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Abstract
CYSTIC FIBROSIS RELATED DIABETES: CLINICAL CASES IN TERMS OF PRINCIPLES OF PEDIATRIC PALLIATIVE CARE
Chaychenko T., Gonchar M., Muratov G., Kharkova M., Rybka O., Buginskaya N., Shulga N., Lutay T., Dolgareva S., Bashkirova N.
Cystic fibrosis related diabetes (CFRD)- is the most common comorbidity in people with cystic fibrosis , occurring in 20% of adolescents and 40–50% of adults. According to the Guidetothe Development of Children's Palliative Care Services life-limiting conditions may be delineated at four broad groups. Cystic fibrosis is a problem from group 2 - conditions where there may be long periods of intensive treatment aimed at prolonging life and allowing participation in normal childhood activities, but premature death is still possible.
Basic aspects of CFRD were discussed, such as cause of the disease, genetic defects, differential diagnosis and principles of treatment. Two clinical cases of pediatric CFRD patients resented to highlight problems of management in terms of potential palliative care.
According to our observations, patients with the CFRD, unfortunately, are perceived as patients with cystic fibrosis with no clear attention to diabetes. Both of them were presented with pure diabetes control due to unclear understanding CFRD with concomitant diet incompliance, irregular records, missed insulin injections, exercising limitations.
We concluded that CFRD patients must be assessed by members of multidisciplinary team at CF centers, working by the principle of palliative care. There is necessary since the moment of CF diagnosing to involve endocrinologist and dietician due to of endocrine problems. Psychologist, social worker and well-trained educators are necessary to increase awareness of the CF comorbidities problem.
Key words: Cystic fibrosis, cystic fibrosis related diabetes, pediatric palliative care, multidisciplinary team
ДІАБЕТ, АСОЦІЙОВАНИЙ З МУКОВІСЦИДОЗОМ: КЛІНІЧНІ ВИПАДКИ З ТОЧКИ ЗОРУ ПРИНЦИПІВ ПЕДІАТРИЧНОЇ ПАЛІАТИВНОЇ ДОПОМОГИ
Т. Чайченко, М. Гончарь, Г. Муратов, М. Харькова, О. Рибка, Н. Бужинская, Н. Шульга, Т. Лутай, С. Долгарева, Н. Башкірова
Цукровий діабет асоційований з муковісцидозом - найбільш поширене ускладнення у осіб, що страждають на муковісцидоз, яке реєструється у 20% підлітків та 40-50 % дорослих. Відповідно до рекомендацій з надання паліативної допомоги дітям, умови для обмеження життя можуть бути визначені у чотирьох широких групах. Муковісцидоз є проблемою, що відноситься до другої групи - стани, при яких передчасна смерть неминуча, але тривале інтенсивне лікування може збільшити тривалість життя дитини і дозволить зберегти якість і фізичну активність.
В статті обговорено основні поняття про цукровий діабет асоційований з муковісцидозом, такі як причини захворювання, генетичні дефекти, диференційний діагноз та принципи лікування. Два клінічних випадки педіатричних пацієнтів розглянуто з метою вивчення проблем лікування з точки зору потенційної паліативної допомоги.
Згідно з нашими спостереженнями, пацієнти з цукровим діабетом асоційованим з муковісцидозом, сприймаються як пацієнти з муковісцидозом без чіткої уваги до діабету. Обидва пацієнти не мали адекватного глікемічного контролю із-за нечіткості розуміння проблеми, що призводило до недотримання дієти, нерегулярності записів у щоденниках самоконтролю, пропущені ін'єкцій інсуліну.
Ми прийшли до висновку, що пацієнти з цукровим діабетом асоційованим з муковісцидозом мають спостерігатися членами міждисциплінарної команди в спеціалізованих центрах, що функціонують за принципом паліативної допомоги. Починаючи з моменту діагностики муковісцидозу до таких команд необхідно залучати ендокринолога та дієтолога із-за високого ризику ендокринних розладів. Психолог, соціальний працівник та добре навчені педагоги необхідні для підвищення обізнаності про проблему та супутні захворювання.
