Pemeriksaan Spirometri pada Anak Talasemia di Rumah Sakit Sardjito Yogyakarta dan Faktor Klinis yang Mempengaruhi

Azwar Aruf, dr. Amalia Setyati, Sp.A(K): dr. Bambang Ardianto, MSc, Ph.D, Sp.A(K)

2025 | Tesis-Subspesialis | SUBSPESIALIS ILMU KESEHATAN ANAK

Latar Belakang: Talasemia adalah penyakit kelainan bawaan  autosomal resesif gangguan pembentukan hemoglobin. Indonesia termasuk dalam daerah sabuk talasemia. Beberapa penelitian melaporkan adanya gangguan fungsi paru pada talasemia terutama talasemia beta mayor yang mendapatkan transfusi darah berulang. Gangguan fungsi paru pada anak talasemia belum menjadi perhatian, dikarenakan sering tidak menunjukkan gejala respiratori.

Tujuan: Mengetahui angka kejadian gangguan fungsi paru pada anak talasemia serta mengidentifikasi faktor klinis yang mempengaruhinya

Metode: Sebanyak 54 anak talasemia beta mayor usia 6-<18>forced vital capacity (FVC), forced expiratory volume 1 (FEV1), FEV1/FVC. Pasien dengan penyakit jantung, paru, dan yang tidak dapat melakukan manuver spirometri dengan baik diekslusi. Analisis bivariat, multivariat, regresi logistik dilakukan untuk mencari adakah hubungan faktor klinis dengan gangguan fungsi paru. 

Hasil: Kejadian gangguan fungsi paru dengan menggunakan batas nilai normal spirometri ?80% nilai prediksi, didapatkan hanya 1 anak (1,9%) dengan gangguan fungsi restriktif ringan, tidak didapatkan gangguan fungsi paru obstruktif maupun campuran. Subanalisis dengan menggunakan batas nilai spirometri ?90% nilai prediksi menunjukkan 18 anak (33,3%) dengan FVC <90>p=0,046), ferritin serum ?4962,5 (OR 12,875; CI: 1,174-155,299. p=0,004)

Simpulan: Gangguan fungsi paru restriktif hanya 1,9%. Faktor klinis yang dapat menjadi prediksi lebih rendahnya fungsi paru adalah usia terdiagnosis ?17,5 bulan dan ferritin serum ?4962.5 

Background: Thalassemia is an autosomal recessive inherited disorder of hemoglobin formation. Indonesia is included in the thalassemia belt area. Several studies have reported impaired lung function in thalassemia, especially beta thalassemia major who receive repeated blood transfusions. Impaired lung function in pediatric thalassemia has not been a concern, because it often does not show respiratory symptoms.

Objective: To determine the prevalence of pulmonary dysfunction and identify clinical predictors of early, subclinical lung function decline in children with transfusion-dependent ?-thalassemia major.

Method: 54 children aged 6 to < 18>Spirometry examination was performed to measure the forced vital capacity (FVC), forced expiratory volume 1 (FEV1), FEV1/FVC. Patients with known comorbid cardiopulmonary disease or who were unable to perform acceptable spirometry were excluded. Analysis bivariate, multivariate, and logistic regression analyses were conducted to find out whether there was a relationship between clinical factors and impaired lung function.

Results: Using the normal spirometry value limit ?80% of predicted value, only 1 child (1.9%) with mild restrictive function disorders, no obstructive or mixed lung function disorders were found. Subanalysis using ?90% predicted value, 18 children (33.3%) had an FVC <90>Independet t-test and Mann-Whitney result showed that median age at diagnosis in children with FVC<90>logistic regression on the subanalysis of spirometry results using the ?90% predicted value, two factors were found associated with lower FVC values: age at diagnosis ?17.5 months (OR 14.39; 95% CI: 1.242-232.2. p=0.046), serum ferritin ?4962.5 (OR 12.875; CI: 1.174-155.299. p=0.004)

Conclusions: Restrictive lung function disorder was only 1.9%. Clinical factors that could predict lower lung function were age at diagnosis ?17.5 months and serum ferritin ?4962.5

Kata Kunci : spirometry, thalassemia, children or pediatric, : spirometri, talasemia, anak