DiGeorge Sendromlu Çocuk Hastalarda Cerrahi̇ Müdahale ve Hemşi̇reli̇k Bakım Yöneti̇mi̇
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DOI:
https://doi.org/10.5281/zenodo.14237922Anahtar Kelimeler:
Çocuk Cerrahisi, DiGeorge Sendromu, Hemşirelik YönetimiÖzet
DiGeorge Sendromu (22q11.2 delesyon sendromu), kardiyovasküler anomaliler, immün yetmezlikler ve gelişimsel gecikmeler gibi çoklu organ sistemlerini etkileyen genetik bir bozukluktur. Bu sendromlu çocuk hastalarda cerrahi müdahaleler, özellikle kardiyak cerrahiler, sıklıkla gerekmekte ve bu süreçlerde multidisipliner bir yaklaşımın önemi ortaya çıkmaktadır. Bu makalede, DiGeorge Sendromlu çocuklarda cerrahi süreçlerin yönetimi ve bu süreçlerde hemşirelik bakımının rolü incelenmiştir. Makale, cerrahi öncesi ve sonrasında hemşirelerin üstlendiği rollerin yanı sıra, beslenme, immün proflaksi ve enfeksiyon riskinin yönetimi gibi kritik bakım alanlarına odaklanmaktadır. Ayrıca, sendromun karakteristik özellikleri nedeniyle gelişebilecek komplikasyonların izlenmesi ve bu komplikasyonlara yönelik proaktif hemşirelik müdahaleleri detaylandırılmıştır. Multidisipliner ekibin bir parçası olarak hemşirelerin, hastaların ve ailelerinin yaşam kalitesini artırmak, komplikasyonları önlemek ve tedavi sürecini optimize etmek için kritik bir role sahip olduğu vurgulanmaktadır. Sonuç olarak, DiGeorge Sendromlu çocuk hastaların cerrahi müdahaleleri ve bakım süreçlerinde multidisipliner bir yaklaşımın benimsenmesi, hasta sonuçlarını iyileştirmede ve komplikasyon riskini azaltmada hayati öneme sahiptir.
Referanslar
Al Habib, H. F., Jacobs, J. P., Mavroudis, C., Tchervenkov, C. I., O'Brien, S. M., Mohammadi, S., & Jacobs, M. L. (2010). Contemporary patterns of management of tetralogy of Fallot: data from the Society of Thoracic Surgeons Database. The Annals of thoracic surgery, 90(3), 813-820.
Andersen, R. D., Langius-Eklöf, A., Nakstad, B., Bernklev, T., & Jylli, L. (2017). The measurement properties of pediatric observational pain scales: A systematic review of reviews. International journal of nursing studies, 73, 93-101.
Anderson, M., Elliott, EJ., Zurynski, YA. (2013) Australian families living with rare disease: experiences of diagnosis, health services use and needs for psychosocial support. Orphanet Journal of Rare Diseases, 8(22). doi: 10.1186/1750-1172-8- 22.
Andsoy, I. I., & Alsawı, S. O. M. (2017). Determination of knowledge and anxiety levels of fathers of children who will undergo surgical intervention about surgery. Journal of Contemporary Medicine, 8(3), 264-270.
Bassett, A. S., Chow, E. W., Husted, J., Weksberg, R., Caluseriu, O., Webb, G. D., & Gatzoulis, M. A. (2005). Clinical features of 78 adults with 22q11 deletion syndrome. American journal of medical genetics Part A, 138(4), 307-313.
Biggs, S. E., Gilchrist, B., & May, K. R. (2023). Chromosome 22q11. 2 deletion (DiGeorge syndrome): immunologic features, diagnosis, and management. Current Allergy and Asthma Reports, 23(4), 213-222.
Chiu, S. N., Wang, J. K., Chen, H. C., Lin, M. T., Wu, E. T., Chen, C. A., ... & Wu, M. H. (2012). Long-term survival and unnatural deaths of patients with repaired tetralogy of Fallot in an Asian cohort. Circulation: Cardiovascular Quality and Outcomes, 5(1), 120-125.
Çalbayram, N. Ç., Altundag, S., & Aydin, B. (2016). The anxiety states of fathers of hospitalized children and its causes. Health Science Journal, 10(6), 1.
DeMarco, R. T., Casale, A. J., Davis, M. M., Yerkes, E. B., O’Neill, J. A., Grosfeld, J. L., & Caldamone, A. A. (2004). 137 Surgical Diseases of the Newborn: Urologic Disorders. Management, Procedures, On-Call Problems, Diseases, and Drugs, 892.
Frizzell, K. H., Cavanaugh, P. K., & Herman, M. J. (2017). Pediatric perioperative pain management. Orthopedic Clinics, 48(4), 467-480.
Gehdoo, R. P. (2004). Post operative pain management in paediatric patients. Indian Journal of Anaesthesia, 48(5), 406-414.
Gennery, A. R. (2012). Immunological aspects of 22q11. 2 deletion syndrome. Cellular and Molecular Life Sciences, 69, 17-27.
Goldmuntz, E. (2020). 22q11. 2 deletion syndrome and congenital heart disease. In American Journal of Medical Genetics Part C: Seminars in Medical Genetics (Vol. 184, No. 1, pp. 64-72). Hoboken, USA: John Wiley & Sons, Inc..
Groft, SC., Posada de la Paz, M. (2017). Rare diseases: joining mainstream research and treatment based on reliable epidemiological data. Advances in Experimental Medicine and Biology, 1031, 3-21. doi: 10.1007/978-3-319-67144-4_1.
Haskoloğlu, Z. Ş., & İkincioğulları, A. (2014). Chromosome 22q11. 2 Deletion Syndrome (Digeorge Syndrome/Velocardiofacial Syndrome). Turk J Immunol, 2(3), 57-66.
