Authors: D. Pospíšilová; V. Mihál; E. Klásková; K. Petrtýlová 1; J. Starý 1; H. Ptoszková 2 Authors‘ workplace: Dětská klinika LF UP a FN, Olomouc Published in: Čes-slov Pediat 2000; (7): 415-423. Category:
In cooperation with paediatric haematologists from the Czech Republic, the Diamond-Blackfan anaemia (DBA)registry was created. Twenty six patients with chronic hypoplastic anaemia were investigated. Twenty two patientsmet the diagnostic criteria for DBA. The incidence of DBA in our country estimated from our registry is 7 DBA cases per million live births. The other 4 patients suffered from chronic parvovirus B 19 infection (2), Fanconianaemia (1), transient erythroblastopenia (1). In the group of DBA patients are 11 boys and 11 girls, the age rangeis 4 months to 20 years. There are 3 families with more than one case of DBA in the registry, two families withautosomal dominant and one with an autosomal recessive inheritance. Diagnosis of DBA was established duringfirst 10 months of life in all cases. Eleven patients (50%) were anaemic already in neonatal age. Four patients(18 %) with hypotrophy at births and 4 (18 %) premature births (35th - 37th week of pregnancy) were identified.The average birth weight of boys was significantly lower than of mature healthy newborn boys (p
Key words: Diamond-Blackfan anaemia, growth factors, associated anomalies, adenosine deaminase, parvovi-
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