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Factors Associated to Growth Disorder in Children with Thalassemia Major
Abstract
Growth disorders in short stature are often found in patients with β-thalassemia major. It is caused by several factors such as hypoxia, hemosiderosis, deficiency of nutritional intake, and micronutrient. Disorder in growth will affect the patient's quality of life. This study aims to determine the prevalence of growth disorders and analyze the factors associated with thalassemia child growth disorders. This study used an observational analytic study with a cross-sectional design on 167 patients with β-thalassemia major at the Palang Merah Indonesia Hospital, Bogor, West Java, in October–December 2018. Data was collected using a transfusion compliance questionnaire and the Morisky Medication Adherence Scale (MMAS-8), while growth was assessed using the CDC 2000 height/age curve. Data analysis used SPSS for Windows version 21.0. Of 167 subjects, 86 subjects (51.5%) were not adherent to transfusion, 97 subjects (58.1%) had low consumption of chelation iron, and 146 subjects (87.4%) had growth problems. The results of bivariate data analysis using the chi-square test for transfusion compliance and parental education on growth obtained p=0.000 and p=0.032. Likewise, for compliance with iron chelation consumption and parents' income to growth, the p value=0.000 was obtained. It was concluded that the prevalence of growth disorders was 87.4%, and there was a relationship between transfusion compliance, parental education level, parents' income, and compliance with iron chelation consumption on growth disorders in thalassemia children.
FAKTOR-FAKTOR YANG BERHUBUNGAN DENGAN GANGGUAN PERTUMBUHAN PADA ANAK TALASEMIA MAYOR
Gangguan pertumbuhan berupa perawakan pendek sering ditemukan pada penderita talasemia β mayor. Hal ini disebabkan oleh beberapa faktor seperti hipoksia, hemosiderosis, kekurangan asupan nutrisi, dan mikonutrien. Gangguan pertumbuhan akan memengaruhi kualitas hidup pasien. Penelitian ini bertujuan mengetahui prevalensi gangguan tumbuh kembang dan menganalisis faktor-faktor yang berhubungan dengan gangguan tumbuh kembang anak talasemia. Penelitian ini menggunakan jenis penelitian observasional analitik dengan desain cross-sectional pada 167 pasien talasemia β mayor di RS Palang Merah Indonesia, Bogor, Jawa Barat pada bulan Oktober–Desember 2018. Pengumpulan data dilakukan dengan menggunakan kuesioner kepatuhan transfusi dan Morisky Medication Adherence Scale (MMAS-8), sedangkan pertumbuhan dinilai menggunakan kurva tinggi/usia CDC 2000. Analisis data menggunakan SPSS for Windows versi 21.0. Dari 167 subjek, 86 subjek (51,5%) tidak patuh pada transfusi, 97 subjek (58,1%) memiliki konsumsi kelasi besi rendah, dan 146 subjek (87,4%) mengalami gangguan pertumbuhan. Hasil analisis data bivariat menggunakan uji chi-square untuk kepatuhan transfusi dan pendidikan orangtua tentang pertumbuhan diperoleh p=0,000 dan p=0,032. Begitu pula untuk kepatuhan konsumsi kelasi besi dan pendapatan orangtua terhadap pertumbuhan diperoleh p=0,000. Disimpulkan bahwa prevalensi gangguan tumbuh kembang sebesar 87,4% dan terdapat hubungan kepatuhan transfusi, tingkat pendidikan orangtua, pendapatan orangtua, dan kepatuhan konsumsi kelasi besi dengan gangguan tumbuh kembang anak talasemia.
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Galanello R, Origa R. Beta-thalasssemia. Orphanet J Rare Dis. 2010;5:11.
Batubara JRL, Faisal. Perawakan pendek. In: Batubara JRL, Trijaya B, Pulungan AB, editors. Buku ajar endrokrinologi anak. 2nd Edition. Jakarta: Badan Penerbit Ikatan Dokter Anak Indonesia; 2017. p. 30–9.
World Health Organization. The global burden of disease. 2012 Update. Geneva: World Health Organization; 2012 [cited 2019 June 20]. Available from: http://www.who.int/healthinfo/global_burden_disease/GBD_reportupdate_full.pdf.
Kementerian Kesehatan Republik Indonesia. Hari talasemia sedunia 2019: putuskan mata rantai talasemia mayor [Internet]. Jakarta: Kementerian Kesehatan Republik Indonesia; 2019 May 8 [cited 2019 June 20]. Available from: http://p2ptm.kemkes.go.id/kegiatan-p2ptm/pusat-/hari-talasemia-sedunia-2019-putuskan-mata-rantai-talasemia-mayor.
