Hereditary spherocytosis is best tretaed with
Correct Answer: Splenectomy
Description: Treatment of choice for hereditary spherocytosis is splenectomy especially in case of severe hemolysis,done after 6 years of age. The treatment of hereditary spherosytosis (HS) is dependent on the severity of the condition and recommendations vary a bit in the medical literature. In general, folate therapy (a type of vitamin B to suppo red blood cell production) is recommended for those with moderate to severe anemia, although some doctors may also recommend it for those with mild anemia. Red blood cell transfusions may be required in severe cases of anemia, paicularly in the first years of life or during infections and pregnancy. If red blood cell transfusions are needed repeatedly, iron chelating therapy may be required to reduce iron overload. Regular monitoring for anemia and gallstones is advised. Removal of the spleen (splenectomy) is usually only performed in severe HS or in moderate to severe cases with significant anemia and gallstone complications. Splenectomy is not recommended in cases of mild HS except in specific cases. The majority of medical researchers no longer recommend that the spleen be removed during gallbladder removal (cholecystectomy), unless there are other reasons to do so. In some cases only removing of pa of the spleen is advised. Expe evaluation is recommended in order to avoid unnecessary spleen removal. Reference: GHAI Essential pediatrics, 8th edition
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