Changes in physical development in children with cystic fibrosis as a universal mechanism of body adaptation
DOI:
https://doi.org/10.15574/SP.2024.7(143).3445Keywords:
children, cystic fibrosis, physical development, weight, height, adaptationAbstract
Cystic fibrosis (CF) is the most common monogenic disorder with an autosomal recessive inheritance pattern. The disease leads to damage to the respiratory system, pancreas, intestines, gallbladder and liver. In Ukraine, about 300 children are born with CF each year. As of 2024, there are 16 children with CF living in the Ternopil region.
Aim - to analyze the parameters of physical development in children with CF to understand the processes of adaptation and maladaptation of the child's body under conditions of chronic hypoxia, which causes dehydration.
Materials and methods. The study was conducted by analyzing the records of hospitalized 15 patients who were receiving inpatient treatment at the Ternopil Regional Clinical Children's Hospital and conducting a literature search of scientific publications from sources such as PubMed, ResearchGate, and Science.
Results. Impairment of physical development, growth and weight gain is one of the most common problems faced by children with CF. The comparison of body mass-to-height ratio (WFLz) and body mass index (BMIz) indicators was carried out as an as assessments of physical development and possible malnutrition in children with CF. Problems with gaining and maintaining weight indicate that the digestive system is not absorbing the nutrients necessary for the child's growth, disrupting adaptation processes.
Conclusions. As a result of the study, no significant impairment of the physical development of children with CF was found, in 10 (66.7%) patients physiological norms were established based on the ratio of body weight to height. As a result of the comparison of BMI indicators, 13 (86.7%) patients were within the physiological deviation range.
No conflict of interests was declared by the authors.
References
Araujo RP, Liotta LA. (2023). Universal structures for adaptation in biochemical reaction networks. Nat Commun. 14: 2251. https://doi.org/10.1038/s41467-023-38011-9; PMid:37081018 PMCid:PMC10119132
Begum N, Byrnes CA, Cheney J, Cooper PJ, Fantino E, Gailer N et al. (2022) Factors in childhood associated with lung function decline to adolescence in cystic fibrosis. J Cyst Fibros. 21(6): 977-983. https://doi.org/10.1016/j.jcf.2022.03.008; PMid:35341694
Berezenko VS, Reznikov YuP, Krat VV. (2017). Cystic fibrosis in children. Early diagnosis as important predictor of treatment efficiency (clinical case). Perinatologiya i pediatriya. 3(71): 74-80. https://doi.org/10.15574/PP.2017.71.74
Bogdani M, Blackman SM, Ridaura C et al. (2017). Structural abnormalities in islets from very young children with cystic fibrosis may contribute to cystic fibrosis-related diabetes. Sci Rep. 7(1): 17231. https://doi.org/10.1038/s41598-017-17404-z; PMid:29222447 PMCid:PMC5722914
Boiarchuk OR, Kosovska VO, Kosovska TM. (2019). Osoblyvosti perebihu mukovistsydozu v ditei Ternopilskoi oblasti. Aktualni pytannia pediatrii, akusherstva ta hinekolohii. 1: 5-9. https://doi.org/10.11603/24116-4944.2019.1.10173
Granados A, Chan CL, Ode KL et al. (2019). Cystic fibrosis related diabetes: Pathophysiology, screening and diagnosis. J Cyst Fibros. 18(2): 3-9. https://doi.org/10.1016/j.jcf.2019.08.016; PMid:31679726
Jentsch TJ, Pusch M. (2018). CLC Chloride Channels and Transporters: Structure, Function, Physiology, and Disease. Physiol Rev. 98(3): 1493-1590. https://doi.org/10.1152/physrev.00047.2017; PMid:29845874
Khan MA, Ali ZS, Sweezey N, Grasemann H, Palaniyar N. (2019) Progression of Cystic Fibrosis Lung Disease from Childhood to Adulthood: Neutrophils, Neutrophil Extracellular Trap (NET) Formation, and NET Degradation. Genes (Basel). 10(3): 183. https://doi.org/10.3390/genes10030183; PMid:30813645 PMCid:PMC6471578
Koivula FNM, McClenaghan NH, Harper AGS, Kelly C. (2016) Islet-intrinsic effects of CFTR mutation. Diabetologia. 59(7): 1350-1355. https://doi.org/10.1007/s00125-016-3936-1; PMid:27033560 PMCid:PMC4901107
Kolb S, Burchartz A, Oriwol D, Schmidt SCE, Woll A, Niessner C. (2021). Indicators to Assess Physical Health of Children and Adolescents in Activity Research-A Scoping Review. Int J Environ Res Public Health. 18(20): 10711. https://doi.org/10.3390/ijerph182010711; PMid:34682457 PMCid:PMC8535832
Kyrychok O.M. (2002). Morpho-functional features of skeletal bones in animals adapted and unadapted to dehydration in general dehydration of the organism. Vestnik naukovykh researches. 1: 118-120.