Rachmilewitz EA, Giardina PJ. How I treat thalassemia. Blood. 2011;118(13):3479–88.
Bhala AK, Marwaha RK, Kau H. Longitudinal growth attainments of transfusion dependent beta-thalassemia children. Mankind Quarterly. 2008;48(4):484–93.
Made A, Ketut A. Profil pertumbuhan, hemoglobin pre-transfusi, kadar feritin, dan usia tulang anak pada thalassemia mayor. Sari Pediatri. 2011;13(4):299–304.
Robbiyah N, Hakimi, Deliana M, Mayasari S. Gangguan pertumbuhan sebagai komplikasi talasemia mayor. MKN. 2014;47(1):44–50.
Gomber S, Dewan P. Physical growth patterns and dental caries in thalassemia. Indian Pediatr. 2006;43(12):1064–9.
Auda R, Idjradinata PS, Djais JTB. Risk factors to growth retardation in major thalassemia. MKB. 2011;43(1):21–5.
Rathaur VK, Imran A, Pathania M. Growth pattern in thalassemic children and their correlation with serum ferritin. J Family Med Prim Care. 2020;9(2):1166–9.
Kurtoglu AU, Kurtoglu E, Temizkan AK. Effect of iron overload on endocrinopathies in patients with beta-thalassaemia major and intermedia. Endokrynol Pol. 2012;63(4):260–3.
Sanctis VD, Soliman AT, Elsedfy H, Skordis N, Kattamis C, Angastiniotis M, et al. Growth and endocrine disorders in thalassemia: the international network on endocrine complications in thalassemia (I-CET) positon statement and guidelines. Indian J Endocrinol Metab. 2013;17(1):8–18.
Jaya IK, Sari DS, Zen NF. Gambaran usia tulang pada pasien thalassemia dengan perawakan pendek di Bagian Ilmu Kesehatan Anak RSUP Dr. Moh Hoesin Palembang. JKK. 2015;2(2):217–22.
Origa R, Galanello R. Pathophysiology of beta thalassaemia. Pediatr Endocrinol Rev. 2011;8 (Suppl 2):263–70.
Galanello R, Origa R. Beta-thalassemia. Orphanet J Rare Dis. 2010;5:11–5.
Wong SH, Omar J, Ismail TST. Multiple endocrinologic complications in thalassemia major. Korean J Clin Lab Sci. 2017;49(4):495–7.
Inati A, Noureldine MHA, Mansour A, Abbas HA. Endocrine and bone complications in β-thalassemia intermedia: current understanding and treatment. Biomed Res Int. 2015;2015:813098.
De P, Mistry R, Wright C, Pancham S, Burbridge W, Gangopadhayay K, et al. A review of endocrine disorders in thalassaemia. Open J Endocr Metab Dis. 2014;4(2):25–34.
Perera NJ, Lau NS, Mathews S, Waite C, Ho PJ, Caterson ID. Overview of endocrinopathies associated with β -thalassemia major. Intern Med J. 2010;40(10):689–96.
Skordis N, Kyriakou A. The multifactorial origin of growth failure in thalassaemia. Pediatr Endocrinol Rev. 2011;8(Suppl 2):271–7.
Huang KE, Mittelman SD, Coates TD, Geffner ME, Wood JC. A significant proportion of thalassemia major patient have adrenal insufficiency detectable on provocative testing. J Pediatr Hematol Oncol. 2015;37(1):54–9.
Riza M, Mulatsih S, Triasih R. Factor associated with insulin-like growth factor-1 in children with thalassemia major. Paediatr Indones. 2019;59(2):72–8.
Abu Samra O, Auda W, Kamhawy H, Al-Tonbary Y. Impact of educational programme regarding chelation therapy on the quality of life for β-thalassemia major children. Hematology. 2015;20(5):297–303.
Mettananda S, Pathiraja H, Peiris R, de Silva U, Mettananda C, Premawardhena A. Health related quality of life among children with transfusion dependent β-thalassaemia major and haemoglobin E β-thalassemia in Sri Lanka: a case control study. Health Qual Life Outcomes. 2019;17(1):137.
DOI: https://doi.org/10.29313/gmhc.v9i2.7674
pISSN 2301-9123 | eISSN 2460-5441
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