Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:dateCreated
2005-11-23
pubmed:abstractText
Disorders of the erythrocyte membrane, including hereditary spherocytosis, hereditary elliptocytosis, hereditary pyropoikilocytosis, and hereditary stomatocytosis, comprise an important group of inherited hemolytic anemias. These syndromes are characterized by marked clinical and laboratory heterogeneity. Recent molecular studies have revealed that there is also significant genetic heterogeneity in these disorders. This is particularly true for the spherocytosis syndromes where each kindred has a private mutation in one of the spherocytosis genes. Treatment with splenectomy is curative in most patients. Splenectomy via a laparoscopic approach has become the surgical method of choice. Growing recognition and understanding of the long-term risks and complications of splenectomy, including cardiovascular disease, thrombotic disorders, and pulmonary hypertension, and the emergence of penicillin-resistant pneumococci, a concern for infection in overwhelming postsplenectomy infection, have led to reevaluation of the role of splenectomy. Recent management guidelines acknowledge these important considerations when entertaining splenectomy and recommend detailed discussion between health care providers, patient, and family.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
1520-4383
pubmed:author
pubmed:issnType
Electronic
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
13-8
pubmed:meshHeading
pubmed:year
2005
pubmed:articleTitle
Red cell membrane disorders.
pubmed:affiliation
Department of Pediatrics, Yale University School of Medicine, 333 Cedar Street, P. O. Box 208064, New Haven, CT 06520-8064, USA. patrick.gallagher@yale.edu
pubmed:publicationType
Journal Article, Research Support, N.I.H., Extramural