Growth pattern in children with beta-thalassemia major and its relation with serum ferritin, IGF1 and IGFBP3

Abstract

Objectives: Growth impairment in children with Beta-thalassemia major (BTM) has several possible etiologies including excess iron overload and endocrinologic abnormalities. We aimed to assess growth in children with BTM and its relation with serum ferritin, thyroid hormones, IGF1 and IGFBP3. Materials and methods: Thirty-three children with BTM and 30 healthy children (control group) matched in age, sex and height were subjected to full clinical history and examination, including anthropometric measurements. Fasting blood samples from both groups were taken for complete blood counts, fasting blood sugar, liver function tests, serum ferritin, thyroid profiles, IGF1 and IGFBP3. Bone ages for both groups were determined radiologically. Results: A total of 57.6% of BTM group had retarded linear growth and 45.5% of them were with delayed puberty. There was statistical significant decrease in upper/lower segment (U/L) ratio in the BTM group compared to the control group (p=0.035). No apparent hypothyroidism was found in the thalassemia group, but significant decreases were found in both IGF1 and IGFBP3 levels of the thalassemia group compared with the control group (p=0.022 and p=0.037, respectively). There was a significant correlation between T4 and U/L ratio (p

Keywords

• Beta-thalassemia major, children, growth, thyroid profile, IGF1, IGFBP3, ferritin

Growth pattern in children with beta-thalassemia major and its relation with serum ferritin, IGF1 and IGFBP3

Öz

Amaç: Beta-talasemi major\'lu (BTM) çocuklarda büyüme geriliği aşırı demir yükü ve endokrinolojik anormallikleri de içeren birçok muhtemel etyoloji nedeniyledir. Biz BTM\'li çocuklarda büyümeyi ve serum ferritin, tiroid hormonları, IGF1 ve IGFBP3 ile ilişkisini araştırmayı amaçladık. Gereç ve yöntem: Beta-talasemi majorlu 33 çocuk ve yaş, cinsiyet ve boy bakımından eşleştirilmiş 30 sağlıklı kontrol grubu çocuk için tam bir öykü ve fizik muayene yapılarak, antropometrik ölçümleri alındı. Her iki grupta açlık kan örnekleri alınarak tam kan sayımı, açlık kan şekeri, karaciğer fonksiyon testleri, serum ferritin, tiroid profile, IGF1 ve IGFP3 düzeyleri çalışıldı. Her iki grubun kemik yaşı radyolojik olarak belirlendi. Bulgular: Beta-talasemi major grubunda boy kısalığı %57.6 ve gecikmiş puberte % 45.5 oranında saptandı. Üst/Alt ekstremite oranı BTM grubunda kontrollere nazaran anlamlı düşük bulundu (p=0.035). Talasemi grubunda açık hipotiroidi görülmedi ancak BTM grubunda IGF1 ve IGFBP3 düzeyleri kontrollerden anlamlı düşük bulundu (sırasıyla, p=0.022 ve p=0.037). T4 ile üst/alt ekstremite oranı arasında anlamlı bağıntı mevcuttu. IGF1, boy, transfüzyon süresi ve şelasyon süresi ile anlamlı ilişkili bulundu. Serum ferritin ile diğer değişkenler arasında anlamlı bir korelasyon saptanmadı. Sonuç: Beta-talasemi major\'lu çocuklarda düzenli transfüzyonlar ve şelasyon tedavisine ragmen özellikle büyük çocuklarda büyüme geriliği belirgindir ve bunun nedeni asıl olarak büyüme hormone-IGF1 ve IGBP3 eksenindedir.

Anahtar Kelimeler

• Beta-talasemi major, çocuklar, büyüme, tiroid fonksiyonları, IGF1, IGFBP3, ferritin

Kaynakça

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