suppl (June 2012): 1. http://dx.doi.org/10.1258/jrsm.2012.12s011.

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23

Kovell, Lara C., Jiangxia Wang, Stacey L. Ishman, Pamela L. Zeitlin, and Emily F. Boss. "Cystic Fibrosis and Sinusitis in Children." Otolaryngology–Head and Neck Surgery 145, no. 1 (March 10, 2011): 146–53. http://dx.doi.org/10.1177/0194599811400816.

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24

Gerdes, Jeffrey S., and Shirley Murphy. "Cystic Fibrosis in Pueblo Indian Children." Clinical Pediatrics 24, no. 2 (February 1985): 104–6. http://dx.doi.org/10.1177/000992288502400209.

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MACDONALD, NONI E., ROBERT F. MORRIS, and PIERRE H. BEAUDRY. "Varicella in children with cystic fibrosis." Pediatric Infectious Disease Journal 6, no. 4 (April 1987): 414–16. http://dx.doi.org/10.1097/00006454-198704000-00014.

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26

Aktay, Atiye N., Steven L. Werlin, and Heidi E. Hoeppner. "Electrogastrography in children with cystic fibrosis." Gastroenterology 118, no. 4 (April 2000): A1158—A1159. http://dx.doi.org/10.1016/s0016-5085(00)80454-0.

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David, T. J. "Cystic Fibrosis in Children and Adults." Paediatric Respiratory Reviews 14 (May 2013): 1. http://dx.doi.org/10.1016/j.prrv.2013.01.004.

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David, T. J. "Cystic Fibrosis in Children and Adults." Paediatric Respiratory Reviews 15 (June 2014): 1. http://dx.doi.org/10.1016/j.prrv.2014.04.001.

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Kumru, B., N. Emiralioğlu, and H. Gökmen Ozel. "Malnutrition in children with cystic fibrosis." Clinical Nutrition 37 (September 2018): S90—S91. http://dx.doi.org/10.1016/j.clnu.2018.06.1353.

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Singh, Meenu, Rajendra Prasad, and Lata Kumar. "Cystic fibrosis in north Indian children." Indian Journal of Pediatrics 69, no. 7 (July 2002): 627–29. http://dx.doi.org/10.1007/bf02722693.

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David, T. J. "Cystic fibrosis in children and adults." Paediatric Respiratory Reviews 16 (October 2015): 1. http://dx.doi.org/10.1016/j.prrv.2015.07.005.

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David, T. J. "Cystic Fibrosis in Children and Adults." Paediatric Respiratory Reviews 20 (August 2016): 1. http://dx.doi.org/10.1016/j.prrv.2016.06.002.

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Sermet-Gaudelus, Isabelle, Muriel Le Bourgeois, Catherine Pierre-Audigier, Catherine Offredo, Didier Guillemot, Sophie Halley, Chantal Akoua-Koffi, et al. "Mycobacterium abscessusand Children with Cystic Fibrosis." Emerging Infectious Diseases 9, no. 12 (December 2003): 1587–91. http://dx.doi.org/10.3201/eid0912.020774.

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Natochin, Yury V., Alla A. Kuznetsova, Anastasia V. Prokopenko, Gregorio P. Milani, Sebastiano A. G. Lava, and Anna S. Marina. "Osmoregulation in children with cystic fibrosis." European Journal of Pediatrics 179, no. 5 (January 2, 2020): 835–38. http://dx.doi.org/10.1007/s00431-019-03550-1.

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Balfour-Lynn, I. M., K. Malbon, J. F. Burman, and S. J. Davidson. "Thrombophilia in children with cystic fibrosis." Pediatric Pulmonology 39, no. 4 (2005): 306–10. http://dx.doi.org/10.1002/ppul.20181.

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36

Liabina, N. V., O. I. Simonova, I. V. Shirokova, E. L. Semikina, E. F. Mavrikidi, I. I. Kondrakhina, Yu V. Gorinova, and V. A. Kerimova. "Significance of fructosamine in the diagnosis of carbohydrate metabolism disorders in children with cystic fibrosis." Meditsinskiy sovet = Medical Council, no. 1 (March 3, 2022): 226–34. http://dx.doi.org/10.21518/2079-701x-2022-16-1-226-234.

