الفهرس | Only 14 pages are availabe for public view |
Abstract SUMMARY ron over load produces toxic build up in many organs, including liver, endocrine glands, and heart (Vogiatzi et al., 2009). Deposition of iron in the anterior pituitary gland may cause hypogonadotropic hypogonadism and growth hormone deficiency, leading to delayed puberty as the most frequent endocrine complication of beta thalassemic patients (Jensen et al., 1997). In this cross sectional, case-control study we have evaluated 60 patients with β-thalassemia major who were regularly attending the Pediatric Hematology Clinic, Ain Shams University, In the period from April 2011 to January 2012 with mean age (13.55±1.34 years), they were compared to 40 healthy individuals of same age & sex groups served as the control group. Our patients were divided according to their compliance to iron therapy into 2 groups; Group A: compliant to iron chelating therapy and Group B: Non-compliant to iron chelating therapy. Those with other causes of delayed puberty were excluded from our study. After obtaining a verbal consent from the included subjects they were subjected to careful history taking and a full physical examination including pubertal assessment by Tanner I Summary 92 scoring scale (Tanner et al., 1966). In addition to serum ferritin level in the last three months and evaluating the ferritin trend in the past three years. All our patients were subjected to brain MRI & previously done LIC. The aim of our study is to reveal abnormal brain MRI findings in patients in patients with beta-thalassemia major & uncover relation between MRI findings & iron overload in those patients. Our results showed that weight, height and BMI were significantly lower in cases compared to control. As regards laboratory data, Hb level was significantly lower, Serum ferritin level was significantly higher in cases compared to control. Significant correlation was found between serum ferritin & liver iron concentration (LIC) dry tissue. Results revealed that puberty was significantly delayed in cases compared to control. In the present study we found that delayed puberty was significantly higher in patients treated with DFO & DFX., while normal puberty was significantly higher in patients treated with DFP & combined therapy. Summary 93 Our study showed that cases with delayed puberty had significantly higher serum ferritin level compared to cases with normal puberty. Our MRI results showed that pituitary height and signal intensity were significantly reduced in cases compared to control. Additionally, cases with delayed puberty showed significantly reduced pituitary height compared to cases with normal puberty. Also cases with delayed puberty showed more reduction in signal intensity of anterior pituitary gland compared to cases with normal puberty. Our results showed no significant association between MRI findings of reduced pituitary height and signal intensity with serum ferritin. Our results showed no significant difference between cases with normal pituitary height & cases with reduced pituitary height as regards puberty. In the present study we showed that none of our patients had silent brain infarct or frontal lobe atrophy. While 78.3% of thalassemic patients had iron deposition in choroid plexus |