Spherocytosis, hereditary (HS)
50 chance to have hereditary spherocytosis.
The treatment of hereditary 0spherocytosis is to remove the spleen (splenectomy). Although the red cell defect persists, the breakup of the red cells (hemolysis) ceases. Splenectomy, however, is a hazard in young children. Young children without a spleen are at increased risk for overwhelming sepsis (bloodstream infection), particularly with the pneumococcus bacteria. Splenectomy is therefore usually postponed if possible until the age of 3 years. Before having a splenectomy, anyone with hereditary spherocytosis should have the pneumococcal vaccine. Ideally one should also receive the vaccines against Helophilus B and Meningococcal infection. Such vaccines should be given 14 days before splenectomy if possible. A yearly flu shot is also recommended after splenectomy. Persons with hereditary spherocytosis (or another cause of brisk ongoing hemolysis) should take supplemental folic acid as it is used up quickly due to the increased need to produce more red blood cells.
The prognosis (outlook) after splenectomy is for a normal life and a normal life expectancy.
HS is also known as congenital hemolytic jaundice, severe atypical spherocytosis, spherocytosis type II, ankyrin deficiency, erythrocyte ankyrin deficiency, ankyrin-R deficiency, and ankyrin1 deficiency.
Read Also:
- Sphingomyelin lipoidosis
the classical infantile form (type A), the visceral (organ) form (type B), the subacute or juvenile form (type C), the Nova Scotian variant (type D), and the adult form (type E). The disease is named for the German physicians Albert Niemann (1880-1921) and Ludwig Pick (1868-1944). Other names for the disease include sphingomyelinase deficiency.
- Sphingomyelinase deficiency
the classical infantile form (type A), the visceral (organ) form (type B), the subacute or juvenile form (type C), the Nova Scotian variant (type D), and the adult form (type E). The disease is named for the German physicians Albert Niemann (1880-1921) and Ludwig Pick (1868-1944). Other names for the disease include sphingomyelinase deficiency.
- Sphygmomanometer
systolic and diastolic. Systolic refers to systole, the phase when the heart pumps blood out into the aorta. Diastolic refers to diastole, the resting period when the heart refills with blood. At each heartbeat, blood pressure is raised to the systolic level, and, between beats, it drops to the diastolic level. With the cuff inflated […]
- Spider bites
Bites from spiders. These are usually irritating, but not poisonous. Localized reddening and swelling are common and pass within a few days. A few spiders are poisonous, in the US notably the black widow and brown recluse (brown fiddler). Bites from these spiders require emergency treatment, especially for children. However, most spiders do not pose […]
- Spider veins
A group of widened veins that can be seen through the surface of the skin. Their wheel-and- spoke shape resembles a spider. Also known as spider telangiectasia.