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Introduction. Cystic fibrosis associated (related) diabetes mellitus (CFDM) is one of the most common severe complications of cystic fibrosis. Today, the issue of the goodness of a single measurement of fasting blood glucose, glycated hemoglobin and fructosamine to assess the degree of carbohydrate metabolism disorders in children with cystic fibrosis is a subject of debate. The gold standard for diagnosing carbohydrate metabolism disorders (impaired fasting glycemia, various pre-diabetic conditions, diabetes mellitus) is the oral glucose tolerance test. Data about the diagnostic value of fructosamine for predicting CFDM aren’t dispositive. No credible studies of fructosamine in children with cystic fibrosis have been found in the Russian medical literature.Aim. To determine the diagnostic informative value of the blood fructosamine and other single-measured glycemic marker levels in the early diagnosis of carbohydrate metabolism disorders at the pre-diabetes stage in children with cystic fibrosis.Materials and methods. The results of laboratory tests and instrumental examinations of 87 children with cystic fibrosis aged 5–18 years were reviewed. A cross-sectional study of fructosamine, fasting blood sugar and glycated hemoglobin level (some patients underwent an oral glucose tolerance test), and liver fibroelastometry were also performed.Results and discussion. An increase in the fructosamine level was observed, which indicates a possible CFDM in children (p = 0.049): CFDM is highly likely, if the level of fructosamine is ≥255.5 µmol/l (reference ranges for children are 272–285 µmol/l; sensitivity = 57.1%, specificity = 85.2%). It has been found that children with cystic fibrosis and liver cirrhosis have increased fructosamine levels: cut-off = 253.0 µmol/l (Sn – 70.0%, Sp – 85.2%).Conclusion. The fructosamine level, a single determination of fasting blood glucose and glycated hemoglobin are not informative markers for the diagnosis of pre-diabetic conditions in cystic fibrosis in children. It is important to bear in mind that concomitant liver cirrhosis can affect the fructosamine levels. CFDM can be diagnosed at the lower fructosamine level, despite the generally accepted ranges.

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Diaconu, Ramona, Laura Bozomitu, Emil Anton, Paula Popovici, Carmen Anton, Daniel Timofte, Ciobica Alin, and Evelina Moraru. "Infectious and nutritional mechanisms in children with cystic fibrosis." Archives of Biological Sciences 67, no. 3 (2015): 1063–66. http://dx.doi.org/10.2298/abs140228070d.

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Cystic fibrosis is a polymorphic disease characterized by severe genetic dysfunctions. Besides the complex genetic background, most patients with cystic fibrosis also have increased susceptibility to infections and and their nutritional status is affected. Chronic pulmonary infection and gastrointestinal or nutritional abnormalities are characteristics of this disorder. Of our selected 56 subjects, 21.28% presented a pulmonary condition, and 28.57% digestive deregulation. We also observed that the infectious status in cystic fibrosis was dominated by respiratory infections (71.42%), and the main pathogens were Streptococcus. pneumoniae, Haemophilus. influenzae, Moraxella catarrhalis, Pseudomonas aeruginosa, Burkholderia cepacia, Staphylococcus aureus and Aspergillus. Additionally, it seems that while pulmonary function is strongly linked with adequate nutrition and weight gain, monitoring weight and pulmonary function are fundamental aspects in understanding the mechanisms of cystic fibrosis, and a very important parameter for a better management of this disorder. Early identification of nutrition and infectious risk factors is necessary for the effective and timely interventions that can have a positive impact on disease outcome. Infectious and nutritional aspects and interactions between the two are described. It is expected that management of cystic fibrosis will significantly improve.

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Weber, Ronja, Nathan Perkins, Tobias Bruderer, Srdjan Micic, and Alexander Moeller. "Identification of Exhaled Metabolites in Children with Cystic Fibrosis." Metabolites 12, no. 10 (October 17, 2022): 980. http://dx.doi.org/10.3390/metabo12100980.

