Pulmonary embolism after snake bite in a child with Diamond-Blackfan anemia (Journal article)

Makis, A./ Kattamis, A./ Grammeniatis, V./ Sihlimiri, P./ Kotsonis, H./ Iliadis, A./ Siamopoulou, A./ Chaliasos, N.

Diamond-Blackfan anemia (DBA) is a rare, congenital, pure red blood cell aplasia owing to gene defects affecting the function of ribosomal proteins, essential for erythroid maturation. Iron overload is a serious complication of chronic transfusions, which may lead to cardiac toxicity and endothelial damage. We report a case of pulmonary embolism, observed after viper bite in a transfusion-dependent child with DBA without known inherited thrombophilic factors. Embolic events are uncommon after viper bites, which they usually cause consumption coagulopathy, resulting in hypocoagulable state. DBA has not been earlier correlated with thrombotic episodes. In our patient, we suggest an iron overload-induced hypercoagulability state, which in the presence of a procoagulant substance lead to the development of a thromboembolic event.
Institution and School/Department of submitter: Πανεπιστήμιο Ιωαννίνων. Σχολή Επιστημών Υγείας. Τμήμα Ιατρικής
Keywords: Adrenal Cortex Hormones/therapeutic use,Anemia, Diamond-Blackfan/*complications/genetics/therapy,Blood Transfusion, Autologous,Child,Erythrocyte Transfusion,Hematopoietic Stem Cell Transplantation,Humans,Iron Overload/complications/genetics/therapy,Male,Mutation,Pulmonary Embolism/*complications/genetics/therapy,Ribosomal Proteins/genetics/metabolism,Snake Bites/*complications/genetics/therapy,Transplantation, Homologous
URI: http://olympias.lib.uoi.gr/jspui/handle/123456789/18902
ISSN: 1536-3678
Link: http://www.ncbi.nlm.nih.gov/pubmed/20881870
Appears in Collections:Άρθρα σε επιστημονικά περιοδικά ( Ανοικτά)

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