RT Book, Section A1 Bissonnette, Bruno A1 Luginbuehl, Igor A1 Engelhardt, Thomas SR Print(0) ID 1164072791 T1 Hereditary Spherocytosis T2 Syndromes: Rapid Recognition and Perioperative Implications, 2e YR 2019 FD 2019 PB McGraw-Hill Education PP New York, NY SN 9781259861789 LK accesspediatrics.mhmedical.com/content.aspx?aid=1164072791 RD 2024/03/29 AB Hereditary spherocytosis is clinically characterized by anemia, jaundice, splenomegaly, and generalized fatigue. Most cases are detected soon after birth. However, it can manifest itself at the age of 4 or 5 years. It is a membrane defect within red blood cells (RBCs) resulting in a shortened survival time. The red cells have low amounts of lipid within the bilayer membrane that lead to an abnormally small amount of surface area that causes them to change their and become spherocytic. It affects vascular flow, resulting in hemolysis. It is caused by an inherited metabolic defect. There is no cure for this medical condition and the current management option is splenectomy (except for mild condition). Post-splenectomy risk includes sepsis and will require antibiotics or immunization to limit possible infection to streptococcus pneumoniae, meningococcus, and influenza.