Ключові слова: муковісцидоз, цукровий діабет асоційований з муковісцидозом, дитяча паліативна допомога, міждисциплінарна команда
ДИАБЕТ, АССОЦИИРОВАННЫЙ С МУКОВИСЦИДОЗОМ: КЛИНИЧЕСКИЕ СЛУЧАИ С ТОЧКИ ЗРЕНИЯ ПРИНЦИПОВ ПЕДИАТРИЧЕСКОЙ ПАЛІАТИВНОЙ ПОМОЩИ
Т. Чайченко, М. Гончарь, Г. Муратов, М. Харькова, О. Рыбка, Н. Бужинская, Н. Шульга, Т. Лутай, С. Долгарева, Н. Башкирова
Сахарный диабет, ассоциированный с муковисцидозом - наиболее распространенное осложнение у лиц, страдающих муковисцидозом, которое регистрируется у 20 % подростков и 40-50 % взрослых. В соответствии с рекомендациями по оказанию паллиативной помощи детям, условия для ограничения жизни могут быть определены в четырех широких группах. Муковисцидоз является проблемой, которая относится ко второй группе - состояния, при которых преждевременная смерть неизбежна, но длительное интенсивное лечение может увеличить продолжительность жизни ребенка и позволит сохранить качество и физическую активность.
В статье обсуждены основные понятия о сахарном диабете ассоциированного с муковисцидозом, такие как причины заболевания, генетические дефекты, дифференциальный диагноз и принципы лечения. Два клинических случая педиатрических пациентов рассмотрено с целью изучения проблем лечения с точки зрения потенциальной паллиативной помощи.
Согласно нашим наблюдениям, пациенты с сахарным диабетом ассоциированным с муковисцидозом, воспринимаются как пациенты с муковисцидозом без четкой внимания к диабету. Оба пациента не имели адекватного гликемического контроля из-за нечеткости понимания проблемы, что приводило к не соблюдению диеты, не регулярности записей в дневниках самоконтроля, пропущенных инъекций инсулина.
Мы пришли к выводу, что пациенты с сахарным диабетом ассоциированным с муковисцидозом должны наблюдаться членами междисциплинарной команды в специализированных центрах, которые функционируют по принципу паллиативной помощи. Начиная с момента диагностики муковисцидоза к таким командам необходимо привлекать эндокринолога и диетолога из-за высокого риска эндокринных расстройств. Психолог, социальный работник и хорошо обученные педагоги необходимы для повышения осведомленности о проблеме и сопутствующих заболеваниях.
Ключевые слова: муковисцидоз, сахарный диабет ассоциированный с муковисцидозом, детская паллиативная помощь, междисциплинарная команда
References
World Health Organization. WHO Definition of Palliative Care. Retrieved from: http://www.who.int/cancer/palliative/definition/en/
World Health Organization: WHO definition of palliative care. (2012). Retrieved from: www.who.int/cancer/palliative/definition/en/
Caruso Brown, A. E., Howard, S. C., Baker, J. N., Ribeiro, R. C., & Lam, C. G. (2014). Reported Availability and Gaps of Pediatric Palliative Care in Low- and Middle-Income Countries: A Systematic Review of Published Data. Journal of Palliative Medicine, 17(12), 1369–1383. Retrieved from: http://doi.org/10.1089/jpm.2014.0095
World Health Organization: Strengthening palliative care as a component of integrated treatment within the continuum of care. (2014). Retrieved from: http://apps.who.int/gb/ebwha/pdf_files/EB134/B134_R7-en.pdf (Last accessed February1, 2014)
Gonchar', M.O., Ryga, O. O., Pen'kov, A. Ju. (2016). Pryncypy nadannja paliatyvnoi' dopomogy ditjam . [Principles of Palliative Care for Children ]. Harkiv: HNMU, 112 , 18.
A Guide to the Development of Children's Palliative Care Services. Report of a Joint Working Party of the Association for Children with Life-threatening or Terminal Conditiona and their Families and the Royal College of Paediatrics and Child Health. (1997). Bristol—London.
Retrieved from: http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Expert=586
Kapranov, N.I., Kashirskaja, N. Ju., Petrova, N.V. (2004). Medicinskaja genetika"«Mukoviscidoz. Dostizhenija i problemy na sovremennom jetape» [Medical genetics "" Mucoviscidosis: achievements of the problem at the present stage "Children]. Moskva: GUMGNCRAMN, №9., 398-412.
Marshall, B. C., Butler, S.M., Stoddard, M., Moran, A.M., Liou, T.G., Morgan, W.J., Pediatr, J. (2005). Epidemiology of cystic fibrosis-related diabetes. 146(5):681, 7.
Moran, A., Dunitz, J., Nathan, B., Saeed, A., Holme, B., Thomas, W. (2009). Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality. Diabetes Care, 32(9):1626, 31.
Moran, A., Brunzell, C., Cohen, R. C., et al. (2010). Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes: A position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care, 33(12):2697-2708. doi:10.2337/dc10-1768.