Lynn C, Muther E. (2021). Expаnding the Cystic Fibrosis Foundаtion's mentаl heаlth screening guidelines: Eаrly childhood behаviorаl heаlth аssessment for children with cystic fibrosis аged 4 to 11. J Cyst Fibros. 20; 2: S148-S149. URL: https://www.cysticfibrosisjournаl.com/аrticle/S1569-1993. https://doi.org/10.1016/S1569-1993(21)01734-3
Mason KA, Rogol AD. (2022). Trends in Growth and Maturation in Children with Cystic Fibrosis Throughout Nine Decades. Front Endocrinol (Lausanne). 13: 935354. https://doi.org/10.3389/fendo.2022.935354; PMid:35903281 PMCid:PMC9317724
Misbahuddin M. (2017). Rafeeq and Hussam Aly Sayed Murad. Cystic fibrosis: current therapeutic targets and future approaches. Rafeeq and Murad. J Transl Med. 15: 84. https://doi.org/10.1186/s12967-017-1193-9; PMid:28449677 PMCid:PMC5408469
Monasor-Ortolá D, Quesada-Rico JA, Nso-Roca AP, Rizo-Baeza M, Cortés-Castell E et al. (2021). Degree of Accuracy of the BMI Z-Score to Determine Excess Fat Mass Using DXA in Children and Adolescents. Int J Environ Res Public Health. 18(22): 12114. https://doi.org/10.3390/ijerph182212114; PMid:34831872 PMCid:PMC8625214
MOZ Ukrainy (2016). Mukovistsydoz. Unifikovanyi klinichnyi protokol pervynnoi, vtorynnoi (spetsializovanoi) ta tretynnoi (vysokospetsializovanoi) medychnoi dopomohy. Nakaz Ministerstva okhorony zdorov'ia Ukrainy vid 15.07.2016 No. 723. URL: https://dec.gov.uа/wpcontent/uploаds/imаges/dodаtki/2016_723_Mykoviscidoz/2016_723_YKPMD_Mkvsz.pdf.
Mwangome MK, Berkley JA. (2014). The reliability of weight-for-length/height Z scores in children. Matern Child Nutr. 10(4): 474-480. https://doi.org/10.1111/mcn.12124; PMid:24785183 PMCid:PMC4282477
Norris AW, Ode KL, Merjaneh L et al. (2019). Survival in a bad neighborhood: pancreatic islets in cystic fibrosis. J Endocrinol. 241(1): R35-R50. https://doi.org/10.1530/JOE-18-0468; PMid:30759072 PMCid:PMC6675675
Ntimbane T, Mailhot G, Spahis S et al. (2016). CFTR silencing in pancreatic β-cells reveals a functional impact on glucosestimulated insulin secretion and oxidative stress response. Am J Physiol Endocrinol Metab. 310(3): 200-212. https://doi.org/10.1152/ajpendo.00333.2015; PMid:26625901
Nugent K. (2023). Cystic fibrosis аnd pulmonаry biofilms. The Southwest Respirаtory аnd Criticаl Cаre Chronicles. 11; 49: 19-26. https://doi.org/10.12746/swrccc.v11i49.1233
Okhotnikova OM, Hladush YI, Ivanova TP. (2013). Cystic fibrosis in children: early diagnosis errors and their analysis. Klinichna imunolohiia. Alerhholohiia. Infektolohiia - Clinical Immunology. Allergology. Infectology. 5(64): 15-21.