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The early detection of inflammation and infection is important to prevent irreversible lung damage in cystic fibrosis. Novel and non-invasive monitoring tools would be of high benefit for the quality of life of patients. Our group previously detected over 100 exhaled mass-to-charge (m/z) features, using on-line secondary electrospray ionization high-resolution mass spectrometry (SESI-HRMS), which distinguish children with cystic fibrosis from healthy controls. The aim of this study was to annotate as many m/z features as possible with putative chemical structures. Compound identification was performed by applying a rigorous workflow, which included the analysis of on-line MS2 spectra and a literature comparison. A total of 49 discriminatory exhaled compounds were putatively identified. A group of compounds including glycolic acid, glyceric acid and xanthine were elevated in the cystic fibrosis group. A large group of acylcarnitines and aldehydes were found to be decreased in cystic fibrosis. The proposed compound identification workflow was used to identify signatures of volatile organic compounds that discriminate children with cystic fibrosis from healthy controls, which is the first step for future non-invasive and personalized applications.

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Werkhaven, Jay, and Lauren D. Holinger. "Bronchial Casts in Children." Annals of Otology, Rhinology & Laryngology 96, no. 1 (January 1987): 86–92. http://dx.doi.org/10.1177/000348948709600121.

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Bronchial casts were found in four children during a 2-year period at the Children's Memorial Hospital. These four children represent 0.2% of the 1,943 endoscopic cases during that time. Thirty other cases of bronchial casts have been reported previously in children 9 years of age or younger, only 12 of which were not associated with cystic fibrosis. Casts may be a complication of many diseases; cystic fibrosis, asthma, bronchitis, allergic bronchopulmonary aspergillosis, and bronchocentric granulomatosis are the most common. With the exception of cystic fibrosis, these diseases may all have an allergic pulmonary reaction as the underlying cause. In all four cases reported here, the diagnosis was made at endoscopy because the clinical picture is not dissimilar from that of bronchial foreign bodies. Management includes endoscopic removal of the cast, chest physical therapy with nebulized acetylcysteine, and an evaluation to determine the underlying condition.

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Strawhacker, MaryAnn Tapper, and Joyce Wellendorf. "Caring for Children With Cystic Fibrosis: A Collaborative Clinical and School Approach." Journal of School Nursing 20, no. 1 (February 2004): 5–15. http://dx.doi.org/10.1177/10598405040200010301.

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Earlier diagnosis and more effective treatments have improved both morbidity and mortality associated with cystic fibrosis, making regular school attendance a reality. School nurses have a unique opportunity to assist students with cystic fibrosis successfully manage their disease. Medical treatment for cystic fibrosis can be complex, leaving students and families in need of health consultation and support. The clinic and school nurse each brings a unique perspective to cystic fibrosis care management. Working to understanding perspectives across settings and looking for ways to collaborate through mutual planning and goal setting is an ideal way to support families and promote achievement of optimal health status for students.

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Leclercq‐Foucart, Jacqueline, Philippe Forget, FrançLoise Sodoyez‐Goffaux, and Ada Zappitelli. "Intestinal Permeability to [⁵¹Cr]EDTA in Children with Cystic Fibrosis." Journal of Pediatric Gastroenterology and Nutrition 5, no. 3 (May 1986): 384–87. http://dx.doi.org/10.1002/j.1536-4801.1986.tb09091.x.

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Summary: Intestinal permeability was investigated in 14 children with cystic fibrosis making use of [51Cr]EDTA as probe molecule. Ten normal young adults and 11 children served as controls. After oral administration of [51Cr]‐EDTA, 24 h urine was collected. Urinary radioactivity was calculated and results expressed as percentage of oral dose excreted in 24 h urine. Mean and SEM were as follows: 2.51 ± 0.21, 2.35 ± 0.24, and 13.19 ± 1.72 for control children, normal adults, and cystic fibrosis patients, respectively. The permeability differences between cystic fibrosis patients and either control children or control adults are significant (p < 0.001).

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Mbonda, E., P. Forget, Z. Saye, and J. Leclercq‐Foucart. "Usefulness of Random Fecal α₁‐Antitrypsin and Chymotrypsin Determinations in Children." Journal of Pediatric Gastroenterology and Nutrition 8, no. 1 (January 1989): 85–88. http://dx.doi.org/10.1002/j.1536-4801.1989.tb09706.x.