Ooi, C. Y., Durie, P.R. J. (2012). Cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations in pancreatitis. Cyst Fibros, 11(5):355-62.
Yi,Y., Norris, A.W., Wang, K., et al. (2016). Abnormal Glucose Tolerance in Infants and Young Children with Cystic Fibrosis. American Journal of Respiratory and Critical Care Medicine, 194(8):974-980. doi:10.1164/rccm.201512-2518OC.
Classification and Diagnosis of Diabetes. American Diabetes Association.Diabetes Care. (2017).40(Suppl 1):S11-S24.
Rana, M., Munns, C.F., Selvadurai, H., Donaghue, K. C., Craig, M. E. (2010). Cystic fibrosis-related diabetes in children—gaps in the evidence? Nature Reviews Endocrinology , V.6, 371–378. doi:10.1038/nrendo.2010.85
Kerem, E., Conway, S., Elborn, S., Heijerman, H. (2005). Standarty terapii bol'nyh mukoviscidozom: Evropejskij consensus. [Standards of care for patients with cystic fibrosis: a European consensus]. For the Consensus Committee Department of Pediatrics and CF center, Mount Scopus, Jerusalem 91240, Israel Journal of сystic fibrosis, 4 7–26
Belle-van, Meerkerk, G., de Valk, H. W., Stam-Slob, M.C., et al. (2016). Cystic Fibrosis-Related Diabetes with strict glycaemic control is not associated with frequent intravenous antibiotics use for pulmonary infections. Diabetes Res ClinPract, 116:230.
Moran, A., Brunzell, C., Cohen, R.C., Katz, M., Marshall, B. C., Onady, G., Slovis, B. (2010). Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes. the CFRD Guidelines Committee. Diabetes Care, 33 (12) 2697-2708; DOI: 10.2337/dc10-1768.
Senatorova, G.S., Tel'nova, L.G., Chernenko, L.M., Dolgareva, S.B. (2017). [On the issue of the combined course of cystic fibrosis and diabetes in children]. Materіali naukovo-praktichnoї konferencії «Aktual'nі pitannja fіzіologії, patologії ta organіzacії medichnogo zabezpechennja dіtej shkіl'nogo vіku ta pіdlіtkіv. Problemnі pitannja komorbіdnih stanіv u dіtej ta pіdlіtkіv». Harkіv.
Liou, T.G., Adler, F.R., Fitzsimmons, S.C., Cahill, B.C., Hibbs, J.R., Marshall, B.C. (2001). Predictive 5-year survivorship model of cystic fibrosis. Am J Epidemiol. 153: 345–352
Mayer-Hamblett, N., Rosenfeld, M., Emerson, J., Goss, C.H., Aitken, M.,L. (2002). Developing cystic fibrosis lung transplant referral criteria using predictors of 2-year mortality. Am J RespirCrit Care Med. 166: 1550–1555.
Moran, A., Diem, P., Klein, D.J., Levitt, M.D., Robertson, R.P. (1991). Pancreatic endocrine function in cystic fibrosis. J Pediatr, 118:715–723.
Moran, A., Brunzell, C.,Cohen, R. C., Katz, M., Marshall, B.C., Onady, G., Slovis, B. (2010). Clinical Care Guidelines for Cystic Fibrosis–Related Diabetes. the CFRD Guidelines Committee. Diabetes Care, 33 (12) 2697-2708; DOI: 10.2337/dc10-1768.
Schwarzenberg, S.J., Thomas, W., Olsen, T. W., Grover, T., Walk, D., . Moran, A. (2007). Microvascular Complications in Cystic Fibrosis–Related Diabetes. Diabetes Care, 30 (5) 1056-1061; DOI: 10.2337/dc06-1576.
Millington, K., Miller, V., Rubenstein, R. ,C., & Kelly, A. (2014). Patient and parent perceptions of the diagnosis and management of cystic fibrosis-related diabetes. Journal of Clinical & Translational Endocrinology, 1(3), 100–107. Retrieved from: http://doi.org/10.1016/j.jcte.2014.07.003
Moran, A., Pillay, K., Becker, D.J., Acerini, C.L. (2014). ISPAD Clinical Practice Consensus Guidelines 2014. Management of cystic fibrosis-related diabetes in children and adolescents Management of cystic fibrosis-related diabetes in children and adolescents. Pediatric Diabetes, (Suppl. 20): 65–76.
Kerem, E., Conway, S., Elborn, S., Heijerman, H. (2005). Consensus Committee Department of Pediatrics and CF center. Israel Journal of Cystic Fibrosis, 4 7–26.
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