AI Summary & Key Takeaways
AI InsightMost cases of aplastic crises associated with HPV B-19 in patients with hereditary Spherocytosis have been reported in children and adolescents.
📌 3 Key Takeaways
Abstract
Hereditary Spherocytosis is heterogeneous disorder characterized by alterations in the interactions between proteins of the red blood cell membrane skeleton, with reported molecular defects in ankyrin, spectrin, band 3, band 4.1 or band 4.2. The resulting instability of the membrane skeleton can be detected by altered morphology, osmotic fragility or deformability. Human Parvovirus B19 (HPV B-19) is usually associated with an acute, self-limited disease in children. In patients with a congenital hemolytic ane-mia, infection with this virus can cause an aplastic crisis. In children HPV B-19 causes an acute self-limited illness known as erythema infectiosum (fifth disease). Most cases of aplastic crises associated with HPV B-19 in patients with hereditary Spherocytosis have been reported in children and adolescents.
Özet
Herediter Sferositoz (HS) eritrositlerin membran iskeletindeki ankrin ve spektrindeki defekt sonucu oluşan heterojen bir hastalıktır. Bu proteinlerdeki kantitatif eksiklik veya fonksiyonel bozukluklar sonucu eritrosit yüzeyinden lipid kaybı oluşur ve eritrosit frajil hale gelerek hemolitik ane-miye neden olur. Human Parvovirus B19 (HPV B-19) HS'lu çocuklarda hemolitik anemiye ve aplastik krize neden olur. HPV B-19 aslında beşinci hastalık (eritema infeksiyozum) etkenidir. Bu makalede HPV B-19'a bağlı gelişen aplastik krizle ilk defa başvuran 11 yaşındaki kız hasta sunulmuştur.