Research Information System
Turkiye Klinikleri Pediatri, vol.20, no.3, pp.221-231, 2011 (Scopus)
Objective: There are different registry and research centers for every bone marrow failure syndromes (BMFS), and most of these centers work internationally and give their results periodically. In Turkey, studies about BMFS are limited to Fanconi aplastic anemia, and the data about other BMFS are limited. In this study, we aimed to document our experience about the children with BMFS. Material and Methods: We retrospectively evaluated the medical records of the children with BMFS followed in ou clinic, in between January 1, 1995 and May 30,2008. We discarded the patients with aplastic anemia secondary to malign infiltrative diseases, chemotherapy and radiotherapy together with patients who did not have enough data in their medical records. Results: Among 41 patients who enrolled to the study, 24 (58.5%) of them was boy, and 17(41.5%) of them was girl. Their age at the time of diagnosis was 5.9 ± 4.8 year, and they were followed up for 3.6 ± 3.1 year. There were 15 (36.6%) Fanconi aplastic anemia, 7 (17%) non-Fanconi aplastic anemia, 5 (12.2%) dyskeratosis congenita, 5 (12.2%) pure red cell aplasia, 5 (12.2%) acquired aplastic anemia, 3 (7.3%) osteopetrozis, and 1 (2.4%) severe congenital neutropenia. Conclusion: In conclusion, the most frequent BMFS seen in children in this part of Turkey is Fanconi aplastic anemia, and other BMFS are seen in decreasing frequency. There are some sort of insufficiencies about the diagnosis and follow up of this patient group as seen in our study. To overcome these problems, we need national multicenter studies about this diseases. Copyright © 2011 by Türkiye Klinikleri.