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Random fecal chymotrypsin activity and fecal α1‐antitrypsin (FA‐1‐AT) concentrations were determined in 11 children with cystic fibrosis, 5 children with Crohn's disease, 9 children with chronic aspecific diarrhea, 85 children with acute gastroenteritis, and 54 control children. Cystic fibrosis patients showed only very low fecal chymotrypsin values that did not overlap with values obtained in patients with either acute or chronic diarrhea. When compared with our control group, a significant increase of FA‐1‐AT concentrations was found only in children with Crohn's disease. Normal values were found in all patients with either chronic aspecific diarrhea or cystic fibrosis, while 12 of 85 children with acute gastroenteritis showed FA‐1‐AT concentrations above the 95th percentile of control children. We conclude that diarrhea (either acute or chronic) does not significantly decrease the clinical usefulness of fecal chymotrypsin activity measurements in the diagnosis of pancreatic insufficiency, while acute (gastroenteritis) but not chronic (chronic aspecific diarrhea, cystic fibrosis) diarrhea can give rise to protein losing and FA‐1‐AT concentrations similar to those found in Crohn's disease.

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Kondratyeva, E. I., A. I. Tlif, A. Yu Voronkova, E. L. Amelina, N. Yu Kashirskaya, S. A. Krasovsky, M. A. Starinova, and T. L. Kuraeva. "Characteristics of patients with cystic fibrosis-dependent diabetes mellitus in childhood according to the register of patients with cystic fibrosis of the Russian Federation in 2021." Diabetes mellitus 26, no. 5 (October 23, 2023): 418–26. http://dx.doi.org/10.14341/dm13064.

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BACKGROUND: In recent decades, the life expectancy of patients with CF has increased, which leads to an increase in the frequency of conditions associated with impaired carbohydrate metabolism.AIM: to analyze the impact of cystic fibrosis-associated diabetes mellitus (CFDM) in children and adolescents on the course of cystic fibrosis according to the 2021 register of patients with cystic fibrosis of the Russian Federation (RF).MATERIALS AND METHODS: the data of the register of patients with cystic fibrosis of the Russian Federation for 2021 were analyzed. The study included 122 patients with diabetes mellitus requiring the use of insulin — 33 children (27%) and 89 adults (73%), the average age of children was 13.5±4.1, the average age of adult.patients aged 18 years and older was 27.4±6.6. To compare the course of cystic fibrosis in patients with and without diabetes mellitus, groups were formed that were comparable in age, gender, genotype — the group of patients without diabetes included 827 patients, 33 patients made up the group of patients with CFRD using insulin. Diagnostic criteria, indicators of respiratory function, microbiological status, nature of complications, volume of therapy were compared.RESULTS: Patients with CFRD have a lower FEV1 compared to children without diabetes mellitus — M±SD FEV1 (%) 85.2±27.5 in the group of patients without diabetes mellitus and M±SD FEV1 (%) 72.4±26.0 in patients with diabetes mellitus (p < 0.016), compared in the microbiological seeding groups — a trend towards more frequent chronic growth of Pseudomonas aeruginosa 54.50% versus 39.4% in the group without CFRD. An increase in MRSA was also more often detected — 9.1% compared with the group of children without diabetes — 3.1%. Antibacterial therapy is more commonly used — inhaled in 54.6% of patients without diabetes mellitus while children with CFRD received inhaled antibiotic therapy in 75.8% (p=0.017). There was a significant difference in the used antibacterial tablet therapy (p=0.013). A significant difference in the number of patients on oxygen therapy in the group with CFRD — 12.1%, versus 3.4% without CFRD (p=0.01) confirms a more severe course of cystic fibrosis in patients with CFRD.CONCLUSION: The prevalence of CFDM with the need for insulin therapy among children in the Russian Federation is 1.3%. Cystic fibrosis-associated diabetes mellitus significantly worsens the course of cystic fibrosis in terms of lung function, the growth of gram-negative and resistant flora, the presence of severe complications and the frequent use of antibiotic therapy, which is obviously associated with frequent exacerbations of the bronchopulmonary process in cystic fibrosis in patients with developed cystic fibrosis-associated diabetes mellitus.

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Chaudhry, Sunil. "Cystic Fibrosis: Improving quality of life." Journal of Community Health Management 8, no. 2 (July 15, 2021): 91–96. http://dx.doi.org/10.18231/j.jchm.2021.021.

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Cystic Fibrosis (CF) or Mucoviscidosis is an inherited condition. In cystic fibrosis transmembrane conductance regulator (CFTR) protein does not functions properly i.e regulation of fluids and salts outside the cells. Cystic fibrosis affects exocrine glands eg., the mucus-secreting and sweat glands in the respiratory and digestive systems. The frequency of common mutation F508del (deletion of phenylalanine residue at position 508) in children is between 19% and 34%. The estimate frequency of CF as 1:10,000 to 1:40,000 in children. There is no cure for cystic fibrosis, but treatment can reduce symptoms and complications to improve quality of life. Close monitoring and early, aggressive intervention is recommended to slow the progression of CF, which can lead to possible longer life.