Karacı, A. R., Aydemir, N. A., Şaşmazel, A., Harmandar, B., Erdem, A., Yurtsever, N., ... & Bilal, MS (2012). Early and mid-term results of total truncus arteriosus correction surgery. Turkish Chest Heart Vascular Journal, 20(2), 194-199.
Lackey AE, Muzio MR. DiGeorge syndrome. StatPearls Publishing [Internet]. 2020 Jan [cited 2023 Sep].
Land, M. H., Garcia-Lloret, M. I., Borzy, M. S., Rao, P. N., Aziz, N., McGhee, S. A., ... & Stiehm, E. R. (2007). Long-term results of bone marrow transplantation in complete DiGeorge syndrome. Journal of allergy and clinical immunology, 120(4), 908-915.
Marino, B., Digilio, M. C., Toscano, A., Anaclerio, S., Giannotti, A., Feltri, C., ... & Dallapiccola, B. (2001). Anatomic patterns of conotruncal defects associated with deletion 22q11. Genetics in Medicine, 3(1), 45-48.
Markert, M. L., Devlin, B. H., Alexieff, M. J., Li, J., McCarthy, E. A., Gupton, S. E., ... & Hoehner, J. C. (2007). Review of 54 patients with complete DiGeorge anomaly enrolled in protocols for thymus transplantation: outcome of 44 consecutive transplants. Blood, 109(10), 4539-4547.
McDonald-McGinn DM, Tonnesen MK, Laufer-Cahana A, Finucane B, Driscoll DA, Emanuel BS., (2001). Phenotype of the 22q11.2 deletion in individuals identified through an affected relative: cast a wide FISHing net! Genet Med. 3:23-9.
McDonald-McGinn, D. M., & Sullivan, K. E. (2011). Chromosome 22q11. 2 deletion syndrome (DiGeorge syndrome/velocardiofacial syndrome). Medicine, 90(1), 1-18.
McGhee SA, Lloret MG, Stiehm ER. (2009). Immunologic reconstitution in 22q deletion (DiGeorge) syndrome. Immunol Res. 45:37-45.
McLean-Tooke, A., Spickett, G. P., Gennery, A. R., & Hague, R. (2008). "Immunodeficiency and autoimmunity in 22q11.2 deletion syndrome." Scandinavian Journal of Immunology, 67(6), 479-490.
Molster, C., Urwin, D., Di Pietro, L., Fookes, M., Petrie, D., van der Laan, S., Dawkins, H. (2016). Survey of healthcare experiences of Australian adults living with rare diseases. Orphanet Journal of Rare Diseases, 11(1).
Moons P, Bovijn L, Budts W, Belmans A, Gewillig M. (2010). Temporal trends in survival to adulthood among patients born with congenital heart disease from 1970 to 1992 in Belgium. Circulation 122:2264-72.
Okyay, R. D., & Ayoğlu, H. (2018). Postoperative pain management in children. Pediatric Practice and Research, 6(2), 16-25.
Ozer Z, Bahcecik AN, Caglar B, Zengiri A. (2019). Di George Syndrome and Nursing Care: Case Report. Istanbul Sabahattin Zaim University Journal of the Institute of Science. 3(1):35-39.
Ozer, S., & Ay, A. (2023). Rare Diseases and Nursing. Ege University Faculty of Nursing Journal, 39(3), 438-442.
Pelentsov, LJ., Fielder, AL., Esterman, AJ. (2016). The supportive care needs of parents with a child with a rare disease: a qualitative descriptive study. Journal of Pediatric Nursing, 31(3), e207–18. doi: 10.1016/j.pedn.2015.10.022.
Reisli I, Gokturk B. DiGeorge syndrome. Asthma Allergy Immunology. 2016;14(3):129-142.
Rosenkranz, E.R. (2001). Congenital cardiac anomalies, In: Pediatric Surgical Secrets. P.L. Glick, M.G. Caty, R.H. Pearl, M.S. Irish, Eds., Hanley and Belfus.
Scully BB, Morales DLS, Zafar F, et al. (2010). Current Expectations for Surgical Repair of Isolated Ventricular Septal Defects. Ann Thorac Surg. 89(2):544-51.
Shields L, Pratt J, Hunter J. (2006). Family centred care: a review of qualitative studies. J Clin Nurs. 15:1317–23.
Valente AM, Cook S, Festa P, Ko HH, Krishnamurthy R, Taylor AM., (2014). Multimodality imaging guidelines for patients with repaired tetralogy of fallot: A report from the American Society of Echocardiography: Developed incollaboration with the Society for Cardiovascular Magnetic Resonance and the Society for Pediatric Radiology. J Am Soc Echocardiogr. 27:111-41.
Walkowiak, D., Domaradzki, J. (2020). Needs assessment study of rare diseases education for nurses and nursing students in Poland. Orphanet Journal of Rare Diseases,15(1). doi: 10.1186/S13023-020-01432-6.
Wu, H. Y., Rusnack, S. L., Bellah, R. D., Plachter, N., Mcdonald-Mcgınn, D. M., Zackai, E. H., & Canning, D. A. (2002). Genitourinary malformations in chromosome 22q11. 2 deletion. The Journal of urology, 168(6), 2564-2565.
Yeoh, T. Y., Scavonetto, F., Hamlin, R. J., Burkhart, H. M., Sprung, J., & Weingarten, T. N. (2014). Perioperative management of patients with DiGeorge syndrome undergoing cardiac surgery. Journal of Cardiothoracic and Vascular Anesthesia, 28(4), 983-989.
Yıldırım, Ö., & Aliyev, B. (2020). Early Results After Ventricular Septal Defect Surgical Repair in Patients Over One Year Old.
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