Panagopoulou P, Fotoulaki M, Nikolaou A, Nousia-Arvanitakis S. (2014, Feb). Prevalence of malnutrition and obesity among cystic fibrosis patients. Pediatr Int. 56(1): 89-94. https://doi.org/10.1111/ped.12214; PMid:24003895
Pereira SV, Ribeiro JD, Bertuzzo CS, Marson FAL. (2017). Association of clinical severity of cystic fibrosis with variants in the SLC gene family (SLC6A14, SLC26A9, SLC11A1 and SLC9A3). Gene. 629: 117-126. https://doi.org/10.1016/j.gene.2017.07.068; PMid:28756021
Rafeeq MM, Murad HAS. (2017). Cystic fibrosis: current therapeutic targets and future approaches. J Transl Med. 15(1): 84. https://doi.org/10.1186/s12967-017-1193-9; PMid:28449677 PMCid:PMC5408469
Ridley K, Condren M. (2020). Elexаcаftor-Tezаcаftor-Ivаcаftor: The First Triple-Combinаtion Cystic Fibrosis Trаnsmembrаne Conductаnce Regulаtor Modulаting Therаpy. J Pediаtr Phаrmаcol Ther. 25; 3: 192-197. https://doi.org/10.5863/1551-6776-25.3.192; PMid:32265602 PMCid:PMC7134581
Rout-Pitt N, Farrow N, Parsons D, Donnelley M. (2018). Epithelial mesenchymal transition (EMT): a universal process in lung diseases with implications for cystic fibrosis pathophysiology. Respir Res. 19(1): 136. https://doi.org/10.1186/s12931-018-0834-8; PMid:30021582 PMCid:PMC6052671
Saiman L, Siegel JD, LiPuma JJ, Brown RF et al. (2014). Cystic Fibrous Foundation Infection Prevention and Control Guideline for Cystic Fibrosis: 2013 Update Recommendations for healthcare professionals. Infection Control and Hospital Epidemiology. 35; Suppl 1: S1-S67. URL: http://www.jstor.org/stable/10.1086/676882. https://doi.org/10.1086/676882
PMid:25025126
Schmidt BZ, Haaf JB, Leal T, Noel S. (2016). Cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis: current perspectives. Clin Pharmacol. 8: 127-140. https://doi.org/10.2147/CPAA.S100759; PMid:27703398 PMCid:PMC5036583
Southern KW, Addy C, Bell SC, Bevan A, Borawska U et al. (2024, Jan). Standards for the care of people with cystic fibrosis; establishing and maintaining health. J Cyst Fibros. 23(1): 12-28. https://doi.org/10.1016/j.jcf.2023.12.002; PMid:38129255
WHO Multicentre Growth Reference Study Group (2007). WHO Child Growth Standards: Head circumference-for-age, arm circumference-for-age, triceps skinfold-for-age and subscapular skinfoldfor-age: Methods and development. Geneva: World Health Organization; pp 217 http://www.who.int/childgrowth/publications
Zysman-Colman Z, Munsar Z, Sheikh S, Rubenstein RC, Kelly A. (2022). Infant Body Mass Index or Weight-for-Length and Risk of Undernutrition in Childhood Among Children with Cystic Fibrosis. J Pediatr. 243: 116-121.e3. https://doi.org/10.1016/j.jpeds.2021.11.059; PMid:34871592
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