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International Journal of Recent Trends in Science and Technology, ISSN 2277-2812 E-ISSN: 2249-8109

Volume 5, Issue 1, 2012 pp 01-02

Case Report

Congenital Dyserythropoitic Anemia Type I Presenting as Congestive Cardiac Failure in Neonatal Life

Anilkumar M. Khamkar†, Manish M. Tiwari†, Pallavi P. Saple†, Amol Suryawanshi†, P.M.Zadke#, Manisha Surwade*

{†Department of Pediatrics, #Department of Pathology, *Department of Anaesthesia} S.R.T.R.G. Medical College and Hospital, Ambajogai, Beed (MS) INDIA

Academic Editor:  Dr. Aher K. R.

Abstract

 

Abstract: Congenital dyserythropoietic Anemias (CDAs) constitute a heterogeneous group of rare inherited disorders, characterized by moderate to severe anemia due to a markedly ineffective erythropoiesis with an erythroblastic bone-marrow and specific erythroid morphological anomalies. Three major types of CDA and a number of variants have been described. The diagnosis and categorization of these disorders are facilitated by microscopic examination of the blood and bone marrow and by serologic testing. The severity of anemia varies considerably within and between families. We report a case of CDA type I in a neonate who presented as congestive cardiac failure secondary to severe Anemia who also had PPHN due to LVF. Baby succumbed to death on day 1 of life itself. Clinical profile and laboratory investigations were consistent with CDA type I.