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previous hydrops fetalis - collezione «Medicina: Ematologia: Talassemie e trapianto del midollo osseo» - Deborah Olmi next
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Medicina: Ematologia: Talassemie e trapianto del midollo osseo

English
hydrops fetalis clicca per ingrandire
Attestation 3
Part of speech Noun syntagm
Grammatical label Countable
Definition In hydrops Fetalis (or Homozygous Alpha Thalassemia) all the 4 a genes are abnormal. There is no a chain production hence no HbF production and death in utero. At autopsy the cord blood shows severe anemia, less than 60g/L. There is no HbA or HbF on electrophoresis, most of the hemoglobin is hemoglobin Bart´swhich consists of 4 gamma chains.
Definition source Rice, FA. Thalassemia. (INTEEN01)
Context The classic alpha-thalassemia clinical syndromes studied extensively in Southeast Asians include hydrops fetalis due to homozygous alpha-thalassemia, hemoglobin H disease, alpha-thalassemia trait, and the silent carrier form of alpha- thalassemia.
Context source Ohene-Frempong, K., et al. (1987). ‘Thalassemia syndromes. Recent advances’. Hematology Oncology Clinics of North America 1(3):503-19. (RISCEN177)
Subject field Haemopoiesis
Sub-field (level 1) Thalassemias
Generic concept Alpha-thalassemias
it Idrope Fetale
Reliability code 3



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