Publication date Print : April Hereditary spherocytosis must always be suspected in children with anemia, hyperbilirubinemia, splenomegaly or cholelithiasis, in the asymptomatic individual with an affected relative, and in the neonate with hyperbilirubinemia with no blood group incompatibility; its early detection is key to avoid kernicterus. Follow-up of these patients is based on periodical control and supply of information on the adequate management of hemolytic or aplastic crisis, and early detection of cholelithiasis. The decision to perform splenectomy is usually associated with quality of life rather than life-threatening risk, and it should result from a consensus between patient, parents and physicians.
|Published (Last):||13 July 2012|
|PDF File Size:||5.52 Mb|
|ePub File Size:||13.88 Mb|
|Price:||Free* [*Free Regsitration Required]|
Red cell membrane protein deficiencies in Mexican patients with hereditary spherocytosis. Blood Cells Mol Dis ; 31 3 : Additional erythrocytic and reticulocytic parameters helpful for diagnosis of hereditary spherocytosis: results of a multicentre study. Ann Hematol ; 90 7 : Guidelines for the diagnosis and management of hereditary spherocytosis update. Br J Haematol ; 1 : Delaunay J. The molecular basis of hereditary red cell membrane disorders.
Blood Rev ; 21 1 : Mutation of a barrier insulator in the human ankyrin-1 gene is associated with hereditary spherocytosis. J Clin Invest ; 12 : Gac Med Mex ; 5 : Casale M, Perrotta S. Splenectomy for hereditary spherocytosis: complete, partial or not at all? Expert Rev Hematol ; 4 6 : Contemporary pediatric splenectomy: continuing controversies. Pediatr Surg Int ; 27 11 : Splenectomy in hereditary spherocytosis: Review of 1, patients and application of the pediatric quality indicators.
Pediatr Blood Cancer ; 52 7 : Laparoscopic splenectomy has become the gold standard in children. Am Surg ; 68 3 : Is expertise in pediatric surgery necessary to perform laparoscopic splenectomy in children? An experience from a department of general surgery. Updates Surg ; 64 2 :
Laparoscopic Splenectomy in Pediatric Age: Long-Term Follow-Up
Important User Information: Remote access to EBSCO's databases is permitted to patrons of subscribing institutions accessing from remote locations for personal, non-commercial use. However, remote access to EBSCO's databases from non-subscribing institutions is not allowed if the purpose of the use is for commercial gain through cost reduction or avoidance for a non-subscribing institution. Affected patients can remain asymptomatic, with a minimum hemolysis, or develop a severe hemolytic anemia. It is transferred as an autosomal dominant disease, less frequent as an autosomal recessive one, or with no medical history in the family.
2012, Número 4