ÖZETAmaç: 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, büyü-me, tiroid fonksiyonları, IGF1, IGFBP3, ferritin ABSTRACT Objectives: Growth impairment in children with Betathalassemia 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<0.05); IGF1 was significantly correlated with height, duration of transfusions and duration of chelation (p<0.05). No significant correlation was found betw...
Symptoms suggesting occurrence of otitis media (OM) in infants and young children are not always right. Objective: Assessment of those infants and children with symptoms suggesting otitis media. Patients & Methods: 113 infants and children were complaining of ear symptoms suggesting otitis media as well as 63 infants and children of same age and sex were not complaining of any of these symptoms suggesting otitis media, were fully investigated prospectively, by full clinical and ear, nose examination as well as swab culture from the ear discharge. Results: There was no statistical significant difference between the prevalence of otitis related symptoms and the risk factors in both groups. There was no correlation found between the otoscopic diagnosis of OM and the main complaint suggesting OM in the patients, mainly ear pain and ear discharge. There was a positive significant correlation between otoscopic diagnosis of OM and fever, diarrhea, and bronchiolitis (significance was at 0.01, 0.01 and 0.05 levels respectively). Conclusion: Otoscopic examination is very important in any infant or child complaining of ear pain, ear discharge or complaining of otitis related symptoms.
We studied gall bladder contractility in 61 children with beta-thalassaemia who were asymptomatic for gall bladder disease and 51 sex-and age-matched controls in Cairo, Egypt, using real-time ultrasonography. Multiple gall bladder stones were present in 18.0% of thalassaemia patients and sludge in 6.6%. There were statistically significant differences between thalassaemia patients and controls in gall bladder fasting volume, residual volume, emptying time and contraction index. There was significant positive correlation between fasting and residual volumes and age, weight and height, and between fasting volume and body mass index and serum ferritin level. Contraction index was negatively correlated with serum total bilirubin. Impaired gall bladder motility was evident in patients with betathalassaemia and it may be related to disease duration, serum ferritin and total serum bilirubin level.
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