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Pizzignacco, Tainá Maues Peluci, Débora Falleiros de Mello, and Regina Aparecida Garcia de Lima. "Stigma and cystic fibrosis." Revista Latino-Americana de Enfermagem 18, no. 1 (February 2010): 139–42. http://dx.doi.org/10.1590/s0104-11692010000100021.

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Cystic Fibrosis (CF), also known as Mucoviscidosis, is a chronic disease of autosomal recessive origin and so far incurable. This analysis considers some characteristics of patients and family members that indicate it is a stigmatizing disease. The CF stigma’s impact on the lives of children and adolescents can affect treatment adherence, socialization, family relationships and the formation of their life histories, with direct consequences on their quality of life.

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Laskowska-Klita, T., and M. Chełchowska. "Antioxidant status in erythrocytes of cystic fibrosis children." Acta Biochimica Polonica 48, no. 1 (March 31, 2001): 283–85. http://dx.doi.org/10.18388/abp.2001_5137.

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Activities of superoxide dismutase, catalase and glutathione peroxidase in erythrocytes of cystic fibrosis children were studied in order to estimate the severity of their deficiency. Our results point to increased susceptibility of erythrocytes of cystic fibrosis subjects to oxidative injury and indicate that the antioxidant status of patients should be carefully monitored.

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King, Paul T., Lovisa Dousha, Nadeene Clarke, Jennifer Schaefer, Rosemary Carzino, Roleen Sharma, Ken L. Wan, et al. "Phagocyte extracellular traps in children with neutrophilic airway inflammation." ERJ Open Research 7, no. 2 (March 11, 2021): 00883–2020. http://dx.doi.org/10.1183/23120541.00883-2020.

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Childhood lung infection is often associated with prominent neutrophilic airway inflammation and excess production of proteases such as neutrophil elastase (NE). The mechanisms responsible for this inflammation are not well understood. One potentially relevant pathway is the production of extracellular traps by neutrophils (NETs) and macrophages (METs). The aim of this study was to measure NET and MET expression in children and the effect of deoxyribonculease (DNase) 1 and α1-antitrypsin (AAT) on this process.We studied 76 children (median age of 4.0 years) with cystic fibrosis or chronic cough who underwent investigational bronchoscopy. NETs, METs and neutrophil elastase activity in bronchoalveolar lavage (BAL) samples were measured using confocal microscopy and functional assays. The effects of DNase 1 and AAT on NET/MET expression and neutrophil elastase activity were examined in vitro.Both subject groups had airway neutrophilia with prominent BAL production of NETs with neutrophil elastase co-expression; the mean %±standard error of the mean of neutrophils expressing NETs in the cystic fibrosis group was 23.3±2.8% and in the non-cystic fibrosis group was 28.4±3.9%. NET expression was higher in subjects who had detectable neutrophil elastase activity (p≤0.0074). The percentage of macrophages expressing METs in the cystic fibrosis group was 10.7±1.2% and in the non-cystic fibrosis group was 13.2±1.9%. DNase 1 decreased NET/MET expression (p<0.0001), but increased neutrophil elastase activity (p≤0.0137). The combination of AAT and DNase 1 reduced neutrophil elastase activity (p≤0.0049).We observed prominent extracellular trap formation in symptomatic children with and without cystic fibrosis. This innate inflammatory response was down-regulated by a combination of currently available therapeutics.

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Machura, Edyta, Katarzyna Ziora, Maria Szczepańska, Elżbieta Świętochowska, Franciszek Halkiewicz, Małgorzata Barć-Czarnecka, and Dariusz Ziora. "Serum levels of chemerin, omentin and vaspin in children with cystic fibrosis." Pediatric Endocrinology 16, no. 4 (2017): 255–62. http://dx.doi.org/10.18544/ep-01.16.04.1679.

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Huang, Ting, Caihong Li, and Daishun Liu. "Recurrent Cough and Expectoration for 10 Years: A Case Report." Global Pediatric Health 6 (January 2019): 2333794X1983372. http://dx.doi.org/10.1177/2333794x19833725.

