Dergiler / SSK Tepecik Eğitim Hastanesi Dergisi / 2003 / Cilt: 13 - Sayı: 3
Talasemi intermedia ve kemik displazisi
- Sayfa
- 155–161
- DOI
- —
Özet
Amaç: Talasemi majörlü hastalar, kemik displazileri dahil pek çok komplikasyon yönünden incelenmiş, ancak daha az transfüzyon ve şelasyon gereksinimi olan talasemi intermedia (Tl)' lı olgular iskelet displazileri yönünden araştırılmamıştır. Çalışmamızda, TI'lı olgularda kemik displazisi sıklığı ve displaziyi etkileyen faktörlerin araştırılması amaçlanmıştır. Yöntem: Yaş ortalaması 17.4 ±10.1 (8-37) yıl, desferrioxamine (DFO) başlama yaşı 3 yıl ve üzeri, DFO dozu 4 yıl, DFO dozu >30 mg/kg/G olmasının olgularda kemik displazisi geliştirme riskini arttırmadığı anlaşılmıştır. PTH, IGF-1, IGFBP-3 tüm olgularda normal bulunmuştur. Sonuç: Kemik displazilerinin TI'lı olgularda yakından izlenmesi gereken patogenezi karmaşık bir komplikasyon olduğu, vertebral displastik değişikliklerin daha erken başladığı, <50 mg/kg/G DFO dozu, süresi ve başlama yaşından bağımsız, yaşla doğru orantılı olarak artan oranlarda görüldüğü, olguların displazi yönünden yakından izlenmesi gerektiği kanısına varılmıştır.
Abstract
Aim: Up to date, thalassemia major patients have been investigated in terms of the related complications including bone dysplasia but thalessemia intermedia (TI) patients who require less transfusion and chelationhave not been studied for bone dysplasia. Our objective was to assess the frequency and the risk factors for bone dysplasia in TI patients. Methods: Twenty-one TI patients between the ages of 8-37 years, DFO age $\geq$3 yrs, DFO dose $\leq$50 mg/kg/d and with mean pretransfusion Hb and mean ferritin values 8.1$\pm$0.4 gr/dl and 2656±1579 ng/dl respectively were included in the study. The X rays of the left hand-wrist, knee and PA and lateral view vertebrae were evaluated by two radiologists simultaneously. Metaphyseal irregularity, osteoscferotic linear streaks, subchondral pseudocysts, compression of the vertebrae, marginal sclerosis, dorsal kyphosis and scoliosis mere accepted as dysplastic changes. Osteoporosis was evaluated by DEXA. Parathormone, IGF-1 and IGFBP-3 levels were analysed by radioimmunoassay. For statistical analysis, "SPSS for MS Windows Release 11.0" independent samples t-test, Mann- Whitney U, Wilcoxon Rank Sum W test, Pearson Q Square Test and odds ratio were used. Results: Metaphyseal and/or spinal bone dysplasia was found in 13 (61.9%) TI patients with mean age of 21.2$\pm$11 years. All of the patients with displasia showed vertebral dysplastic changes. Five (23.8%) patients who were $\leq$12 years of age within the study group showed only spinal dysplasia, whereas 8 (38.1%) patients who were $\geq$20 years of age showed metaphyseal dysplasia in addition to spinal dysplasia. Height SDS and sitting height SDS were found <-2.5 only in one patient and sitting height SDS was at -2.5 in two other patients. Osteoporosis and other endocrine complications accompanied dysplasia in 4 patients. Chronological age showed a statistical difference between patients with and without bone dysplasia. DFO age $\leq$5 yrs, DFO duration >4 yrs and DFO dose >30mg/kg/day did not show a statistical importance for bone dysplasia in risk analysis. Conclusion: Bone dysplasia is an important multifactorial complication seen in TI patients and must be followed up carefully. Incidence of skeletal dysplastic changes is higher in older patients.