%0 Journal Article
%T Neonatal familial Evans syndrome associated with joint hypermobility and mitral valve regurgitation in three siblings in a Saudi Arab family
%A Ahmed Fathelrahman
%A Albakrah Mohameed
%J Annals of Saudi Medicine
%D 2009
%I King Faisal Specialist Hospital and Research Centre
%X The occurrence of autoimmune hemolytic anemia and immune thrombocytopenia in the absence of a known underlying cause led to the diagnosis of Evans syndrome in a 9-month-old male. Subsequently, a similar diagnosis was made in two siblings (a 3-year-old boy and a 1-day-old girl). The 9-month-old had a chronic course with exacerbations. He was treated with steroids, intravenous immunoglobulin and colchiccine with a variable response. He died of congestive heart failure at the age of 8 years. The brother¡äs disease course was one of remission and exacerbation. With time, remissions were prolonged and paralleled an improvement in joint hypermobility. The sister died of sepsis after a chronic course with severe exacerbattions. Only two families with Evans syndrome have been reported in the English medical literature. In one report (in a Saudi Arab family), the disease was associated with hereditary spastic paraplegia.
%U http://www.saudiannals.net/article.asp?issn=0256-4947;year=2009;volume=29;issue=3;spage=227;epage=230;aulast=Ahmed