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Rationale. Most cases of cystic fibrosis occur in Europe, with only a few occurring in Asia. Pulmonary cystic fibrosis is not a rare disease, but in children it is a potentially life-threatening condition. Children suffering from pulmonary cystic fibrosis rarely survive to adulthood, and responses to treatment are generally poor. The most common cause of cystic fibrosis is a genetic mutation on chromosome 7. Patient concerns. A 15-year-old boy with healthy parents suffered from a recurrent cough and expectoration for nearly 10 years. Six years previously, a definitive diagnosis of pulmonary cystic fibrosis and hepatic cirrhosis was made at the Beijing Children’s Hospital. The first occurrence of hematemesis occurred 1 year ago. The main symptoms, which caused this period of hospitalization, were cough, expectoration, and hematemesis. Diagnoses. The underlying cause was finally determined to be the cystic fibrosis transmembrane conductance regulator gene (p.G970D). After genetic and sweat testing performed at the Beijing Children’s Hospital in 2012, a definitive diagnosis of cystic fibrosis was made. Interventions. The patient was administered hemostatic treatment, antibiotics, and cough relief and sputum reduction therapy. Outcomes. The patient’s condition rapidly improved and continued to remain stable, though future relapse is possible following respiratory tract infections. Lessons. This case indicates that in the case of any child that presents a recurrent cryptogenic cough and expectoration, whether accompanied by hematemesis or not, pulmonary cystic fibrosis should be considered. In order to determine underlying causes and prepare for cystic fibrosis transmembrane conductance regulator modulator therapy, genetic and sweat testing are recommended to be conducted if available.

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Skachkova, T. S., E. V. Kniazeva, E. N. Goloveshkina, T. V. Tronza, E. I. Kondratyeva, A. Y. Voronkova, and V. G. Akimkin. "The Prevalence of Genetic Determinants of Antibiotic Resistance, which are of Particular Epidemiological Consequences, in the Microbiota of the Oropharyngeal Swabs in Patients with Cystic Fibrosis." Epidemiology and Vaccinal Prevention 22, no. 4 (September 20, 2023): 44–48. http://dx.doi.org/10.31631/2073-3046-2023-22-4-44-48.

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Relevance. Antibiotic resistance of microorganisms can contribute to the chronicity of the inflammatory process, lead to an increase in the cost of treating patients and make it difficult to eradicate the pathogen. Patients with cystic fibrosis constantly require medical supervision, regular visits to medical institutions, and therefore there is a high risk of infection with nosocomial antibiotic- resistant strains. In addition, the necessary intake of antibacterial drugs provides an advantage for the reproduction of resistant microorganisms.Aim. Comparison of the frequency of detection of antibiotic resistance determinants in oropharyngeal swabs in children with cystic fibrosis and conditionally healthy children using molecular biological methods.Materials and methods. A PCR study of oropharyngeal discharge from 100 children with cystic fibrosis and 100 children from the control (healthy comparison subject) group was performed. Genetic antibiotic resistance locus: metallo-b-lactamases of the VIM, IMP and NDM groups; carbapenemase genes of the KPC and OXA-48 groups; extended-spectrum beta-lactamase genes of the CTX-M group and the mecA gene were detected by polymerase chain reaction (PCR) with hybridization-fluorescence detection.Results and discussion. As a result of the analysis, a statistically significant increase in the frequency of detection of genetic determinants of antibiotic resistance in the microbiota of the oropharyngeal discharge in children with cystic fibrosis was found compared with healthy children (p<0.001). The chances of detecting antibiotic resistance loci in the discharge of the oropharynx among children with cystic fibrosis are 38.5 times higher than among healthy children (95% CI: 5.1-289.5). In 28% of children with cystic fibrosis, DNA of the genetic determinants of antibiotic resistance was detected in the microbiome of the discharge of the oropharynx. A high percentage of the presence of genetic determinants of antibiotic resistance may be the reason for the ineffectiveness of antibiotic therapy.Conclusion. Due to the high occurrence in the microbiome of the oropharyngeal discharge of patients with cystic fibrosis of genetic antibiotic resistance locus that are of particular clinical and/or epidemiological significance, and the high risk of the spread of antibiotic-resistant strains outside medical institutions, it is necessary to include this group of patients in regular epidemiological monitoring.

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