[Red cell membrane defects].
Genetic defects of red cell membrane proteins constitute in Europe the most common cause of congenital hemolytic anemias. During the past decennium the defects and the pathogenetic mechanisms have been eludicated. Relatively simple hematologic tests allow for differentiation into groups with different severity of the disease. This allows prognostic assessment and careful risk-benefit evaluation for splenectomy.
Eber
S W
SW
Kinderklinik der Technischen Universität und Praxis für Kinder- und Jugendmedizin, Schwerpunkt Pädiatrische Hämotologie/Onkologie, München. praxis@kid-z.de
Huber
A R
AR
D000745:Anemia, Hemolytic, Congenital / Q000097:blood / Q000175:diagnosis* / Q000235:genetics* / Q000601:surgery; D002986:Clinical Trials as Topic; D004913:Erythrocytes, Abnormal; D006403:Hematologic Tests / Q000379:methods*; D006801:Humans; D017410:Practice Guidelines as Topic; D010818:Practice Patterns, Physicians'; D011379:Prognosis; D018570:Risk Assessment / Q000379:methods*; D012307:Risk Factors; D013156:Splenectomy